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Complications of Neonatal Surgical Cases: NEC and Birth Defects Dr. Phillip Pan, MD Neonatologist Bellevue Woman’s Center, a service of Ellis Medicine.

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Presentation on theme: "Complications of Neonatal Surgical Cases: NEC and Birth Defects Dr. Phillip Pan, MD Neonatologist Bellevue Woman’s Center, a service of Ellis Medicine."— Presentation transcript:

1 Complications of Neonatal Surgical Cases: NEC and Birth Defects Dr. Phillip Pan, MD Neonatologist Bellevue Woman’s Center, a service of Ellis Medicine Albany Medical Center

2 Complications of Neonatal Surgical Cases: Objectives Necrotizing EnterocolitisNecrotizing Enterocolitis Birth DefectsBirth Defects Tracheoesophageal FistulaTracheoesophageal Fistula MyelomeningoceleMyelomeningocele Abdominal wall defects (gastroschisis, omphalocele)Abdominal wall defects (gastroschisis, omphalocele) Hydrocephalus (congenital, post-hemorrhagic)Hydrocephalus (congenital, post-hemorrhagic) Gastrostomy tubeGastrostomy tube

3 Necrotizing Enterocolitis Characterized by injury to the intestines with variable degrees of intestinal ischemia, usually including the terminal ileumCharacterized by injury to the intestines with variable degrees of intestinal ischemia, usually including the terminal ileum Gas develops within the muscular and submucosal layers and may be seen as pneumatosis intestinalisGas develops within the muscular and submucosal layers and may be seen as pneumatosis intestinalis When full-thickness necrosis occurs, perforation and peritonitis developWhen full-thickness necrosis occurs, perforation and peritonitis develop

4 Characteristics 1-5% of all NICU admissions1-5% of all NICU admissions usually onset between 3 rd - 10 th day of lifeusually onset between 3 rd - 10 th day of life no association between sex, race, season or socioeconomic statusno association between sex, race, season or socioeconomic status predominantly affects premature infants, but about 10% of those affected are term infantspredominantly affects premature infants, but about 10% of those affected are term infants mortality is between 30-40%mortality is between 30-40%

5 Theories Variable theories – infection, inflammation, ischemia/reperfusion injuryVariable theories – infection, inflammation, ischemia/reperfusion injury Probably multifactorial and requires interaction of immaturity, previous gastrointestinal ischemia, enteral feeding and bacterial invasionProbably multifactorial and requires interaction of immaturity, previous gastrointestinal ischemia, enteral feeding and bacterial invasion Contradicting the theory is NEC occurs in infantsContradicting the theory is NEC occurs in infants with no identifiable risk factors,with no identifiable risk factors, who were never fed enterally, andwho were never fed enterally, and among otherwise healthy full-term infantsamong otherwise healthy full-term infants

6 Bell Clinical Staging Stage I – Suspected NEC Stage I – Suspected NEC Temp instability, apnea, gastric residuals, abdominal distension. No radiographic abnormalitiesTemp instability, apnea, gastric residuals, abdominal distension. No radiographic abnormalities NPO, antibiotics for 2-3 days pending cultureNPO, antibiotics for 2-3 days pending culture Stage II – Definite NEC Stage II – Definite NEC Also includes absent bowel sounds, abdominal tendernessAlso includes absent bowel sounds, abdominal tenderness Ileus, pneumatosis (see photo)Ileus, pneumatosis (see photo) NPO, antibiotics x14 daysNPO, antibiotics x14 days

7 Copyright ©2001 American Academy of Pediatrics Dimmitt, R. A. et al. Neoreviews 2001;2:110-e117 A plain anteroposterior radiograph demonstrating pneumatosis

8 Bell Clinical Staging Stage III – Advanced NEC hemodynamic instability, DIC, neutropenia, metabolic acidosis, respiratory compromisehemodynamic instability, DIC, neutropenia, metabolic acidosis, respiratory compromise generalized peritonitis, marked tenderness and distension of abdomengeneralized peritonitis, marked tenderness and distension of abdomen antibiotics, inotropes, assisted ventilationantibiotics, inotropes, assisted ventilation Includes bowel perforation, pneuomoperitoneumIncludes bowel perforation, pneuomoperitoneum

9 Copyright ©2001 American Academy of Pediatrics Dimmitt, R. A. et al. Neoreviews 2001;2:110-e117 An anteroposterior radiograph showing extensive pneumatosis and free air

10 Surgical Management Indications include pneumoperitoneum, persistent metabolic acidosis, rapidly worsening pulmonary status and unremitting neutropenia or thrombocytopeniaIndications include pneumoperitoneum, persistent metabolic acidosis, rapidly worsening pulmonary status and unremitting neutropenia or thrombocytopenia Emergently a peritoneal drain may be placedEmergently a peritoneal drain may be placed Staged exploratory laporatomies to identify ischemic, perforated, and necrotic bowelStaged exploratory laporatomies to identify ischemic, perforated, and necrotic bowel

11 Complications stricture formation occurs in 25-35% of survivorsstricture formation occurs in 25-35% of survivors “silent” strictures may be identified by barium enema“silent” strictures may be identified by barium enema late-onset intestinal obstruction following medical and/or surgical management includes adhesions, scarring and short bowel syndromelate-onset intestinal obstruction following medical and/or surgical management includes adhesions, scarring and short bowel syndrome neurodevelopmental delays occur in 50% of survivorsneurodevelopmental delays occur in 50% of survivors most serious intestinal complication is short bowel syndrome (25%)most serious intestinal complication is short bowel syndrome (25%)

12 Short Bowel Syndrome NEC is the leading cause of SBSNEC is the leading cause of SBS other causes include congenital malformationsother causes include congenital malformations More than 50% of small bowel must be removed before substantial malabsorption occursMore than 50% of small bowel must be removed before substantial malabsorption occurs normal lengths cm at 28 weeks vs 250 cm at termnormal lengths cm at 28 weeks vs 250 cm at term an important determinant of future function is presence of ileocecal valvean important determinant of future function is presence of ileocecal valve 20 cm is minimal length sufficient to allow dependence on TPN20 cm is minimal length sufficient to allow dependence on TPN following ileocolic anastomasis, painless rectal bleeding or melena from anastomotic ulcers can occur years after NEC surgeryfollowing ileocolic anastomasis, painless rectal bleeding or melena from anastomotic ulcers can occur years after NEC surgery

13 Prognosis prognosis of patients with short bowel syndrome is guardedprognosis of patients with short bowel syndrome is guarded infants are followed by the primary physician, pediatric gastroenterologist and surgeoninfants are followed by the primary physician, pediatric gastroenterologist and surgeon Some of these complications may be present at discharge from the NICU, and some require continued surveillanceSome of these complications may be present at discharge from the NICU, and some require continued surveillance Poor growth is a frequent outcomePoor growth is a frequent outcome Dumping syndrome is observed in infants with ostomies or severe diarrhea during GI infectionsDumping syndrome is observed in infants with ostomies or severe diarrhea during GI infections Dumping syndrome may cause rapid dehydration and electrolyte imbalanceDumping syndrome may cause rapid dehydration and electrolyte imbalance

14 Changing Pattern of Causes and Mortality of SBS Date of Report Study size Intestinal Anomalies : NEC (%) Mortality Rate(%) Reference : 032Wilmore : 29-Ziegler : 4412Sondheimer : 4330Andorsky : 2027Zuiros-Tejeira : 4411Salvia : 9650Cole

15 Bowel and Liver transplantation Boston Children’s HospitalBoston Children’s Hospital Children Hospital of PhiladelphiaChildren Hospital of Philadelphia New York cityNew York city liver transplantation - for 1 year following the date of transplantation liver transplantation - for 1 year following the date of transplantation

16 TPN-related cholestasis Characterized by direct hyperbilirubinemia, hepatomegaly and increased serum transaminasesCharacterized by direct hyperbilirubinemia, hepatomegaly and increased serum transaminases Risk factor includes prolonged enteral fastingRisk factor includes prolonged enteral fasting Treatment includes enteral nutrition, increasing bile acid production (actigall, phenobarbitol) and stopping TPNTreatment includes enteral nutrition, increasing bile acid production (actigall, phenobarbitol) and stopping TPN

17 Gastrostomy tube Anatomic, neurologic and developmental disordersAnatomic, neurologic and developmental disorders Memorable casesMemorable cases Congenital myotonic dystrophyCongenital myotonic dystrophy Pierre Robin sequencePierre Robin sequence Tracheo-esophageal fistulaTracheo-esophageal fistula Severe bronchopulmonary dysplasiaSevere bronchopulmonary dysplasia Need for supplemental daily enteral feeding via a g-tube for children < 3 years Need for supplemental daily enteral feeding via a g-tube for children < 3 years

18 Gastrostomy tube

19 Mickey button

20 G-tube for GER some children who have antireflux surgery also benefit from a G-tubesome children who have antireflux surgery also benefit from a G-tube G-tube should be considered in children with neurologic impairment, poor gastric motility, or poor nutritional statusG-tube should be considered in children with neurologic impairment, poor gastric motility, or poor nutritional status In addition to facilitating postoperative feeding, it allows drainage or venting of the stomachIn addition to facilitating postoperative feeding, it allows drainage or venting of the stomach

21 Birth Defects: outline Tracheoesophageal FistulaTracheoesophageal Fistula MyelomeningocelesMyelomeningoceles Abdominal wall defectsAbdominal wall defects GastroschisisGastroschisis OmphaloceleOmphalocele HydrocephalusHydrocephalus CongenitalCongenital Post-hemorrhagicPost-hemorrhagic

22 Birth Defects An abnormality of structure, function or metabolism that results in physical or mental disabilities or deathAn abnormality of structure, function or metabolism that results in physical or mental disabilities or death 3% of all children (120,000 babies) are born with a serious structural defect3% of all children (120,000 babies) are born with a serious structural defect The leading cause of death in the 1 st year of lifeThe leading cause of death in the 1 st year of life

23

24 Tracheoesophageal Fistula

25

26 Surgical Repair of TEF Surgical repair depends on presence of esophageal atresia, gap between proximal and distal esophagus ends, degree of respiratory compromiseSurgical repair depends on presence of esophageal atresia, gap between proximal and distal esophagus ends, degree of respiratory compromise Initial timing must consider aspiration of gastric contents into the lower lungs with urgent decompression and ligation of the distal TEFInitial timing must consider aspiration of gastric contents into the lower lungs with urgent decompression and ligation of the distal TEF May involve gastric pull-up, colonic graft, gastrostomy tubeMay involve gastric pull-up, colonic graft, gastrostomy tube

27 Complications Early – anastomotic leak, recurrent TEF and anastomotic stricturesEarly – anastomotic leak, recurrent TEF and anastomotic strictures Late – GER, esophageal dysmotility and tracheomalaciaLate – GER, esophageal dysmotility and tracheomalacia

28 Outcome and Prognosis Statistics are constantly changing and improvingStatistics are constantly changing and improving Mortality ranges from 7-63% and 0-11% depending on classification technique and reporting statisticsMortality ranges from 7-63% and 0-11% depending on classification technique and reporting statistics Fetuses with prenatal diagnoses of esophageal atresia have a worse prognosis with a mortality rate as high as 75% vs 21 % without esophageal atresiaFetuses with prenatal diagnoses of esophageal atresia have a worse prognosis with a mortality rate as high as 75% vs 21 % without esophageal atresia

29 Omphalocele vs gastroschisis OmphaloceleGastroschisis PathogenesisAbnormality during body folding Compromise of vascular supply EtiologySporadic. Increased assoc with genetic syndrome Sporadic. Increased assoc with young mothers Birth weight (< 5 th %ile)20%77% Mortality at 1 yr40%7.7% PEAGA infant, enclosed sac, may include liver and other organs SGA infant, exposed intestines.

30 Management of Open Ventral Wall Defects Multidisciplinary teamworkMultidisciplinary teamwork Protect exposed bowel/mucosaProtect exposed bowel/mucosa Prevent heat lossPrevent heat loss Prevent insensible water lossPrevent insensible water loss Prevent vascular compromisePrevent vascular compromise Nasogastric decompressionNasogastric decompression Broad-spectrum antibiotic coverageBroad-spectrum antibiotic coverage Total parenteral nutritionTotal parenteral nutrition Surgical correctionSurgical correction

31 Gastroschisis

32 Compromised bowel

33 Omphalocele

34 Large with wharton jelly

35 Bowel in silo

36

37 Neural Tube defects Neural tub defects affect about 3000 pregnancies each year in the USNeural tub defects affect about 3000 pregnancies each year in the US Risk may be reduced by taking at least 0.4 mg of vitamin B folic acid before and during early pregnancyRisk may be reduced by taking at least 0.4 mg of vitamin B folic acid before and during early pregnancy The neural tube folds inward to form a tube by the 28 th day after conceptionThe neural tube folds inward to form a tube by the 28 th day after conception Includes spina bifida, anencephaly, encephaloceleIncludes spina bifida, anencephaly, encephalocele

38 Spina Bifida The most common NTD, affect 1500 babies each year in the USThe most common NTD, affect 1500 babies each year in the US 3 classic forms3 classic forms Occulta – mildest form with usually no symptomsOcculta – mildest form with usually no symptoms Meningocele – rarest from with just cyst or fluid-filled sac without spinal nervesMeningocele – rarest from with just cyst or fluid-filled sac without spinal nerves MyelomeningoceleMyelomeningocele

39 Myelomeningocele both the membranes and nerve roots of the spinal cord are enclosed in cyst or fully exposedboth the membranes and nerve roots of the spinal cord are enclosed in cyst or fully exposed In spite of surgical repair, affect babies have some degree of leg paralysis, encoparesis and eneuresisIn spite of surgical repair, affect babies have some degree of leg paralysis, encoparesis and eneuresis

40

41 Hydranencephaly

42 Classic Dandy-Walker variant Melissa A. Parisia and William B. Dobyns. Human malformations of the midbrain and hindbrain: review and proposed classification scheme. Molecular Genetics and Metabolism. 80 (2003): 36–53.

43 Dandy-Walker Variants

44 Incidence of IVH % LBW infants in 1970’s % LBW infants in 1970’s % LBW infants in 1980’s % LBW infants in 1980’s Incidence correlates with degree of prematurityIncidence correlates with degree of prematurity Survival rates of smallest infants continue to increaseSurvival rates of smallest infants continue to increase

45 Risk of IVH Risk is inversely proportional to gestational ageRisk is inversely proportional to gestational age 35% of infants with birth weights of g have grade 3 or 4 IVH35% of infants with birth weights of g have grade 3 or 4 IVH If posthemorrhagic ventricular dilatation occurs after intraventricular hemorrhage does, it is usually apparent on cranial ultrasonography within 2-3 weeksIf posthemorrhagic ventricular dilatation occurs after intraventricular hemorrhage does, it is usually apparent on cranial ultrasonography within 2-3 weeks Infants who have had intracranial hemorrhage must always be monitored in neurodevelopmental follow-up clinicsInfants who have had intracranial hemorrhage must always be monitored in neurodevelopmental follow-up clinics

46 IVH: Grade I

47 IVH Pathology

48 IVH Pathology - Severe bleed

49 Outcomes for IVH ELBW preterm infants with grade I or II IVH may have poor neurodevelopmental outcomesELBW preterm infants with grade I or II IVH may have poor neurodevelopmental outcomes Grade III or grade IV IVH is associated with the least favorable neurodevelopmental results Grade III or grade IV IVH is associated with the least favorable neurodevelopmental results the degree of prematurity and the presence of chorioamnionitis may also be major contributors to severe long-term disabilitiesthe degree of prematurity and the presence of chorioamnionitis may also be major contributors to severe long-term disabilities IVH may lead to posthemorrhagic hydrocephalusIVH may lead to posthemorrhagic hydrocephalus

50 Post-hemorrhagic Ventricular Dilation 65% no progressive ventricular dilation65% no progressive ventricular dilation Majority of infants with slight/moderate IVHMajority of infants with slight/moderate IVH Minority of infants with severe IVHMinority of infants with severe IVH 35% slowly progressive ventricular dilation35% slowly progressive ventricular dilation 65% spontaneous arrest within 4 weeks with some or complete resolution65% spontaneous arrest within 4 weeks with some or complete resolution 30% continue to progress > 4 weeks, 1/3 of these become rapid30% continue to progress > 4 weeks, 1/3 of these become rapid 5% rapidly progress < 4 weeks5% rapidly progress < 4 weeks

51 Outcomes for PHH/PVL parenchymal hemorrhage causes cerebral infarction and can lead to cerebral or cerebellar porencephalyparenchymal hemorrhage causes cerebral infarction and can lead to cerebral or cerebellar porencephaly Porencephaly and posthemorrhagic hydrocephalus are among the most devastating CNS events in premature infants.Porencephaly and posthemorrhagic hydrocephalus are among the most devastating CNS events in premature infants. Cystic PVLCystic PVL High risk for CPHigh risk for CP Cognitive delays in children with severest lesions on u/s and with spastic quadriplegiaCognitive delays in children with severest lesions on u/s and with spastic quadriplegia

52 Ventriculo-peritoneal shunt Although ventricular dilatation may be reversible, infants with severe ventricular enlargement are at high risk for neurodevelopmental handicapAlthough ventricular dilatation may be reversible, infants with severe ventricular enlargement are at high risk for neurodevelopmental handicap Rapidly progressive posthemorrhagic hydrocephalus may require permanent placement of a cerebrospinal fluid (CSF) shuntRapidly progressive posthemorrhagic hydrocephalus may require permanent placement of a cerebrospinal fluid (CSF) shunt The long-term neurodevelopment of ELBW infants who require shunt insertion is very unfavorable compared with ELBW infants with intraventricular hemorrhage who do not have ventricular enlargementThe long-term neurodevelopment of ELBW infants who require shunt insertion is very unfavorable compared with ELBW infants with intraventricular hemorrhage who do not have ventricular enlargement head circumference should be monitored for rapid or slow growthhead circumference should be monitored for rapid or slow growth

53 Shunt Malfunction the NICU graduate with a VP shunt must be monitored for for shunt infections or malfunctionsthe NICU graduate with a VP shunt must be monitored for for shunt infections or malfunctions Malfunctions are typically due to an occlusion of the proximal or distal cannula with a subsequent increase in intracranial pressureMalfunctions are typically due to an occlusion of the proximal or distal cannula with a subsequent increase in intracranial pressure Poor feeding, vomiting, irritability, lethargy, sleepiness, apnea, and seizuresPoor feeding, vomiting, irritability, lethargy, sleepiness, apnea, and seizures shunt infection and meningitis should be suspected with fever and malaiseshunt infection and meningitis should be suspected with fever and malaise

54 PVL sequelae Other postnatal events leading to PVL include CSF infections or intraventricular hemorrhage, life-threatening apnea and bradycardia, and cardiorespiratory arrestOther postnatal events leading to PVL include CSF infections or intraventricular hemorrhage, life-threatening apnea and bradycardia, and cardiorespiratory arrest The condition is otherwise identified on cranial sonograms as echogenic areas in the periventricular white matter. Injuries in these areas evolve into cystsThe condition is otherwise identified on cranial sonograms as echogenic areas in the periventricular white matter. Injuries in these areas evolve into cysts PVL is highly associated with subsequent neurodevelopmental disabilities, particularly cerebral palsy PVL is highly associated with subsequent neurodevelopmental disabilities, particularly cerebral palsy Persistence of cysts is known to increase the risk of severe neuromotor abnormalitiesPersistence of cysts is known to increase the risk of severe neuromotor abnormalities


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