Biliary atresia (BA)

• A progressive inflammatory and fibrosing destruction of the extrahepatic bile ducts in the first weeks of life. Bile cannot reach the intestine → conjugated (direct) hyperbilirubinemia, cholestatic liver injury, and, without treatment, biliary cirrhosis and death within the first years.
• Cause is unknown (immune-mediated injury, possibly triggered by viruses); a minority have associated anomalies such as polysplenia and situs abnormalities.
• Time-critical: outcomes are best when surgical drainage (Kasai procedure) is done early — ideally before about 60 days of age, and earlier is better.
• BA is the most common reason for liver transplantation in children.

Necrotizing enterocolitis (NEC)

• Ischemic and inflammatory necrosis of the bowel wall, most often the terminal ileum and colon, with bacterial invasion. Gas produced by bacteria dissects into the bowel wall (pneumatosis intestinalis). Necrosis can progress to perforation, peritonitis, sepsis, and death.
• The most common GI emergency in the NICU. Prematurity is the major risk factor; most cases occur in very low birth weight infants after enteral feeding has begun, commonly in the second to third week of life (later in the most preterm).
• Other risk factors: formula feeding (human milk is protective), intestinal hypoxia–ischemia (asphyxia, congenital heart disease, polycythemia, exchange transfusion, umbilical catheters), red cell transfusion, acid-suppressing drugs, and prolonged empiric antibiotics that disturb gut flora. Term infants with NEC usually have heart disease or another predisposing condition.

Biliary atresia

• Jaundice persisting beyond 2 weeks of age (or beyond 3 weeks in a breastfed infant) — often dismissed as "breast milk jaundice"
• Pale, clay-colored (acholic) stools — the most important clue; stool color may be variable at first
• Dark urine (conjugated bilirubin is water-soluble; normal newborn urine is pale)
• Firm hepatomegaly; later splenomegaly and ascites (portal hypertension)
• Initially looks well and feeds normally; later poor weight gain (fat malabsorption), irritability, pruritus
• Bleeding from vitamin K malabsorption — bruising, oozing, and even intracranial hemorrhage

NEC

• GI signs: feeding intolerance, increasing or bilious gastric residuals or vomiting, abdominal distension, tenderness, visible bowel loops, bloody or occult-blood-positive stools, abdominal wall redness or bluish discoloration (perforation/peritonitis)
• Systemic signs: apnea and bradycardia, temperature instability, lethargy, poor perfusion, hypotension — similar to sepsis
• Decreased or absent bowel sounds

Biliary atresia

Stool color cards given to parents are used in some countries to speed recognition of pale stools.

NEC

Modified Bell staging: Stage I — suspected (nonspecific signs, nondiagnostic X-ray); Stage II — definite (pneumatosis ± portal venous gas); Stage III — advanced (critically ill; IIIB = perforation).

Biliary atresia

• Kasai hepatoportoenterostomy: the fibrotic ducts are removed and a Roux-en-Y loop of jejunum is attached to the liver hilum (porta hepatis) so that bile can drain from microscopic ducts. Success is judged by clearance of jaundice and return of colored stools.
• Post-Kasai care:
  • Ursodiol (ursodeoxycholic acid) to promote bile flow — diarrhea, abdominal discomfort
  • Prophylactic antibiotics in many centers to prevent cholangitis (e.g., trimethoprim–sulfamethoxazole — rash, bone marrow suppression, hyperkalemia; labeled as contraindicated under 2 months of age because sulfonamides displace bilirubin (kernicterus risk); some centers use it after Kasai under specialist direction, others choose another agent)
  • Corticosteroids are sometimes used, but benefit is uncertain
• Nutrition: medium-chain triglyceride (MCT)–enriched formula (MCTs are absorbed without bile), higher calories, continued breast milk as advised; fat-soluble vitamins A, D, E, and K with blood-level monitoring (toxicity: vitamin A — raised intracranial pressure and liver injury; vitamin D — hypercalcemia).
• Pruritus: ursodiol; bile acid binders such as cholestyramine (constipation, binds other medicines and fat-soluble vitamins — give other drugs 1 hour before or 4–6 hours after); rifampin (hepatotoxicity, orange body fluids, many drug interactions); newer ileal bile acid transport inhibitors are used in some cholestatic liver diseases.
• Cholangitis: fever, worsening jaundice, pale stools again → IV antibiotics urgently.
• Liver transplantation when Kasai fails or cirrhosis progresses (portal hypertension, variceal bleeding, ascites, growth failure). Lifelong immunosuppression afterward (infection risk, nephrotoxicity with calcineurin inhibitors, drug-level monitoring).

NEC

• Bowel rest: NPO, orogastric tube to low intermittent suction for decompression
• IV fluids and parenteral nutrition; correct acidosis, electrolytes, and coagulopathy; platelet and blood transfusion as needed
• Broad-spectrum IV antibiotics after blood culture, commonly including anaerobic coverage (e.g., ampicillin + gentamicin + metronidazole, or per unit protocol); monitor aminoglycoside levels and renal function
• Respiratory and circulatory support; inotropes for shock
• Typical duration of bowel rest and antibiotics for definite NEC is about 7–14 days, then gradual reintroduction of feeds, preferably human milk
• Surgery for perforation or clinical deterioration despite treatment: laparotomy with resection of necrotic bowel and an ostomy, or a bedside peritoneal drain in very small, unstable infants

Prevention of NEC

• Human milk (mother's own milk; pasteurized donor milk when unavailable) is the most effective preventive measure
• Standardized, cautious feeding advancement protocols
• Avoid unnecessary acid suppression and prolonged empiric antibiotics
• Probiotics: evidence of benefit exists, but in 2023 the FDA warned that preterm infants given probiotic products developed invasive, sometimes fatal infections from the product organisms; there are no FDA-approved probiotic products for preventing NEC. Use only under institutional policy with informed parental consent.

Listed in priority order.

1. Airway, breathing, circulation — NEC can cause apnea, respiratory failure, and shock: continuous monitoring, oxygen/ventilation support, fluid resuscitation as ordered
2. Early recognition of NEC — before each feed check abdominal girth, residuals (per unit policy), stools, and tolerance; stop feeds and notify for distension, bilious aspirates, bloody stools, or apnea with feeding intolerance
3. Bowel decompression — maintain OG tube patency and suction; record drainage color and volume
4. Abdominal assessment — measure abdominal girth at the same marked point every few hours; assess tenderness, discoloration, visible loops, bowel sounds
5. Positioning — supine or side-lying without pressure on the distended abdomen; loose diaper; avoid rectal temperatures and rectal medications
6. Infection control and medications — antibiotics on time; hand hygiene; central-line care for parenteral nutrition
7. Fluid and nutrition — strict intake and output, daily weight, glucose and electrolytes; TPN safety (line care, glucose monitoring)
8. Biliary atresia care
   • Check stool color every diaper and document; monitor jaundice, urine color, weight, head circumference
   • Give MCT formula and fat-soluble vitamins as ordered; bleeding precautions until vitamin K deficiency is corrected
   • After Kasai: monitor for fever and cholangitis, wound healing, stool color return; NG decompression and pain control early postoperatively
   • Skin care for pruritus: keep nails short, soft cotton clothing, lukewarm baths, moisturizer
9. Family support — explain the urgency of timely surgery, the possibility of future transplant, and coping resources; teach ostomy care after NEC surgery

Biliary atresia

• Look at stool color every day; report pale, white, or gray stools, or jaundice returning
• Seek care immediately for fever after Kasai — cholangitis needs urgent IV antibiotics
• Give vitamins and ursodiol exactly as prescribed; do not give extra vitamins without advice
• Watch for bleeding, abdominal swelling, vomiting blood, or black stools (portal hypertension)
• Nutrition is critical for growth; high-calorie feeding plans may include tube feeds
• Vaccines should be completed on schedule before any transplant (live vaccines are not given after transplant)

NEC

• Breast milk protects the baby — support pumping and delivery of milk to the NICU
• After NEC surgery: ostomy care, signs of stoma problems, and dehydration (fewer wet diapers, sunken fontanel, lethargy)
• Report vomiting (especially green), abdominal swelling, blood in stools, or poor feeding weeks later — possible stricture
• Keep developmental follow-up appointments

• BA: jaundice beyond 2 weeks + pale (acholic) stools + dark urine + hepatomegaly
• Prolonged jaundice → check direct/conjugated bilirubin; above 1.0 mg/dL (17 µmol/L) is abnormal
• Ultrasound first; intraoperative cholangiogram confirms BA
• Kasai procedure early (ideally before about 60 days); most common cause of pediatric liver transplant
• After Kasai: watch for cholangitis (fever); give MCT formula and vitamins A, D, E, K
• NEC: preterm + enteral feeds; formula feeding raises risk, human milk protects
• NEC signs: feeding intolerance, distension, bilious residuals, bloody stools, apnea, temperature instability
• X-ray hallmark: pneumatosis intestinalis; free air = perforation → surgery
• Treatment: NPO, OG decompression, IV antibiotics, TPN, serial abdominal films and girths
• Labs in NEC: thrombocytopenia and metabolic acidosis
• No rectal temperatures in suspected NEC; measure abdominal girth at a marked point
• Late complications: stricture and short bowel syndrome