Clinical Signs and Syndromes in Surgery

Clinical Signs andSyndromes in Surgery

Clinical Signs andSyndromes in Surgery

JAYPEE BROTHERS MEDICAL PUBLISHERS (P) LTD

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®

Shivananda PrabhuProfessor of Surgery

Kasturba Medical CollegeMangalore, Karnataka

India

ForewordG Rajagopal

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Clinical Signs and Syndromes in Surgery

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It had been an extremely pleasant experience goingthrough the pages of Clinical Signs and Syndromes inSurgery.

In an era, where technology is rapidly trying toreplace clinical skills, like recording a detailed history,eliciting clinical signs, etc., this work nudges you as agentle reminder of the unquestionable relevance ofclinical examination of a patient. I feel it has been along-felt need of both undergraduate and postgraduatestudents, to have a ready-reckoner like this, and its utilityis not restricted to students of surgery alone. It is ofimmense value to students of all disciplines of modernmedicine.

In an examination scenario, to be able to group yourfindings and/or to know the names of various ‘namedsigns’ and syndromes while presenting a case, isdefinitely a great advantage and would impress anexaminer no end. Also, it will have immense utility inviva voce as well, and will make a topper standout froma mediocre. I am convinced that Dr Shivananda Prabhuhas worked hard and researched well to collect thiswealth of information and I am sure the medical students

Foreword

Clinical Signs and Syndromes in Surgeryvi

community will appreciate the value of this collectionfor years to come.

I congratulate and compliment Dr ShivanandaPrabhu for this effort and wish him all the best in all hisfuture academic endeavors.

Maj Gen (Retd) Dr G Rajagopal AVSMMS FRCS

Dean and Professor of Surgery and OncosurgeryKasturba Medical College

Mangalore, Karnataka, India

Ever since I was an undergraduate student, eliciting ofclinical signs has always fascinated me. I rememberwatching in awe as seniors demonstrated clinical signs.

This wonderment at such skills reached its peakduring discussion of central nervous system (CNS)disorders in the medical wards. Neurological disordersare nothing but a collection of signs, one used to think.Such thoughts brought anxiety with them as one wasnot sure how to cope.

Those times are long gone, but the fascination withsigns remains. Having chosen general surgery as myfield, it is only natural that I would now be interestedmainly in signs pertaining to surgical conditions. Lifeof a surgeon is in many ways easier than that of aphysician inasmuch as many of surgical conditionsproduce distinctive symptoms and signs unlike mostmedical illness. Also, surgical conditions most oftenthan not lead to some anatomical and physiologicaldistortions discernible by clinical examination asclinical signs. Only there have not been many booksdedicated to this aspect of clinical examination. Thereare many excellent books dealing with clinicalexamination as a whole, but they do not segregateclinical signs from rest of the process of clinicalevaluation. Hence, for a student preparing for clinicalexamination, it becomes a tough task to brush up hisknowledge. Hence, the need for a book dealing

Preface

Clinical Signs and Syndromes in Surgeryviii

exclusively with clinical signs. Also, while we do knowabout a particular sign as an indication of a particulardisease often we do not really know the best way toelicit the sign. Easy access to diagnostic imaging hasonly made the ignorance deeper. This book attempts toaddress this problem. This should hopefully help notonly students preparing for examinations but alsopractising surgeons.

I have included a brief account of syndromes, as Ifound these to be the scourge of exam-going students.Examiners revel in catching students off-guard bythrowing questions at them about some obscuresyndrome or the other. I hope to reduce such a threatby touching upon them. While this book may not haveanything new in its content, I am sure the idea ofpresenting “signs and syndromes” in a concise book isa novel one.

Shivananda Prabhu

Chapter One: Abdominal Signs ............................ 1• Signs on Inspection .............................................. 3• Signs on Palpation ............................................... 9• Signs on Percussion and Auscultation of

Abdomen ........................................................... 17• Radiological Signs in Abdomen ........................ 21

Chapter Two: Thyroid Signs ............................... 27• Signs in Thyrotoxicosis ..................................... 28• Signs Associated with Thyroid Pathology

other than Thyrotoxicosis .................................. 34

Chapter Three: Signs Pertaining to OtherOrgan Systems ...................................................... 37• Signs in Torsion Tests ........................................ 38• Signs in Latent Tetany ....................................... 40• Signs in Deep Vein Thrombosis ........................ 41• Signs of Visceral Malignancy ............................ 43• Signs in Peripheral Vascular Disease................. 44• Signs in Arterial Aneurysm................................ 46• Signs of Hernia .................................................. 49• Miscellaneous Signs .......................................... 50

Chapter Four: Clinical Syndromes ..................... 55• Auriculotemporal Nerve Syndrome .................. 57• Afferent Loop Syndrome ................................... 58• Beckwith-Wiedemann Syndrome ...................... 59• Bland-White-Garland Syndrome ....................... 60

Contents

Clinical Signs and Syndromes in Surgeryx

• Blind Loop Syndrome ....................................... 60• Boerhaave’s Syndrome ...................................... 62• Budd-Chiari Syndrome ...................................... 63• Carcinoid Syndrome .......................................... 64• Chilaiditi’s Syndrome ........................................ 65• Compartment Syndrome .................................... 66• Crush Syndrome ................................................ 67• CRST Syndrome ................................................ 67• Cronkhite-Canada Syndrome ............................ 68• Crigler-Najjar Syndrome ................................... 68• Cushing’s Syndrome .......................................... 68• Cruveilhier-Baumgarten’s Syndrome ................ 69• Carotid Steal Syndrome ..................................... 70• Dubin-Johnson Syndrome ................................. 70• Dumping Syndrome ........................................... 70• Gardner’s Syndrome .......................................... 72• Gilbert’s Syndrome ............................................ 72• Horner’s Syndrome ............................................ 73• Hepatorenal Syndrome ...................................... 74• Job Syndrome .................................................... 75• Kearns-Sayre Syndrome .................................... 75• Klippel-Trenaunay-Weber Syndrome ................ 76• Koenig’s Syndrome ........................................... 76• Lamb Syndrome ................................................ 77• Lambert-Eaton Syndrome.................................. 77• Leriche’s Syndrome ........................................... 77• Lynch Syndrome ................................................ 78• Munchausen’s Syndrome ................................... 79

Contents xi

• Murphy’s Syndrome .......................................... 79• Meigs’ Syndrome ............................................... 80• Mallory-Weiss Syndrome .................................. 81• Marfan’s Syndrome ........................................... 82• Malabsorption Syndrome .................................. 83• Mirizzi’s Syndrome ........................................... 83• Mafucci’s Syndrome .......................................... 84• Mendelson’s Syndrome ..................................... 85• Naffziger’s Syndrome ........................................ 86• Nelson’s Syndrome ............................................ 88• Ogilvie’s Syndrome ........................................... 88• Ormond’s Syndrome .......................................... 89• Ortner’s Syndrome ............................................ 90• Poland’s Syndrome ............................................ 90• Pendred’s Syndrome .......................................... 91• Pickwickian Syndrome ...................................... 91• Plummer-Vinson Syndrome............................... 92• Peutz-Jeghers Syndrome ................................... 93• Postcholecystectomy Syndrome ........................ 94• Prune Belly Syndrome ....................................... 95• Parker-Weber Syndrome .................................... 95• Postconcussion Syndrome ................................. 96• Postsplenectomy Syndrome ............................... 96• Postphlebitic Syndrome ..................................... 97• Paraneoplastic Syndrome .................................. 98• Pierre-Robin Syndrome ..................................... 98• Pseudo-Zollinger-Ellison Syndrome ................. 99• Rotor’s Syndrome ............................................ 100

Clinical Signs and Syndromes in Surgeryxii

• Rapunzel Syndrome ......................................... 100• Raynaud’s Syndrome ....................................... 101• Sezary Syndrome ............................................. 101• Sipple Syndrome.............................................. 102• Stein-Leventhal Syndrome .............................. 103• Sturge-Weber Syndrome .................................. 104• Sheehan’s Syndrome ....................................... 104• Stewart-Treves Syndrome................................ 105• Sjögren’s Syndrome ......................................... 105• Scheuermann’s Syndrome ............................... 106• Stevens-Johnson Syndrome ............................. 107• Sandifer’s Syndrome ....................................... 108• Sump Syndrome .............................................. 108• Short-Bowel Syndrome ................................... 108• Seat-belt Syndrome.......................................... 109• Turcot’s Syndrome ............................................ 110• Tumor Lysis Syndrome ..................................... 111• Torre Syndrome ................................................ 112• Tietze’s Syndrome ............................................ 112• Takayasu’s Syndrome ....................................... 113• Verner-Morrison Syndrome .............................. 113• Wilkie’s Syndrome ........................................... 114• Wermer’s Syndrome ......................................... 115• Weak Vein Syndrome ....................................... 115• Waltman Walter Syndrome ............................... 116• Von Hippel-Lindau Syndrome .......................... 117• Zollinger-Ellison Syndrome ............................. 117

Index ...................................................................... 119

Clinical examination is the most exciting as well aschallenging part of a surgical residents’ daily routine.Even in today’s world where the advancement in thefields of laboratory sciences and diagnostics make thediagnosis of disease conditions less difficult than before,clinical acumen retains its importance. One needs soundclinical judgement to be able to make proper use ofdiagnostic technology. Hence, students of surgeryshould endeavor to acquire a level of clinical skillswhich allows them to narrow down the diagnosticpossibilities and order for investigations accordingly.

Often, while examining a patient one relies on certainclinical finding elicited during examination to arrive ata plausible conclusion. Of course a detailed historytaken from patient by a sympathetic and astute clinicianwill go a long way in pointing towards the pathologythat the patient has. It is beyond the scope of the bookto go into details of history taking. Good history alongwith well-detected clinical findings, when analyzedtogether will make the clinical picture clearer. If all thesymptoms and clinical observations could be explainedby a single pathological entity, then the diagnosis isnear certain. Hence, only one diagnosis need be putforth and investigation asked for just to confirm or ruleit out. On the other hand if all facets of the case cannotbe explained by a single pathological lesion, then

Introduction

Clinical Signs and Syndromes in Surgeryxiv

differential diagnosis should be thought of andinvestigations ordered accordingly.

What are these clinical findings which help us reacha definitive conclusion? These are observations madeby the clinician during inspection, palpation,auscultation, or percussion. They are objective findingswhich can be corroborated by any clinician. There isno subjective element in them.

In other words, they are called “clinical signs”.A clinical sign when properly elicited gives a clue

to underlying pathology. Its presence makes thediagnosis more of a probability and less of a guess.When many such observations or signs are put togetherit is possible to arrive at a conclusive opinion regardingthe disease process. It all looks simple andstraightforward at first look. But one is well advised tokeep the following facts in mind before embarking onthe pursuit of this art of eliciting clinical signs.• Just knowing the theory underlying a clinical sign

is not enough. One should be familiar with the exacttechnique of eliciting the sign. One may not be ableto demonstrate a sign, even when it is present if oneemploys incorrect technique. Even books will helponly to a limited extent. There is no substitute forobserving an expert clinician eliciting the sign.

• If an attempt at eliciting a sign is likely to causediscomfort to the patient then it is necessary thatclinician explains to him the nature of the test and

Introduction xv

enlists his cooperation, e.g. rebound tenderness. Anuncooperative and distressed patient is sure recipefor failure. If one fails to elicit such a sign withinone or two attempts it is better to let it go asinconclusive or absent. One should persist in tryingto elicit a sign only if it is vital for the diagnosis.There are very few signs of such singular clinicalimportance.

• If a simple laboratory test can avoid prolongedclinical examination and laborious analysis thenchoose it, especially in an emergency setting, e.g.chest X-ray with domes of diaphragm to check forfree gas under diaphragm will clear the diagnosisimmediately and should not be unnecessarily delayedpending detailed examination.

• One should be able to elicit the sign even when thediagnosis is as yet unclear. Anybody can elicit thesign once the diagnosis is established and known,e.g. even a beginner will be able to observe visiblegastric peristalsis once endoscopy has revealed thepresence of gastric outlet obstruction. But thatobservation will only serve academic purpose. Onthe other hand, if visible gastric peristalsis isobserved by an astute clinician in the OPD itself,patient will be saved a lot of time and of coursemoney. Such skill at observing the signs is especiallyuseful while one is working in mofussil areas andnot a city.

Clinical Signs and Syndromes in Surgeryxvi

• Remember a particular sign need not be present inall cases of particular pathology. Atypicalpresentation of a disease condition is quite commonand one needs to maintain a high degree of clinicalsuspicion to be able to diagnose a condition even inthe absence of typical signs.

With these few facts in mind let us now acquaintourselves with clinical signs, system-by-system.

AbdominalSigns

Clinical Signs and Syndromes in Surgery2

It is only natural that we are discussing abdominal signsfirst. It has been rightly said that abdomen is a‘Pandora’s Box’. Even the most experienced cliniciansoften stumble when it comes to abdomen. There aretimes when the final truth about abdominal pathologyis only revealed at laparotomy. True, ultrasound,contrast CT, MRI, etc. have made diagnosing abdominalpathology less difficult but not yet easy.

There are many reasons why an abdominalpathology is more difficult to diagnose than lesionsanywhere else. The foremost of the reasons is the factthat abdomen is the seat of so many organs of variedanatomy and physiology. That being so, ordinarily whenany of these organs is involved it should produce distinctsigns and symptoms, but unfortunately most of theseorgans are interlinked both anatomically as well asfunction-wise. Hence, a disease in any one organ willdisrupt not only its function but also that of the othersthat are linked to it, thereby confusing the picture. Andnature adds to the confusion by introducing its ownsigns and symptoms via body’s protective mechanism.For example, vomiting caused by protectivepylorospasm in case of acute appendicitis. Anotherreason why abdomen is still an enigma is that a properevaluation of abdomen requires not only a skilledclinician but also a relaxed and cooperative patient.Many a time to expect a patient having severe painabdomen to be relaxed is to expect too much from him.

Abdominal Signs 3

Clumsy attempts at palpation will only serve to raisehis anxiety. Hence, it is really necessary to make apatient feel at ease before starting any examination. Afew minutes spent in sympathetic explanation will go along way in making the examination worthwhile.

Before starting to look for abdominal signs one has tomake the patient lie comfortably on the examination couchbreathing deeply but steadily. It goes without saying thatthe whole of the abdomen should be exposed right fromnipple level to mid thigh level. Of course, one has toprovide adequate privacy. Ensure that a lady assistant ispresent if the patient is a female, for obvious reasons.

SIGNS ON INSPECTION

Looking for abdominal signs should start with carefulinspection of the abdominal wall as well as itsmovements. For ease of presentation we will discussthe inspectory signs first followed by palpatory onesand so on.

The signs have not been listed in any particular orderof their perceived importance. They are discussed inalphabetic order to eliminate any personal biasregarding their clinical significance.

Auenbrugger’s Sign

This sign is said to be present when there is an epigastricprominence produced by marked pericardial effusion.


The differentiation should not be difficult as underlyingpericardial effusion will definitely produce other signsand symptoms referable to the poor cardiac functionlike features of congestive cardiac failure, muffled heartsounds, etc. When in doubt, simple investigation likeX-ray chest AP view or echocardiography should clearthe doubt.

Beevor’s Sign

When the infraumbilical portion of both rectusabdominis muscles is paralyzed umbilicus moves closerto the xiphisternum. This is due to the lack of tone ofthe lower part of muscles.

Cullen’s Sign (Umbilical Black Eye)

This is usually seen in hemorrhagic pancreatitis. Thereis periumbilical discoloration due to seepage of bloodeither transperitonially or along the falciform ligament.

It should be borne in mind that this is relatively latesign in the process of pancreatitis and hence should nothe sought as an aid to diagnose acute pancreatitis. Whileits presence indicates grave prognosis for the patient itsabsence in no way rules out pancreatic inflammation.Diagnosis of acute pancreatitis is essentially clinicalbased on detailed history and careful examination of theabdomen followed by laboratory tests like serumamylase.

Abdominal Signs 5

One should remember that the sign is not exclusivefor pancreatitis. Any massive intraperitoneal bleed alsocan lead to the development of this sign. For example,ruptured ectopic.

Grey Turner’s Sign

This is bluish discoloration of the flank seen mostcommonly in acute hemorrhagic pancreatitis. Onceagain this is due to hemorrhage into retroperitonealspace due to acute pancreatic inflammation. This blooddissects through tissues and appears in flanks. It goeswithout saying that this is another sign of graveprognosis indicating the need for urgent resuscitation.

One has to remember that this sign may also beassociated with other equally serious condition likeleaking abdominal aortic aneurysm (AAA),retroperitoneal bleed due to trauma, etc.

Fox Sign

Occasionally there is discoloration of inguinal region incases of hemorrhagic pancreatitis due to trickling ofhemorrhagic fluid.

Hippocratic Facies

Evident during advanced stages of any acute peritonealinflammation. Patient has in drawn but bright eyes,anxious look with pinched face and cold skin.


Ransohoff’s Sign

Yellow pigmentation of umbilicus and periumbilicalregion in rupture of common bile duct. The extravasatedbile traverses along the falciform ligament to reach theumbilical region.

Sign de dance (Dance’s Sign)

This is seen in some cases of intussusception, especiallythe iliocolic type. There is emptiness in the right iliacfossa because of progressive telescoping of the ileumand cecum in to distal colon leaving the right iliac fossaempty. There may be some in drawing of the parietalwall noticeable during inspection. This can beconfirmed by palpation. Also on palpation one may beable to feel a sausage shaped mass, with its concavitytowards the umbilicus. The consistency of the massmight change from time-to-time depending uponperistalsis. One may be able to appreciate visibleperistalsis in these individuals. Barium enema isconfirmatory and shows the claw sign, which isdiscussed later. History from the patient might revealintestinal colic, obstruction and red current jelly stools.

Tanyol’s Sign

Normally umbilicus lies midway between symphysispubis and xiphisternum. But a mass arising from thepelvis may lead to displacement of umbilicus upwards

Abdominal Signs 7

nearer to xiphisternum. The opposite happens whenthere is gross ascites. This is known as Tanyol’s sign.

Visible Peristalsis

While inspecting the abdomen for any abdominalcondition one is supposed to look for movements, be itrespiratory, peristaltic or pulsatile. Of these, peristalticmovements, if made out during clinical examination,are more likely to be of clinical significance.

Peristalsis is normal forward propulsive movementsof entire gastrointestinal tract. Whenever there is anymechanical obstruction to the forward propulsion ofits contents these peristaltic waves become stronger andmore frequent in an effort to overcome the obstruction.Patient is likely to have colicky abdominal painassociated with vomiting. These strong peristaltic wavesare often visible in not so obese patients. Dependingupon the site of obstruction, the clinical nature of visibleperistalsis changes. For example, if the obstruction isat pylorus of the stomach, as occurs in chronic duodenalulcer patient has epigastric pain and peristalsis is visiblein upper abdomen. To induce peristalsis, whenevergastric outlet obstruction is suspected on history givenby the patient, he is made to drink substantial amountof water and asked to lie down. The clinician shouldpreferably sit beside patient’s bed and watch forperistaltic wave starting in the left hypochondrium andmoving slightly downwards and to the right. The wave


appears as an area of fullness preceded by constriction.Patient will complain of colicky pain during the test.Other clinical tests like succussion splash andauscultopercussion are done to confirm the dilated stateof stomach following outlet obstruction. Succussionsplash is the splashing sound of retained gastric contentsheard in the epigastrium with the help of stethoscopewhen patient is gently shaken. Auscultopercussioninvolves marking the borders of a dilated stomach withthe help of change in sound heard when the bell ofstethoscope is kept on the epigastrium and abdominalwall is scratched in a radial fashion moving away fromthe stethoscope. As long as the scratching finger liesover the dilated stomach there will be tympanic notewhich will abruptly change in character when the fingermoves beyond the boundaries of the stomach. One canmark multiple such points, which when joined willindicate roughly the position of the dilated stomach inthe abdomen.

Visible peristalsis can also be induced by flickingthe abdominal wall with the fingers or putting a fewdrops of ether on the abdominal wall.

Peristaltic wave will travel in a step ladder patternprogressively moving downwards on case ofobstruction of small bowel. In case of obstruction ofdistal colon the peristaltic wave may be seen to passfrom right to left at or just above the level of umbilicus.The direction of movement and other signs of colonic

Abdominal Signs 9

obstruction help to differentiate this from visible gastricperistalsis.

SIGNS ON PALPATION

Whenever a case of abdominal pathology presents itselfin the OPD or casualty the tendency amongst surgicalresidents is to go and start palpating the abdomen evenbefore a decent history is taken. This practice isimproper, insensitive and often counter productive.

Before palpating the abdomen one should try todetermine the nature of the pathology that the patient ismostly likely to have. This necessitates taking a goodhistory eliciting details of all the symptoms. Any doubtsthat the clinician might have should be clarified askingnecessary questions. At the end of the history takingclinician will have developed a rapport with the patient.Then the examination proper should start, beginningwith general physical examination. Inspection of theabdomen should be done after exposing the abdominalwall fully and allowing the patient to breathe regularly.Any inspectory sign should be recorded forcorroboration during palpating. If it is deemed thatpalpation is likely to be painful then percussion andauscultation should be done before going in forpalpation. It is a good practice to explain to the patientthe nature of palpatory maneuver that will be needed.


Aaron’s Sign

This sign is said to be present when the patient expressesa feeling of discomfort in the epigastric region or theprecordial region on applying sustained pressure overthe McBurney’ point. But it should be noted that thereare other signs and symptoms, which cause much lessdiscomfort at the same time pointing towardsappendicular inflammation. Hence, this sign is rarelysought when one is suspecting acute appendicitis.

Alder’s Sign

This is a useful sign in diagnosing acute appendicitisduring the later half of pregnancy.

In a pregnant patient with pain in the right iliac fossathe source of pain can be differentiated by asking herto lie on the left side. After waiting for a few minutes iftenderness is again sought for, if it is of uterine originthen the point of maximum tenderness will be move tothe left with the uterus while if it is of appendicularorigin then it will stay where it was.

Baid Sign

It is one of the abdominal signs described by an Indiansurgeon.

In a thin patient suffering from pseudocyst of thepancreas, the stomach is often pushed anteriorly towards

Abdominal Signs 11

the abdominal wall. In such a patient if a Ryle’s tube isin place it is often palpable beneath the thin abdominalwall. This fact can easily be confirmed by a lateral X-rayof the abdomen.

Bapat’s Bed Shaking Test

An ingenious method to elicit inflammation of theparietal peritoneum is to gently shake the bed. Theresultant body movement is enough to induce pain in apatient with peritonitis. This test is considered morehumane than repeated palpation of the abdomen in apatient with peritonitis.

Boa’s Sign

It is a sign of acute cholecystitis. An area of hyperes-thesia can be detected over the posterior abdominal wallbetween T11 and L1 and starting from 1 inch lateral tothe midline to posterior axillary line. This is an exampleof referred pain.

Carnett’s Sign

Whenever there is tenderness over any part of theabdomen, if the tenderness decreases on contractingthe abdominal muscles then the source of pain isintraperitoneal. If it remains the same then the pathologyis in the parietal wall.


Cope’s Psoas Test

This is elicited in evaluating a case of possible retrocecalappendicitis. When the appendix lies retrocecally, as itdoes very often, it lies in close proximity to psoas majormuscle. Hence, when such an appendix is inflamedpsoas muscle may get irritated enough to go into spasm.In such cases hyperextension of the hip joint will causepain to the patient. In well-established cases patientmight have fixed flexion deformity of the right hip joint.

Cope’s Obturator Test

Cope’s obturator test has the same underlying principleas Cope’s psoas test only, it is positive in pelvicappendicitis when the appendix is in close proximityto obturator internus muscle. Due to the proximity tothe inflamed organ the muscle fibers irritated. Hence,when such patient is asked to internally rotate the righthip joint he will experience pain.

Fothergill’s Sign

Presence of a tender mass overlying one of the rectiwhich does not cross the midline and is palpable evenwhen the muscle is made taut is indicative of rectussheath hematoma.

A careful history will tell the clinician that theswelling was of acute onset and had etiological factors

Abdominal Signs 13

like a bout of severe cough, or any sudden strain on therectus abdominis.

Klein’s Sign (Cf. Alder’s Sign)

Useful in differentiating acute appendicitis frommesenteric lymphadenitis. In many instances the clinicalfeatures of acute appendicitis are indistinguishable fromthose of mesenteric adenitis. In such cases if the patientis asked to lie on the left side the point of maximumtenderness will more to the left side in mesentericadenitis but not in acute appendicitis. But one has toremember that in Meckel’s diverticulum also thetenderness might move to the left in a similar manner.

Mallet-Guy Sign

It sometimes looked for in chronic pancreaticinflammation. Patient is asked to lie on his right side.Deep palpation in the left subcostal and epigastricregion will elicit pain if pancreas is inflamed.

Murphy’s Sign

This is a sign of acute cholecystitis. This helps to clarifythe situation when doubt exists as to the underlyingpathology causing pain in the right hypochondrium.

Patients asked to take a deep breath while theclinician does deep palpation in the right


hypochondrium. Patient will hold his breath midwaythrough inspiration as the inflamed gallbladder touchesthe abdominal wall. Admittedly this causes discomfortto the patient and hence the test should not be repeatedwithout sufficient reason.

Rebound Tenderness (Blumberg’s Sign)

All the abdominal signs this one is perhaps the mostsignificant inasmuch as its presence usually indicatesproblem of a serious nature. Often the patient wouldrequire a laparotomy as rebound tenderness is anindicator of inflammation of the parietal peritoneum.And most cases of peritoneal inflammation or peritonitisdo need surgical intervention. Hence, it is imperativethat we become adept at this sign.

One should remember that rebound tenderness islooked for only when there are other clinical featuresof peritoneal pathology like pain abdomen of acuteonset which is exaggerated by cough or movement,vomiting, constipation, etc. Hence, one should be gentlein trying to elicit rebound tenderness. If the diagnosisis already beyond doubt (e.g. X-ray showing gas underthe diaphragm, etc.) one should not inflict more painon the patient just to satisfy one’s curiosity.

Once it has been decided that rebound tendernessshould be looked for to clarify the situation, the natureof the test should be explained to the patient. Then he/she should be requested to keep the abdomen as relaxed

Abdominal Signs 15

as possible. A round of superficial palpation will usuallypoint to the site of maximum tenderness. Here theclinician should palpate deeply watching patients faceall the while. Then he should maintain the pressure fora brief while then release the pressure abruptly. If onewere to observe patient’s face during the wholemaneuver one can notice that patient winces duringinitial palpation, shows sign of less discomfort duringthe phase of sustained pressure and winces in pain againwhen the palpating hand is abruptly lifted.

The pain during the test is due to the movement ofthe sensitive parietal peritoneum. During initial deeppalpation movement of the parietal peritoneum causespain, but during sustained pressure, as there is no furthermovement there is a dip in the painful stimulus. Butwhen the hand is released abruptly parietal peritoneumsprings back into it original state causing even morepain to the patient.

Gently performed this test can clarify the diagnosisin condition like acute appendicitis, perforativeperitonitis in its early stages, pelvic inflammation, etc.But one should remember that it needs a highlycooperative patient for the successful eliciting of thissign. An apprehensive patient would simply resist anypalpation by keeping the abdomen guarded. Of coursebeyond a certain threshold of pain, nature takes overby making the abdomen wall rigid precluding anyfurther palpation.


While checking for rebound tenderness the activepart of the test is the abrupt release the palpating hand.One should resist the temptation to make exaggeratedmovements during the release. Often it is seen that afterrelease of the pressure the forearm is taken back in adramatic arc by the clinician. But one would do well toremember that once off the abdominal wall forearmmovement has no effect other than dramatics. Suchantics only succeed in making the patient anxious.

Rosenbach’s Sign

Loss of abdominal reflex when the viscera are inflamedis called Rosenbach’s sign. Normally, when skin of theabdominal wall is stimulated there is contraction of theabdominal wall muscles. This is called superficialabdominal reflex. Contraction of the same muscleswhen the neighboring bony points are tapped is nameddeep abdominal reflex.

Abdominal reflex is also sometimes called epigastricreflex or supraumbilical reflex.

When the underlying intraperitoneal viscera areinflamed abdominal muscles tend to go into spasm as aprotective response. Hence, there is loss of abdominalreflex.

Rovsing’s Sign

This is a sign which is sometimes present in acuteappendicitis. If the clinician presses the abdomen inthe left iliac fossa patient may complain of pain in the

Abdominal Signs 17

right iliac region. Displacement of air and fluid withinthe colon proximally may be the reason for thisphenomenon. The displaced air stimulates the inflamedappendix and cecal mucosa thus causing pain. Directdisplacement of the abdominal contents, thus disturbingthe inflamed appendix may be another factorcontributing to the increased pain.

Rovsing’s sign can be elicited with less discomfortto the patient when compared to rebound tendernessbut it is not very reliable. Absence of Rovsing’s signdoes not rule out appendicitis.

Ten Horn’s Sign

This sign is said to be present when the patient feelspain on gentle traction of the right spermatic cord. Ifpresent, it indicates the presence of acute appendicitis.Traction applied on the right spermatic card producesdownward movement of the cord contents some ofwhich like gonadal vessels lie in close proximity to theviscera in the right iliac fossa. The disturbance thenproduced of these viscera especially cecum andinflamed appendix may induce pain. Of course one hasto rule out funiculitis before seeking out this sign.

SIGNS ON PERCUSSION ANDAUSCULTATION OF ABDOMEN

These are considered together as the number of signsunder these headings are comparatively small. But one


should remember that both auscultation and percussioncause much less discomfort to a patient with abdominalpain than palpation. Hence, auscultation of the abdomenhas to be completed immediately after inspectionfollowed by percussion keeping potentially paininducing palpation to the last.

Let us examine a few signs under this category.

Balance’s Sign

This refers to persistent dullness in the lefthypochondrium and left lumbar regions and shiftingdullness in the right flank typically present in ruptureof the spleen. When there is bleeding due to splenictrauma there is hemoperitoneum which is the reasonfor shifting dullness in the right flank, but left flankwill have persistent dullness due to the presence ofperisplenic hematoma and clots.

Relevant history of trauma, signs of internalhemorrhage, external evidence of injury like laceratedwounds, contusion, rib fractures, etc. should make thediagnosis clear without having to wait for Balance’ssign to appear. This is a surgical emergency and earlierthe bleeding is controlled surgically the better. Hence,if splenic injury is suspected, once the airway, breathingand circulation are stabilized the diagnosis should beconfirmed by imaging studies like USG and /or CT andarrangements are made for immediate blood transfusionand operative management.

Abdominal Signs 19

Federici’s Sign

In massive pneumoperitoneum, cardiac sounds areclearly heard with the stethoscope kept over theabdomen wall. This is due to unhindered conductionof sounds through air contained within the peritonealcavity.

Kehr’s Sign

It is the pain felt in the left shoulder by the patient whohas ruptured spleen. The underlying mechanism is theirritation of left dome of diaphragm by the blood. Theresultant pain stimulus traveling via the phrenic nerveis referred to the left shoulder also as supraclavicularnerves share the same root value as the phrenic nerve.

This sign is almost always present in a case of splenicrupture. But it is for the clinician to ask about it andanalyze it. Patient is unlikely to volunteer thisinformation as he is more likely to be bothered aboutthe severe abdominal pain he would certainly havebecause of ruptured spleen. Also in a case of polytraumathere are likely to be other direct injuries on the thoraxand upper limb. Hence, presence of Kehr’s sign getsmasked.

This sign is discussed here for convenience ofreference even though it is not elicited on percussion.


Kenawy’s Sign

It is the venous hum heard when one auscultates justbelow the xiphoid process. It is due to splenic veinengorgement associated with portal hypertension withsplenomegaly. The hum gets accentuated duringinspiration as the flow to the splenic vein increases dueto compression of the spleen by the descendingdiaphragm.

Puddle Sign

This is used to detect minimal ascites. As low as 150 mlof intraperitoneal fluid can be detected by this test, ifcarefully done. Patient is asked to lie prone for 5 minutesthen assume knee elbow position. The freeintraperitoneal fluid collects in the dependentperiumbilical area. This usually manifests as dullnessin the periumbilical region or in the most dependentpart of the abdomen.

The level of the fluid can be deduced by keepingthe bell of the stethoscope on the most dependant partand auscultating while one of the flanks is flickedregularly with fingers. Stethoscope is gradually movedtowards the opposite flank with the patient maintainingthe knee elbow position. A sharp increase in the intensityof the sound indicates the level of the fluid.

Abdominal Signs 21

Toma’s Sign

This is used to distinguish between inflammatory ascitesfrom ascites due to noninflammatory causes like portalhypertension, cardiac failure, hypoproteinemia, etc.

In inflammatory ascites there is gradual contractionof the mesentery which ultimately draws most of theintestines to the right. This is logical to expect, as theline of attachment of the mesentery is from left to theright, starting just to the left of L2 vertebra to the upperend of right sacroiliac joint. Hence, when the patientwith inflammatory ascites lies supine one can elicittympanic note on the right side whereas the note is moreuniformly dull on the left side.

RADIOLOGICAL SIGNS IN ABDOMEN

In evaluating a case of abdominal pathology, eitheracute or chronic, many a time clinical examinationproves inconclusive. One has to resort to investigationsin such cases to arrive at a diagnosis. One of the simplestinvestigation that is can be done easily in any hospitalat any time of the day or night is an X-ray of theabdomen, taken both in erect and supine position. Achest X-ray with domes of diaphragm is also useful inclassifying doubtful cases of upper abdominalpathology. Let us now examine a few important andrelatively easy radiological signs so as to finetune one’s


diagnostic acumen without having to resort toradiologist’s help all the time.

Benz’s Sign (Crow Foot or Seagull Sign)

It is indicative of gallbladder stones. About 90% of thegallbladder stones are radiolucent and hence do notshow up on plain X-ray. But when they do containenough calcium to be radiopaque one might be able toappreciate lucent areas within the radiopaque stonesindicating gradual build-up of the stones around aninflammatory focus. If the lucent area is tri-radiate thenit is called Mercedes Benz sign or crowfoot sign afterthe emblem of the famous car company. If it is biradiateit is called “seagull in flight” sign, as it resembles seagullwith its wings spread apart.

Claw Sign

It is a sign indicative ileocolic intussusception on bariumenema study. The barium enema casts a claw likeopaque shadow around the apex of the intussusceptum.It is safe to do a barium enema study whenever largebowel obstruction is suspected, one might emphasizehere.

Coffee-bean Sign (Omega Sign)

This is seen on plain X-ray in a case of sigmoidvolvulus. There is a huge loop of bowel occupying

Abdominal Signs 23

almost the whole of the abdomen with a centralbifurcation resembling one-half a coffee-bean.Occasionally, the gas shadow may resemble the Greekletter Omega. Then it is called Omega sign.

In both the above cases it is apparent that the loop inquestion is arising from the pelvis.

Colon Cut-off Sign

It is a sign detected on plain X-ray of the abdomen takenin a patient suspected of acute pancreatitis preferablyin erect posture. The radiolucent shadow cast by thegas in the transverse colon seems to come to an abruptend near the mid transverse colon due to the proximityof this part of the bowel to the inflamed pancreas,producing the classical appearance of colon “cut-off”.

Cupola Sign

It is a sign of free gas within peritoneum. If a chestX-ray including both the domes of the diagram is takenin erect posture then the free gas if present will collectbeneath the domes of diaphragm as a dark radiolucentcrescent.

Free air in the peritoneal cavity may be due toperforation of a hollow viscus, postlaparotomy orlaparoscopy or may follow certain gynecologicalprocedures live vaginal douche, hysteroscopy, etc.

It is said a good quality radiograph can detect aslittle as 1 ml of free gas in the peritoneum.


Double Bubble Sign

This is a sign of duodenal obstruction. A plain X-ray insuch a case will show two bubbles of gas in the upperabdomen. This is due to distension of the stomach aswell as the proximal duodenum, separated by thepylorus.

Duodenal obstruction is not so uncommon in thenewborn due to duodenal atresia. X-ray helps in earlydiagnosis of this conduction.

In the adults, stricture, annular pancreas, neoplasm,etc. can be the cause of duodenal obstruction. Herebarium study, upper GI endoscopy, etc. can be done toclarify the diagnosis further.

Football Sign

In young children all the free air might collect underthe anterior abdominal wall, which in a supineabdominal X-ray will cast a football like shadow. Thisis called football sign.

Medusa Lock Sign

This is characteristic of intestinal obstruction due toroundworms. These worms will have narrow columnsof gas in their alimentary canals. These may show upon a plain film as coiled locks of hair when there arelarge numbers of worms obstructing the lumen. Hence,

Abdominal Signs 25

the name “Medusa Lock sign” after the Greekmythological character.

Reverse 3 Sign

It is barium study sign seen in cases of carcinoma ofampulla of vater. Barium in the widened C loop ofduodenum with the lesion in the ampulla contribute tothis appearance.

Stierlin’s Sign

It is a radiological sign discernible on barium mealfollow through study.

There is constant emptying of the terminal ileumand the cecum with barium remaining either proximalto it or distal. This is due to the intestinal hurry causedby inflammatory process most commonly tuberculosis.

ThyroidSigns


Clinical examination of the thyroid gland is anotherfascinating experience for students of surgery. A goiteris a quintessentially surgical problem which excites anysurgeon worth his salt. Hence, a patient who happensto have a goiter is likely to be popular amongst students,through he/she is not likely to relish such popularity.

SIGNS IN THYROTOXICOSIS

All the clinical conditions affecting thyroid glandthyrotoxicosis perhaps has the most florid features. Aproperly taken history and careful examination makethe diagnosis clear enough in most cases. But somehowmore than the effects of excess thyroid hormone on thetarget organs, exophthalmos or ophthalmopathy thatoccurs simultaneously has been the source of most ofthe signs associated with thyrotoxicosis. While there isa long list of such “eye signs” which impressesbeginners no end, one has to realize that the presenceof these signs not really necessary to diagnose and treata case of thyrotoxicosis. The names that these myriadeye signs go by are of academic interest only. Studentsshould try to understand the underlying pathologywhich results in the particular sign. Whether or not onecan recollect the name should not matter. Butunfortunately in our exams sometimes more stress islaid on the students’ recollection of such names thanhis grasp of the underlying mechanism. Hence, an

Thyroid Signs 29

attempt has been made to list as many eye signs as onecould collect arranged in alphabetical order. If readerscan send me more signs with adequate references itwill be gratefully acknowledged.

Abadie’s Sign

This refers to spasm of levator palpebrae superiors dueto excessive sympathomimetic activity.

Ballet’s Sign

External ophthalmoplegia without internal ophthalmop-legia. External ophthalmoplegia can be partial orcomplete.

Becker’s Sign

This refers to abnormal pulsation of the retinal vessels.Needless to say, this necessitates endoscopicexamination.

Boston’s Sign

This refers to the jerky lowering of upper eyelid whenthe eyeball is rotated downwards. One has todifferentiate this from Von Graefe’s sign where thereference is to upper lid lag and not to its jerkymovement.


Dalrymple’s Sign

There is widening of palpebral fissure such that theupper sclera is clearly visible, in Grave’s disease. Thisis primarily due to spasm of the levator palpebralsuperioris causing retraction of the upper eyelid.

Enroth’s Sign

This refers to abnormal fullness of eyelid seen inGrave’s disease. This is due to edema and is moremanifest in the upper eyelid than lower eyelid.

Gifford’s Sign

In thyrotoxicosis due to Grave’s disease upper eyelidcannot be everted easily. This is again due to spasm oflevator palpebrae superioris.

In cases of proptosis of the eye due to intraorbitaltumors though the outward clinical picture may appearsimilar, Gifford’s sign is absent and upper eyelid canbe everted easily.

Graefe’s Sign

Syn: Von Graefe’s SignIn Grave’s disease when the patient is asked to followa finger moved slowly up and down in front of his uppereyelids persistently lag behind. One can confirm the

Thyroid Signs 31

presence of this lag by noticing the corneoscleral limbusclearly moving down ahead of the upper eyelid.

This sign has to be differentiated from ‘pseudo-Graefe’s sign which is the lagging behind of the uppereyelid due to paralysis of oculomotor nerve.

Griffith’s Sign

This is the opposite of Graefe’s sign. When the patientis asked to follow a finger moved in upward directionin front of his eyes the lower lid lags behind thecorneoscleral limbus persistently.

Grocco’s Sign

In Grave’s disease whenever the patient puts in amuscular effort there is acute dilalation of the heart.

Jellinek’s Sign

Brownish pigmentation of the upper eyelid seen in casesof Grave’s ophthalmopathy is referred to as Jellenek’ssign.

Jendrassik Sign

This refers to the paralysis of extraocular muscles. Thedifficulty in moving the eyeball upwards and outwardsis the most commonly noticed defect.


Joffroy’s Sign

When a patient with exophthalmos is asked to lookupwards with the head is bent downwards there will beno noticeable wrinkling of the forehead as would occurin normal individuals.

Knies’ Sign

Inequality in pupillary dilatation seen Grave’sophthalmopathy.

Kocher’s Sign

Mean’s Sign, Globe Lag SignHere when examiner’s hand held at the level of patients’eyes is suddenly raised higher the upper eyelid movesup more rapidly than the eyeball.

Loewi’s Sign

Pupils dilate readily with epinephrine in primarythyrotoxicosis.

Mobius’s Sign

Inability to achieve convergence of the eyeballs whilelooking at a near object. This is due to paresis of themedial rectus muscle.

Thyroid Signs 33

Mann’s Sign

Syn: Dixon Mann’s signIn Grave’s ophthalmopathy the two eyeballs do notappear to be at the same level.

Plummer’s Sign

If refers to patients inability to walk up a stair case.This is mainly due to proximal myopathy weakeningthe muscles necessary for such activity. This is one signin primary thyrotoxicosis which is not related toophthalmopathy.

Rosenbach’s Sign

It is the fine tremor of the upper eyelid, noticeableespecially when the eyes are gently closed. This signshares its name with the abdominal sign where theabdominal reflex is lost due to inflamed viscera.

Sainton’s Sign

In Grave’s ophthalmopathy, frontalis muscle contractsafter the cessation of levator action during upward gaze.Normally, these two muscles act simultaneously whenthe patient gaze upwards.


Stellwag’s Sign

This refers to infrequent blinking of the eyelids in Grave’sdisease. Also, the blinking is incomplete. The reasonsare obvious.

Snellen’s Sign

Syn: Reisman’s SignBruit heard over closed eyes in Grave’sophthalmopathy.

Topolanski’s Sign

There is pericorneal congestion in Grave’sophthalmopathy.

SIGNS ASSOCIATED WITH THYROIDPATHOLOGY OTHER THANTHYROTOXICOSIS

Berry’s Sign

Disappearance of the carotid pulse in a patient withgoiter is an ominous sign. If usually means theinfiltration of carotid sheath by thyroid malignancy.

Pemberton’s Sign

This sign is present when the patient has a retrosternalgoiter. Patient is asked to raise both his/her hands above

Thyroid Signs 35

the head and maintain that position for a few minutes.In case of retrosternal goiter patient will develop facialcongestion and engorgement of major neck veins. Thisis due to obstruction to venous drainage occurring atthe thoracic inlet due to pressure by the goiter. One hasto keep in mind that patient might actually faint duringthis maneuver. Hence, the test should be done only tillthe first signs of congestion are apparent.

Kocher’s Test

This has to be differentiated from Kocher’s sign seenin thyrotoxicosis.

This test helps to detect tracheomalacia in patientswith long standing large goiters. Mild pressure withthumb and fingers of examiners right hand on both thelobes will produce strider due to loss of elasticity ofthe tracheal rings. Such patients might have strider andbreathlessness postoperatively following removal of thegland.

SignsPertaining toOther Organ

Systems


These signs are discussed together as they arecomparatively fewer in number. Once again studentsof surgery are urged to understand the mechanismbehind these signs rather than just trying to memorizethem. And these signs are more useful when analyzedtogether with relevant history and proper clinicalexamination. One should not rely on any one clinicalsign only to include or rule out pathology of a particularorgan.

Let us now examine a few of these signs:

SIGNS IN TORSION TESTS

Angell’s Sign

This sign is present in cases of torsion of testis wherethe predisposing factor is the presence of a longmesorchium.

The presence of mesorchium cannot be made out inthe already torted testis, but can easily be made out onthe opposite side. The opposite testis lies horizontally.One can also feel the gap between the testis andepididymis palpation.

Deming’s Sign

This is another sign of torsion tests which helps todifferentiate if from acute epididymorchitis. In case oftorsion testis the affected testis lies at a higher level

Signs Pertaining to Other Organ Systems 39

than its fellow. This is because of the shortening of thecord due to the twist in it. Cremasteric muscle spasmalso contributes to the higher placement of the testis.

Prehn’s Sign

Elevation and support to the scrotum on the side of thepathology relieves the pain in epididymorchitis but notso in torsion testis.

The reason for this is obvious, i.e. inepididymorchitis at least part of the pain is due to thedragging effect of having an edematous testis on thespermatic cord. It gets relieved with support. But intorsion testis the pain is due to ischemia which remainsunaltered even in elevated position.

Differentiating torsion tests from acute epididy-morchitis is of great importance because of the dangerof gangrene of the testis in case of torsion if promptsurgical correction is not done. Hence, the significanceof these signs cannot be over emphasized. However, ifone is not sure about the diagnosis in an emergencysetting it is better to follow that time tested adage “whenin doubt, open and see”. Of course, if it can be arrangedpromptly Doppler ultrasound study of the testicularblood flow will be helpful, but it should not be at thecost of too much valuable time. A negative explorationis never as important as delayed surgery resulting inloss of testis.


SIGNS IN LATENT TETANY

Tetany is a state of abnormal muscular spasm inducedby deficiency of ionic calcium in blood. It can occur inhypoparathyroidism, alkalosis, massive bloodtransfusion, etc. Since it is a potentially life threateningcondition one has to recognize tetany when it is still inits latent stage. This is where knowledge of signs oflatent tetany will help. A few of the more importantsigns have been discussed below:

Chvostek’s Sign (Syn: Weiss Sign)

This is present whenever there is hypocalcemic tetany.Normally, this sign is elicited by tapping lightly in frontof the tragus to stimulate the branches of the facialnerve. In case of tetany there is muscular twitching ofthe facial muscles most notably at the angle of themouth.

The presence of these signs should alert the clinicianto the lurking danger of cardiac problems. Hence,immediate parenteral calcium supplementation isnecessary whenever this sign is evident. Hence, itsclinical significance cannot be over emphasized.

Escherich’s Sign

This is another, albeit less popular sign of latent tetany.Tapping the skin near the angle of the mouth leads toprotrusion of the lips.


Peroneal Sign

Tapping ever the peroneal nerve at the fibular neck willtrigger dorsiflexion and abduction in latent tetany.

Trousseau’s Sign

This refers to the induction of carpopedal spasm bytemporarily blocking arterial supply to the hand. Thisis done by the application of sphygmomanometer cuffto the arm, inflating above the systolic pressure andmaintaining it for about 3-5 minutes. During this timedue to lack of blood supply latent tetany becomes overtand the intrinsic muscles of the hand go into and spasmproducing the so-called “obstetrician’s hand(accoucheur’s hand)”. Of course do not forget to releasethe pressure after you have confirmed the presence ofthe sign.

One has to remember that the name Trousseau’s signalso refers to another phenomenon, i.e. occurrence ofmigrating thrombophlebitis in patients with visceralmalignancies especially with carcinoma of the pancreas.

SIGNS IN DEEP VEIN THROMBOSIS

Deep vein thrombosis is a condition which can developsilently in a hospitalized patient and has the potentialto kill the patient via pulmonary embolism. It is widelyaccepted that presence of deep vein thrombosis is muchwider than is commonly believed. Hence, clinicians


have always made efforts to detect it in time. Hence,the different signs. Students are warned that some ofthese signs are highly subjective and mostly elicitedclumsily. Occasionally vigorous attempts at eliciting asign may cause more harm than good by dislodging ahitherto quiescent thrombus. In today’s world wheremost well equipped hospitals have access to colorDoppler study of the venous system, these signs areslowly losing their significance. However, they arementioned here for the sake of completion.

Homan’s Sign

Dorsiflexion of the foot with the knee bent producespain in the calf whenever there is established thrombosisin the veins of the leg. One has to dorsiflex the footslowly and gently for fear of dislodging the clot. Alsosudden dorsiflexion might induce calf pain even inotherwise normal individuals.

Rupture of a popliteal cyst also produces a similarpicture with positive Homan’s sign. Inadvertentinitiation of anticoagulants in this clinical scenario is,needless to say, disastrous.

Moses’ Sign

Squeezing of the calf muscle produces pain when thereis established deep vein thrombus.


This sign has been deleted from most of thetextbooks now as repeated attempts to elicit it aredisastrous to the patient.

Whenever there is any doubt about the presence itis better to perform duplex scan to confirm or excludedeep vein thrombosis.

The most prudent approach for a surgeon would beto take prophylactic measures like low doseanticoagulants, early mobilization, calf muscleexercises, elastocrepe bandage application, etc. in theimmediate postoperative period. Better be safe thansorry!

Payr’s Sign

This is a sign of local thrombophlebitis and not of deepvein thrombosis. It has been included here forconvenience of study.

In cases of thrombophlebitis of the foot vein the factmay not be clinically obvious on inspection due to thethick skin of the sole. Pain on palpation over the soleindicates the presence of thrombophlebitis.

SIGNS OF VISCERAL MALIGNANCY

Leser-Trélat Sign

This refers to the sudden appearance and rapid increasein the number of seborrheic keratosis in patients


harboring internal malignancy. The condition is usuallyassociated with pruritus.

Trousseau’s Sign

Patients harboring internal malignancies like carcinomaof the pancreas, stomach, etc. often have migratingthrombophlebitis even before the primary lesionbecomes symptomatic. Hence, a clinician with highindex of suspicion should look for these life threateningconditions when a patient presents with innocuouslooking thrombophlebitis.

It may be noted that this sign shares its name withthe obstetricians hand sign of latent tetany.

Troisier’s Sign

It is the enlargement of Virchow’s node in the leftsupraclavicular region in malignancies ofintraperitoneal organs or genitalia. Though it may rarelyoccur as the presenting symptom in these cases, itinvariably signifies poor prognosis.

SIGNS IN PERIPHERAL VASCULAR DISEASE

Sign of Disappearing Pulse (De Weese Sign)

This sign may be present in cases were peripheralvascular deficit is present but clinically the pulses arepalpable. If in such a patient ischemia is stronglysuspected based on history then the patient is asked to


exercise or walk to the point of claudication and thenthe pulse is again looked for. The pulse which wasearlier present will now be absent. This is due tovasodilatation that results from exercise. The deficientblood flow which was enough for pulse to be felt at aresting stage will now become insufficient to impart apalpable pulsation.

Harvey’s Sign

This is an indirect method of inferring reduced arterialflow to the limbs. In case of arterial deficit obviously thevenous flow is sluggish as well. This can be deduced byassessing venous refilling. A segment of vein is emptiedof the blood by placing index fingers of both hands firmlyon the vein, initially side by side and later moving themapart. Now if the finger farther from the heart is lifted therefilling of vein is slower in an ischemic limb than normal.

A similar deduction can be drawn by lifting the limbabove the level of the heart to empty the veins and thenplacing it horizontal. In a normal limb veins fill upwithin 5 seconds in a severely ischemic limb this maytake up to 30 seconds.

Fuchsig Sign

This helps to detect the presence of popliteal pulse. Thispulse is difficult to palpate even is normal individual.Hence, this indirect method is sometimes employed todetect its presence. Patient is asked to sit on a chair


cross legged with the leg in question over its fellow,knee crossing knee. In a relaxed patient if the poplitealpulse is present then the upper leg will oscillatesynchronous with the pulse. A negative test may meaneither a very weak pulse or absent one.

SIGNS IN ARTERIAL ANEURYSM

In today’s world of increasing subspecialization ageneral surgeon is rarely called upon to manage a knowncase of aneurysm. Hence, the signs which are going tobe described are rarely, if ever, going to be seen in asurgical ward. But in a patient who is in the surgicalward for some other complaint, if any of these signsare noticed it is worth while ascertaining the cause. Earlydiagnosis of a major artery aneurysm might mean thedifference between life and death for the patientconcerned.

Again the signs are described in alphabetic order.

Bozzolo’s Sign

Visible pulsations of the arterioles in the nasal mucousmembrane is believed to indicate the presence ofaneurysm of thoracic aorta.

Cardarelli’s Sign

In aneurysms the aortic arch there will be transversepulsations of an endotracheal tube, if the patient has


been intubated. This is due to transmission of arterialpulsation to the nearby trachea.

Dorendorf’s Sign

In aneurysm of the aortic arch there will be fullness ofone of the supraclavicular fossae.

Delbet’s Sign

In case of aneurysm of the main artery of a limb, absentdistal pulses, if nutrition of the distal limb is maintainedthen the collateral circulation is deemed to be sufficientfor maintaining the vascularity of the distal limb. Thenutrition of the distal limb can be deduced by lookingfor signs of chronic ischemia like thin and shining skin,loss of subcutaneous fat brittle nail, etc. Loss of distalpulse alone does not indicate poor vascularity.

Drummonds Sign

Puffing sound synchronous with cardiac systole heardfrom the nostrils especially when the mouth is closedin some cases of aortic aneurysm.

Glasgow Sign

Systolic murmur heard over the brachial artery inaneurysm of the aorta.

Porter’s Sign (Oliver’s Sign)

Tracheal tug noticeable in aneurysms of arch of aorta.


Similar tug may be there when carcinoma of the mainbronchus becomes adherent to arch of aorta.

Queenu Muret Sign

This is another sign indicating the maintenance of distalblood supply in a case of aneurysm of main artery of alimb proximally. If in such a case a needle prick is madedistal to the aneurysm, free flow of blood indicatessatisfactory collateral circulation.

Both this as well as Delbet’s sign have to benecessarily looked for if the proposed treatment for theaneurysm is ligation of the artery. Of course, with theadvent of aneurysmorrhaphy, grafts and stents, ligationof an aneurysm is only of academic interest now.

Perez’s Sign

Crepts heard over the upper chest when the arms arealternatively raised and lowered in case of aneurysmof aortic arch.

This is also positive in case of fibrous mediastinitis.

Branham’s Sign (Nicoladoni’s Sign)

Strictly speaking, this is not a sign of any aneurysm butof arterio-venous malformation.

Whenever arteriovenous fistula’s is suspected, if thefeeding artery to the lesion is compressed then not only


the size of the AV malformation reduces but theassociated bruit disappears. Also there is a fall in pulserate and normalization of pulse pressure. Of course inthese days of duplex scanning this sign is hardly eversought for. Blocking the feeding vessel will interruptthe hyperdynamic circulation caused by shunting ofblood. This results in all the changes in hemodynamicslisted above.

SIGNS OF HERNIA

It is indeed surprising that diagnosing a hernia shouldneed the assistance of signs. Of course diagnosing ahernia is a straightforward act most of the time but notalways, especially when dealing with rare types or earlystages of hernia.

Silk Glove Sign

This helps the clinician to distinguish between a normalcord and a cord with empty inguinal hernial sac.Sometimes especially in indirect inguinal herniae thecontents may not prolapse into the sac at the patient’swill nor after standing. In such cases, if one palpatesthe cord, the cord will feel as if it is wrapped in silk.The cord contents cannot be felt distinctly. This is dueto the empty sac made of parietal peritoneum.


Howship-Romberg Sign

This may point to an obturator hernia but needs a veryhigh index of clinical suspicion to detect its presence.In cases of obturator hernia pressure on the obturatornerve by the hernia contents might produce pain on theinner aspect of patient’s knee.

Border Sign

This radiological sign indicates the occurrence ofventral hernia in the early postoperative period. If abarium study is done in the early postoperative periodthe presence of a ventral hernia or incisional hernia isdetected by the sharp outlining of the inferior and lateralborders by the barium within the level loops whilemedial and upper borders are not well delineated.

This occurs due to the dependant state of either thelateral for inferior border of hernia in various positionsof the body.

MISCELLANEOUS SIGNS

Here we discuss a group of signs which are notinterconnected but are interesting all the same. Theyare listed under the heading ‘miscellaneous’ only forconvenience. This arbitrary clustering of signs in noway diminishes their significance in the appropriateclinical setting.


Battle’s Sign

This refers to ecchymosis over the mastoid process incases of fracture of the middle cranial fossa. In pre CTera this sign was a very significant clinical findingindicating the gravity of the trauma.

Boyee’s Sign

This is a sign of esophageal diverticulum. Often in anesophageal diverticulum food residue and saliva canbe found.

If one presses the side of the neck, the diverticulummight empty the contents with a gurgling sound.

Eyelash Sign

This is used to differentiate true unconsciousness frommalingering, hysteria and such other functional states.In the latter case if the eyelashes are stroked gently therewill be movement of eyelids, but not in a true organic ortraumatic brain lesion.

Hoover’s Sign

Another sign which might help detect genuine organiclesion from functional one. If a patient having genuineparalysis one lower limb, is asked to lift that leg he/sheinvoluntarily makes counter pressure with the heel ofthe other leg against the bed in an effort to lift theparalyzed leg. This counter pressure can be noticed


whether the affected limb could be moved or not. Nosuch counter pressure is discernible in hysteria ormalingering.

Macewan’s Sign (Cracked Pot Sign)

Percussion over the skull of a child with hydrocephaluswill give the sound skin to tapping a cracked pot.

Nikolsky’s Sign

In pemphigus vulgaris rubbing the skin with slightpressure might lead to pealing off of the epidermis.

Milian’s Sign

This differentiates between erysipelas and streptococcalcellulitis. Erysipelas is a streptococcal infection of theskin while cellulitis is inflammation of the subcutaneoustissue. In the head and neck region the two can bedistinguished by the fact that erysipelas spreads to theexternal ear but cellulitis does not as there is nosubcutaneous tissue in the pinna.

Kanavel’s Sign

In inflammation of the ulnar bursa the point ofmaximum tenderness lies between the transverse palmarcreases.


Ripault’s Sign

It is a sign of death. It refers to permanent change inthe shape of pupil by unilateral pressure on the eyeball.

Tinel’s Sign (DTP Sign)

If the skin over the point of injury to a peripheral nerveis gently tapped and patient complains of tingling or“pins and needles” along the distribution of the nerve,it means that the regeneration of the nerve is under way.Similar “Distal tingling on percussion” (DTP) alsooccurs when the injury to the nerve is only partial.

Setting Sun Sign

As the name suggests this is a sign of grave prognosisin conditions like hydrocephalus, intracranial bleed,brain tumor, etc.

The iris appears to set behind the lower eyelidexposing large area of sclera above the corneoscleraljunction.

Slip Sign

This is a popular “sign” amongst students. All it refersto is that in a lipoma the edge of the swelling slips awayfrom the palpating finger. This palpating feel hasattained the status of a ‘sign’ with time.


Wrist Sign

Present in Marfan’s syndrome the abnormally longfingers of the patient will make thumb and little fingeroverlap each other when the opposite wrist is gripped.

ClinicalSyndromes


A clinical syndrome can best be defined as a collectionof signs and symptoms which are commonly associatedwith a particular morbid process. For example, if anypathological condition is associated with a particularclinical picture more often than not, then all the signsand symptoms which contribute to such clinical picturemay be said to constitute a clinical syndrome, e.g.Murphy’s syndrome in acute appendicitis.

But a particular set of signs and symptoms in termeda syndrome only if it is consistently associated with thesame morbid process and knowledge of such asassociation between the clinical picture and diseaseprocess helps in arriving at a diagnosis, e.g. Meig’ssyndrome in fibromyoma of the ovary.

Often it has been noticed that our students areworried about not knowing a particular syndrome. Onewho knows the most syndromes is likely to beconsidered the most well read amongst his peers. Thisstate of affairs is partly due to the undue importanceattached by some examiners to the recalling of namesof vague clinical syndromes. But it is necessary toremember that it is all very well if one knows aboutthese obscure syndromes after knowing commonconditions. If not, it is far better to know only commonconditions now, only then notice syndromes. After all,one’s clinical career involves treating commonconditions 99% of the time. One is not likely to beaccused of missing a rare syndrome. But even a master

Clinical Syndromes 57

of syndromes is likely to be held responsible if acommon disease process is overlooked in the searchfor exotica. Some one has rightly said “if you make arare diagnosis you are rarely correct”.

Why then should we have a section on syndromes?The reason is simple. I have observed students spendinghours in the library looking up reference books just toget the name of a particular syndrome. Many books,especially the western ones mention only moresignificant syndromes and skip the rest. In order thatan interested student gets basic information on mostsurgical syndromes at one place this chapter has beenincluded. Once again I remind my students that knowingthese syndromes is secondary to a proper study ofcommon clinical conditions.

Here too the syndromes have been discussed in thealphabetical order of their names. I have tried to discussin brief only fen amongst them which I think are mostrelevant. Wherever possible synonyms have beenmentioned within brackets.

AURICULOTEMPORAL NERVE SYNDROME

Syn: Frey’s syndrome: Gustatory SweatingThis refers to flushing, sweating and hyperaesthesia inthe region of the ear and cheek in response tomastication of food. This occurs due to erroneoustransmission of parasympathetic stimulus meant for


stimulating salivation through the sympathetic channelsinnervating skin and its integuments. Often such an errorfollows trauma to the region or surgery on the parotidgland. Following such insult there is cross connectionbetween parasympathetic fibers from otic ganglion andsympathetic fibers from the superior cervical ganglion.Hence, whenever the patient eats the impulses meantto stimulate the parotid gland actually go to the skinstimulating sweat glands, blood vessels etc. Hence theclinical picture of sweating and flushing.

If the syndrome follows surgery or trauma, then onlyreassurance and local anti perspirant spray is sufficient.Usually symptoms improve in 6 months. Occasionally,if the symptoms become annoying enough, then surgicalsection of Jacobson’s nerve or tympanic branch ofglossopharyngeal nerve or vidian nerve.

AFFERENT LOOP SYNDROME

This occurs following gastrectomy and Billroth IIanastomosis. If there is obstruction to the flow of contentof afferent loop into the gastric stump, then there isstagnation and increased pressure within the proximalloop. Immediately after surgery this might result induodenal blow out. If blow out does not happen thenthe patient will complain of pain and bloating sensationin the epigastrium. The bilious contents might suddenlyget discharged into the gastric stump when the pressure


builds up beyond a threshold level, resulting invomiting. Increased pressure within the duodenummight lead to acute pancreatitis due to contents enteringpancreatic duct.

If left untreated continued dilatation of the proximaljejunum and duodenum may lead to gangrene of thebowel.

Afferent loop obstruction needs surgical treatmentmost of the time. Common operation done areenteroenterostomy between proximal dilated andefferent loops. Alternatively the distal portion of theafferent loop can be resected and the anastomosisconverted to a Roux-en-Y type.

The common causes for afferent loop syndrome aretoo long an afferent loop, kink in the loop, volvulus,jejunogastric intussusception internal herniation etc.Hence it is advisable to keep the afferent loop as shortas possible and close all potential spaces for internalherniation.

BECKWITH-WIEDEMANN SYNDROME

Syn: EMG SyndromeIn this neonatal syndrome, there is association betweenexomphalos, macroglossia and gigantism. Hypoglyce-mia is another dangerous feature of this syndrome whichneeds to be expected and promptly dealt with to avoidfatal outcome.


This syndrome has an autosomal recessiveinheritance. It is also called the “EMG Syndrome”, anacronym for its three important clinical features, i.e.,exomphalos, macroglossia and gigantism.

BLAND-WHITE-GARLAND SYNDROME

This syndrome refers to the anomalous origin of leftmain coronary artery from the pulmonary artery; thereis flow of deoxygenated blood from pulmonary arteryto the left main coronary. Patient will have exertionaldyspnoea due to resultant ischemia and cardiac failure.The infant may survive into adulthood only if there isgood collateral circulation between right and leftcoronary arteries.

Cardiac catheterization and angiography arediagnostic as the clinical picture is usually not specific.Treatment is surgical re-implantation of left maincoronary artery directly to the aorta. Mortality inuntreated cases is very high.

BLIND LOOP SYNDROME

This is the clinical picture usually seen in patients whohave undergone bowel surgery, most classically end toside anastomosis. If the proximal residual segment ofeither limit of an end to side anastomosis be it jejunum,ileum or colon is allowed to be more than 1.5 cm beyondthe anastomosis then that segment will not drain


properly leading to stagnation and bacterial overgrowth.This change in intestinal microflora will lead tomalnutrition. Patient will have malabsorption especiallyfor fat, vitamins and other micronutrients. Patient willhave symptoms of dyspepsia, flatulence, abdominalpain, anemia, etc. and may even suffer from frankintestinal obstruction.

Treatment involves giving rest to the bowel keepingthe patient nil by mouth and inserting a Ryle’s tubeespecially if there is subacute intestinal obstruction. Ifthe patient can take oral feeds, then broad spectrumantibiotics like doxycycline with or withoutmetronidazole should be given to control the luminalbacterial overgrowth. This coupled with probiotics likelacto bacillus spores given orally should be able torestore the intestinal micro flora. The only problem withthis line of management is that the patient is likely tohave only a temporary relief before the condition recurs.The final court of appeal is surgery wherein the blindloop is resected if technically feasible. Hence one hasto prevent the occurrence of blind loop syndrome duringthe first surgery itself, by keeping the blind loop as shortas possible under the circumstances.

Similar clinical picture might be seen following sideto side anastomosis by-passing a non-resectable growthor in a particularly difficult case of adhesive obstructionwhere the exact point of obstruction can not be reacheddue to dense adhesions. Since these are unavoidable


situations one has to warn the patient about possibilityof the blind loop syndrome and he should be advisedabout appropriate nutritional support.

Another condition where occasionally a blind looplike picture develops spontaneously is intestinaltuberculosis or any other condition causing multiplestrictures of the bowel. The segment between twoconsecutive strictures is partially obstructed and henceleads to stagnation and all its associated problems. Herethe treatment involves resection of the entire segmentor strictureplasty to relieve obstruction.

BOERHAAVE’S SYNDROME

This refers to an acute surgical emergency where inthere is perforation of the lower oesophagus due tobarotrauma. It usually follows a violent bout of vomitingand retching. Patient complains of severe pain in theepigastrium and retrosternal region. There might besymptoms of the pleural effusion. On examinationpatient appears toxic and is most cases has surgicalemphysema. This condition needs a high index ofsuspicion on the part of clinician for diagnosis. Andunless emergent management is instituted patient likelyto succumb to massive thoracic inflammation thatensues.

If suspected then a contrast film with water solublecontrast confirms the diagnosis in most cases. Onlyrarely thin barium may have to be used. One has to


bear in mind that too much time should not be wastedin arriving at a diagnosis. A relevant history along withthe clinical picture including surgical emphysema isalmost confirmatory.

Management usually involves emergencythoracotomy to deal with the contamination and toinstitute drainage. The usual and prudent approach isto do an oesophageal diversion and toilet. In very earlycases, direct two layers repair of the oesophagus canbe attempted. Attempting oesophageal resection in analready compromised patient is a very risky affair andhence not usually practised.

In a few cases where the diagnosis has been madebut the clinical picture is mild a conservative approachcan be followed by keeping the patient nil per orallystarting TPN and higher antibiotics. Of course if thecondition of the patient does not improve rapidly thensurgical intervention becomes unavoidable.

BUDD-CHIARI SYNDROME

Syn: Robi Tansky’s DiseaseThis syndrome refers to the clinical picture which resultsfrom obstruction to the hepatic veins. The cause ofobstruction is thrombosis in most cases and onlyoccasionally is the venous obstruction due to web orneoplastic encasement of the hepatic veins. Theunderlying pathology for spontaneous thrombosiswithin the hepatic veins may occasionally be apparent


like thrombocytosis, polycythemia or oral contraceptivepills but in most cases idiopathic.

The onset can be acute or more insidious. In its acuteform there will be gross hepatomegaly associated withsevere right hypochondrial pain, portal hypertensionwith gross intractable ascites and may rapidly progressto hepatic coma and death.

When the onset is more protracted patient maydevelop cirrhosis, portal hypertension, oesophagealvarices etc resulting in slow deterioration in health.

Diagnosis depends on high index of suspicion andgetting relevant investigations like color Doppler,contrast CT, liver function tests, etc.

Definitive treatment may be possible in rare caseswhen the obstruction is due to a web obstructingvascular flow. Endovascular meatotomy is theprocedure of choice and is done transatrially underguidance. In other cases porta systemic shunts can bedone if the patient’s general condition permits it butthe results are not always satisfactory.

CARCINOID SYNDROME

As the name suggests this syndrome is associated withcarcinoid tumors. More than 95% carcinoid tumorsoccur in the gastrointestinal tract and produce thesyndrome complex only when they metastasize to theliver. These tumors arising from Kulchitsky cellsproduce substances like serotonin, kinins, histamine,


prostaglandins, etc. which are responsible for the signsand symptoms of carcinoid syndrome. The classicalpicture occurs in about 10% of the patients and includesflushing, angiomas, bronchospasm, diarrhoea andoccasionally mental variations. There might be stenosisof tricuspid and pulmonary valves leading to cardiacdysfunction. The symptoms are sometimes induced byintake of alcohol, chocolate cheese, etc.

Diagnosis can be clinical by detecting high levelsof 5-Hydroxy indole acetic acid (5-HIAA) in urine.Nuclear scintigraphy may help in localizing the tumor.

Treatment is usually symptomatic as the establishedsyndrome usually means metastatic disease. However,it worth while resecting the primary if it is feasible.

CHILAIDITI’S SYNDROME

This syndrome causes diagnostic confusion duringevaluation of acute abdomen cases. Occasionally,especially in children and in the elderly there will beinterposition of transverse colon between the right lobeof the liver and the right dome of the diaphragm. Inerect abdominal and chest x rays it appears as gas underthe diaphragm causing diagnostic dilemma.

Chilaiditi’s syndrome can be distinguished from freegas by the following features.

The gas in chilaiditis syndrome is within the boweland hence does not form a sharp crescent. It is borders


may show slight undulation and haustral markings ifseen carefully.

There will be no gas under the left dome ofdiaphragm in chilaiditi’s syndrome.

Classical clinical picture of perforative peritonitisis absent.

COMPARTMENT SYNDROME

It is a situation wherein there is increased pressure withina confined anatomic space leading to obstruction ofvascular flow. If not treated early there may be threat tothe function and viability of the tissues supplied.Classically, such compartment syndrome occurs in thelower limb following trauma and hemorrhage. Infectionand gangrene is another life threatening condition thatcan contribute to such patients death. Increasedcollection of extracellular fluid in infection and of bloodin trauma leads to increased pressure within themusculofascial compartments of the lower limb leadingto reduced blood supply to the distal limb. There willbe pallor, pulselessness and paresthesia of the foot alongwith severe pain.

The only way to prevent gross limb oedema andgangrene of distal limb is to do an urgent decompressivefasciotomy of the deep fascia, thus relieving the pressurebuilds up.

Similar pathology may occur in the upper limb,peritoneal cavity or any other closed body space.


Treatment almost always involves immediate surgeryif one wishes to avoid distal ischemia.

Also one has to remember the possibility of crushsyndrome if the aetiology involves severe crushingtrauma.

CRUSH SYNDROME

This is a shock like state that follows major crush injuryof the muscles. In its classical form, it occurs after therelease of the compressive force crushing the muscles,as is often the case in case of road-traffic accidents,mining accidents, earthquake, etc. The sudden releaseof myoglobin into the circulation may damage the renaltubules resulting in oliguria and renal shutdown.Occasionally similar unfortunate result may follow therelease of tourniquet.

CRST SYNDROME

It is a syndrome usually observed in scleroderma. It ischaracterized by:• Calcinosis cutis — C• Raynaud’s phenomenon — R• Sclerodactyly — S• Telangiectasia. — T

Scleroderma is a disease in which there isprogressive thickening of the skin due to thickening offibrous tissue with eventual atrophy of the epidermis.


CRONKHITE-CANADA SYNDROME

It is a rare condition in which there are gastrointestinalpolyps, alopecia and nail dystrophy.

CRIGLER-NAJJAR SYNDROME

It is a type of familial non hemolytic jaundice whereinthere is unconjugated hyperbilirubinemia. There is acongenital defect in conjugation of bilirubin to formbilirubin diglucuronide due to absence of glucuronyltransferase.

During early infancy it can cause permanent braindamage resembling kernicterus, especially whenglucuronyl transferase is completely absent (type ICrigler-Najjar syndrome). In type II disease, there isonly partial deficiency of the enzyme and hence thedisease runs a milder course. Type I disease isfortunately autosomal recessive whereas Type II isdominant.

Phenobarbitone has been used in the treatment toinduce glucuronyl transferase in the liver.

CUSHING’S SYNDROME

This refers to the clinical picture observed when thereis inappropriately elevated plasma cortisol levels. Theincrease in plasma cortisol levels may be:a. ACTH dependent like in the case of pituitary

adenoma (also called Cushing’s disease) external


administration of excess of ACTH, ectopic ACTHsecretary tumors, etc.

b. Independent of ACTH, e.g. hyperfunctioningneoplasm of adrenal cortex or excessive exogenousadministration of corticosteroids.

Clinical features include a diabetes like status,hypertension myopathy, osteoporosis cutaneous striae,central obesity including moon face and buffalo humpetc. Women may develop hirsutism, acne andamenorrhoea. In some patients mental depression isapparent.

Treatment depends on the underlying cause. Furtherdetails are beyond the scope of this book.

CRUVEILHIER-BAUMGARTEN’SSYNDROME

In cirrhosis of the liver umbilical and paraumbilicalveins open up and there will be varicosities of theperiumbilical veins. The clinical picture of dilated andtortuous veins around the umbilical is called caputmedusae (medusa head appearance) after the Greekmythological character. The flow of blood will be awayfrom the umbilical. This forms one of the sites ofportosystemic anastomosis. This whole clinical entityis named Cruveilhier-Baumgarten’s disease orsyndrome.


CAROTID STEAL SYNDROME

Signs and symptoms of vertebrobasilar insufficiencyresulting from siphoning of the blood from the vertebralartery to the external carotid artery are referred to asthe carotid steal syndrome. Patient has transientischemic attacks and spells of dizziness and loss ofbalance.

DUBIN-JOHNSON SYNDROME

It is a type of congenital non-hemolytic hyper-bilirubinemia. There is defective transport of conjugatedbilirubin into the biliary canaliculi and hence conjugatedhyperbilirubinemia occurs.

Diagnosis is established by the presence ofbilirubinemia poor bromsulphthalein (BSP) clearanceand typical picture on liver biopsy. Prognosis isgenerally good.

DUMPING SYNDROME

Syn: Postcibal syndromeIt is the most distressing post gastrectomy clinicalsyndrome. It is of two types. In the early dumpingsyndrome patient suffers from vasomotor symptomslike hypotension, tachycardia, sweating and giddinesswithin a few minutes of food ingestion. There may be


colicky abdominal pain followed by diarrhoea. Theunderlying mechanism is simple. It is basically adisorder of a carbohydrate metabolism. In a patient whohas had gastrectomy especially Billroth II type, there issudden presentation of large quantities of carbohydrateto the small bowel as the “antral pump” no longer exists.This large carbohydrate load leads to sudden transienthyperglycemia which prevents further carbohydrateabsorption. The carbohydrate thus left behind in thebowel, lumen draws in water from the mucosa alongosmotic gradient. This results in increased bowelactivity, diarrhoea as well as features of hypotension.Ingestion of more carbohydrates worsens this condition.Patient is advised to take small, dry meals poor incarbohydrates at frequent intervals. Octreotide may beof use in preventing symptoms but it is too expensivefor routine use. A few cases might need surgery in theform of conversion of Billroth II to Billroth I if possibleor interposition of antiperistaltic bowel segmentbetween the stomach and the jejunum.

On the other hand, late dumping is less common aswell as less distressing. Here patient develops featuresof hypoglycemia about 2 hrs after food intake. Herethe initial hyperglycemia stimulates exaggerated insulinsecretion leading to hypoglycemia. Ingestion of morefood usually relieves the symptoms. This syndrome isusually treated conservatively.


GARDNER’S SYNDROME

It is one of the polyposis coli syndromes. It is inheritedas an autosomal dominant trait and is associated withmultiple tumors like osteomas, fibromas, desmoids, andepidermoid cysts early in life followed later by thedevelopment of multiple colonic polyps. There isincreased risk of colorectal malignancy in this condition.The only definitive surgical procedure which eliminatesthe risk of colorectal malignancy is total procto-colectomy followed by permanent ileostomy or ileoanalanastomosis. Ileorectal anastomosis after resection ofthe whole colon sans rectum is less morbid but alsoless protective.

GILBERT’S SYNDROME

It is a type of familial non hemolytic jaundice. Itgenerally inherited as autosomal dominant and presentsitself only during adolescence. Patient develops mildjaundice (usually below 6 mg/dl) and may haveanorexia, malaise and upper abdominal pain. Suchepisodes may be triggered by any stress like infection,starvation, etc.

Investigations reveal increase in indirect bilirubin.There is no evidence of hemolysis nor any bilirubinuria.

The underlying pathology is a deficiency ofglucuronyl transferase. Occasionally the uptake of


unconjugated bilirubin from the plasma itself isimpaired.

Mild cases do not require any treatment. In somecases treatment with phenobarbitone has been used tostimulate liver enzymes including glucuronyltransferase thus relieving symptoms.

HORNER’S SYNDROME

It occurs when there is involvement of cervicalsympathetic pathway anywhere from hypothalamus topost ganglion nerves. It consists of ptosis (drooping)of the upper eyelid, miosis (constriction) of the pupil,enophthalmos (small, indrawn eyeball) and anhidrosis(absence of sweating in the ipsilateral hemiface. Whenthe Horner’s syndrome is due to central cause like abrain stem tumor or infarct usually other localizing signsappear making the diagnosis clear. Most of the commonsurgical conditions like cervical rib, Pancoast’s tumor,brachial plexus trauma carcinoma thyroid etc involvethe preganglionic nerves.

In post-ganglion Horner’s syndrome, nerves beyondthe superior cervical ganglion are affected by base skullfracture, tumor, cavernous sinus thrombosis, etc. It isnoteworthy that sweating is normal when postganglionfibers only are involved.

Horner’s syndrome is important clinically in that itdraws one’s attention to the underlying pathology whichis almost always more sinister.


HEPATORENAL SYNDROME

This term is now used to indicate any renal failureoccurring in patients with parenchymal liver diseasewhen no other cause can be found. This is especiallyso in the immediate post operative period after surgeryon the biliary tract in a jaundiced patient. Liver cirrhosisis another cause making patients vulnerable forhepatorenal syndrome. It may be precipitated bygastrointestinal bleeding, surgical intervention,abdominal paracentesis and sometimes even forceddiuresis. Initially, it resembles pre-renal azotemia butdoes not respond well to correction of volume depletion.It may rapidly progress to acute tubular necrosis.

It is important to rule out other causes ofsimultaneous liver and renal damage like sepsis, shock,drugs like halothane and aminoglycoside, toxemia etcwhich can mimic the clinical picture of hepatorenalsyndrome.

It is interesting to note that the kidneys in suchpatients are histologically normal and function normallywhen transplanted onto a patient without liver disease.Only late in the progression of hepatorenal syndromechanges of acute lobular necrosis (ATN) set in.

It is not clear what causes hepatorenal syndrome.Some believe it is the direct damage caused by bilirubinand bile salts to renal tubules. Others believe there isan element of ischemic damage in the development ofthis process. It is possible that there are toxins in the


serum of jaundiced patients which are noxious to therenal tubules.

Prevention as well as treatment consists ofmaintaining high effective plasma volume both beforeand after surgical intervention. As long as there are nochanges of ATN then the kidneys recover fully. OnceATN is established then the treatment has to be alteredaccordingly. It goes without saying that one has to ruleout or eliminate other causes of acute renal damage.

JOB SYNDROME

It is a variant of hyperimmunoglobulin E syndrome seenin fair skin individuals. Patient starts developingsymptoms from the first year of life itself. They includerecurrent seborrheic eruptions of the scalp, dystrophicnails, follicular eruptions resembling herpes and anunexplained tendency to develop multiple staphy-lococcal ‘cold’ abscesses. There are no inflammatorysigns perhaps due to defective erythema response.Patient may also have recurrent otitis media, respiratorytract infection and liver and lung abscesses.

This syndrome is named after the famous biblicalcharacter Job who is supposed to have suffered fromchronic furunculosis and multiple boils.

KEARNS-SAYRE SYNDROME

It is disorder of mitochondrial function. It consistsof hypoparathyroidism, progressive external


ophthalmoplegia, pigmentary retinopathy andcardiomyopathy. Patient presents with ophthalmicsymptoms at an early age, but the possibility ofhypoparathyroidism should be kept in mind. Thissyndrome has been noted to occur with diabetesmellitus.

KLIPPEL-TRENAUNAY-WEBER SYNDROME

Elephantiasis Congenita Angiomatosa

Usually seen in the extremities wherein there iscombination of angiomatosis and localized gigantismdue to hypertrophy of bone and muscle. Hence, it issometimes called angiosteohypertrophy. The tissuehypertrophy is presumably due to abundant vascularityof the part resulting from angiomatosis.

KOENIG’S SYNDROME

This refers to the alternating attacks of constipation anddiarrhea as well as intestinal colic and gurgling in theright iliac fossa associated with ileocecal tuberculosis.Ileocecal TB, especially the ulcerative type often leadsto multiple strictures in the distal ileum. This initiallytends to cause sub acute obstruction and stasis (i.e.constipation). The resultant bacterial over growthultimately causes enteritis and diarrhea.


LAMB SYNDROME

The name is an acronym for the following clinicalfeatures, i.e.• L — Lentiginosis• A — Atrial myxoma• M — Mucocutaneous myxoma• B — Blue naevi

Its recognition is important as there is increased riskof melanoma in these patients.

LAMBERT-EATON SYNDROME

Syn: Eaton-Lambert Syndrome

It is the proximal myopathy associated with internalmalignancy. The muscle weakness is progressive andnot associated with any cutaneous lesions ofdermatomyositis.

Its clinical significance lies in the fact that it drawsone’s attention to the underlying internal malignancy.

LERICHE’S SYNDROME

First described by Leriche in 1923. Typical symptomcomplex as described by Leriche affects men betweenthe age of 35 to 60 in whom there is thromboticocclusion of aortic bifurcation. It includes extremefatiguability of both lower limbs, symmetrical atrophy


of both lower limbs, pallor of lower limbs and mosttypically inability to achieve stable penile erection. Thelast symptom occurs due to reduced flow throughinternal pudendal artery and hence poor filling ofcorpora cavernosa.

The most common cause for thrombotic occlusion ofaortic bifurcation is atherosclerosis. And as the processof narrowing develops slowly over a period of months,acute symptoms are rare.

Finally, there may be ulceration and gangrene of thelower limbs.

Diagnosis is established by color Doppler studiesand arteriography. Treatment involves by passing theocclusion in most cases though in selected casesthromboendarterectomy might give satisfactory results.

LYNCH SYNDROME

Syn: 1. Familial Atypical Multiple Mole Melanoma(FAMMM) Syndrome

2. BK Mole Syndrome (after the familiesstudied)

It is a familial disorder with multiple dysplastic naevi,transmitted as dominant trait. Many of these patientsdevelop melanoma in these naevi. These patients are alsoat risk of developing primary melanoma. When two ormore members of the same family are affected the chanceof melanoma is deemed to be more than 50%.


MUNCHAUSEN’S SYNDROME

Syn: Hospital Hopper SyndromeIt is a term coined by Lord Asher in 1951 to describefactitious disorders wherein the patient eitherexaggerates or simply invents varied symptoms in orderto gain attention. Typically he will have a long pasthistory, sometimes interspersed with multipleoperations, and history of having consulted manydoctors.

The description of symptoms by the patient is oftenvery convincing as the patient will have learnt over timehow to present his case. Munchausen’s syndrome needsto be differentiated from plain malingering wherein thepatient’s motivation is purely external, e.g. sicknessabsenteeism.

Baron Munchausen was an German army officer inthe 18th century who, it was believed, told fantastictales about his own exploits just to gain attention.

MURPHY’S SYNDROME

Syn: Murphy’s triadThis phrase is sometimes used to refer to the symptomcomplex which accompany is early acute appendicitisi.e. the sequence of pain, vomiting and fever (hencethe name Murphy’s triad).


Initially, the pain is felt around the umbilicus as thesource of pain is visceral. As the parietal peritoneum inthe right iliac fossa becomes inflamed later in the courseof acute appendicitis pain gets localized to right iliacfossa. Pain is usually followed by vomiting and not viceversa. The vomiting is the result of protectivepylorospasm. Increased body temperature may bepresent as a result of inflammation but rarely beyond38-39°C.

It has to be kept in mind that diagnosis of acuteappendicitis is not always easy and the aforementionedclassical syndrome may not always be present. One mayhave to depend on one or more of the factors liketenderness over McBurney’s point including reboundtenderness, increased WBC count, sonological evidenceof appendicitis etc to arrive at a plausible diagnosis. Somecases might even warrant CT abdomen and/or diagnosticlaparoscopy.

MEIGS’ SYNDROME

This is an infrequent accompaniment of benign solidovarian tumors, most commonly ovarian fibroma. Thesyndrome comprises of hydrothorax (more common onthe right side) and ascites.

Believed the ascites is due to seepage of fluid fromthe tumor into the peritoneal cavity. Hydrothorax resultswhen such peritoneal fluid reaches the thorax viadiaphragmatic lymphatics.


One has to remember that malignant ovarian tumorscan also cause ascites and pulmonary involvement.There is no need to emphasize that the outlook for suchpatients is bleak unlike those having Meig’s syndrome.

MALLORY-WEISS SYNDROME

In this there is longitudinal tear in the mucosa at thecardioesophaged junction following a bout of severevomiting and retching. It can cause significanthematemesis and melaena. The vomiting episodeusually follows a binge of alcohol but may be due toany other cause.

Typical history of vomiting first of gastric contentsfollowed later by nonproductive retching andhematemesis should alert the clinician to the possibilityof Mallory-Weiss syndrome. Fiber optic endoscopyshows the mucosal tear confirming the diagnosis. Thetear is rarely deeper than the submucosa. But thepossibility of through and through perforation(Boerhaave’s syndrome) should be kept in mind andthe same ruled out.

Treatment is conservative initially as for any othercause of upper GI bleed. Sengstaken tube tamponade iscontraindicated as it may further extend the tear.Vasopressin analogues or direct adrenaline injectionaround the tear might be needed in some cases.

Surgery may be needed in rare cases and involves along gastrotomy and under running the tear with


non-absorbable suture. One has to remember thatoccasionally there may be more than one tear.

MARFAN’S SYNDROME

It is an autosomal dominant disorder with highpenetrance and variable expressivity. The genetic defectis said to be in chromosome 15. There is an inheriteddefect in collagen formation and function in theseindividuals.

The leading defects are skeletal, ocular andcardiovascular. The skeletal deformities are abnormallywide arm span, greater pubis to sole length than vertexto pubis length, arachnodactyly, hyper extensible joints,kyphoscoliosis pectus excavatum and flat feet. Greattoes may be longer than other toes and skull deformitieslike dolichocephaly may be seen. Large deformed ears,ectopia lentis, aortic aneurysm, cutaneous striae, etc.are some of the other defects seen in Marfan’ssyndrome.

Mental retardation is not a feature of this syndrome.In fact Abraham Lincoln one of the greatest presidentsof USA is said to have suffered from this syndrome.

Cardiovascular involvement is the most commoncause of death. Propranolol is said to be of some benefitin limiting progression of the vascular defects. Surgerymight be needed for aneurysm, kyphoscoliosis or oculardefects.


MALABSORPTION SYNDROME

This is a clinical condition caused by poor absorptionof various nutrients from the gastrointestinal tract.Symptoms include weight loss, lassitude, anorexia,anemia, oedema due to protein deficiency etc apart fromabdominal symptoms like diarrhoea, flatulence anddiscomfort. Specific symptoms of deficiency of specificnutrients may be present, e.g. night blindness due tovitamin A deficiency, peripheral neuropathy caused byvit. B12 deficiency.

Etiology includes failure of digestion as in chronicpancreatitis, cholestasis, gastric bypass surgeries, blindloop syndrome etc; or failure of mucosal absorption asin gluten enteropathy (celiac sprue) or hypogamma-globulinemia. Short bowel syndrome causesmalabsorption due to rapid transit of bowel contents.Radiation enteritis and mesentenie ischemia are some ofthe other causes.

Investigation include endoscopic jejunal mucosalbiopsy, breath tests for lactose and lactulose,barium studies and immunologic tests for hypogamma-globulinemia.

Treatment depends on the cause and has not beendiscussed here for obvious reasons.

MIRIZZI’S SYNDROME

It is one of the unusual causes for obstructive jaundice.Here a stone in the Hartmann’s pouch presses on the


bile duct producing obstruction. In many cases theremay be a cholecysto choledochal fistula at the site ofobstruction. Cholangiography will show the site ofobstruction but differentiation from cholangiocar-cinoma may be difficult.

Treatment usually involves surgery, but one has tobear in mind that the separation of gall bladder fromthe common hepatic duct may damage the latter.

Another variant of Mirizzi’s syndrome is when alarge stone in the cystic duct just above the junctionwith the bile duct, obstructs common hepatic duct.

This is commonly misdiagnosed as stone in the bileduct. ERCP will clarify the picture.

MAFUCCI’S SYNDROME

It is a clinical condition in which multipleenchondromas are associated with hemangiomas,usually cavernous type. It is not a genetic disorder andhence not hereditary. There is thought to be severemesodermal dysplasia. Hemangiomas of the skin or softtissues is the presenting feature. Phleboliths may be seenon X-ray. Enchondromas affect metacarpals, phalangesor even long bones, not necessarily on the same limbas hemangioma.

Though there is an increased risk of chondrosarcomain these patients, they usually live fairly long as thetumors are low grade and are more likely to die of nonskeletal causes than the tumor itself.


It is worth while noting here that multipleenchondromatosis without hemangiomas is referred toas Ollier’s disease.

MENDELSON’S SYNDROME

This refers to the clinical picture due to aspiration thegastric contents into the respiratory tract especially inobstetric patients following vomiting or regurgitation.

During pregnancy there are several factors whichmight induce vomiting or regurgitation. One is theobviously increased intra-abdominal pressure.Progesterone also is known to induce vomiting andregurgitation by its relaxing action on the gastricesophageal sphincter. Hormonal influences (probablychorionic gonadotrophin) predominate during the earlypregnancy in the causation of emesis. Also psychogenicand neurogenic factors are believed to play a role inaggravating emesis.

Aspiration of gastric contents leads to a chainreaction with obvious deleterious effect. There will bea deteriorating clinical picture representing the ill effectsof lower respiratory infection, pulmonary oedema andfinally respiratory distress syndrome. Added to this willbe the ketoacidosis which might result from severevomiting itself.

The major decision to be made in this situation iswhen to terminate the pregnancy. The guiding principle


is to make sure that the crisis should not be allowed toworsen till both mother and foetus are at risk.

NAFFZIGER’S SYNDROME

Scalenus Anticus syndrome, Adson-CaffeysyndromeIn this syndrome vascular and neurological symptomsare produced by pressure on the subclavian artery andbrachial plexus passing through the scalene triangle.Scalene triangle is bounded anteriority by the scalenusanticus muscles, posteriorly by the scalenus medius andinferiorly by first rib.

In some people because of the excessive resting toneof the scalenus anticus muscle the first rib is slightlyelevated producing compression on the subclavianartery and nerve roots especially C8, T1.

This is one of the causes for a symptom complexcalled thoracic outlet syndrome. Other causes arecervical rib, long transverse process of C7, malunionof fracture of the first rib or clavicle, tumors or exostosisin the region, etc. Symptoms include:1. Neurological symptoms like pain, paraesthesia and

numbness usually in the region of ulnar distributionin the hand and fingers. The symptoms might laterspread to the rest of the upper limb and may finallyproduce severe sensory motor loss and muscularatrophy.


2. Symptoms of arterial compression like claudicationpain fatigue, coldness and weakness of upper limb.These symptoms are aggravated by exercise orexposure to cold.

3. There may be symptoms of embolization into endarteries of the fingers. Emboli are thrown by thethrombus that forms in the dilated part of thesubclavian artery beyond the compression (poststenotic dilation). Patient might suddenly developpain, pallor, paresthesia of the distal fingersuggesting acute ischemia. Frank gangrene maydevelop later.

4. Venous compression occurs less often but canproduce edema and cyanosis.

Diagnosis is by clinical tests like scalene maneuver(Adson’s test) costoclavicular compression maneuver(exaggerated military posture) and three minuteselevated arm stress test, etc. Clinical examination willreveal signs of ischemia as well as neurological deficit.Conformation can be sought by X-ray, Doppler study,angiography, nerve conduction study, electro-myography, etc.

Management depends on the specific cause if one isdetected. Otherwise thoracic outlet decompressionthrough supra clavicular approach usually benefits morethan 90% of the patients. It involves removal of thescalene muscles, resection of cervical rib if present andresecting first rib if thought to be the culprit.


NELSON’S SYNDROME

Syn: Postadrenalectomy Syndrome

This syndrome follows total adrenalectomy. Totaladrenal ablation was being practised for Cushing’ssyndrome of non-adrenal origin. Not only was thesurgery associated with significant mortality andmorbidity, it was noticed that postoperatively there aspituitary hypertrophy, abnormally high ACTH levelsand hyperpigmentation. The morbidity levels used toreach so high as to need pictuitary ablation. This clinicalpicture was referred to as Nelson’s syndrome. It is rarenowadays.

OGILVIE’S SYNDROME

Idiopathic MegacolonIt is otherwise called acute intestinal pseudo obstruction.

It is etiology is not fully understood. It usually occursin elderly individuals following trauma or pelvicsurgery. Excessive pelvic sympathetic discharge isbelieved to be the cause.

Patient has acute distension of the abdomenassociated with vomiting and pain. Usually patient hasconstipation but occasionally there may be diarrhea.X-ray shows massive dilatation of cecum and ascendingcolon without evidence of organic obstruction.


If the cecal diameter is < 10 cm then conservativegentle enema or flatus tube insertion might relieve theobstruction. Of course, electrolyte imbalance or othercauses of paralytic ileus should be ruled out.

Patient should be monitored by serial X-rays andclinical evaluation. If the cecal diameter is measured tobe > 12 cm then either endoscopic or operativeintervention is needed as otherwise chances of cecalperforation are high. Endoscopic decompression needsexperience and is potentially dangerous. Also there is achance of recurrence of obstruction. Thus surgery maybe required in case endoscopy is not available or isunsuccessful, if there are more than two recurrences andwhen there is endoscopic evidence of ischemia andnecrosis. Patient might need colostomy or colonicresection depending on the operative findings.

ORMOND’S SYNDROME

Syn: Ormond’s DiseaseIf refers to idiopathic retroperitoneal fibrosis. Somecases may have positive family history. In such casesother fibrosing conditions like sclerosing cholangitis,Reidel’s thyroiditis etc may coexist.

Patient usually presents with oliguria. The cause ofobstruction is dense periureteric fibrosis especially ator below the pelvic brim. Without treatment theobstruction progresses and may lead to renal failure.


Intravenous urogram shows both ureters obstructedand displaced towards the midline. CT scan isdiagnostic.

Ureteric stenting or percutaneous nephrostomyrelieves the obstruction temporarily. Though high dosesteroids have been advocated by some, definitivetreatment involves ureterolysis (i.e. releasing of fibrousadhesions) and omental wrapping around the ureters.The only other alternative is hemodialysis.

ORTNER’S SYNDROME

This is one of the unusual reasons for recurrentlaryngeal nerve palsies. Here the recurrent laryngealnerve gets paralyzed by the enlarged heart. Thecommonest cause for such cardiomyopathy is the leftatrial enlargement seen in mitral stenosis.

It may be added here that pre-existing recurrentlaryngeal nerve palsy should be sought for via indirector direct laryngoscopy before posting any patient forthyroid surgery. It discovered post operatively, it isdifficult for any surgeon not to get blamed for the palsy,however impeccable the surgery may have been.

POLAND’S SYNDROME

It refers to a rare condition where there is absence ofone breast ipsilateral pectoralis major and sometimes


pectoralis minor associated with absent costal cartilages.In some cases there may be syndactyly.

In some patients the defect may only be restricted tomuscle and breast. In them the anomaly becomesobvious only as the child grows up. In patients whohave full blown syndrome with hypoplastic chest wallearly reconstruction might become necessary.

PENDRED’S SYNDROME

Described by Vaughan Pendred in 1896. It refers to adyshormonogenetic goiter associated with sensorineuraldeafness. The hormone deficient is peroxidase whichis involved in the iodine binding of tyrosyl units withinthe thyroglobulin. Usually the deficit is moderate andthe patient is euthyroid.

The condition is autosomal recessive and hence afamily history should be sought.

If hypothyroid state exists, then hormonereplacement may be needed. On the other hand, thesensorineural deafness may need further evaluation andexpert management to ensure normal development ofthe infant.

PICKWICKIAN SYNDROME

This syndrome is named after the famed fictionalcharacter created by Charles Dickens.


It refers to hypoventilation and its related eventsoccurring in morbidly obese individuals especially inlying down position.

Obesity leads to poor compliance of chest wall andincreased intra-abdominal pressure. This in turn reducesthe functional residual volume of the lungs. This stateof affairs may result in ventilation perfusion mismatchespecially in the lung bases. Patient may develophypercapnia and hypoxemia. Many patients haveobstructive sleep apnea. All these may ultimately resultin polycythemia pulmonary hypertension and right heartfailure. The term “Pickwickian” is especially appliedwhen the patient is somnolent even during day time.

Needless to say weight loss forms the mainstay oftreatment. Correction of sleep apnoea may be of benefit.Progesterone has been tried in the treatment in an effortto improve respiratory drive.

This clinical entity has been discussed here becausemorbid obesity is increasingly being recognized as asurgical problem.

PLUMMER-VINSON SYNDROME

Syn: Paterson Kelly SyndromeIt is one of the less common causes of dysphagia. Mostof the patients are women and hence this condition hasto be differentiated from globus hystericus whereinthere is no mechanical obstruction in the oesophageallumen.


Plummer Vinson syndrome is invariably found inpatients suffering from iron deficiency anemia. Therewill be a mucosal web in the oesophagus in the postcricoid region, obstructing the passage of food bolus.Elsewhere also the oesophageal mucosa is unhealthyand friable. Patients present with dysphagia and chokingsensation. Other features of sideropenic anemia likekoilonychia, bald tongue, angular cheilosis etc may bepresent.

If diagnosed accurately, treatment is dilatation of thepin hole opening in the esophageal web. Great care isneeded as the mucosa is friable and may bleed.Simultaneous treatment of iron deficiency anemiacompletely reverses the oesophageal changes.Untreated this condition may progress to malignancy.Hence Plummer Vinson syndrome is considered aprecancerous state of the oesophagus.

PEUTZ-JEGHERS SYNDROME

This syndrome is often mistakenly considered as partof the familial adenomatous polyposis syndrome.Actually, it is an autosomal dominant condition causingthe development of hamartomas and not adenomasthrough out the gastrointestinal tract. Small intestine ismost commonly affected though stomach and colonicpolyps are also well documented.

Patients become symptomatic early in life and maysuffer from colicky pain, hemorrhage and anemia. There


may be circumoral and labial pigmentation. Unlike infamilial adenomatous polyposis syndrome, the risk ofmalignant change in the polyps is significantly less butis present nevertheless.

If the symptoms are troublesome, then conservativeresection might be needed. Per operative enteroscopyto detect and snare other lesions, if present will reducethe need for early reoperation for recurrent symptoms.

POSTCHOLECYSTECTOMY SYNDROME

Following cholecystectomy, though a large percentageof patients are relieved of their symptoms a significantnumber continue to have symptoms of dyspepsia andreflux disease. In about 5 percent of patients thesesymptoms are severe enough to seek medical attention.This symptom complex has been arbitrarily learned aspost cholecystectomy syndrome.

In some of these patients common bile duct stonesmay be the cause for symptoms. These patients needendoscopic retrieval of stones. In a few others biliarydyskinesia and sphincter of Oddi dysfunction may bethe underlying pathology. In these patients endoscopicsphincterotomy may be of benefit.

It is worth while to note that occasionally biliarytract disease coexists with irritable bowel syndrome ordiverticulosis. In such cases colonic pathology mightvery well be the cause of residual symptoms. Hiatushernia is another such coexisting condition.


Even after ruling out all possible organic causes fordyspepsia a definite subset of patients remains whereinthere is no apparent cause. The only hope for thesepatients is counseling to divert their focus off theabdominal symptoms.

PRUNE BELLY SYNDROME

Syn: 1. Eagle Barret syndrome2. Triad syndrome

In this condition there is a typical triad of abnormalities.They are deficiency of anterior abdominal wall (hence“prune belly”) cryptorchidism and malformations ofurinary tract. Surgical correction of the abdominal walllaxity is difficult. Many children die during infancymostly due to uncontrolled urinary tract infection.

The exact etiology is unknown.

PARKER-WEBER SYNDROME

In this condition there are multiple arteriovenousmalformations in the lower limb and sometimes in theadjoining pelvis. These congenital AV malformationmay be localized to a small area or may involve thewhole limb. There may be local gigantism. The AVfistula will cause hyper dynamic circulation and mayprogress eventually to cardiac failure. Branham’s signcan be demonstrated in many cases (i.e. heart rate falls


when the feeding artery to the AV malformation isblocked.)

Skin pigmentation and ulceration are the othersymptoms of this rare condition.

POSTCONCUSSION SYNDROME

This follows head injury resulting in cerebralconcussion. If there is no focal lesion demonstrable onCT then these patients are supposed to have suffereddiffuse axonal injury. Most patients recover fully, butsome patients continue to have problems with memory,dizziness, nausea, hypersensitivity to stimuli anddiminished concentration.

POSTSPLENECTOMY SYNDROME

Following splenectomy patients become vulnerable tobacterial infection, especially by capsulated organismslike Pneumococcus, Meningococcus and H. influence.Aggressive treatment is mandatory to preventoverwhelming postsplenectomy septicemia.

In elective splenectomy it is prudent to vaccinatethe patients at least 2 weeks before surgery againstcapsulated organisms. In emergency splenectomiesespecially in children perioperative antibiotic cover isnecessary till vaccine becomes effective. During surgeryfor traumatic rupture of spleen all attempts should be


made to salvage at least a part of the spleen(splenorrhaphy). Splenunculi, if found should bemeticulously saved. Of course, splenunculi cannot besaved when splenectomy is done for hematologicalcauses.

POSTPHLEBITIC SYNDROME

Syn: Post-thrombotic SyndromeThis term refers to the long-term complications of deepvein thrombosis (DVT) DVT can occur due to a varietyof causes which impair mobility like serious illness,trauma, major surgery, pregnancy, etc. There may notbe any symptoms in many patients.

Some may develop limb edema, pain and pyrexia.With time if life threatening complications likepulmonary embolism do not develop the clot is lysedand veins recanalize but the valves are damagedpermanently resulting in deep venous incompetence.The resultant venous hypertension leads to edema,lipodermatosclerosis, eczematous lesions and finallyvenous ulcer. Treatment of this condition is mostlyconservative. Valvuloplasty is the surgical option forthese patients.

One has to remember that deep vein incompetenceis not a must for venous ulceration. Pure superficialvein varicosities also cause the same picture and canbe treated by simple ligation procedures.


PARANEOPLASTIC SYNDROME

This refers to a symptom complex which associatedwith a neoplasia but not caused directly by the tumor.The neoplasia cause the clinical syndrome mostly bythe production of either a hormone or any othersystemically active factor.

In some causes these paraneoplastic syndrome mightnot be of life threatening nature like polycythemia inrenal cell carcinoma and acanthosis in gastric and uter-ine cancers. But some other neoplasia produce syndromeswhich add significantly to the morbidity and mortality.For example, syndrome of inappropriate secretion ofADH (i.e. SIADH) seen in small cell carcinoma of thelung. Small cell tumor of the lung can also produce aCushing’s syndrome like condition by secreting anACTH like substance. Dermatomyositis in lung andbreast cancers, carcinoid syndrome in bronchial ad-enoma, migrating thrombophlebitis in carcinoma pan-creas are some of the other examples of paraneoplasticsyndrome.

PIERRE-ROBIN SYNDROME

Consists of micrognathia, small tongue, cleft palate andeye defects. Eye defects may be in the form ofcongenital glaucoma, myopia or retinal detachment. Thesmallness of the mandible and the resultant posterior


displacement of the chin will result in tongue fallingback and obstructing the airway. In its severe form theairway obstruction may be life threatening for thenewborn child. The infant might also have chokingepisodes during feeding most of these unfortunatechildren need early surgical correction

The exact etiology is not known.

PSEUDO-ZOLLINGER-ELLISON SYNDROME

This is seen sometimes in multiple endocrine neoplasia(MEN) type I due to exaggerated gastrin response tohypercalcemia due to hyperparathyroidism. Though thepattern of ulceration and symptomatology resemblesthat of true Zollinger-Ellison syndrome, this conditionis termed ‘pseudo’ because it disappears as soon asparathyroid hyperfunction is dealt with.

Rendu-Osler-Weber Syndrome

In this hereditary syndrome there are multipletelangiectasias of gastrointestinal tract, nose, skin, liver,CNS, etc. Epistaxis is the most common symptom butgastrointestinal bleed can be life threatening. Since thetelangiectasias are small they are not detectable byimaging. Hence, angiography or endoscopy is themainstay of diagnosis. A positive family history is oftensuggestive. Photocoagulation or electrocoagulation of


these lesions has been tried with mixed results.Treatment is difficult especially when there are multiplelesions. Massive GI bleed may necessitate majorresection.

ROTOR’S SYNDROME

It is a type of congenital nonhemolytic jaundice in whichthere is conjugated bilirubinemia and bilirubinuria.

Liver is normal histologically and usually thejaundice is mild. The underlying pathology is said tobe poor storage of bilirubin in the liver cells.

Prognosis is excellent compared to other types ofconjugated hyperbilirubinemia syndromes.

RAPUNZEL SYNDROME

This term is sometimes used to describe the presenceof extensive trichobezoar occupying the stomach,duodenum and down into the jejunum.

Patient is usually mentally challenged and will havealopecia, halitosis along with an epigastric mass. Historyof eating ones own hair may be available if elicitedcarefully. Patient might have features of intestinalobstruction. Gastrointestinal bleeding and perforationare other serious complications.

If diagnosed in time surgery relieves the symptomscompletely.


RAYNAUD’S SYNDROME

This refers to the sequence of local pallor, cyanosis anderythema that occurs when a limb with peripheralvascular disease is exposed to cold.

It was earlier classified as primary or Raynaud’sdisease which was idiopathic, and secondary orRaynaud’s phenomenon which occurred in associationwith other diseases like scleroderma, cervical rib, SLE,carpal tunnel syndrome, etc. This distinction is not veryclear. Hence has been given up and the term Raynaud’ssyndrome is used for all cases.

This condition is due to the abnormal sensitivity ofthe arterioles to cold. When exposed to cold thesearterioles constrict and the part becomes blanched orpale (local syncope). The hypoxia thus induced initiallyresults in capillary dilatation. These dilated capillariesget filled with deoxygenated blood leading to cyanosis(local asphyxia). Finally, the arterioles themselves dilateand fresh oxygenated blood again flows leading toerythema and engorgement.

The condition may slowly to progress to ischemiculceration and local gangrene.

SEZARY SYNDROME

It is a distinct variety of mycosis fungoides or cutaneousT-cell lymphoma.


It presents with erythroderma, pruritus andlymphadenopathy. Peripheral blood shows specific cellscall Sezary cells.

Clinical course is protracted over years.Lymphadenopathy is an indicator of poor prognosis.

If the disease is limited to the skin, then total skinelectron beam irradiation and topical chemotherapy maybe tried. PUVA has also been tried.

SIADH (Syndrome of Inappropriate Secretionof ADH)

This is seen in some cancers (like small cell cancer ofthe lung) burns and head injury. There is excesssecretion of ADH hormone leading to concentratedurine, expanded extracellular fluid volume anddilutional hyponatremia. Patient may suffer fromlethargy and dizziness. If correctly diagnosed, fluidrestriction is all that is necessary. Use of loop diureticslike furosemide and isotonic saline may be neededoccasionally.

SIPPLE SYNDROME

Syn: Multiple Endocrine Neoplasia type IIIn this familial condition, there is association ofmedullary carcinoma of thyroid, phaeochromocytomaand parathyroid adenomas.


Sipple syndrome or MEN type II is again subdividedinto type II a and II b. In MEN type II b in addition tothe neoplasms mentioned above patients will havemultiple mucosal neuromas especially of the lips andthe tongue (bumpy lips) diffuse ganglioneuromas ofthe gastrointestinal tract, skeletal abnormalities andmarfanoid features. Sometimes MEN type II b is alsoreferred to as “MEN type III”.

The clinical significance of Sipple syndrome lies inthe fact that medullary carcinoma thyroid that occursis more aggressive than its counterpart in MEN type IIa. Unless the diagnosis is made early and totalthyroidectomy done, the disease rapidly becomes fatal.But one has to be conscious of the possibility ofcoexisting phaeochromocytoma in order to avoidcatastrophe on operating table.

Phaeochromocytoma in MEN II is always benignand restricted to adrenal medulla. It may or may not besymptomatic.

Parathyroid adenomas are much rarer in MEN typeII b than II a.

Sipple syndrome has a autosomal dominantinheritance.

STEIN-LEVENTHAL SYNDROME

Polycystic Ovary SyndromeTypical triad of Stein-Leventhal syndrome is largepolycystic ovaries, secondary amenorrhea and


infertility. Other features commonly observed areobesity, hirsutism and dysfunctional uterine bleeding.

The exact etiology is not known. It is thought thereis excessive androgen production from the enlargedovaries.

The surgical importance lies in the presence of apelvic mass. Awareness of this syndrome makesrecognition of its typical features easy. Of course themanagement of the condition has to be left forgynecologists.

STURGE-WEBER SYNDROME

Syn: Sturge-Klischer-Weber SyndromeIt consists of a triad of angiomas namely congenitalcutaneous angioma in the region of trigeminal nerve,ipsilateral meningeal angioma and angioma of thechoroids.

While the cutaneous angioma (flame nevus) isobvious at sight, the presence of other two angiomascan be suspected by the focal neurological signs andsecondary glaucoma that they produce. Surgeon’s roleis restricted to recognizing the syndrome thereby pavingthe way for specific management by experts in the field.Hence, details have not been discussed.

SHEEHAN’S SYNDROME

It is hypopituitarism resulting from pituitary infarctionfollowing severe postpartum hemorrhage. Patient may


later present with features of hypothyroidism, loss oflibido, features of adrenal insufficiency, hypoglycemiadue to increased sensitivity to insulin, etc.

If not properly treated patients rapidly progress tocoma. Treatment involves replacement of deficienthormones.

STEWART-TREVES SYNDROME

In its classic form it refers to the lymphangiosarcomaoccurring in a lymphoedematous upper limb followingradical mastectomy. Some people include other causesof lymphedema also in the above definition, e.g.postradiation lymphedema.

The possibility of this long-term complication shouldbe borne in mind and the patient asked to report anyulceration or local pathology especially when thelymphedema is significant. Lymphangiosarcoma is avery aggressive tumor and may need a majoramputation.

SJÖGREN’S SYNDROME

It is an autoimmune disorder characterized by dry eyes(kerato-conjunctivitis sicca) and dry month(xerostomia). It may be primary, otherwise called “siccasyndrome” wherein the clinical features are severe butthere are no other associated connective tissue disorder.On the other hand, secondary Sjögren’s syndrome refers


to that which is associated with other connective tissuedisorders like rheumatoid arthritis, systemic lupuserythematosus, myasthenia gravis, primary biliarycirrhosis.

Investigation usually involves assessing the level ofdamage to parotid and lacrimal glands. Schirmer’s testfor lacrimation, other tests like sialography, FNAC ofthe gland, technetium scan, etc. are also occasionallynecessary to clarify the diagnosis.

Autoantibody estimation might point to anyassociated connective tissue disorder like rheumatoidarthritis which needs specific therapy.

The treatment of this syndrome mainly symptomaticand basically involves moistening the mucousmembranes with artificial tears, saliva, etc. Occasionallytransplantation of parotid duct to lacrimal sulcus maybe necessary to save vision when severe keratitisendangers it.

SCHEUERMANN’S SYNDROME

Syn: SarcoidosisIt is a multisystem granulomatous disorder producinglesions similar to that of tuberculosis.

It can run subacute and self limiting course or maybe chronic with multiple extrapulmonary manifestationsincluding parotid involvement, erythema nodosum,polyarthralgia, hepatosplenomegaly nephrocalcinosis,


etc. The last named manifestation occurs due todisturbed calcium metabolism leading tohypercalcemia.

For definitive diagnosis either a lymph node or askin lesion has to be biopsied. Transbronchial lungbiopsy is always confirmatory. The granulomas arenoncaseating unlike in tuberculosis. Kveim intradermaltest using antigen from human sarcoid tissue issuggestive.

Though many subacute cases resolve spontaneously,chronic and more severe forms need prolongedcorticosteroid treatment to suppress the manifestations.

Involvement of parotid gland by sarcoidosis withassociated facial palsy, fever and anterior uveitis is sometimes referred to as “Heerfordt’s syndrome”.

STEVENS-JOHNSON SYNDROME

Syn: Erythema Multiforme BullosumA severe form of bullous erythema multiformeinvolving large parts of the body surface and the mucousmembranes. It is a potentially life threatening rarecomplication of sulphonamides. Needless to say thedrug has to be stopped immediately.

Occasionally, Stevens-Johnson syndrome isassociated with ocular lesions like conjunctivitis, iritis,etc. It is then referred to as “ocular-mucous membranesyndrome”.


SANDIFER’S SYNDROME

It is a peculiar phenomenon where a patients ofgastroesophageal reflux disease has spasmodiccontortions of the head. Fortunately, it disappears withthe elimination of reflux either medically or by surgicalintervention.

SUMP SYNDROME

This is a syndrome that develops many years followinga choledochoduodenostomy. Patient develops clinicalfeatures of recurrent cholangitis like fever, pain in theright hypochondrium, etc.

The reason for this syndrome is the collection ofbiliary sludge and vegetable matter in the retroduodenalpart of the bile duct. Partial stenosis of the anastomoticsite might be a contributory factor in the causation.

Treatment by way of endoscopic sphincterotomyand/or balloon dilatation of the anastomosis has beentried, but the results are not always satisfactory.

SHORT-BOWEL SYNDROME

As the name suggests this clinical condition results fromresection of too much small bowel.

Such massive bowel resection might be necessaryin superior mesenteric artery infarction or superiormesenteric venous thrombosis. Other causes includenecrotizing enterocolitis, intestinal agenesis in neonates,


radiation enteritis, inflammatory bowel disease, massivetrauma, etc.

Characteristic features of this syndrome reflect thealtered physiology of the GI tract. Patient will havediarrhea, steatorrhea weight loss, nutritional deficiency,etc. due to short and inadequate bowel length foradequate digestion or assimilation.

Treatment involves use of antidiarrheal drugs and H2receptor antagonists. H2 receptor blockers reverse thehypergastrinemia that is seen following short bowelsyndrome. Antibiotics may occasionally be needed if gutsepsis is diagnosed but may worsen diarrhea.

Surgical treatment involves interposition of a reversed(anti-persistatic) intestinal segment or intestinallengthening. The drawbacks of interposition surgery arethe technical difficulty in a patients with short bowel.On the other hand intestinal lengthening operation(Bianchi operation) involves sectioning the availablebowel longitudinally using stapler and then anastomosingthem end to end. This relies on the fact that the two sidesof the bowel are independently supplied by mesentericvessels.

Intestinal allo-transplantation offers hope but is asyet in experimental stage only.

SEAT-BELT SYNDROME

This occurs in road traffic accident due to an improperlyworn seat-belt. The massive deceleration force will


result in the seat-belt impinging on the abdominal wall.This can lead to avulsion of rectus abdominis from thepubic bone. Needless to say such an injury will resultin trauma to the soft viscera within the abdominal cavity.

Another feature of this syndrome is the fracture ofthe posterior process and/or body of the lumbarvertebrae. This results from hyper flexion of the spine.

The contusion caused by improperly applied seat-belt may be seen across the abdomen (seat-belt sign).

Treatment is as for any other blunt abdominal traumawith willingness to do laparotomy at the slightest doubtof visceral trauma.

TURCOT’S SYNDROME

It is a part of the familial adenomatous polyposissyndrome. In Turcot’s syndrome gastrointestinal polypsare associated with brain tumors like gliomas ormedulloblastomas. It is inherited as autosomal dominantand is definitely a premalignant state as far as the GItract is concerned. By the second decade of life thereare multiple polyps mostly in the colon but also in thestomach and small bowel. The higher the number thegreater is the risk of malignancy.

Patient may have loose stools, bleeding per rectumor features of overtmalignancy like weight loss,intestinal obstruction, etc.

Family history might suggest the diagnosis.Colonoscopy will confirm the diagnosis.


Total proctocolectomy with ileoanal anastoneosis isthe only way to prevent an almost certain malignanttransformation of one of the polyps. If conservativeresection sparing the rectum has been done then regularfollow-up for endoscopic screening for rectal polyps isadvisable.

Sulindac has been used in the treatment of thiscondition. This NSAID is apparently successful insuppressing these polyps.

TUMOR LYSIS SYNDROME

This sometimes occurs following chemotherapy. Lysisof tumor cells releases large quantities of intracellularsubstances like uric acid, potassium phosphate, etc. intocirculation. Hyperkalemia may induce life threateningcardiac arrhythmias. Hyperkalemia is worsened by renalfailure caused by hyperphosphatemia. Increase inphosphate also causes hypocalcemia leading to cramps,cardiac arrhythmia and tetany.

This syndrome is more likely to occur is large tumorshighly sensitive to chemotherapy, e.g. lymphomas.

Anticipating this syndrome in the appropriate settingand taking precautionary measures is very important.Acid-base balance, hydration, electrolytes, uric acidshould all be montored and if necessary corrected beforeinitiation of chemotherapy. Once manifest treatment isas for individual abnormality. Some patients might needhemodialysis.


TORRE SYNDROME

Syn: Muir-Torres SyndromeThis is another syndrome associated with visceral tumorespecially small bowel neoplasm.

Patient might have sebaceous cysts, sebaceousadenomas, fibromas, lipomas, etc. together with visceralmalignancy. Occasionally, the patient might have doublemalignancy, e.g. fibrosarcoma and small bowel cancer.

The clinical significance of this syndrome lies in thefact that awareness of its existence might lead to anearlier diagnosis of the visceral tumor in an occasionalpatient even when the symptoms are not suggestive.Of course it needs a very high index of suspicion onthe part of the clinician.

TIETZE’S SYNDROME

It is a condition of unknown etiology wherein there ispainful non-supperative inflammation of costochondralcartilages. It is sometimes referred to as peristernalperichondritis. Local tenderness may be the onlysymptom. There is no gender predilection.

The disease usually self-limiting and hence needsonly symptomatic treatment. Occasionally steroids,either local or systemic may be needed. If the symptomscontinue then excision of the involved cartilage maybe needed.


TAKAYASU’S SYNDROME

Syn: Takayasu’s Disease; Pulseless ArteritisThis is an arteritis of probably immunological originthat affects the branches of aortic arch, most commonlythe subclavian artery. Most of the patients are youngwomen.

Patient presents with claudication pain in the upperlimb with absent pulses. Constitutional symptoms likefever, vomiting myalgia, arthralgia may be present. Ifthe cerebral circulation has been involved thensymptoms of cerebrovascular insufficiency appear.Ischemic gangrene of the upper extremity andmyocardial infarction are lurking dangers in thiscondition.

Treatment involves immunosuppressive drugs likeprednisolone or cyclophosphamide. Direct arterialsurgery has proven disappointing as reocclusion is verycommon.

VERNER-MORRISON SYNDROME

Syn: WDHA Syndrome• Watery diarrhea hypokalemia, achlorhydria• Pancreatic cholera syndrome• Vipoma syndrome

Vasoactive intestinal peptide secreted by vipoma, an isletcell tumor produces severe diarrhea which is resistant to


conventional treatment. This in turn leads tohypokalemia, hypochlorhydria and hypovolemia.Patients have abdominal cramps and weakness.Occasionally, there may be hypercalcemia. Diagnosis canbe confirmed if fasting plasma VIP level is more than500 mg /ml.

Definitive treatment is surgical removal of the tumor.But electrolyte and acid-base balance has to be restoredbefore surgery. Octreotide has revolutionized thepresurgical management. Type of surgery will dependupon the exact nature of the tumor and its location withinthe pancreas.

WILKIE’S SYNDROME

Syn: Superior Mesenteric Artery (SMA) Syndrome

This refers to the compression of distal part of theduodenum between superior mesenteric artery anteriorlyand the aorta and vertebral body posteriorly. Suddenweight loss and immobilization in bed have been citedas triggering factors. Abnormal attachment of ligamentof Trietz is another contributing factor. It is rare in obesepatients.

It is onset is insidious and patient presents withvomiting. Contrast study will reveal dilated stomach andduodenum proximal to the crossing of superiormesenteries artery.


If conservative measures fail then duodeno-jejunostomy is the surgical procedure of choice forrelieving obstruction. Simple gastrojejunostomy mayfail to relieve duodenal obstruction adequately.

WERMER’S SYNDROME

Syn: MEN – Type I

It is the less commonly known name for multipleendocrine neoplasia type I. The commonest feature ofthis autosomal dominant condition is hyperpara-thyroidism. Other features are pancreatic islet celltumors and pituitary adenomas. Rarely adrenal tumorare also seen.

The pathology in parathyroid glands is usually ahyperplasia and not adenoma whereas elsewhere it isadenoma. Pancreatic adenoma may occasionallyproduce excess gastrin leading to Zollinger-Ellisonsyndrome.

Treatment is as for the individual tumor dependingon site histopathology and clinical features.

WEAK VEIN SYNDROME

This is one of the causes for gross primary varicositiesof the lower limb venous system, especially of thesuperficial system. The wall of the veins is congenitally


defective and lacking in tone. Even the valves withinthe vein are weak and likely to be incompetent. Patientmay not have overt complications of these varicositiesas long as the deep system is efficiently functioning.

WALTMAN WALTER SYNDROME

This is a shock like state that can rarely occur in theearly postoperative period following CBD explorationor even simple cholecystectomy. After about 2-3 daysafter the procedure patient suddenly complains of severeepigastric and right hypochondrial pain and may havecirculatory collapse. Sometimes, the features maymimic myocardial infarction.

The etiopathogenesis of this syndrome involves bileleak either due to ligature slipping from cystic ductstump or due to malfunctioning T-tube. The leaked bilefirst gets collected in the Morison’s pouch and then inright subphrenic space and paracolic gulter. The livergets pushed downward and to the left thus impingingon the inferior vena cava. The reduced venous returnto the heart may cause circulatory collapse and anginalike picture.

Diagnosis should be suspected on clinical groundsalone as it is an acute emergency. Only if patient isrelatively stable, ultrasound can be done to confirm thediagnosis. One should not unnecessarily delay re-exploring the patient. T-tube should be repositioned and


cystic duct stump checked after drainage and wash.Abdomen should be closed after keeping a drain in theMorison’s pouch.

VON HIPPEL-LINDAU SYNDROME

It is an autosomal dominant condition. The geneticdefect found in this condition is known to suppressexpression of endothelial growth factor. Hence, themain characteristic of this syndrome is vascular tumorsof the central nervous system especially cerebellum andretina. Adenocarcenoma of the kidney is anothercommonly associated condition whose incidenceincreases with age. Phaeochromocytoma is the otherneoplasm sometimes seen in this condition.

ZOLLINGER-ELLISON SYNDROME

Syn: Gastrinoma Syndrome

This syndrome was described in 1955 in associationwith pancreatic islet cell tumor secreting gastrin. Manyof these patients (up to 20%) will have multipleendocrine neoplasia type I.

The characteristic triad of symptoms of thissyndrome is jejunal ulceration, hypersecretion of gastrinand pancreatic islet cell tumor. This condition is morecommon is males and occurs in middle aged people.ZE syndrome associated with MEN type I occurs in


the young. Patients have multiple peptic ulcers even atunusual sites like jejunum. Gastroesophageal refluxdisease and secretory diarrhea are seen in some. Pepticulcer perforation is a life threatening complication ofthis syndrome.

Diagnosis is by measuring serum gastrin level(> 100 pg/ml). But one should stop all antiacid secretorydrug at least 1 week before estimation of gastrin levels.Acid secretion studies also may give a clue to thediagnosis.

Treatment involves excision of the offendingneoplasm. But one should remember that at least halfof these tumors may be malignant and many a timethey are on the duodenal wall. If there are nodemonstrable metastasis then exploration is done andtumor localized with the help of intraoperativeultrasound. However, the results of surgery are notalways good. But at least it offers the hope of completecure if a single benign tumor is the cause of thesyndrome. Alternatively, proton pump inhibitor may beable to control symptoms of peptic ulcerationadequately. Occasional patient might need hear totalgastrectomy for symptom relief.

AAaron’s sign 10Abadie’s sign 29Abdominal signs 1Adson-Caffey syndrome 86Afferent loop syndrome 58Alder’s sign 10, 13Angell’s sign 38Auenbrugger’s sign 3Auriculotemporal nerve

syndrome 57

BBaid sign 10Balance’s sign 18Ballet’s sign 29Bapat’s bed shaking test 11Battle’s sign 51Becker’s sign 29Beckwith-Wiedemann

syndrome 59Beevor’s sign 4Benz’s sign 22Berry’s sign 34BK mole syndrome 78Bland-White-Garland syndrome

60Blind loop syndrome 60Blumberg’s sign 14Boa’s sign 11Boerhaave’s syndrome 62Border sign 50Boston’s sign 29Boyee’s sign 51

Index

Bozzolo’s sign 46Branham’s sign 48Budd-Chiari syndrome 63

CCarcinoid syndrome 64Cardarelli’s sign 46Carnett’s sign 11Carotid steal syndrome 70Chilaiditi’s syndrome 65Chvostek’s sign 40Claw sign 22Clinical syndromes 55Coffee-bean sign 22Colon cut-off sign 23Compartment syndrome 66Cope’s

obturator test 12psoas test 12

Cracked pot sign 52Crigler-Najjar syndrome 68Cronkhitee Canada syndrome 68Crow foot sign 22CRST syndrome 67Crush syndrome 67Cruveilhier-Baumgarten

syndrome 69Cullen’s sign 4Cupola sign 23Cushing’s syndrome 68

DDalrymple’s sign 30Dance’s sign 6


De Weese sign 44Delbet’s sign 47Deming’s sign 38Dixon Mann’s sign 33Dorendorf’s sign 47Double bubble sign 24Drummonds sign 47DTP sign 53Dubin-Johnson syndrome 70Dumping syndrome 70

EEagle Barret syndrome 95Eaton-Lambert syndrome 77Elephantiasis congenita

angiomatosa 76EMG syndrome 59Enroth’s sign 30Erythema multiforme bullosum

107Escherich’s sign 40Eyelash sign 51

FFamilial atypical multiple mole

melanoma 78Federici’s sign 19Football sign 24Fothergill’s sign 12Fox sign 5Frey’s syndrome 57Fuchsig sign 45

GGardner’s syndrome 72Gastrinoma syndrome 118

Gifford’s sign 30Gilbert’s syndrome 72Glasgow sign 47Globe lag sign 32Graefe’s sign 30Grey Turner’s sign 5Griffith’s sign 31Grocco’s sign 31Gustatory sweating 57

HHarvey’s sign 45Hepatorenal syndrome 74Hippocratic facies 5Homan’s sign 42Horner’s syndrome 73Hospital hopper syndrome 79Howship-Romberg sign 50

IIdiopathic megacolon 88

JJellinek’s sign 31Jendrassik sign 31Job syndrome 75Joffroy’s sign 32

KKanavel’s sign 53Kearns-Sayre syndrome 75Kehr’s sign 19Kenawy’s sign 20Klein’s sign 13Klippel-Trenaunay-Weber

syndrome 76

Index 121

Knies’ sign 32Kocher’s

sign 32test 35

Koenig’s syndrome 76

LLamb syndrome 77Lambert-Eaton syndrome 77Leriche’s syndrome 77Leser-Trélat sign 43Loewi’s sign 32Lynch syndrome 78

MMacewan’s sign 52Mafucci’s syndrome 84Malabsorption syndrome 83Mallet-Guy sign 13Mallory-Weiss syndrome 81Mann’s sign 33Marfan’s syndrome 82Mean’s sign 32Medusa lock sign 24Meigs’ syndrome 80Mendelson’s syndrome 85Milian’s sign 52Mirizzi’s syndrome 83Miscellaneous signs 50Mobius’s sign 32Moses’ sign 42Muir-Torres syndrome 112Multiple endocrine neoplasia

type II 102Munchausen’s syndrome 79

Murphy’ssign 13syndrome 79triad 79

NNaffzigger’s syndrome 86Nelson’s syndrome 88Nicoladoni’s sign 48Nikolsky’s sign 52

OOgilvie’s syndrome 88Oliver’s sign 47Omega sign 22Ormond’s

disease 89syndrome 89

Ortner’s syndrome 90

PPandora’s box 2Paraneoplastic syndrome 98Parker-Weber syndrome 95Paterson Kelly syndrome 92Payr’s sign 43Pemberton’s sign 34Pendred’s syndrome 91Perez’s sign 48Peroneal sign 41Peutz-Jegher’s syndrome 93Pickwickian syndrome 91Pierre-Robin syndrome 98Plummer-Vinson syndrome 92Plummer’s sign 33


Poland’s syndrome 90Polycystic ovary syndrome 103Porter’s sign 47Postadrenalectomy syndrome 88Postcholecystectomy syndrome

94Postcibal syndrome 70Postconcussion syndrome 96Postphlebitic syndrome 97Postsplenectomy syndrome 96Post-thrombotic syndrome 97Prehn’s sign 39Prune Belly syndrome 95Pseudo-Zollinger-Ellison

syndrome 99Puddle sign 20Pulseless arteritis 113

QQueenu Muret sign 48

RRadiological signs in abdomen

21Ransohoff’s sign 6Rapunzel syndrome 100Raynaud’s syndrome 101Rebound tenderness 14Reisman’s sign 34Rendu-Osler-Weber syndrome

99Reverse 3 sign 25Ripault’s sign 53Robi Tansky’s disease 63Rosenbach’s sign 16, 33

Rotor’s syndrome 100Rovsing’s sign 16

SSainton’s sign 33Sandifer’s syndrome 108Sarcoidosis 106Scalenus anticus syndrome 86Scheuermann’s syndrome 106Seagull sign 22Seat-belt syndrome 110Setting sun sign 53Sezary syndrome 101Sheehan’s syndrome 104Short-Bowel syndrome 108Sign de dance 6Sign of

disappearing pulse 44visceral malignancy 43

Signs inarterial aneurysm 46deep vein thrombosis 41latent tetany 40peripheral vascular disease44thyrotoxicosis 28torsion tests 38

Signs of hernia 49Signs on

inspection 3palpation 9percussion and auscultationof abdomen 17

Silk glove sign 49Sipple syndrome 102

Index 123

Sjögren’s syndrome 105Slip sign 54Snellen’s sign 34Stein-Leventhal syndrome 103Stellwag’s sign 34Stevens-Johnson syndrome 107Stewart-Treves syndrome 105Stierlin’s sign 25Sturge-Klischer-Weber syndrome

104Sturge-Weber syndrome 104Superior mesenteric artery

syndrome 114Syndrome of inappropriate

secretion of ADH 102

TTakayasu’s

disease 113syndrome 113

Tanyol’s sign 6Ten horn’s sign 17Thyroid signs 27Tietze’s syndrome 112Tinel’s sign 53Toma’s sign 21Topolanski’s sign 34Torre syndrome 112

Triad syndrome 95Troisier’s sign 44Trousseau’s sign 41, 44Tumor lysis syndrome 111Turcot’s syndrome 110

UUmbilical black eye 4

VVerner-Morrison syndrome 114Visible peristalsis 7von Graefe’s sign 30von Hippel-Lindau syndrome

117

WWaltman Walter syndrome 116WDHA syndrome 114Weak vein syndrome 116Weiss sign 40Wermer’s syndrome 115Wilkie’s syndrome 114Wrist sign 54

ZZollinger-Ellison syndrome 116,

117

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Clinical Signs and Syndromes in Surgery