Surgical Emergencies in the Newborn-1

8/7/2019 Surgical Emergencies in the Newborn-1

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Surgical Emergencies inSurgical Emergencies in

the Newbornthe Newborn

University of North Carolina at Chapel HillUniversity of North Carolina at Chapel Hill

Pediatric Surgery DivisionPediatric Surgery Division

Patty LangePatty Lange

Last revised 4/15/06Last revised 4/15/06


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EmergenciesEmergencies

TypesTypes

Airway/RespiratoryAirway/Respiratory

Intestinal ObstructionIntestinal Obstruction Intestinal PerforationIntestinal Perforation

SignsSigns

Respiratory distressRespiratory distress

Abdominal distensionAbdominal distension

PeritonitisPeritonitis

PneumoperitoneumPneumoperitoneum


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Airway/RespiratoryAirway/Respiratory

Neck MassesNeck Masses Cystic HygromasCystic Hygromas

Tracheal anomaliesTracheal anomalies

Thoracic masses/pulmonary lesionsThoracic masses/pulmonary lesions Congenital lobar emphysemaCongenital lobar emphysema

Overdistension of one ormore lobes (nl histological lung)Overdistension of one ormore lobes (nl histological lung)

Congenital cystic adenomatous malformationCongenital cystic adenomatous malformation

Multicysticmass of lung tissue, proliferation of bronchial structuresMulticysticmass of lung tissue, proliferation of bronchial structuresat the expense of alveoliat the expense of alveoli

Pulmonary agenesisPulmonary agenesis Absence of lungAbsence of lung

Congenital diaphragmatic herniaCongenital diaphragmatic hernia

Tracheoesophageal fistulaTracheoesophageal fistula


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Cystic HygromaCystic Hygroma

Multiloculated cystic spaces lined by endothelialMultiloculated cystic spaces lined by endothelial

cellscells Separated by fine walls containing numerous smoothmuscleSeparated by fine walls containing numerous smoothmuscle

cellscells Result ofmaldevelopment of lymphatic spacesResult ofmaldevelopment of lymphatic spaces

Incidence about 1 in 12,000 birthsIncidence about 1 in 12,000 births 5050--65% appear at birth, 8565% appear at birth, 85--90% appear by age 290% appear by age 2

NeckNeck--75%, Axilla 20%; can be seen in mediastinum,75%, Axilla 20%; can be seen in mediastinum,

retroperitoneum, pelvis, groinretroperitoneum, pelvis, groin

Nuchal/post cervical CHs have been associated withNuchal/post cervical CHs have been associated with

chromosomal abnormalitieschromosomal abnormalitieshighmortality ratehighmortality rate


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ComplicationsComplications RespiratoryRespiratorylarge hygromas can extend into oropharynx andlarge hygromas can extend into oropharynx and

tracheatrachea

Inflammation/InfectionInflammation/Infection

HemorrhageHemorrhage

TreatmentTreatment Dependent on size, location, symptoms/complicationsDependent on size, location, symptoms/complications

Some pts require emergent surgery due to airway compromiseSome pts require emergent surgery due to airway compromise

Best treatm

ent is com

plete excisionBest treatm

ent is com

plete excision Aspiration typically not effective due to rapid refilling of fluidAspiration typically not effective due to rapid refilling of fluid

SclerotherapySclerotherapyBleomycin, OKBleomycin, OK--432 (no longer available in US),432 (no longer available in US),doxycycline, fibrin gluedoxycycline, fibrin glue


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Postnatal overdistension of one ormore lobes ofPostnatal overdistension of one ormore lobes of

histologically normal lunghistologically normal lung Probably due to cartilaginous deficiency in the tracheobronchialProbably due to cartilaginous deficiency in the tracheobronchial

treetree Obstruction causing the overdistension may be due toObstruction causing the overdistension may be due to

11chondromalacia of bronchichondromalacia of bronchi

22extrinsic pressure on bronchus by anomalous pulmonary vein orextrinsic pressure on bronchus by anomalous pulmonary vein or

abnormally large PDAabnormally large PDA

33idiopathicidiopathic LocationLocation

LUL 47%, RML 28%, RUL 20%; lower lobes


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Congenital LobarEmphysemaCongenital LobarEmphysema

DiagnosisDiagnosis Usually can be made by plain CXR; Chest CT and V/P scansUsually can be made by plain CXR; Chest CT and V/P scans

may be helpfulmay be helpful

TreatmentTreatment May require urgent surgical decompression with lobectomyMay require urgent surgical decompression with lobectomy

Selective bronchial intubationSelective bronchial intubation

Sometimes see spontaneous resolutionSometimes see spontaneous resolutionneed closeneed close

observationobservation


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Congenital LobarEmphysemaCongenital LobarEmphysema


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Congenital Cystic AdenomatousCongenital Cystic Adenomatous

Malformation (CCAM)Malformation (CCAM)

Mass of cysts lined by ciliated cuboidal orMass of cysts lined by ciliated cuboidal or

columnar pseudostratified epitheliumcolumnar pseudostratified epithelium

Three typesThree types IIfew large cysts >2cm; thick walls, normal alveoli between thefew large cysts >2cm; thick walls, normal alveoli between the

cysts; ciliated pseudostratified columnar epitheliumcysts; ciliated pseudostratified columnar epithelium

IIIInumerous small cysts


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CCAMCCAM

DiagnosisDiagnosis CT scan allows differentiation of typesCT scan allows differentiation of types

Some can be diagnosed on prenatal USSome can be diagnosed on prenatal US

TreatmentTreatment

Surgical excision, typically anatomical lobe resection, due to riskSurgical excision, typically anatomical lobe resection, due to risk

of infection,malignant transformationof infection,malignant transformation

Some are performing fetal aspirationSome are performing fetal aspiration


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CCAMCCAM


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Congenital Diaphragmatic HerniaCongenital Diaphragmatic Hernia

IntroIntro 1 in 2001 in 200--5000 live births, females >males5000 live births, females >males

Etiology unknownEtiology unknown

Large percentage of fetuses are stillbornLarge percentage of fetuses are stillborn

Still highmortality of those that make it to birthStill highmortality of those that make it to birth

DXDX

Frequentlymade prenatallyFrequentlymade prenatally

CXRCXR

TreatmentTreatment

Respiratory supportRespiratory support

ECMOECMO

Primary closure or patch closure when pt stablePrimary closure or patch closure when pt stable


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Tracheoesophageal Fistula andTracheoesophageal Fistula and

Esophageal AtresiaEsophageal Atresia


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Intestinal ObstructionIntestinal Obstruction Incidence approx 1 per 500Incidence approx 1 per 500--1000 live1000 live

birthsbirths

Approx 50% due to atresia or stenosisApprox 50% due to atresia or stenosis

Majority of neonates present shortlyMajority of neonates present shortly

after birthafter birth


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Anatomic DifferentiationAnatomic Differentiation

Upper GIUpper GI

Duodenal atresias/websDuodenal atresias/webs

small bowel atresiassmall bowel atresias

malrotation/midgut volvulusmalrotation/midgut volvulus

GERDGERD

Meconium ileusMeconium ileus

pyloric stenosispyloric stenosis

Inguinal herniaInguinal hernia

NECNEC


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Lower GILower GI

Colonic atresiaColonic atresia

Meconium plugMeconium plug

HirschsprungsHirschsprungs

Small Left Colon SyndromeSmall Left Colon Syndrome

MagalocystisMagalocystis--MicrocolonMicrocolon--IntestinalIntestinalHypoperistalsis SyndromeHypoperistalsis Syndrome

Imperforate anusImperforate anus

Anatomic DifferentiationAnatomic Differentiation


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Urgency to TreatUrgency to Treat

EmergenciesEmergencies

Free air on KUBFree air on KUB

PeritonitisPeritonitis

Acute increase in abd distensionAcute increase in abd distension

Clinical deterioration (incr pressors, decClinical deterioration (incr pressors, dec

platelets, worsening acidosis)platelets, worsening acidosis) Abd wall cellulitis/discolorationAbd wall cellulitis/discoloration


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Urgency to TreatUrgency to Treat

Further workupFurther workup

Contrast enemas for distal obstructionsContrast enemas for distal obstructions

KUB/CrossKUB/Cross--table lateraltable lateral

Milk Scans for GERDMilk Scans for GERD

UGI formalrotation/proximal atresiasUGI formalrotation/proximal atresias


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Comm

on DisordersComm

on DisordersNECNEC

Duodenal AtresiaDuodenal Atresia

Small Bowel AtresiaSmall Bowel Atresia

Malrotation/VolvulusMalrotation/Volvulus



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NEC ContNEC Cont

PresentationPresentation

distension, tachycardia, lethargy, biliousdistension, tachycardia, lethargy, bilious

output, heme pos stools, oliguriaoutput, heme pos stools, oliguria

DXDX

clinicalclinical

KUB may show pneumatosis, fixed loop,KUB may show pneumatosis, fixed loop,free air, portal venous gas, ascitesfree air, portal venous gas, ascites


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NEC TreatmentNEC Treatment

MedicalMedical

NPO, sump tube, Broad Abx after cxsNPO, sump tube, Broad Abx after cxs

drawn, serial KUB/lateral xdrawn, serial KUB/lateral x--rays, frequentrays, frequentabd examsabd exams

Surgical indicationsSurgical indications

Free airFree air Abd wall CellulitisAbd wall Cellulitis

Fixed loop on KUBFixed loop on KUB

Clinical deteriorationClinical deterioration


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NEC OutcomesNEC Outcomes

Overall survival ~ 80%, improving inOverall survival ~ 80%, improving in

LBWLBW

In pts w/perforation, 65% perioperativeIn pts w/perforation, 65% perioperative

mortality, no perfmortality, no perf----30%mortality30%mortality

25% of Survivors develop stricture25% of Survivors develop stricture

6% pts have recurrent NEC6% pts have recurrent NEC

Postop NECPostop NEC----Myelomeningocele,Myelomeningocele,

GastroschisisGastroschisis----4545--65%mortality65%mortality


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PneumatosisPneumatosis


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PneumoperitoneumPneumoperitoneum


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NECNEC----Abd Distension/ErythemaAbd Distension/Erythema


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Necrotic Segment IleumNecrotic Segment Ileum


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ResectionResection


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SpecimenSpecimen----IleocecectomyIleocecectomy


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IleostomyIleostomy


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Comm

on DisordersComm

on DisordersNECNEC



MalrotationMalrotation



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IncidenceIncidence----1 in 5,000 to 10,000 live1 in 5,000 to 10,000 live

birthsbirths

75% of stenoses and 40% of atresias are75% of stenoses and 40% of atresias arefound in Duodenumfound in Duodenum

Multiple atresias in 15% of casesMultiple atresias in 15% of cases

50% pts are LBW and premature50% pts are LBW and premature

Polyhydramnios in 75%Polyhydramnios in 75%

Bilious emesis usually presentBilious emesis usually present


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Duodenal Atresia ContDuodenal Atresia Cont

Associated AnomaliesAssociated Anomalies

Downs (30%)Downs (30%)


Congenital Heart DiseaseCongenital Heart Disease

Esophageal AtresiaEsophageal Atresia

Urinary Tract MalformationsUrinary Tract Malformations AnorectalmalformationsAnorectalmalformations

VACTERLVACTERL


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Duodenal Atresia DiagnosisDuodenal Atresia Diagnosis

RadiographsRadiographs

DoubleDouble--BubbleBubble

Pyloric dimple signPyloric dimple sign

Absence of beak sign seen in pyloricAbsence of beak sign seen in pyloric

obstructionobstruction

Workup of potential associatedWorkup of potential associatedanomaliesanomalies

ECHO, abd US, possible VCUGECHO, abd US, possible VCUG


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Double BubbleDouble Bubble


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Duodenal Atresia TreatmentDuodenal Atresia Treatment

Nasogastric decompression, hydrationNasogastric decompression, hydration

SurgerySurgery

Double diamond duodenoduodenostomyDouble diamond duodenoduodenostomy

Cont prolonged NG decompression,Cont prolonged NG decompression,

sometimesmore than 2 weeks neededsometimesmore than 2 weeks needed


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Comm

on DisordersComm

on DisordersNECNEC






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Jejunal is most common, about 1 perJejunal is most common, about 1 per

2,000 live births2,000 live births

Atresia due to inAtresia due to in--utero occlusion of all orutero occlusion of all or

part of the blood supply to the bowelpart of the blood supply to the bowel

ClassificationClassification----Types ITypes I--IVIV

Presents w/bilious emesis, abdPresents w/bilious emesis, abd

distension, failure to pass meconiumdistension, failure to pass meconium

(70%)(70%)


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Intestinal Atresia ClassificationIntestinal Atresia Classification


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Small Bowel Atresia ContSmall Bowel Atresia Cont

Associated AnomaliesAssociated Anomalies

other atresiasother atresias


Biliary atresiaBiliary atresia

polysplenia syndrome (situs inversus,polysplenia syndrome (situs inversus,

cardiac anomalies, atresias)cardiac anomalies, atresias) CF (10%)CF (10%)


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AtresiaAtresia----Diagnosis and TreatmentDiagnosis and Treatment

Plain films show dilated loops small bowelPlain films show dilated loops small bowel

Contrast enema shows small unused colonContrast enema shows small unused colon

UGI/SBFT shows failure of contrast to passUGI/SBFT shows failure of contrast to passbeyond atretic pointbeyond atretic point

TreatmentTreatment is surgicalis surgical tapered primary anastamosistapered primary anastamosis

check for other atresias/associated anomaliescheck for other atresias/associated anomalies


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Comm

on DisordersComm

on DisordersNECNEC



Malrotation/VolvulusMalrotation/Volvulus



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1 per 6,000 live births1 per 6,000 live births

can be asymptomatic throughout lifecan be asymptomatic throughout life

Usually presents in first 6 months of lifeUsually presents in first 6 months of life 18% children w/short gut had malrotation with18% children w/short gut had malrotation with

volvulusvolvulus

EtiologyEtiology physiologic umbilical herniaphysiologic umbilical hernia----4th wk gestation4th wk gestation

Reduction of hernia 10thReduction of hernia 10th -- 12th wks of gestation12th wks of gestation


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Normal EmbryologyNormal Embryology


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Malrotation ClassificationMalrotation Classification

NonrotationNonrotation

when neither duodenojejunal or cecocolicwhen neither duodenojejunal or cecocolic

limbs undergo correct rotationlimbs undergo correct rotation

Abn Rotation ofAbn Rotation of Duodenojejunal limbDuodenojejunal limb

causes Ladds bands to form acrosscauses Ladds bands to form across

duodenumduodenum Abn rotation ofAbn rotation of Cecocolic limbCecocolic limb

cecum lies close to midline, narrowcecum lies close to midline, narrow

mesenteric basemesenteric base


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Abnormal Rotation/FixationAbnormal Rotation/Fixation


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Malrotation DiagnosisMalrotation Diagnosis

Varying symptoms from very mild toVarying symptoms from very mild to

catastrophiccatastrophic

**Bilious em

esis is Volvulus until proven**Bilious em

esis is Volvulus until provenotherwise**otherwise**

Bilious emesis, bloody diarrhea, abdBilious emesis, bloody diarrhea, abd

distension, lethargy, shockdistension, lethargy, shock

UGI shows abnormal position ofUGI shows abnormal position of

DuodenumDuodenum

if Volvulus, see birds beak in duodenumif Volvulus, see birds beak in duodenum


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Malrotation UGIMalrotation UGI


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Intraop VolvulusIntraop Volvulus


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Bowel NecrosisBowel Necrosis----VolvulusVolvulus


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MalrotationMalrotation----TreatmentTreatment

SurgicalSurgical----Ladds ProcedureLadds Procedure

EviscerationEvisceration

Untwisting of volvulus (counterclockwise)Untwisting of volvulus (counterclockwise)

Division of Ladds BandsDivision of Ladds Bands

Widening mesenteric baseWidening mesenteric base

Relief of Duodenal obstructionRelief of Duodenal obstruction AppendectomyAppendectomy

Recurrence 10% after LaddsRecurrence 10% after Ladds


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Common DisordersCommon Disorders

NECNEC






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Hirschsprungs DiseaseHirschsprungs Disease

Migratory failure of neural crest cellsMigratory failure of neural crest cells

Incidence 1 in 5,000 live births, malesIncidence 1 in 5,000 live births, males

affected 4:1 over femalesaffected 4:1 over females

90% of pts w/Hsprungs fail to pass90% of pts w/Hsprungs fail to pass

meconium in first 24meconium in first 24--48 hrs48 hrs

Abd distension, bilious emesis,Abd distension, bilious emesis,obstructive enterocolitisobstructive enterocolitis


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Hirschsprungs DiagnosisHirschsprungs Diagnosis

BariumEnemaBariumEnema

Transition zoneTransition zone

Anorectal ManometryAnorectal Manometry

shows failure of reflexive relaxationshows failure of reflexive relaxation

not very helpful in infants, young childrennot very helpful in infants, young children

Rectal BiopsyRectal Biopsy Absence of Ganglion cells and hypertrophyAbsence of Ganglion cells and hypertrophy

of nervesof nerves


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Transition Zone on BETransition Zone on BE


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Hirschsprungs TreatmentHirschsprungs Treatment

In neonates, can do primary pullIn neonates, can do primary pull--

throughthrough----bringing normal colon down tobringing normal colon down to

anorectal junctionanorectal junction In older infants, may need divertingIn older infants, may need diverting

colostomy first to decompresscolostomy first to decompress

May need prolonged dilatations andMay need prolonged dilatations andirrigationsirrigations


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PullPull--Through ProcedureThrough Procedure


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SummarySummary BILIOUS EMESIS IS VOLVULUSBILIOUS EMESIS IS VOLVULUS

UNTIL PROVEN OTHERWISEUNTIL PROVEN OTHERWISE

Signs of surgical emergencySigns of surgical emergency

free air, abd wall cellulitis, fixed loop onfree air, abd wall cellulitis, fixed loop on

xray, rapid distension, peritonitis, clinicalxray, rapid distension, peritonitis, clinical

deteriorationdeterioration

History and plain films will guideHistory and plain films will guidesequence of additional studiessequence of additional studies

Remember associated anomaliesRemember associated anomalies

Documents

Surgical Emergencies in the Newborn-1