Surgical Emergencies in the Newborn University of North Carolina at Chapel Hill Pediatric Surgery...

Surgical Emergencies in Surgical Emergencies in the Newbornthe Newborn

University of North Carolina at Chapel HillUniversity of North Carolina at Chapel HillPediatric Surgery DivisionPediatric Surgery Division

Patty LangePatty LangeLast revised 4/15/06Last revised 4/15/06

EmergenciesEmergencies

TypesTypes Airway/RespiratoryAirway/Respiratory Intestinal ObstructionIntestinal Obstruction Intestinal PerforationIntestinal Perforation

SignsSigns Respiratory distressRespiratory distress Abdominal distensionAbdominal distension PeritonitisPeritonitis PneumoperitoneumPneumoperitoneum

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 or more lobes (nl histological lung)Overdistension of one or more lobes (nl histological lung) Congenital cystic adenomatous malformationCongenital cystic adenomatous malformation

• Multicystic mass of lung tissue, proliferation of bronchial structures Multicystic mass of lung tissue, proliferation of bronchial structures at the expense of alveoliat the expense of alveoli

Pulmonary agenesisPulmonary agenesis• Absence of lung Absence of lung

Congenital diaphragmatic herniaCongenital diaphragmatic hernia Tracheoesophageal fistulaTracheoesophageal fistula

Cystic HygromaCystic Hygroma

Multiloculated cystic spaces lined by endothelial Multiloculated cystic spaces lined by endothelial cellscells

Separated by fine walls containing numerous smooth muscle Separated by fine walls containing numerous smooth muscle cellscells

Result of maldevelopment of lymphatic spacesResult of maldevelopment of lymphatic spaces

Incidence about 1 in 12,000 birthsIncidence about 1 in 12,000 births 50-65% appear at birth, 85-90% appear by age 250-65% appear at birth, 85-90% appear by age 2 Neck-75%, Axilla 20%; can be seen in mediastinum, Neck-75%, Axilla 20%; can be seen in mediastinum,

retroperitoneum, pelvis, groinretroperitoneum, pelvis, groin Nuchal/post cervical CH’s have been associated with Nuchal/post cervical CH’s have been associated with

chromosomal abnormalities—high mortality ratechromosomal abnormalities—high mortality rate

Cystic HygromaCystic Hygroma

ComplicationsComplications Respiratory—large hygromas can extend into oropharynx and Respiratory—large 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 treatment is complete excisionBest treatment is complete excision Aspiration typically not effective due to rapid refilling of fluidAspiration typically not effective due to rapid refilling of fluid Sclerotherapy—Bleomycin, OK-432 (no longer available in US), Sclerotherapy—Bleomycin, OK-432 (no longer available in US),

doxycycline, fibrin gluedoxycycline, fibrin glue

Cystic HygromaCystic Hygroma

Cystic HygromaCystic Hygroma

Postnatal overdistension of one or more lobes of Postnatal overdistension of one or more lobes of histologically normal lunghistologically normal lung

Probably due to cartilaginous deficiency in the tracheobronchial Probably due to cartilaginous deficiency in the tracheobronchial treetree

Obstruction causing the overdistension may be due toObstruction causing the overdistension may be due to• 1—chondromalacia of bronchi1—chondromalacia of bronchi

• 2—extrinsic pressure on bronchus by anomalous pulmonary vein or 2—extrinsic pressure on bronchus by anomalous pulmonary vein or abnormally large PDAabnormally large PDA

• 3—idiopathic3—idiopathic

LocationLocation LUL 47%, RML 28%, RUL 20%; lower lobes <5%; Bilat rareLUL 47%, RML 28%, RUL 20%; lower lobes <5%; Bilat rare

Congenital Lobar EmphysemaCongenital Lobar Emphysema

Congenital Lobar EmphysemaCongenital Lobar Emphysema

DiagnosisDiagnosis Usually can be made by plain CXR; Chest CT and V/P scans Usually 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 resolution—need close Sometimes see spontaneous resolution—need close

observationobservation

Congenital Lobar EmphysemaCongenital Lobar Emphysema

Congenital Cystic Adenomatous Congenital Cystic Adenomatous Malformation (CCAM)Malformation (CCAM)

Mass of cysts lined by ciliated cuboidal or Mass of cysts lined by ciliated cuboidal or columnar pseudostratified epitheliumcolumnar pseudostratified epithelium

Three typesThree types I—few large cysts >2cm; thick walls, normal alveoli between the I—few large cysts >2cm; thick walls, normal alveoli between the

cysts; ciliated pseudostratified columnar epitheliumcysts; ciliated pseudostratified columnar epithelium II—numerous small cysts <1cm, thin muscular coat, large II—numerous small cysts <1cm, thin muscular coat, large

alveolar-like structures between the cysts; ciliated cuboidal to alveolar-like structures between the cysts; ciliated cuboidal to columnar epithelium; assoc w/other congenital anomaliescolumnar epithelium; assoc w/other congenital anomalies

III—bulky firm masses of folded ciliated and non-ciliated cuboidal III—bulky firm masses of folded ciliated and non-ciliated cuboidal epithelium and thick layer of smooth muscle; often occupy the epithelium and thick layer of smooth muscle; often occupy the entire lobe or lobes of lungentire lobe or lobes of lung

More common on the left side, 2% bilateralMore common on the left side, 2% bilateral

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

of infection, malignant transformationof infection, malignant transformation Some are performing fetal aspirationSome are performing fetal aspiration

CCAMCCAM

Congenital Diaphragmatic HerniaCongenital Diaphragmatic Hernia IntroIntro

1 in 200-5000 live births, females >males1 in 200-5000 live births, females >males Etiology unknownEtiology unknown Large percentage of fetuses are stillbornLarge percentage of fetuses are stillborn Still high mortality of those that make it to birthStill high mortality of those that make it to birth

DXDX Frequently made prenatallyFrequently made prenatally CXRCXR

TreatmentTreatment Respiratory supportRespiratory support ECMOECMO Primary closure or patch closure when pt stablePrimary closure or patch closure when pt stable

Tracheoesophageal Fistula and Tracheoesophageal Fistula and Esophageal AtresiaEsophageal Atresia

Intestinal ObstructionIntestinal Obstruction

Incidence approx 1 per 500-1000 live Incidence approx 1 per 500-1000 live birthsbirths

Approx 50% due to atresia or stenosisApprox 50% due to atresia or stenosis Majority of neonates present shortly Majority of neonates present shortly

after birthafter birth

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

Lower GILower GI Colonic atresiaColonic atresia Meconium plugMeconium plug Hirschsprung’sHirschsprung’s Small Left Colon SyndromeSmall Left Colon Syndrome Magalocystis-Microcolon-Intestinal Magalocystis-Microcolon-Intestinal

Hypoperistalsis SyndromeHypoperistalsis Syndrome Imperforate anusImperforate anus

Anatomic DifferentiationAnatomic Differentiation

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, dec Clinical deterioration (incr pressors, dec

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

Urgency to TreatUrgency to Treat

Further workupFurther workup Contrast enemas for distal obstructionsContrast enemas for distal obstructions KUB/Cross-table lateral KUB/Cross-table lateral Milk Scans for GERDMilk Scans for GERD UGI for malrotation/proximal atresiasUGI for malrotation/proximal atresias

Common DisordersCommon Disorders

NECNEC Duodenal AtresiaDuodenal Atresia Small Bowel AtresiaSmall Bowel Atresia Malrotation/VolvulusMalrotation/Volvulus Hirschsprung’sHirschsprung’s

NEC Con’tNEC Con’t

PresentationPresentation distension, tachycardia, lethargy, bilious distension, 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

NEC TreatmentNEC Treatment

MedicalMedical NPO, sump tube, Broad Abx after cx’s NPO, sump tube, Broad Abx after cx’s

drawn, serial KUB/lateral x-rays, frequent drawn, serial KUB/lateral x-rays, frequent abd examsabd exams

Surgical indicationsSurgical indications Free airFree air Abd wall CellulitisAbd wall Cellulitis Fixed loop on KUBFixed loop on KUB Clinical deteriorationClinical deterioration

NEC OutcomesNEC Outcomes

Overall survival ~ 80%, improving in LBWOverall survival ~ 80%, improving in LBW In pts w/perforation, 65% perioperative In pts w/perforation, 65% perioperative

mortality, no perf--30% mortalitymortality, no perf--30% mortality 25% of Survivors develop stricture25% of Survivors develop stricture 6% pts have recurrent NEC6% pts have recurrent NEC Postop NEC--Myelomeningocele, Postop NEC--Myelomeningocele,

Gastroschisis--45-65% mortalityGastroschisis--45-65% mortality

PneumatosisPneumatosis

PneumoperitoneumPneumoperitoneum

NEC--Abd Distension/ErythemaNEC--Abd Distension/Erythema

Necrotic Segment IleumNecrotic Segment Ileum

ResectionResection

Specimen--IleocecectomySpecimen--Ileocecectomy

IleostomyIleostomy

Common DisordersCommon Disorders

NECNEC Duodenal AtresiaDuodenal Atresia Small Bowel AtresiaSmall Bowel Atresia MalrotationMalrotation Hirschsprung’sHirschsprung’s

Duodenal AtresiaDuodenal Atresia

Incidence--1 in 5,000 to 10,000 live birthsIncidence--1 in 5,000 to 10,000 live births 75% of stenoses and 40% of atresias are 75% of stenoses and 40% of atresias are

found 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

Duodenal Atresia Con’tDuodenal Atresia Con’t

Associated AnomaliesAssociated Anomalies Down’s (30%)Down’s (30%) MalrotationMalrotation Congenital Heart DiseaseCongenital Heart Disease Esophageal AtresiaEsophageal Atresia Urinary Tract MalformationsUrinary Tract Malformations Anorectal malformationsAnorectal malformations VACTERLVACTERL

Duodenal Atresia DiagnosisDuodenal Atresia Diagnosis

RadiographsRadiographs ““Double-Bubble”Double-Bubble” Pyloric dimple signPyloric dimple sign Absence of “beak” sign seen in pyloric Absence of “beak” sign seen in pyloric

obstructionobstruction Workup of potential associated Workup of potential associated

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

““Double Bubble”Double Bubble”

Duodenal Atresia TreatmentDuodenal Atresia Treatment

Nasogastric decompression, hydrationNasogastric decompression, hydration SurgerySurgery

Double diamond duodenoduodenostomyDouble diamond duodenoduodenostomy Con’t prolonged NG decompression, Con’t prolonged NG decompression,

sometimes more than 2 weeks neededsometimes more than 2 weeks needed

Common DisordersCommon Disorders

NECNEC Duodenal AtresiaDuodenal Atresia Small Bowel AtresiaSmall Bowel Atresia MalrotationMalrotation Hirschsprung’sHirschsprung’s

Small Bowel AtresiaSmall Bowel Atresia

Jejunal is most common, about 1 per Jejunal is most common, about 1 per 2,000 live births2,000 live births

Atresia due to in-utero occlusion of all or Atresia due to in-utero occlusion of all or part of the blood supply to the bowelpart of the blood supply to the bowel

Classification--Types I-IVClassification--Types I-IV Presents w/bilious emesis, abd Presents w/bilious emesis, abd

distension, failure to pass meconium distension, failure to pass meconium (70%)(70%)

Intestinal Atresia ClassificationIntestinal Atresia Classification

Small Bowel Atresia Con’tSmall Bowel Atresia Con’t

Associated AnomaliesAssociated Anomalies other atresiasother atresias Hirschsprung’sHirschsprung’s Biliary atresiaBiliary atresia polysplenia syndrome (situs inversus, polysplenia syndrome (situs inversus,

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

Atresia--Diagnosis and TreatmentAtresia--Diagnosis 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 pass UGI/SBFT shows failure of contrast to pass

beyond atretic pointbeyond atretic point TreatmentTreatment is surgical is surgical

tapered primary anastamosistapered primary anastamosis check for other atresias/associated anomaliescheck for other atresias/associated anomalies

Common DisordersCommon Disorders

NECNEC Duodenal AtresiaDuodenal Atresia Small Bowel AtresiaSmall Bowel Atresia Malrotation/VolvulusMalrotation/Volvulus Hirschsprung’sHirschsprung’s

MalrotationMalrotation

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 with 18% children w/short gut had malrotation with

volvulusvolvulus EtiologyEtiology

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

Normal EmbryologyNormal Embryology

Malrotation ClassificationMalrotation Classification

NonrotationNonrotation when neither duodenojejunal or cecocolic limbs when neither duodenojejunal or cecocolic limbs

undergo correct rotationundergo correct rotation Abn Rotation of Abn Rotation of Duodenojejunal limbDuodenojejunal limb

causes Ladd’s bands to form across causes Ladd’s bands to form across duodenumduodenum

Abn rotation of Abn rotation of Cecocolic limbCecocolic limb cecum lies close to midline, narrow mesenteric cecum lies close to midline, narrow mesenteric

basebase

Abnormal Rotation/FixationAbnormal Rotation/Fixation

Malrotation DiagnosisMalrotation Diagnosis

Varying symptoms from very mild to Varying symptoms from very mild to catastrophiccatastrophic

**Bilious emesis is Volvulus until proven **Bilious emesis is Volvulus until proven otherwise**otherwise**

Bilious emesis, bloody diarrhea, abd Bilious emesis, bloody diarrhea, abd distension, lethargy, shockdistension, lethargy, shock

UGI shows abnormal position of UGI shows abnormal position of DuodenumDuodenum if Volvulus, see “bird’s beak” in duodenumif Volvulus, see “bird’s beak” in duodenum

Malrotation UGIMalrotation UGI

Intraop VolvulusIntraop Volvulus

Bowel Necrosis--VolvulusBowel Necrosis--Volvulus

Malrotation--TreatmentMalrotation--Treatment

Surgical--Ladd’s ProcedureSurgical--Ladd’s Procedure EviscerationEvisceration Untwisting of volvulus (counterclockwise)Untwisting of volvulus (counterclockwise) Division of Ladd’s BandsDivision of Ladd’s Bands Widening mesenteric baseWidening mesenteric base Relief of Duodenal obstructionRelief of Duodenal obstruction AppendectomyAppendectomy

Recurrence 10% after Ladd’sRecurrence 10% after Ladd’s

Common DisordersCommon Disorders

NECNEC Duodenal AtresiaDuodenal Atresia Small Bowel AtresiaSmall Bowel Atresia MalrotationMalrotation Hirschsprung’sHirschsprung’s

Hirschsprung’s DiseaseHirschsprung’s Disease

Migratory failure of neural crest cellsMigratory failure of neural crest cells Incidence 1 in 5,000 live births, males Incidence 1 in 5,000 live births, males

affected 4:1 over femalesaffected 4:1 over females 90% of pts w/H’sprung’s fail to pass 90% of pts w/H’sprung’s fail to pass

meconium in first 24-48 hrsmeconium in first 24-48 hrs Abd distension, bilious emesis, Abd distension, bilious emesis,

obstructive enterocolitisobstructive enterocolitis

Hirschsprung’s DiagnosisHirschsprung’s Diagnosis

Barium EnemaBarium Enema 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 hypertrophy Absence of Ganglion cells and hypertrophy

of nervesof nerves

Transition Zone on BETransition Zone on BE

Hirschsprung’s TreatmentHirschsprung’s Treatment

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

In older infants, may need diverting In older infants, may need diverting colostomy first to decompresscolostomy first to decompress

May need prolonged dilatations and May need prolonged dilatations and irrigationsirrigations

Pull-Through ProcedurePull-Through Procedure

SummarySummary BILIOUS EMESIS IS VOLVULUS BILIOUS EMESIS IS VOLVULUS

UNTIL PROVEN OTHERWISEUNTIL PROVEN OTHERWISE Signs of surgical emergencySigns of surgical emergency

free air, abd wall cellulitis, fixed loop on free air, abd wall cellulitis, fixed loop on xray, rapid distension, peritonitis, clinical xray, rapid distension, peritonitis, clinical deteriorationdeterioration

History and plain films will guide History and plain films will guide sequence of additional studiessequence of additional studies

Remember associated anomaliesRemember associated anomalies