SURGICAL SURGICAL EMERGENCIES IN EMERGENCIES IN THE NEWBORNTHE NEWBORN

TRACY L. MCTIERNAN, MA, TRACY L. MCTIERNAN, MA, CPNPCPNP

DIVISION OF PEDIATRIC DIVISION OF PEDIATRIC SURGERYSURGERY

NYU LANGONE MEDICAL NYU LANGONE MEDICAL CENTERCENTER

Congenital Diaphragmatic HerniaCongenital Diaphragmatic Hernia Esophageal AtresiaEsophageal Atresia Congenital Intestinal ObstructionCongenital Intestinal Obstruction

Duodenal AtresiaDuodenal Atresia Ileal and Jejunal Atresia Ileal and Jejunal Atresia Meconium IleusMeconium Ileus Malrotation and VolvulusMalrotation and Volvulus Hirschsprung’s DiseaseHirschsprung’s Disease Imperforate AnusImperforate Anus

Abdominal Wall DefectsAbdominal Wall Defects OmphaloceleOmphalocele GastroschisisGastroschisis

WHAT ARE WE TALKING WHAT ARE WE TALKING ABOUT?ABOUT?

Congenital Diaphragmatic Congenital Diaphragmatic HerniaHernia

IncidenceIncidence Estimated to be between 1/2000 to Estimated to be between 1/2000 to

5000 live births5000 live births 1/3 of infants are stillborn1/3 of infants are stillborn Males > females in live birthsMales > females in live births Approximately 80% are on the left sideApproximately 80% are on the left side Occurrence risk in a 1Occurrence risk in a 1stst degree relative degree relative

~2%~2%

EtiologyEtiology Cause of CDH is unknownCause of CDH is unknown Increasing evidence that it may be Increasing evidence that it may be

related to exposure to environmental related to exposure to environmental factorsfactors

Associated AnomaliesAssociated Anomalies Incidence ranges from 10-50%Incidence ranges from 10-50% Skeletal defects ~32%Skeletal defects ~32% Cardiac anomalies ~24%Cardiac anomalies ~24% Tracheobronchial tree ~18%Tracheobronchial tree ~18%

PathologyPathology In most cases the defect is established by In most cases the defect is established by

gestational week 12gestational week 12 Classic left sided CDH features a 2-4 cm Classic left sided CDH features a 2-4 cm

posterolateral defectposterolateral defect Herniated contents often include the left lobe of Herniated contents often include the left lobe of

the liver, the spleen, and almost the entire GI tractthe liver, the spleen, and almost the entire GI tract Long term compression of the developing fetal Long term compression of the developing fetal

lungs results in pulmonary maldevelopment and lungs results in pulmonary maldevelopment and lung hypoplasialung hypoplasia

All the major bronchial buds are present in the All the major bronchial buds are present in the CDH lung but the number of bronchial branches is CDH lung but the number of bronchial branches is greatly reducedgreatly reduced

Alveolar development is also severely affectedAlveolar development is also severely affected

PathologyPathology Pulmonary vascular bed is abnormal, Pulmonary vascular bed is abnormal,

resulting in increased pulmonary resulting in increased pulmonary vascular resistancevascular resistance

Increasing hypoxia results in increasing Increasing hypoxia results in increasing right-to left shunting leading to severe right-to left shunting leading to severe and progressive respiratory failureand progressive respiratory failure

DiagnosisDiagnosis Prenatal ultrasound is accurate in 40-Prenatal ultrasound is accurate in 40-

90% of cases90% of cases Mean gestational age is 24 weeks with Mean gestational age is 24 weeks with

some discovered as early as 11 weekssome discovered as early as 11 weeks Polyhydramnios is present in up to 80% Polyhydramnios is present in up to 80%

of casesof cases After birth the respiratory symptoms After birth the respiratory symptoms

are determined by the degree of are determined by the degree of pulmonary hypoplasia and reactive pulmonary hypoplasia and reactive pulmonary hypertensionpulmonary hypertension

DiagnosisDiagnosis Most severely affected infants develop Most severely affected infants develop

respiratory distress at birthrespiratory distress at birth Majority demonstrate symptoms within Majority demonstrate symptoms within

the first 24 hoursthe first 24 hours Infants will have scaphoid abdomen and Infants will have scaphoid abdomen and

symmetrically distended chestsymmetrically distended chest Tracheal deviation away from the defectTracheal deviation away from the defect Confirmed by plain chest radiograph Confirmed by plain chest radiograph

which demonstrates loops of bowel in the which demonstrates loops of bowel in the chestchest

ManagementManagement Key to consider that CDH is a physiologic Key to consider that CDH is a physiologic

emergency emergency NOTNOT a surgical emergency a surgical emergency Early surgery further decreases lung complianceEarly surgery further decreases lung compliance The post birth transition of vascular and pulmonary The post birth transition of vascular and pulmonary

function is prolonged in CDHfunction is prolonged in CDH In theory, delayed surgery provides additional time In theory, delayed surgery provides additional time

for this transition to occur resulting in a more for this transition to occur resulting in a more stable infantstable infant

Infants should be intubated and an nasogastric Infants should be intubated and an nasogastric tube passedtube passed

Ventilation by mask or Ambu bag is contraindicatedVentilation by mask or Ambu bag is contraindicated May be managed on a conventional ventilator or a May be managed on a conventional ventilator or a

high frequency ventilatorhigh frequency ventilator

ManagementManagement Key to success is currently thought to Key to success is currently thought to

be gentle ventilation with permissive be gentle ventilation with permissive hypercapnea to reduce barotraumahypercapnea to reduce barotrauma

Preductal pulseoximetry should be Preductal pulseoximetry should be monitoredmonitored

Metabolic acid base disturbances Metabolic acid base disturbances should be corrected with fluid should be corrected with fluid management or bicarbonate management or bicarbonate administrationadministration

ECMO in severe cases - but often NOT ECMO in severe cases - but often NOT necessarynecessary

Surgical ManagementSurgical Management Timing of surgery is dependent on the infants Timing of surgery is dependent on the infants

condition, ideally anywhere from 5 days to 2 condition, ideally anywhere from 5 days to 2 weeks of ageweeks of age

Infant’s ventilator settings are improving and Infant’s ventilator settings are improving and being weanedbeing weaned

Surgical approach is usually through a Surgical approach is usually through a subcostal incision. Laparoscopic and subcostal incision. Laparoscopic and thoracoscopic techniques can also be usedthoracoscopic techniques can also be used

Abdominal viscera are gently reduced into the Abdominal viscera are gently reduced into the abdominal cavityabdominal cavity

Defect is closed primarily or utilizing a patchDefect is closed primarily or utilizing a patch Abdominal wall is closed if possible avoiding Abdominal wall is closed if possible avoiding

increased intra-abdominal pressureincreased intra-abdominal pressure

OutcomesOutcomes Survival rates as high as 80-90% are Survival rates as high as 80-90% are

being reached with current treatment being reached with current treatment modalitiesmodalities

High variation in survival rates among High variation in survival rates among institutions represent different institutions represent different treatment strategiestreatment strategies

Associated anomalies such as Associated anomalies such as congenital heart disease remain a congenital heart disease remain a significant risk factor for poor outcomesignificant risk factor for poor outcome

Esophageal Atresia and Esophageal Atresia and Tracheoesophageal Tracheoesophageal

FistulaFistula IncidenceIncidence

EA and TEF are relatively common congenital EA and TEF are relatively common congenital anomaliesanomalies

1 in 4500 live births in the US1 in 4500 live births in the US Male=FemaleMale=Female 0.5% - 2% increase risk in newborns with one 0.5% - 2% increase risk in newborns with one

affected siblingaffected sibling Risk increases to 20% if more than one sibling Risk increases to 20% if more than one sibling

is affectedis affected

ClassificationClassification

Esophageal atresia rarely occurs as Esophageal atresia rarely occurs as an isolated congenital anomaly. an isolated congenital anomaly.

Esophageal atresia alone is due to Esophageal atresia alone is due to failure of the recanalization of the failure of the recanalization of the esophagus during the 8esophagus during the 8thth week of week of gestation gestation

Gross Classification

85% are type C (Distal TEF)85% are type C (Distal TEF) 7% are type A (Pure atresia)7% are type A (Pure atresia) 4% are type E ( H-type fistula)4% are type E ( H-type fistula) Proximal fistula is the least commonProximal fistula is the least common

Associated Anomalies VACTERL: vertebral, anal, cardiac, TE, renal, limb

Congenital heart disease is associated with higher mortality

VSD is most common

ASD

Tetralogy of Fallot

PDA

Echocardiogram – important to determine position of aortic arch

Associated Anomalies Vertebral and radial anomalies will be

seen on x-ray Sonogram of the kidneys Physical Exam of the anus

Clinical FeaturesClinical Features PrenatallyPrenatally

• Predictive value of prenatal ultrasound ~20-40%Predictive value of prenatal ultrasound ~20-40%• Polyhydramnios (2/3 cases)Polyhydramnios (2/3 cases)• Small or absent stomach bubbleSmall or absent stomach bubble

PostnatallyPostnatally--

• Most infants are symptomatic within the first few hours Most infants are symptomatic within the first few hours of lifeof life

• Excessive salivationExcessive salivation• Regurgitation of first feedRegurgitation of first feed• Cyanosis with/without feedsCyanosis with/without feeds• Respiratory distressRespiratory distress• Inability to pass a catheter into the stomachInability to pass a catheter into the stomach• Gastric distention (with distal fistula)Gastric distention (with distal fistula)

DiagnosisDiagnosis Failure to pass NG tube (not Type E)Failure to pass NG tube (not Type E) CXR- tube coiled in upper esophagusCXR- tube coiled in upper esophagus ““Pouchogram” with air Pouchogram” with air Distal air on AXR confirms the presence Distal air on AXR confirms the presence

of a fistulaof a fistula H-type fistulas are often diagnosed later H-type fistulas are often diagnosed later

DiagnosisConfirm with AP chest x-ray that demonstrates the catheter curled in the upper esophageal pouch.

•Abdominal XR can help distinguish esophageal blind pouch (no gastric air) from distal TEF (gastric air)

DiagnosisDiagnosisAttempt to pass catheter into stomach. Cannot pass more than 10-15 cm.

When diagnosis is uncertain or proximal TEF is suspected, a small amount of water-soluble contrast material can be injected into the esophageal pouch under fluoroscopic guidance (must remove contrast material immediately to avoid regurgitation and aspiration)

Diagnosis

Diagnosis

Proximal Esophageal Atresia

Diagnosis- Type E “H type” TEF

ManagementManagement PreoperativelyPreoperatively

• Minimize complications from aspirationMinimize complications from aspiration• Suction blind pouch continuously with Suction blind pouch continuously with

Replogle tubeReplogle tube• NPO/TPNNPO/TPN• Upright position of childUpright position of child• Early surgery for short gap atresiaEarly surgery for short gap atresia• Long gap atresia may be delayed up to 6-12 Long gap atresia may be delayed up to 6-12

weeksweeks• Long gap atresia should have gastrostomy Long gap atresia should have gastrostomy

tube placed for enteral nutritiontube placed for enteral nutrition

ManagementManagement SurgerySurgery

• Surgical ligation of the fistulaSurgical ligation of the fistula• Primary anastomosis of the esophageal Primary anastomosis of the esophageal

segmentssegments• Primary repair may not be possible if the Primary repair may not be possible if the

distance between esophageal segments is distance between esophageal segments is long. Staged procedures have been long. Staged procedures have been performed that include elongation of the performed that include elongation of the esophagus with circular myotomy, esophagus with circular myotomy, interposition of the colon, and gastric interposition of the colon, and gastric transpositiontransposition

To repair esophageal atresia, a thoracotomy incision is made (A). The proximal and distal esophageal segments are identified (B). The distal fistula is transected (C) and anastomosed to the upper esophageal pouch (D). (With H-type fistula a cervical approach can be used in most cases)

Post Operative Post Operative ManagementManagement

ManagementManagement Post OperativePost Operative

• Adequate fluid resuscitationAdequate fluid resuscitation• Wean ventilatorWean ventilator• No deep tracheal suctioningNo deep tracheal suctioning• Avoid bag/mask ventilationAvoid bag/mask ventilation• No pacifiers/suckingNo pacifiers/sucking• Chest tube remains in place for approximately Chest tube remains in place for approximately

one weekone week• Replogle tube (10 Fr) remains in place +/- one Replogle tube (10 Fr) remains in place +/- one

weekweek• Esophagram 1 week post opEsophagram 1 week post op

Outcome

PROGNOSIS

85% to 95% survival

Infants with increased risk of morbidity and mortality

•Low birth weight (>1500)

•Major CHD

•Severe associated anomalies

•Ventilator dependency

•Long gap atresia

Outcome Complications

• Anastomotic Leak• Esophageal stricture• Recurrent fistula

Other Associated Issues Tracheomalacia Disturbed peristalsis and delayed gastric emptying are

common “Seal Bark” cough Gastroesophageal reflux and aspiration

Congenital Intestinal Congenital Intestinal ObstructionObstruction

Intestinal AtresiaIntestinal Atresia Duodenal atresiaDuodenal atresia Ileal and jejunal atresiaIleal and jejunal atresia

Meconium IleusMeconium Ileus Malrotation and VolvulusMalrotation and Volvulus Hirschspung’s DiseaseHirschspung’s Disease Imperforate AnusImperforate Anus

Duodenal AtresiaDuodenal Atresia IncidenceIncidence

Most common site of neonatal intestinal Most common site of neonatal intestinal obstructionobstruction

1 in 6,000 to 10,000 live births1 in 6,000 to 10,000 live births 75% of stenoses and 40% of atresias are found 75% of stenoses and 40% of atresias are found

in Duodenumin Duodenum Multiple atresias in 15% of casesMultiple atresias in 15% of cases 50% pts are LBW and premature50% pts are LBW and premature

EtiologyEtiology No specific genetic abnormality No specific genetic abnormality Increase incidence in siblingsIncrease incidence in siblings Has been shown to occur in several Has been shown to occur in several

generations of a familygenerations of a family Association with Trisomy 21Association with Trisomy 21

Associated AnomaliesAssociated Anomalies Down Syndrome 28%Down Syndrome 28% Annular pancreas 23%Annular pancreas 23% Congenital heart disease 23%Congenital heart disease 23% Malrotation 20%Malrotation 20% Esophageal atresia/TEF 9%Esophageal atresia/TEF 9% Genitourinary 8%Genitourinary 8% Anorectal 4%Anorectal 4% Other bowel atresia 4%Other bowel atresia 4% None 45%None 45%

PathologyPathology

Type I: The most common type is formed by a membrane composed of mucosa and submucosa and obstructs the lumen. A variation is the windsock deformity.

Type II: The atretic ends of the duodenum are connected by a fibrous cord.

Type III: Complete separation of the atretic segments. Most biliary duct anomalies are associated with this type.

DiagnosisDiagnosis Prenatally Prenatally

• Diagnosed in 32-57% of patientsDiagnosed in 32-57% of patients• Dilated stomach bubble apparent by 3Dilated stomach bubble apparent by 3rdrd

trimestertrimester• Polyhydramnios in 32-59% of casesPolyhydramnios in 32-59% of cases

PostnatallyPostnatally• Symptoms usually appear within the first 24 Symptoms usually appear within the first 24

hourshours• Recognition of partial obstruction can be Recognition of partial obstruction can be

delayeddelayed• Repeated bilious emesis is characteristic – 85%Repeated bilious emesis is characteristic – 85%• Bilious emesis is a surgical emergency Bilious emesis is a surgical emergency

until proven otherwiseuntil proven otherwise• Nonbilious emesis is present when the atresia Nonbilious emesis is present when the atresia

is above the level where the bile duct enters is above the level where the bile duct enters the duodenum (papilla of Vater)the duodenum (papilla of Vater)

DiagnosisDiagnosis Radiologic studiesRadiologic studies

Plain radiograph of the abdomen will generally confirm the diagnosis with a finding of the “double bubble sign

Upper GI series or barium enema may be helpful to differentiate from midgut volvulus

ManagementManagement Replogle tube (10 Fr)Replogle tube (10 Fr) Intravenous fluid resuscitationIntravenous fluid resuscitation Electrolyte correctionElectrolyte correction PICC line may be consideredPICC line may be considered If midgut volvulus has been ruled out, surgical If midgut volvulus has been ruled out, surgical

correction is not urgentcorrection is not urgent Surgery performed is a duodenoduodenostomy Surgery performed is a duodenoduodenostomy

or duodenojejunostomy or duodenojejunostomy Can be performed laparoscopicallyCan be performed laparoscopically

Post-operative ManagementPost-operative Management NPONPO Replogle tube (10 Fr)Replogle tube (10 Fr) IV fluids /hyperalimentationIV fluids /hyperalimentation No feeds until return of bowel functionNo feeds until return of bowel function

ComplicationsComplications Early postoperative complications can Early postoperative complications can

be related to:be related to:• PrematurityPrematurity• Coexisting congenital anomaliesCoexisting congenital anomalies• Parenteral nutritionParenteral nutrition

Anastomotic obstruction/stenosisAnastomotic obstruction/stenosis Anastomotic leakAnastomotic leak AdhesionsAdhesions Prolonged feeding intoleranceProlonged feeding intolerance Duodenal dysmotilityDuodenal dysmotility

OutcomesOutcomes Current survival rates are 90-95%Current survival rates are 90-95% Higher mortality rates are associated Higher mortality rates are associated

with prematurity and multiple with prematurity and multiple congenital anomaliescongenital anomalies

Postoperative complications are Postoperative complications are reported to be 14-18%reported to be 14-18%

Jejunoileal AtresiaJejunoileal Atresia IncidenceIncidence

Has been reported to be as high as 1/3000 live Has been reported to be as high as 1/3000 live births in the USbirths in the US

Wide variation in the reported incidenceWide variation in the reported incidence Males = femalesMales = females 1/3 to ¼ of infants are low birth weight1/3 to ¼ of infants are low birth weight Higher incidence in African-American infantsHigher incidence in African-American infants Increased risk with maternal use of Increased risk with maternal use of

pseudoephedrine alone and in combination pseudoephedrine alone and in combination with acetaminophenwith acetaminophen

Increased in mothers with migraine headaches Increased in mothers with migraine headaches receiving ergotamine tartrate and caffeinereceiving ergotamine tartrate and caffeine

EtiologyEtiology Cause is unknownCause is unknown Most likely associated with a late Most likely associated with a late

intrauterine mesenteric vascular intrauterine mesenteric vascular catastrophecatastrophe

Has been associated with volvulus, Has been associated with volvulus, intussusception, internal hernia and intussusception, internal hernia and constriction of the mesentery in a tight constriction of the mesentery in a tight gastroschisis or omphalocelegastroschisis or omphalocele

Associated anomaliesAssociated anomalies Gastroschisis/OmphaloceleGastroschisis/Omphalocele AscitesAscites Cystic fibrosisCystic fibrosis Malrotation and volvulusMalrotation and volvulus GenitourinaryGenitourinary

PathologyPathology Equally distributed between the Equally distributed between the

jejunum (51%) and the ileum (49%)jejunum (51%) and the ileum (49%) Atresia is usually single (>90%) Atresia is usually single (>90%) Multiple atresias more often involve the Multiple atresias more often involve the

proximal jejunumproximal jejunum Currently 5 classificationsCurrently 5 classifications

Type I: Mucosal atresia with Type I: Mucosal atresia with intact bowel and intact bowel and meseneterymesenetery

TypeII: Blind ends separated TypeII: Blind ends separated by a fibrous cordby a fibrous cord

Type III(a): Blind ends Type III(a): Blind ends separated by a V-shape separated by a V-shape mesenteric defectmesenteric defect

Type III(b): Apple-peel Type III(b): Apple-peel atresiaatresia

Type IV: Multiple atresias Type IV: Multiple atresias (string of sausages)(string of sausages)

DiagnosisDiagnosis Clinical SignsClinical Signs

• Polyhydramnios - more commonly seen in Polyhydramnios - more commonly seen in proximal atresiasproximal atresias

• Bilious emesis – Bilious emesis – SURGICAL EMERGENCY SURGICAL EMERGENCY UNTIL PROVEN OTHERWISEUNTIL PROVEN OTHERWISE

• Abdominal distensionAbdominal distension• JaundiceJaundice• Failure to pass meconiumFailure to pass meconium

Radiologic StudiesRadiologic Studies• Supine and erect abdominal filmsSupine and erect abdominal films• Contrast enema or UGIContrast enema or UGI

ManagementManagement Replogle tube (10 Fr) to continuous suctionReplogle tube (10 Fr) to continuous suction Intravenous fluidsIntravenous fluids Electrolyte correctionElectrolyte correction PICC line placementPICC line placement Operative procedure depends on defectOperative procedure depends on defect

• May require multiple anastomoses May require multiple anastomoses • May require ostomyMay require ostomy• May require tapering of proximally May require tapering of proximally

dilated intestinedilated intestine

Postoperative ManagementPostoperative Management IV fluid resuscitationIV fluid resuscitation NPONPO Replogle (10 Fr) to suctionReplogle (10 Fr) to suction Clear liquids with return of bowel functionClear liquids with return of bowel function Advance to formula- medium chain triglyceride Advance to formula- medium chain triglyceride

or casein hydrolysate formulas should be or casein hydrolysate formulas should be offeredoffered

Malabsorption and diarrhea are common in Malabsorption and diarrhea are common in infants: infants:

• With short bowel lengthWith short bowel length• In whom the ileocecal valve has been resectedIn whom the ileocecal valve has been resected• With multiple atresiasWith multiple atresias• Apple peel atresiaApple peel atresia

OutcomesOutcomes Overall survival rates for jejunoileal Overall survival rates for jejunoileal

atresia are reported to range from 80-atresia are reported to range from 80-90%90%

Most common cause of early deathMost common cause of early death• PneumoniaPneumonia• PeritonitisPeritonitis• SepsisSepsis

Postoperative complicationsPostoperative complications• Intestinal obstruction at the anastomosisIntestinal obstruction at the anastomosis• Anastomotic leakAnastomotic leak

OutcomesOutcomes Factors affecting morbidity and Factors affecting morbidity and

mortalitymortality• Associated anomaliesAssociated anomalies• Short bowel syndromeShort bowel syndrome• PrematurityPrematurity• Respiratory distressRespiratory distress

Meconium IleusMeconium Ileus

IncidenceIncidence Almost always associated with cystic Almost always associated with cystic

fibrosisfibrosis Reported to be the presenting symptom Reported to be the presenting symptom

in 15-20% of casesin 15-20% of cases Incidence is reported to be 30-40% in Incidence is reported to be 30-40% in

all patients with cystic fibrosisall patients with cystic fibrosis

PathologyPathology Simple meconium ileusSimple meconium ileus

• Small intestine mucous glands Small intestine mucous glands produce overly thick secretionsproduce overly thick secretions

• Meconium formed is abnormally Meconium formed is abnormally viscid, sticky and adherentviscid, sticky and adherent

• Proximal ileum dilated with thick, Proximal ileum dilated with thick, sticky meconiumsticky meconium

• Distal ileum and colon are collapsed Distal ileum and colon are collapsed and obstructed by thickly packed and obstructed by thickly packed round mucous plugs – rabbit pelletsround mucous plugs – rabbit pellets

Complicated meconium ileusComplicated meconium ileus

• In utero dilated proximal intestine In utero dilated proximal intestine volvulizesvolvulizes

• Early in gestation may cause one or Early in gestation may cause one or more atresiasmore atresias

• Late in gestation infants may present Late in gestation infants may present with perforation -> meconium with perforation -> meconium peritonitis peritonitis

Associated AnomaliesAssociated Anomalies Cystic fibrosisCystic fibrosis

DiagnosisDiagnosis PrenatallyPrenatally

• Prenatal history of cystic fibrosisPrenatal history of cystic fibrosis• Polyhydramnios is reported in ~20% of mothers – Polyhydramnios is reported in ~20% of mothers –

more common finding in complicated meconium ileusmore common finding in complicated meconium ileus PostnatallyPostnatally

• Intestinal obstruction is evident 24-48 hours after Intestinal obstruction is evident 24-48 hours after birthbirth

-> abdominal distention-> abdominal distention

-> bilious emesis-> bilious emesis

-> failure to pass meconium within 48 hours-> failure to pass meconium within 48 hours• Complicated meconium ileus presents at or shortly Complicated meconium ileus presents at or shortly

after birthafter birth

-> severe abdominal distension-> severe abdominal distension

-> abdominal wall may be red and inflamed-> abdominal wall may be red and inflamed

-> extremely ill appearing infant-> extremely ill appearing infant

Radiologic Studies – Simple MIRadiologic Studies – Simple MI

•Plain radiographsPlain radiographsVarying sized Varying sized loops of distended loops of distended bowelbowel

Absence of air Absence of air fluid levelsfluid levels

Soap bubble Soap bubble appearance appearance particularly in the particularly in the right lower right lower quadrantquadrant

• Contrast EnemaContrast Enema

Water soluble contrast

Microcolon

Pellet-like meconium when contrast is refluxed into the terminal ileum

Curative in 30% of patients

Radiologic studies – Complicated MIRadiologic studies – Complicated MI• Plain filmPlain film

Areas of calcification (calcified meconium)Areas of calcification (calcified meconium) Large dense mass with a rim of calcification Large dense mass with a rim of calcification

– cystic meconium peritonitis– cystic meconium peritonitis Intraperitoneal calcifications can occur Intraperitoneal calcifications can occur

within 4 days of perforationwithin 4 days of perforation

• Contrast enema may not be necessaryContrast enema may not be necessary

Management – Simple MIManagement – Simple MI Aggressive fluid resuscitation Aggressive fluid resuscitation Naso/orogastric tube to suctionNaso/orogastric tube to suction Hyperosmolar contrast enema Hyperosmolar contrast enema

(Gastrografin)(Gastrografin)• May be curative in 30-60% of patientsMay be curative in 30-60% of patients

Goal of surgery is to completely Goal of surgery is to completely evacuate the obstructing plugs and evacuate the obstructing plugs and meconiummeconium

• Enterotomy and irrigationEnterotomy and irrigation• Intestinal resection and primary Intestinal resection and primary

anastomosisanastomosis• Ostomy may be necessaryOstomy may be necessary

Management – Complictated MIManagement – Complictated MI• Typically a much sicker infantTypically a much sicker infant• Aggressive fluid resuscitationAggressive fluid resuscitation• Electrolyte correctionElectrolyte correction• Findings at surgery dictate the Findings at surgery dictate the

procedureprocedure• More likely to have an ostomyMore likely to have an ostomy

OutcomesOutcomes Current 5 year survival is approaching 100%Current 5 year survival is approaching 100% 72% survival at 10 years72% survival at 10 years Current operative mortality is reported to be Current operative mortality is reported to be

10-20%10-20% Current life expectancy for patients with cystic Current life expectancy for patients with cystic

fibrosis is 35 yearsfibrosis is 35 years Complications are related to the CFComplications are related to the CF Most common gastrointestinal problem in Most common gastrointestinal problem in

patients with CF is distal intestinal obstruction patients with CF is distal intestinal obstruction syndromesyndrome

¼ of patients will develop gallstones¼ of patients will develop gallstones Intussusception occurs in 1%Intussusception occurs in 1% Rectal prolapse develops in 1/3 to 1/5 of Rectal prolapse develops in 1/3 to 1/5 of

patientspatients Colonic strictures secondary to high dose oral Colonic strictures secondary to high dose oral

enzyme therapyenzyme therapy

Intestinal Malrotation and Intestinal Malrotation and VolvulusVolvulus

IncidenceIncidence Occurs in 1/500 live birthsOccurs in 1/500 live births Male to female ration is 2:1 in neonatal Male to female ration is 2:1 in neonatal

presentationpresentation No sexual predilection in patients over 1 No sexual predilection in patients over 1

yearyear As many as 40% present within the first As many as 40% present within the first

weekweek 50% present by 1 month50% present by 1 month 75% present by 1 year75% present by 1 year

EtiologyEtiology Rotation and fixation of the intestine Rotation and fixation of the intestine

takes place during the first 3 months of takes place during the first 3 months of gestationgestation

As rotation begins, the intestine moves As rotation begins, the intestine moves outside the abdomenoutside the abdomen

At 10 weeks of gestastional age the At 10 weeks of gestastional age the intestine returns to the abdomenintestine returns to the abdomen

Normal mesenteric attachment extends Normal mesenteric attachment extends from the ligament of Treitz to the cecumfrom the ligament of Treitz to the cecum

Ascending and descending colon are Ascending and descending colon are fixed retroperitoneally fixed retroperitoneally

EtiologyEtiology Malrotation is when the normal process Malrotation is when the normal process

of rotation is arrested or deviated at of rotation is arrested or deviated at various stagesvarious stages

Anomalous fixation may also occurAnomalous fixation may also occur Dense fibrous bands extending from the Dense fibrous bands extending from the

cecum and right colon across the cecum and right colon across the duodenum to the retroperitoneum may duodenum to the retroperitoneum may form – Ladd’s Bandsform – Ladd’s Bands

PathologyPathology Midgut may be supported by a narrow pedicle Midgut may be supported by a narrow pedicle

that contains the entire blood supplythat contains the entire blood supply Narrow pedicle predisposes the bowel to a Narrow pedicle predisposes the bowel to a

clockwise twisting from the duodenum to the clockwise twisting from the duodenum to the transverse colontransverse colon

Distension and peristalsis may initiate torsion Distension and peristalsis may initiate torsion of the intestine on the pedicle - Volvulusof the intestine on the pedicle - Volvulus

Acute midgut volvulus occurs when the blood Acute midgut volvulus occurs when the blood supply to the midgut is disrupted by the torsionsupply to the midgut is disrupted by the torsion

Vascular obstruction and necrosis of the Vascular obstruction and necrosis of the complete midgut develops rapidlycomplete midgut develops rapidly

Ladd’s Bands can cause a mechanical duodenal Ladd’s Bands can cause a mechanical duodenal obstruction without vascular compromiseobstruction without vascular compromise

Associated AnomaliesAssociated Anomalies Are found in 30 to 60% of patientsAre found in 30 to 60% of patients Malrotation is almost always associated Malrotation is almost always associated

withwith• Diaphragmatic herniaDiaphragmatic hernia• Omphalocele/gastroschisisOmphalocele/gastroschisis• Prune belly syndromePrune belly syndrome

Can be associated withCan be associated with• Duodenal atresiaDuodenal atresia• Ileal atresiaIleal atresia• Meconium ileusMeconium ileus• Congenital heart diseaseCongenital heart disease• Imperforate anusImperforate anus• Annular pancreasAnnular pancreas• Biliary atresia Biliary atresia

DiagnosisDiagnosis Clinical symptomsClinical symptoms

• Most frequent symptom is Most frequent symptom is bilious emesisbilious emesis• Pain and irritability in the toddler or older childPain and irritability in the toddler or older child• Abdomen is soft and non-tender to palpation Abdomen is soft and non-tender to palpation

initiallyinitially• Becomes distended and tender with Becomes distended and tender with

strangulation of bowelstrangulation of bowel• Stool may be bloodstainedStool may be bloodstained

Radiologic studiesRadiologic studies• Abdominal radiographs may show a dilated Abdominal radiographs may show a dilated

duodenem with a fluid level, however can be duodenem with a fluid level, however can be read as normal in 20% of casesread as normal in 20% of cases

• Barium enema is unreliable – position of cecum Barium enema is unreliable – position of cecum variesvaries

DiagnosisDiagnosis Radiologic studiesRadiologic studies• Upper GI series with small bowel follow Upper GI series with small bowel follow

through is the most reliable – 96% through is the most reliable – 96% specificity in one reportspecificity in one report Dilated duodenum with a typical corkscrew Dilated duodenum with a typical corkscrew

appearanceappearance Absence of Ligament of TreitzAbsence of Ligament of Treitz Small bowel on right side of abdomenSmall bowel on right side of abdomen UGI may be misleading if duodenal UGI may be misleading if duodenal

obstruction is complete (also seen with obstruction is complete (also seen with duodenal atresia)duodenal atresia)

ManagementManagement Once the diagnosis is made on a Once the diagnosis is made on a

symptomatic child, the patient should symptomatic child, the patient should be taken to the operating room be taken to the operating room immediatelyimmediately

Ladd’s procedure is performedLadd’s procedure is performed• Derotation of the bowel Derotation of the bowel • Division of the Ladd’s bands if presentDivision of the Ladd’s bands if present• Widening the small bowel mesentary by Widening the small bowel mesentary by

lysis of congenital adhesionslysis of congenital adhesions• Return the bowel to a position of Return the bowel to a position of

nonrotationnonrotation• Appendectomy Appendectomy

ManagementManagement If ischemic bowel is present every If ischemic bowel is present every

attempt to preserve bowel length attempt to preserve bowel length should be madeshould be made

Bowel is not surgically fixed into Bowel is not surgically fixed into positionposition

Procedure may also be done Procedure may also be done laparoscopicallylaparoscopically

OutcomesOutcomes Mortality rate with midgut volvulus is at Mortality rate with midgut volvulus is at

least 15%least 15% Return of bowel function is dependent Return of bowel function is dependent

upon duration of obstruction and extent upon duration of obstruction and extent of compromiseof compromise

High incidence of short gut syndrome High incidence of short gut syndrome Recurrent obstructions are rareRecurrent obstructions are rare Higher incidence of intussusception Higher incidence of intussusception

post op than with laparotomies for post op than with laparotomies for other reasons ~3%other reasons ~3%

Hirschsprung’s DiseaseHirschsprung’s Disease

IncidenceIncidence Ranges from 1/4400 to 1/7000 live birthsRanges from 1/4400 to 1/7000 live births Male to female ratio 4:1Male to female ratio 4:1 Long segment disease ratio approaches 1:1 Long segment disease ratio approaches 1:1

but may actually favor femalesbut may actually favor females Incidence increases to 6% for siblings of Incidence increases to 6% for siblings of

affected childrenaffected children Brothers and sons of affected females have Brothers and sons of affected females have

the greatest risk (24% and 29%, the greatest risk (24% and 29%, respectively)respectively)

Etiology Etiology Cause of Hirschsprung’s Disease is Cause of Hirschsprung’s Disease is

unknownunknown Features of the disease are suggestive Features of the disease are suggestive

of a complex pattern of inheritanceof a complex pattern of inheritance RET RET gene mutation was found in~ 35% gene mutation was found in~ 35%

of sporadic cases and 49% of familial of sporadic cases and 49% of familial casescases

Associated AnomaliesAssociated Anomalies Trisomy 21 has been reported in 4-16% Trisomy 21 has been reported in 4-16%

of casesof cases Large and small bowel atresiaLarge and small bowel atresia Anorectal atresiaAnorectal atresia Ondine’s curseOndine’s curse

PathologyAbsence of ganglion cells in the distal intestine is the hallmark of the diseaseGanglion cells are absent in the submucosal plexus and intermuscular plexusRectosigmoid region in ~80% of casesAganglionosis is almost always continuous distallyLong segment disease has been reported 11-26% of casesTotal colonic 3-12%Small bowel 0.4-3%

DiagnosisClinical findings

•Delayed passage of meconium within the first 48 hours of life•95 % of full term infants stool within the first 24 hours•Remainder will stool by 48 hours•This history may be absent in 6 to 42% of patients•Constipation•Abdominal distension•Failure to thrive•Hirschsprung’s Disease should be considered in any child who has a history of constipation dating back to the newborn period

DiagnosisRadiologic studies

•Supine and positional abdominal radiographs - distended loops with paucity of air in the rectum•Contrast Enema with water soluble contrast – classic finding is a narrow spastic distal segment with a dilated proximal segment•Point of caliber change or transition zone is the key radiographic finding•Diagnostic accuracy of contrast enema has been reported to be 76% to 92%•In any infant with a suspected diagnosis of Hirschspung’s Disease no rectal stimulation of any kind should be performed

Diagnosis•Rectal biopsy is the gold standard•May be done at the bedside•Accuracy of suction rectal biopsy has been reported as high as 99.7%•Major problem is inadequate specimen•Full thickness rectal biopsy may be preformed in the operating room

ManagementPrimary pull-through procedureTransanal approach for normal segment diseaseIntraoperative biopsies are performed to confirm the transition zoneProcedure may be performed with laparoscopic assistanceOlder children may require a colostomy first with the definitive procedure performed several weeks later

Outcome

Early Complications

•Intestinal obstruction 8-13%

•Wound infection 10-15%

•Anorectal stenosis 10-20%

•Anastomotic leak 2%

Late Complications

•Incontinence 3-8% - greatest impact on quality of life

•Constipation reported to range from 6-30%

•Hirschsprung’s enterocolitis

Major cause of morbidity and mortality

Incidence ranges from 20-30%

Cause is unknown but some feel it is related to persistent state of colonic and small bowel stasis which leads to bacterial overgrowth

Outcome

Hirschsprung’s enterocolitis

Manifested by explosive diarrhea, abdominal distension and fever

Treatment is with aggressive washout with a rectal tube, IV antibiotics or oral metronidazole

Quality of life is reasonably good and significantly better when compared to patients with other anorectal malformations

Imperforate Anus Incidence

Average incidence worldwide is believed to be 1 in 5000 live births

There is a slight male preponderance Males are twice as likely to have high

imperforate anus Some families have a genetic predisposition Most common in females – rectovestibular fistula Most common in males – rectourethral fistula Imperforate anus without fistula 5% of patients

and half of these patients have Down’s syndrome Cause is unknown

Associated Anomalies

VACTERL syndrome

50-60% of patients will have one or more anomalies

~ 1/3 will have congenital heart disease

•ASD

•PDA

•Tetrology of Fallot

•VSD

Gastrointestinal

•Tracheoesophageal abnormalities 10%

•Duodenal obstruction 1-2%

Associated Anomalies

Spinal and vertebral

•1/3 to ½ of patients

•Tethered cord is most common

•Spinal lipomas

•Syringomelia

•Myelomemingocele

•Hemivertebrae

•Scoliosis

•Butterfly vertebrae

•Hemisacrum

Associated Anomalies

Genitourinary

•Incidence ranges from 1/3 to 1/2 of patients

•Incidence increases with increasing complexity of malformation

•Vesicoureteral reflux is most common

•Renal agnenesis and dysplasia

•Cryptochidism 3-19%

•Hypospadias 5%

Gynecologic

•Hydrocolpos in the newborn period

•Mullarian anomalies leading to menstrual difficulties

•Uterine abnormalities

•Vaginal anomalies

Pathology

Deformities are divided into high, intermediate and low

Classification depends on how far the rectum is descended into the pelvis and whether there is a fistula

Patients will have varying degrees of striated muscle development from normal appearing to no muscle at all

Majority of patients are born without an anal canal and, consequently, can not discriminate between liquid and gas after surgical repair

Patients will have a spectrum of rectosigmoid motility disorders which can lead to constipation issues

Diagnosis

All suspected cases of anorectal malformation require a thorough perineal examination

Infants should be given sufficient time to pass meconium

Two positional radiographs may be helpful

Invertograms are not useful

Figure 341-3 Imperforate anus in females. A, Vestibular fistula. B, Cloaca. (From Peña A: Atlas of Surgical Management of Anorectal Malformations. Springer-Verlag, New York, 1989, pp 50, 60.)

Figure 341-2 Imperforate anus in males. A, Low lesions. B, High lesions. (From Peña A: Atlas of Surgical Management of Anorectal Malformations. Springer-Verlag, New York, 1989, pp 7, 26.)

Management

IV fluids

Replogle tube (10 Fr)

Workup for VATER syndrome

•Echocardiogram

•Dedicated spinal films/spinal ultrasound

•Renal ultrasound

Newborn Female

Perineal Inspection

Single perineal orifice

Cloaca

Colostomy

Perineal fistula

Anoplasty

or

Dilatations

Vestibular fistula

Primary repair

No visible fistula

Rectum below coccyx

High rectum

Colostomy

Adapted from Pena, A, Levitt, M: Anorectal Malformations. In Grossfeld, J, O’Neill, J, et al (ed) Pediatric Surgery. 6th Ed.

Newborn Male

Perineal Inspection

Perineal fistula

Anoplasty

Rectal gas below coccyx

No associated anomalies

PSARP +/- colostomy

Rectal gas above coccyx

Associated defects

Abnormal sacrum

Flat bottom

Colostomy

Adapted from Pena, A, Levitt, M: Anorectal Malformations. In Grossfeld, J, O’Neill, J, et al (ed) Pediatric Surgery. 6th Ed.

Management

Operative repair

•Anoplasty for perineal fistulas

•Posterior Sagittal Anorectoplasty - PSARP

Electrical stimulation of the perineum to evaluate sphincter contraction

Midline sagittal incision dividing the sphincter mechanism into 2 equal parts

Important nerves and vessels do not cross the midline

•Colostomy with delayed PSARP for high imperforate anus

Management

Postoperatively

•Oral feeding may begin when the infant is awake enough

•Antibiotics for at least 24 hours

•Rectourethral fistula – foley cathether for 7 days

•Rectal dilation program is begun ~ 2 weeks post op

•Colostomy, if present, is closed when anal caliber is adequate (~12Fr Hegar dilator in a 1-4 month old)

•Diligent perianal care – protective barrier cream

•Multiple stools initially

OutcomesComplications

•Wound infections and dehiscence can be catastrophic•Rectal strictures•Vaginal strictures•Rectal mucosal prolapse•Urologic injuries in males•Constipation -> megarectosigmoid -> overflow pseudoincontinence

Studies suggest that 75% of patients achieve good bowel control and continence

Outcomes

Incontinence is generally divided into 2 groups

1. Constipation – aggressive bowel irrigation is suggested

2. Increased motility leading to diarrhea – constipating diet, medication to

decrease bowel motility and colonic irrigation is suggested

May require a bowel management program to achieve continence

Appendicostomy for retrograde enemas may be helpful

Late toilet training

Abdominal Wall DefectsOmphalocele

Incidence 1-2.5 per 5000 live births Male preponderance Second most common abdominal wall

defect

EtiologyCause is unknownRare reports occurring in familiesNo specific gene has been identified

Associated Anomalies

30-70% will have associated anomalies

Congenital heart disease – up to 45%

•VSD

•ASD

•Ectopia Cordis

•Tricuspid Atresia

•Coarctation of the Aorta

•Persistent Pulmonary Hypertension of the Newborn

Chromosomal Abnormalities – up to 40%

•Beckwith-Wiedemann syndrome

•Down’s Syndrome

•Trisomy 13-15

•Trisomy 16-18

Associated Anomalies

Musculoskelatal abnormalities

Neural tube defects

GERD – up to 43% in one study

Cryptorchidism – 33%

Macrosomia

Pathology

Central abdominal wall defect

Usually greater than 4cm in diameter

Covered by a translucent sac unless ruptured inutero

Muscles of the abdominal wall are normal

Sac usually contains the liver, midgut and frequently the spleen and gonad

All have malrotation

Infants are usually full term

Diagnosis

Usually antenatally by ultrasound

In a European study sensitivity of ultrasound was 75%

Usually detected at 18 weeks +/- 6 weeks

Management

May be delivered vaginally

Placed on mechanical ventilation

Replogle (10 Fr) tube should be placed

Intravenous fluids

Prophylactic antibiotics

Cardiology evaluation and echocardiogram – this should not delay surgery

Surgery

•Small defects may be closed primarily

•More likely a staged procedure with the placement of a silo

Silo is reduced daily if tolerated at the bedside – ideally reduced in 7 days

Operating room for final closure

Contents of omphalocele after sac is removed

Management

Post Operative Care

•IV fluids to maintain adequate urine output

•Wean ventilator after final closure

•Replogle tube (10 Fr)

•NPO until return of bowel function

•Return of bowel function may be delayed

•TPN

Outcomes

Post operative complications

•Hypovolemia – very high fluid requirements

•Tight closure -> ventilatory compromise, decreased venous return and low cardiac output, oliguria

•Metabolic acidosis as a result of kinking of the hepatic veins from reduction of the liver

Survival rates range from 70-95%

Mortality is often related to associated anomalies

Abdominal Wall DefectsGastroschisis

Incidence 2-4.9 per 10,000 live births Male preponderance Most common abdominal wall defect

Etiology

Cause is unknown, but may be related to fetal distress

Infants are more likely to be premature

Term babies are more likely to be SGA

Associated Anomalies

Much less common than with omphalocele

Intestinal atresia may be seen in 10-15%

GERD ~ 16%

Undescended testicle ~15%

Malrotation is always present

Pathology

Small abdominal wall defect: usually 2-4cm

Occurs adjacent to the umbilicus almost always to the right

Abdominal muscles are normal

There is no sac

Midgut is herniated through the defect

Matted bowel loops may be present at birth

Exposed bowel may appear normal at birth but quickly becomes matted with fibrinous exudate such that individual loops can not be distinguished

Diagnosis

Antenatally by ultrasound

Sensitivity has been reported to be 83%

Detected at 20 weeks +/- 7 weeks

Management

May be delivered vaginally

Cover exposed bowel with warm saline moistened sterile dressing

Replogle tube (10 Fr)

Support of the premature infant

Monitor for fluid and heat loss

Should be taken to the operating room ASAP

The faster the bowel can be reduced the more likely primary closure can be achieved

If reduction and primary closure can not be achieved a silo is placed

Post operatively requires adequate fluid resuscitation

Management

Post operatively

•Wean ventilator after final closure

•Replogle tube (10 Fr)

•NPO until return of bowel function

•May be more prolonged than with omphalocele

•TPN

Outcomes

Post operative complications

•Hypovolemia

•Closure too tight -> ventilatory compromise, decreased venous return and low cardiac output, oliguria

Survival rate is ~90%

Complications related to prematurity responsible for most morbidity and mortality

Primary fascial closure has been reported in up to 80%; some recommend silos in all cases

Median time to feeding ~30 days with a range reported of 5-60 days

Atresias are repaired several weeks after intestinal reduction and fascial closure

Associated atresia may require more time to feeds

In Summary

BILIOUS EMESIS IS VOLVULUS UNTIL PROVEN OTHERWISE

History and plain films will guide sequence of further studies

Remember the associated anomalies

References

Boloker, J., Bateman, D., Wung, J., Stolar, C. Congenital Diaphragmatic Hernia in 120 Infants Treated Conservatively With Permissive Hypercapnea/Spontaneous Respiration/Elective Repair. Journal of Pediatric Surgery 2002; 37: 357-366

Dalla Vechia, L.K.,MD; Grosfeld, J.L., MD; West, K,. MD; Rescoria, F., MD; Scherer, L., MD; Engum, S., MD. Intestinal Atresia and Stenosis. Arch Surg. 1998;133:490-497

Grosfeld, J., MD; O’Neill, J., MD; Fonkalsrud, E. MD; Coran, A., MD (eds). Pediatric Surgery. 6th ed. Philadelphia, PA: Mosby-Elsevier; 2006.

Lewis, N., MBBS, FRCS; Glick, P., MD, MBA. Duodenal Atresia and Stenosis, Surgical Perspective. eMedicine from WebMD. 2010. http://emedicine.medscape.com/article/935748

Millar, A., Rode, H., Cywes, S. Malrotation and Volvulus in Infancy and Childhood. Seminars in Pediatric Surgery 2003;12: 229-236.

Parish, A., MD, Hately, R., MD. Intestinal Malrotation. Emedicine 2010. http://emedicine.medscape.com/article/930313

Reid, J., MD. Midgut Volvulus. Emedicine 2010. http://emedicine.medscape.com/article/411249-overview

Rowe, M., O’Neill, J., Grosfeld, J., Fonkalsrud, E., Coran, A. (eds). Essentials of Pediatric Surgery. St. Louis, MI: Mosby-Year Book, Inc.; 1995.

University of Michigan Department of Surgery. Meconium Ileus. http://surgery.med.umich.edu/pediatric/clinical/physician_content/a-m/meconium_ileus.shtml