surgical emergencies in the newborn university of north carolina at chapel hill pediatric surgery...
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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