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Toronto Notes 2019 Neonatology BILIARY ATRESIA
Definition
• atresiaoftheextrahepaticbileductswhichleadstocholestasisandincreasedconjugatedbilirubinafter the first wk of life
• progressiveobliterativecholangiopathy
Epidemiology
• incidence:1:10,000-15,000livebirths
• associatedanomaliesin10-35%ofcases:situsinversus,congenitalheartdefects,polysplenia
Clinical Presentation
• darkurine,palestool,jaundice(persistingfor>2wk),abdominaldistension,hepatomegaly
Diagnosis
• conjugatedhyperbilirubinemia,abdominalU/S,operativecholangiogram
• HIDAscan(maybebypassedinfavourofbiopsyiftimingofdiagnosisiscritical) • liverbiopsy
Treatment
• surgicaldrainageprocedure
• hepatoportoenterostomy(Kasaiprocedure;mostsuccessfulif<8wkofage)
• two-thirdswilleventuallyrequirelivertransplantation
• vitaminsA,D,E,andK;dietshouldbeenrichedwithmedium-chaintriglyceridestoensureadequate
fat ingestion
Necrotizing Enterocolitis
Definition
• intestinalinflammationassociatedwithfocalordiffuseulcerationandnecrosis • primarilyaffectingterminalileumandcolon
Epidemiology
• affects1-5%ofpretermnewbornsadmittedtoNICU
Pathophysiology
• postulatedmechanismofbowelischemia:mucosaldamageandenteralfeeding→bacterialgrowth→ bowel necrosis/gangrene/perforation
Risk Factors
• prematurity(immaturedefenses)
• asphyxia,shock(poorbowelperfusion)
• hyperosmolarfeeds
• enteralfeedingwithformula(breastmilkcanbeprotective) • sepsis
Clinical Presentation
• usuallypresentsat2-3wkofage
• distendedabdomen,diminishedbowelsounds,feedingintolerance • increasedamountofgastricaspirate/vomituswithbilestaining
• frankoroccultbloodinstool
• signsofbowelperforation(sepsis,shock,peritonitis,DIC)
Investigations
• AXR:pneumonitisintestinalis(intramuralairisahallmarkofNEC),freeair,fixedloops,ileus, thickened bowel wall, portal venous gas
• CBC,ABG,lactate,bloodculture,electrolytes
• highorlowWBC,lowplatelets,hyponatremia,acidosis,hypoxia,hypercapnea
Treatment
• NPO(7-10d),vigorousIVfluidresuscitation,decompressionwithNGtube,supportivetherapy • TPN
• antibiotics(usuallyampicillin,gentamicin±metronidazoleifriskofperforationx7-10d)
• serialAXRsdetectearlyperforation(40%mortalityinperforatedNEC)
• peritonealdrain/surgeryifperforation
• surgicalresectionofnecroticbowelandsurgeryforcomplications(e.g.perforation,strictures)
Pediatrics P67
Influence of Enteral Nutrition on Occurrences of Necrotizing Enterocolitis in Very-Low-Birth- Weight Infants
J Pediatr Gastroenterol Nutr 2015;61(4):445-50 Study: Case-control study of very-low-birth-weight (VLBW) infants and occurrences of NEC within
30 d of life.
Population: 1028 VLBW infants in neonatal intensive care unit Jan 2003-May 2008.
Main Outcome: NEC defined using stage ≥2 of modified Bell criteria.
Results: 55 infants developed NEC within 30 d
of life (5.4%). Those with NEC had higher odds of having been fed breast milk <7 d (OR: 4.02), not having achieved full enteral feeding during the first mo (OR: 3.50), and having had parenteral feeding (OR: 2.70).
Conclusions: Occurrence of NEC can be reduced with breast milk feeding beyond 7 d and early full enteral feeding.