Emergency Pediatri

8/12/2019 Emergency Pediatri

1/121

FK UNISMA

EMERGENCY PEDIATRI


2/121

HERNIAS AND ABDOMINAL WALL

DEFECT


3/121

Congenital/ Posterolateral Diaphragmatic HerniaCDH)

1. One of most severe conditions of neonate2. Defect in diaphragm during early fetal development3. Left side most commonly affected4. Content of the hernia:

- small bowel- colon

- spleen- stomach- liver, kidney, tail of pancreatic


4/121
http://images.google.co.id/imgres?imgurl=http://services.epnet.com/getimage.aspx?imageiid=6883&imgrefurl=http://www.doctorsofusc.com/condition/document/222855&usg=___Es6sjY6zxBZREiMDxVpWdxABdE=&h=398&w=370&sz=105&hl=id&start=17&tbnid=uHD8lQfd6d2r5M:&tbnh=124&tbnw=115&prev=/images?q=pediatric+Diaphragmatic+Hernias&gbv=2&hl=id


5/121

mbryology

Week8 9division of coelomic cavity into the pleuraland peritoneal cavity by the diaphragm; a

triangular area in the posterolateral site was leftopen.

Week10 12

herniation occur through this opening into thepleural cavity at the return of midgut


6/121

1 Hypoplasia of the lung

Pulmonary weight (ipsilateral+contralateral) Alveoli number H ypertrophy of the media of pulmonary arteriole

R esistance of the vessels

Pathophysiology


7/121

2.Pulmonary hypertension Abdominal viscera into the thoracic cavity compression of the lungPaO2 , PaCO2 acidosis, hypoxemia

PH


8/121

Clinical manifestations

1. Severe respiratory distress cyanosis, vomit2. Breath sounds: diminished on the side of hernia

3. Heart sounds: deviated to the contralateralchest4. Scaphoid abdomen


9/121

Diagnosis

Prenatal diagnosis

Ultrasound : abdominal organ the fetal visibelin chest.


10/121

After birth

X-ray film :

Typical air-filled stomach and bowels in the chest,which continues into the abdominal cavity.

Diaphram can not be seen at the affected side. Absence or scarcity of intestine in the abdominal cavity


11/121


12/121

Treatment Before delivery: cortisone could induce the

maturation of pulmonary tissue

Preoperative preparation:

1 mechanical ventilation with pure oxygen2 nasogastric tube to decompress

stomach and intestine

3 semi-supine and inclined tothe ipsilateral side keep warm

4 i.v. fuild, correction of acidosis

(5

surgical repair


13/121

CONGENITAL DEFECTS OFABDOMINAL WALL( Omphalocele & Gastroschizis )


14/121

HISTORY

14

A.C. Celsus ( First Century AD ) : First Report ofnewborns with Abdominal Wall Defects.Ambroise Pare ( 16 th Century ) : First descriptionof Omphalocele.Lycosthenes ( 16 th Century ) : First description ofGastrochizis.Taruffi ( 1894 ) : Introducing the termGastrochizis. Massabau & Guibal, Moore & Stokes, Bernstein

: Described specific clinical entity toAbdominal Wall defects.


15/121

HISTORY

15

Hey ( 1802 ) : First successful repair of Omphalocele.Visick ( 1873 ) : First successful repair of Gastrochizis.Ahfeld ( 1899 ) : Described painting an Omphalocelesac with Alcohol .Max Grob ( ? ) : The use of Mercurochrome.Ein & Shandling ( ? ) : The use of Semi permeableArtificial Membrane.Olhausen & Gross ( 1887 ) : The use of Skin flap after

membrane removal.Schuster ( 1967 ) : Introducing reduction of largeOmphaloceles with prosthetic material.


16/121

ABDOMINAL WALL DEFECTS SPECTRUM

16

Omphalocele ( Lateral Fold )Cephalic Fold Omphalocele( Pentalogy of Cantrell )Caudal Fold Omphalocele ( CloacalExtrophy, Vesicointestinal Fissure )GastroschizisEctopia Cordis ThoracisUmbilical Cord Hernia


17/121

NORMAL EMBRYOLOGY

17

3 Weeks gestation : the flat cellular disk ofembryo develops four folds enclose thebody cavities.Two lateral folds form the pleuroperitoneal ( PP) canal meet anteriorly in the midline. The cephalic fold will takes its place withinanterior chest wall, and also carries the SeptumTransversum continues posteriorly dividesthe PP canal into pleural and peritonealcavities.


18/121

NORMAL EMBRYOLOGY

18

The Caudal Fold brings with it thedeveloping Bladder or Allantois startedoff distal to the anus.

During the fold process, the gut tube hasformed along the length of the embryo.5 Weeks Gestation : the gut tube begin toelongate and develop within the umbilicalcoelom.10 Weeks Gestation : the gut returns from theumbilical coelom to peritoneal cavity undergoes rotation and fixation.


19/121

NORMAL EMBRYOLOGYCEPHALO CAUDAL FOLD

19


20/121

NORMAL EMBRYOLOGYCEPHALO CAUDAL FOLD

20


21/121

NORMAL EMBRYOLOGYLATERAL FOLD

21


22/121

OMPHALOCELE EMBRYOLOGY

22

Failure of the mostly lateral body foldsto complete the journey, and defectare always at the umbilicus ( midline )

Failure of return of the gut from theumbilical coelom to peritoneal cavity.Stopped at Extra coelomic EviscerationStage

Occurs early in embryogenesis affect other organ system associated anomalies frequently.


23/121

GASTROCHIZIS EMBRYOLOGY

23

Abnormal dissolution of abdominal wallvascularization Failure of the umbilicalcoelom to develop The elongating intestinehas no space to expand, and ruptures out the

body wall.Occurs just the right side of the umbilicus Because the right side is relatively unsupported,as a result of complete abnormal dissolution of

the right umbilical vein ( at 4 weeks gestation ).


24/121

GENETICS CONSIDERATION

24

Rare reports of Abdominal wall defects, mainlyomphalocele, occuring in families and even intwins.No specific genes have been identified.

More likely to be associated withChromosomal Anomaly ( Trisomy 13, 18, or 21 ) Usually do occur as part of syndromes (Beckwith - Wiedemann, Gershoni - Baruch,Donnai Barrow, or Fryns Syndrome )


25/121

CLINICAL FEATURES : OMPHALOCELE

25

Omphalocele = Exomphalos = Amniocele =CoelosmiaThe second most common of the abdominalwall defects.Incidence : 1 2.5 / 5000 live birth.Male preponderanceUsually happened in full term baby .


26/121


26

Centrally Abdominal Wall defect, larger than 4cm in diameter , and always covered by atranslucent membrane from which theumbilical cord extends .

The outer membrane layer is formed byamnion, the inner layer by peritoneum, withmesenchymal tissue, called Wharton jellyamong them.

Variation in defects size. Smaller defect, betterin outcome .


27/121

27


28/121

28


29/121


29

Normal muscles of abdominal wall, and thesac usually contains the liver, midgut, andfrequently other organ such as spleen, orgonad .Associated with anomalies condition, such asBeckwith - Wiedemann, Prune Belly, Gershoni- Baruch, Donnai Barrow, Down, or FrynsSyndrome.


30/121

DIFFERENT DIAGNOSIS : OMPHALOCELE

30

Umbilical Hernia( The defect is covered by normal skin not amembrane. Rarely present at birth, butusually becoming apparent in the first week

or months of life )Persistent Vitelline / Omphalomesenteric Duct( There are no umbilical cord in this defect )


31/121

CLINICAL FEATURES : GASTROCHIZIS

31

Gastroschizis ( Greek ) = Belly CleftThe most common of the abdominal walldefects .Incidence : 2 5 / 10000 live birth.Male preponderanceUsually happened in premature baby, Low BirthWeight, or baby with respiratory problems. It isassociated with Intrauterine Distress, andyounger mother .


32/121

CLINICAL FEATURES : GASTROCHIZIS

32

Periumbilical ( right ) Abdominal Wall defect,less than 4 cm in diameter , with a skin bridgemay be present between umbilical cord andthe defect .

Normal muscles of abdominal walls, with nosac or remnant of a sac.Herniation of midgut, stomach, andoccasionally gonad. Liver is very rare.


33/121

33


34/121

34


35/121


36/121

DIFFERENT DIAGNOSIS :GASTROSCHIZIS

36

Rupture of Omphalocele sac( The umbilical cord is on the tip of thedefect, with is covered by rupturedmembrane )


37/121

DEFECTS PREVIEW

Components Omphalocele GastrochizisLocation Umbilical cord Lateral ( Right ) tocord / Paraumbilical

Defect Size Large ( 2 10 cm ) Small ( 2 4 cm )

Cord Inserts in sac Normal insertion ( toleft of defect )

Abdominal Cavity Small Normal

Bowel Normal Matted, inflamed

Malrotation Present Present

GastrointestinalFunction

Normal Prolonged ileus

Associated Anomalies Common( 30 70 % )

Unusual

Outcome Good Good

37


38/121

DIAGNOSTICS :ANTENATAL INTERVENTION

38

Prenatal Ultrasound DetectionIrregular Bowel Contour, or Dilated bowel freein amniotic fluid adjacent to the umbilical

insertionAmniotic Fluid and Serum TestElevated AFP ( Both Maternal serum andAmniotic Fluid ), and Elevated Amniotic FluidAcH Esterase have been correlated with thisdefects ( when there is no Myelomeningocele)


39/121

MANAGEMENT OF OMPHALOCELECONSERVATIVE / INITIAL CARE

39

Maintenance of body temperature.Naso-gastric Tube insertion to keep theintestines decompressed.Ventilator support and supplementaloxygen.Intravenous fluid are provided atmaintenance rateProphylactic Antibiotics

Keep the Omphalocele sac intact, wet, andsterileCardiologic evaluation andEchocardiography are in order.


40/121

MANAGEMENT OF OMPHALOCELESURGICAL DEFECT CLOSURE

40

Primary ClosureShould be performed for small / moderatesized defect.Reduction of Abdominal contents, Incision ofOmphalocele sac, Abdominal Inspection,Mal rotation correction, and Skin Closure.


41/121

MANAGEMENT OF OMPHALOCELESURGICAL DEFECT CLOSURE

41

Delayed Primary ClosureEspecially for giant Omphalocele, with largedefect that need staged reduction of theintestinal contents by performing Silastic silo

sheeting( Extraabdominal Pouch ), with Antibiotics orSilver Sulfadiazine is applied around theedges as a dressing.


42/121

MANAGEMENT OF GASTROSCHIZISCONSERVATIVE / INITIAL CARE

42

Maintenance of body temperature.Naso-gastric Tube insertion to keep theintestines decompressed.Ventilator support and supplementaloxygen.Intravenous fluid are provided atmaintenance rate, or rehydrating rate if itsneeded.

Prophylactic Broad Spectrum AntibioticsIntestinal contents closure with Saran Wrap,Handi Wrap, or Bogotas Bag.


43/121


44/121

44


45/121

COMPLICATION POSSIBILITIES

45

OmphaloceleRelated with associated anomalies, Ruptureof Omphalocele sac.GastroschizisRelated with prematurity, or gastrointestinaltract anomalies, Dehydration, Hypothermia,and Sepsis


46/121


47/121


48/121

The direct hernia protrudes through the posteriorwall of the inguinal canal, i.e., medial to deep inferiorepigastric vessels, destroying or stretching thetransversalis fascia..


49/121

The Embryology

The indirect inguinal hernia is as follows the duct descending to the testicle is a smalloffshoot of the great peritoneal sac in thelower abdomen.During the third month of gestation, theprocessus vaginalis extends down toward thescrotum and follows the chorda gubernaculumto the scrotum.


50/121

During the seventh month, testicle descend intothe scrotum, processus vaginalis forms acovering for the testicle and the serous sac inwhich it resides.

At about the time of birth, the portion of theprocessus vaginalis between the testicle and theabdominal cavity obliterates, leaving a peritonealcavity separate from the tunica vaginalis that

surrounds the testicle


51/121

Incidence

Approximately 1-3% of children.

Premature babies (9-11%) is higher than full-

term (3-5%), with a dramatic risk ofincarceration (30%).


52/121

Diagnosis

Typical patient intermittent lump or bulge inthe groin, scrotum, or labia noted at times ofincreased intra-abdominal pressure.If a loop of bowel entrapped (incarcerated),

develops pain followed by signs of intestinalobstruction.If not reduced, compromised blood supply(strangulation) leads to perforation andperitonitis.Most incarcerated hernias in children can bereduced


53/121


54/121

Treatment

Simple high ligation of the sac

Elective herniorrhaphy is treatment of choice.

Risk of incarceration, repair should be undertakenshortly after diagnosis.

Pediatric patients are allowed to return to full activityimmediately after hernia repair .


55/121

Patients presenting with incarceration should havean attempt at reduction (possible in greater than 98%with experience), and then admission for repairduring that hospitalization.

Bilateral exploration is done routinely by mostexperienced pediatric surgeons.

Recently the use of groin laparoscopy through thehernial sac permits visualization of the contralateral

side.

H d l


56/121

Hydroceles

A hydrocele is a collection of fluid in the spacesurrounding the testicle between the layers of the

tunica vaginalis.

Hydroceles can be scrotal, of the cord, abdominal, ora combination of the above.


57/121

A hydrocele of the cord is the fluid-filled remnant ofthe processus vaginalis separated from the tunicavaginalis.

A communicating hydrocele is one thatcommunicates with the peritoneal cavity by way of anarrow opening into a hernial sac.

Some are associated with an inguinal hernia. They

are often bilateral, and like hernias, are morecommon on the right than the left.


58/121

Hydrocele noncommunican Hydrocele communican


59/121

Most hydroceles will resolved spontaneously by 1-2years of age.

After this time, elective repair can be performed atany time.

Operation is done through the groin and searchmade for an associated hernia.

Aspiration of a hydrocele should never beattempted.


60/121

INVAGINASI


61/121

INVAGINASIIntusussepsi atau Invaginasi sering terjadi

pada bayi dan anak, dimana satu segmen ususmengalami konstriksi oleh gelombangperistaltik dan tiba tiba masuk ke dalam

segmen distalnya ( Dayal dan DeLellis ,1989). Ujung usus yang masuk : intussuseptum Bagian usus yang menerima : intussussepiensIntussussepsi pertamakali dipublikasikan olehPaul Baebette pada pertengahan abad ke 17,

Salah satu sebab obstruksi usus

Tindakan pembedahan


62/121

Invaginasi usus ini akan diikuti oleh mesenterium yang


63/121

Invaginasi usus ini akan diikuti oleh mesenterium yangberisi pembuluh darah, kelenjar limfe, saraf sertalemak dibelakangnya.

Terjadi strangulasi pembuluh darah dan limfeEdema jaringan usus,Jaringan mukosa membengkak berisi darahdan mukus produksi sel goblet.

Red Current Yelly Stools .

Lebih lanjut darah dan mukus masuk kedalam lumen keluar bersama feses,sehingga feses merah dan berlendir

Jika keadaan ini berlanjut dapat terjadi nekrosis


64/121

Jika keadaan ini berlanjut dapat terjadi nekrosisdan gangren usus (Ong dan Beasley, 1990 ;Spitz,1990 ).Pada Invaginasi terjadi :

cedera vasa mesenterika, pertumbuhan bakteri berlebihan,

malabsorbsi, translokasi kuman ( Spitz, 1990 ).

Semua keadaan ini perlu dipertimbangkan pada

penanganan kasus invaginasi.Keterlambatan diagnosis penatalaksanaanpenderita berbeda satu dengan yang lain disertairesiko kematian menjadi besar.

SEJARAH


65/121

Abad ke 17, Paul Barbatte dari amsterdam menulis

tentang intussussepsi dan dapat direduksi secaraoperatif.

Tahun 1876 Hirschprung dari copenhagen telah

mempublikasikan beberapa laporan mengenaireduksi intussussepsi dengan menggunakantekanan hidrostatis. Hasilnya mempunyaikeunggulan dibanding terapi operatif selama 70tahun berikutnya.

SEJARAH


66/121

INSIDENSI


67/121

Laki-laki dan perempuan 3 : 2.

Kasus intussussepsi terjadi dibawah umur 1 tahun

sebesar 65 %,

Jumlah yang terbanyak ditemukan pada usia 5-9 bl( Mark, 1979 ).

INSIDENSI

I i d di k b d k


68/121

Intussussepsi dapat ditentukan berdasarkan :

a. Atas dasar perjalanan penyakitnya:Gibson, Dockerty dan Dixonmembagi dalam 3 macam :

1. Akut : gejala-gejala terjadi kurang dari1 minggu2. Sub akut : gejala-gejala terjadi antara 1-2

minggu

3. Kronik : gejala-gejala yang sudah berlangsung lebih 2 minggu.

b A d il j l


69/121

b. Atas dasar penampilan gejala :Sven Berghdahl at al, membagi intussussepsi menjadi2 macam :1. Intussussepsi yang typis, yakni intussussepsi

dengan gejala yang khas yang mudahdikenal antara lain muntah-muntah, nyeri perut,

massa abdomen dan perdarahan perectal.2. Intussussepsi yang atypis, golongan inimenampakkan gejala-gejala yang bervariasi darigambaran diatas, sehingga kadang kadang

terlambat didiagnosa. Golongan ini baik denganoperatif maupun dengan ba-enema mudahtereduser secara spontan, terdapat lesi spesifik,mudah rekurens dan sering menjadi kronis.

c. Klasifikasi atas dasar lokalisasi :


70/121

Ellis menggolongkan intussussepsi dalam :1. Enteric : adalah intussussepsi usus kecil ke

usus kecil.2. Colic : adalah invaginasi colon ke colon

(colo-colic)..


71/121


72/121

4. Multiple intussussepsi dan retrograde

5. Appendicular ( jarang )6. Gastroduodenal.

7. Yeyunogastrik.

Insiden tertinggi intussussepsi adalah type :enterocolic/ileosekalis,

Mencapai 95 % dari kasus ( Mark, 1979 ).

A


73/121

PENYEBAB

Pada anak-anak: Umumnya tak diketahuipenyebabnya sehingga ada yang menggolongkanidiopatik atau primer.

Orloff meneliti dari 1424 kasus pada anak:95 % kausa tak diketahui5 % divertikel Meckels, polip, duplikasi usus.

F kt b b d i l idi tik i i t


74/121

Faktor pemyebab dari golongan idiopatik ini antaralain:

Motilitas coecum yang berlebihan,

Perkembangan usus besar yang lebih cepat dari

usus kecil selama masa kanak-kanak

Pembesaran lymphe follikel ileum distal

Lymphadenitis mesenterial

Pada orang dewasa : Kebanyakan disebabkan oleh lesi


75/121

Pada orang dewasa : Kebanyakan disebabkan oleh lesiorganis (pada umumnya tumor), tapi tidak berartikasus idiopatik tidak ada. Donhauser dan Kellymengemukakan penyebab intussussepsi orang dewasaseperti tabel dibawah ini :

Jenis lesi Jumlah %

Neoplasma benigna 213 44 %Neoplasma maligna 123 26 %

Divertikel Meckels 53 9 %Gastroenteeeerostomy 27 6 %Ulcus usus 13 3 %Tidak diketahui 59 12 %


76/121

Dasar : - anamnesa yang baik- pemeriksaan fisisk yang cermat- penunjang : radiologi dan USG

Anamnesis : - Anak sehat , menangis, serangan

nyeri perut (sifat kolik), gelisah

- Bayi . nyeri perut mungkin tak nyata. pendiam. muntah. pucat dan berkeringat

- Anak >2 th keluhan nyeri perut

lebih jelas

DIAGNOSIS


77/121

Pemeriksaan fisik


78/121

Stadium dini : fungsi vital masih baik 6-8 jam setelah serangan sakit pertama :

Bila perut makin kembung :Muntah Dehidrasi Demam Takicardi

bakteremia

Bila perut tak kembung:Palpasi dinding abdomen : Dinding abdomensupel, tidak nyeri tekan. teraba massa berbentuklonjong sosis sign

Dance sign : pada perut kanan bawah terabakosong karena sekum dan colon ascenden bergerak

keatas mengikuti proses invaginasi


79/121

RADIOLOGI


80/121

Plain foto abdomen : Gambaran obstruksi saluran cerna

Distribusi udara yang tidak merata

Perselubungan pada daerah perut kanan bawah,

tengah dan atas

Udara hanya menempati perut kiri atas Pada keadaan lanjut tanda obstruksi berupa

multiple air fluid level


81/121

Barium enema


82/121

Barium enema

Untuk diagnosis sekaligus terapiGambaran foto radiologis Gambaran obstruksi , aliran barium terhenti

pada daerah distal invaginasi

Suatu gambaran berbentuk mangkok padabarium yang terhenti ( Cupping appeareance)

Gambaran yang berbentuk lingkaran pir, daribarium yang terdifusi diantara celah invaginasi(coil spring appearance)


83/121


84/121


85/121

Ultrasonografi

Pada potongan longitudinal tampak masaberbentuk tubulerPada potongan melintang tampak gambarantarget appearance atau doughnut appearance


86/121

Terapi


87/121

psebelum dilakukan tindakan definitif

1. resusitasi2. Pasang NGT3. Pasang kateter4. Periksa lab darah5. persiapan donor darah untuk transfusi6. injeksi antibiotik

Pembedahan dapat dilakukan bila :

1. Produksi urin 0,5-1 ml/kgBB/jam2. suhu tubuh < 38 o C3. nadi < 120 kali/menit4. RR < 40 kali/menit

5. kesadaran baik


88/121

CARA PENGELOLAAN


89/121

1. Tanpa operasi :

Reduksi dengan barium enema, untuk mengerjakatindakan ini kamar operasi harus disiapkan, bila terjadikegagalan reposisi langsung dikerjakan operasi

Indikasi: semua kasus kecuali ada kontra indikasiKontra indikasi:

1. Strangulasi2. KU jelek3. Demam tinggi4. Dehidrasi5. Perforasi

6. Intussusepsi yang rekuren

Keuntungan:


90/121

1. terhindarnya dari stress operasi2. terhindarnya bahaya adesi dan strangulasi dikemudian

hari

Komplikasi :1. perforasi kolon2. kolon yang tereduksi mungkin tidak viable lagi

Peni laian keberhasilan reduksi :a. terjadi pembebasan dari intussusepsib. terlihat cairan barium masuk kedalam ileum terminalc. adanya flatus bersama keluarnya cairan barium

bersama feses

d. tidak ditemukanya lagi massa di abdomen

2 D i


91/121

2. Dengan operasi

Laparotomi Eksplorasi Irisan tranversal diatas/dibawah umbilikus Reduksi dikerjakan dengan manual, distal

intussusepsi dilakukan milking dengan udara usus

sebagai pendorong Bila terjadi reposisi nilai viabilitas usus, bila usus

nekrosis lakukan reseksi dan anatomose end to endbila kondisi memungkinkan

Bila reduksi gagal lakukan reseksi dan anastomoseend to end


92/121


93/121


94/121


95/121

Duodenal Atresia

Incidence--1 in 5,000 to 10,000 livebirths

75% of stenoses and 40% of atresias arefound in DuodenumMultiple atresias in 15% of cases

50% pts are LBW and prematurePolyhydramnios in 75%Bilious emesis usually present


96/121

Duodenal Atresia Cont

Associated AnomaliesDowns (30%)

MalrotationCongenital Heart DiseaseEsophageal Atresia

Urinary Tract Malformations Anorectal malformationsVACTERL


97/121

Duodenal Atresia Diagnosis

RadiographsDouble -Bubble

Pyloric dimple sign Absence of beak sign seen in pyloricobstruction

Workup of potential associatedanomaliesECHO, abd US, possible VCUG


98/121

Double Bubble


99/121


100/121


101/121

Small Bowel Atresia

Jejunal is most common, about 1 per2,000 live births

Atresia due to in-utero occlusion of all orpart of the blood supply to the bowelClassification--Types I-IV

Presents w/bilious emesis, abddistension, failure to pass meconium(70%)

Intestinal Atresia Classification


102/121

Intestinal Atresia Classification


103/121

Small Bowel Atresia Cont

Associated Anomaliesother atresias

Hirschsprungs Biliary atresiapolysplenia syndrome (situs inversus,cardiac anomalies, atresias)CF (10%)


104/121


105/121


106/121

Malrotation

1 per 6,000 live birthscan be asymptomatic throughout lifeUsually presents in first 6 months of life18% children w/short gut had malrotation withvolvulusEtiology

physiologic umbilical hernia--4th wk gestationReduction of hernia 10th - 12th wks of gestation

Normal Embryology


107/121

Normal Embryology


108/121

Malrotation Classification

Nonrotationwhen neither duodenojejunal or cecocolic

limbs undergo correct rotation Abn Rotation of Duodenojejunal limb

causes Ladds bands to form acrossduodenum

Abn rotation of Cecocolic limbcecum lies close to midline, narrowmesenteric base


109/121

Abnormal Rotation/Fixation

l


110/121

Malrotation Diagnosis

Varying symptoms from very mild tocatastrophic**Bilious emesis is Volvulus until provenotherwise**Bilious emesis, bloody diarrhea, abddistension, lethargy, shockUGI shows abnormal position ofDuodenum

if Volvulus, see birds beak in duodenum


111/121

Malrotation UGI


112/121


113/121


114/121

Malrotation--Treatment

Surgical-- Ladds Procedure Evisceration

Untwisting of volvulus (counterclockwise)Division of Ladds Bands Widening mesenteric base

Relief of Duodenal obstruction Appendectomy

Recurrence 10% after Ladds


115/121

Common Disorders

NEC Duodenal Atresia

Small Bowel AtresiaMalrotationHirschsprungs


116/121

Hirschsprungs Disease

Migratory failure of neural crest cellsIncidence 1 in 5,000 live births, males

affected 4:1 over females90% of pts w/Hsprungs fail to passmeconium in first 24-48 hrs

Abd distension, bilious emesis,obstructive enterocolitis


117/121

Transition Zone on BE


118/121

Transition Zone on BE


119/121

Hirschsprungs Treatment

In neonates, can do primary pull-through--bringing normal colon down to

anorectal junctionIn older infants, may need divertingcolostomy first to decompress

May need prolonged dilatations andirrigations


120/121


121/121

Documents

Emergency Pediatri