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Biliary Atresia
Milton J. Finegold
Texas Children's Hospital
Houston, TX
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Clinical Summary - Patient 1:
Twin A was born at 27 4/7 weeks weighing 1090 gm by C-section to 36-year old mother with gestational diabetes and idiopathic thrombocytopenic purpura requiring IVIG x3. Required intubation, assisted ventilation, umbilical venous catheterization. Parenteral alimentation with Trophamine, Intralipid begun on day 2, with oral trophic feeds [Pedi Enfamil] as tolerated. Day 3 treated for sepsis; recurred on day 13. Day 5 Bu 3.8, Bc 2.8, AST 1010, ALT 6, AP 331, GGT 1098, Alb 3. Ultrasound: increased hepatic echogenicity; collapsed gallbladder; CBD not visualized; intrahepatic ducts not dilated. Progressive fall in GGT, AP stayed elevated (500s); TPN stopped day 16. At one month, intubated, on CPAP, good weight gain, Bu 3.7, Bc 1.7, AP 606. Repeat US at 6 weeks - increased hepatic echogenicity; no change in GB or ducts. At 3 mo, Bu 1.6, Bc 3.8, AST 84, ALT 59, AP 417, GGT 556 prompting Ursodeoxycholate treatment; repeat US--contracted GB; hyperechoic liver; HIDA scan--no excretion [stool color never mentioned till now--yellow-brown]; Open biopsy on DOL 145 months
Case 4 - Slide 1
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Case 4A - Figure 1 - portal tract features are consistent with extrahepatic biliary obstruction
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Case 4A - Figure 2 - portal tract features are consistent with extrahepatic biliary obstruction
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Case 4A - Figure 3 - portal tract features are consistent with extrahepatic biliary obstruction
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Case 4A - Figure 4 - portal tract features are consistent with extrahepatic biliary obstruction
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Case 4A - Figure 5 - portal tract features are consistent with extrahepatic biliary obstruction
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Clinical Summary - Patient 2:
Twin B born at 31 3/7 weeks weighing 828 gm. Managed in another city for 3 months with RDS evolving to BPD, complicated by Pseudomonas pneumonia. Candida, Staph and Klebsiella UTI also noted. Microcephalic but CNS functioning intact. Hepatosplenomegaly with increased Bc. Ultrasound--ascites; no organ abnormalities. At 90 days, anemic, thrombocytopenic, Bc 1.5, AST 82, ALT 1001, AP 829, GGT 66, prolonged PT, PTT. Skeletal XR-metaphyseal dysplasia. Repeat US at 100 days: contracted, thick-walled GB, normal intrahepatic ducts. HIDA: poor uptake, but excretion into intestine. Biopsy on DOL 120.
Case 4 - Slide 2
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Case 4B - Figure 1 - portal tract features are consistent with extrahepatic biliary obstruction
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Case 4B - Figure 2 - portal tract features are consistent with extrahepatic biliary obstruction
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Case 4B - Figure 3 - portal tract features are consistent with extrahepatic biliary obstruction
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