P0354 Biliary Atresia: Portal Hypertension and Its Consequences

Earliest Collaterals in Biliary Atresia

“…As most of them didn't submit to an endoscopy before their death, they probably lost the diagnosis of varices among them. 4 Grunert et al found portal hypertension with hepatofugal collaterals in the first year of life. 7 Yokoyama et al found splenoretroperitoneal shunt as early as 1 month of age associated with neonatal hepatitis.…”

“…The great majority of these children develop chronic end-stage liver disease, which leads to portal hypertension and its consequences, such as massive bleeding from rupture of esophageal or gastric varices, ascites, hepato-renal syndrome, and hepatic encephalopathy. 4 Portal hypertension has been documented in 68% of these children. 5 In portal hypertension, portosystemic collaterals decompress the portal circulation and give rise to varices.…”

“…Larger varices at sites of limited soft tissue support, notably the gastroesophageal junction, are at greater risk for variceal rupture and bleeding in patients who have portal hypertension. 6 Yoshitoshi et al 4 found that varices started at a median age of 1.6 years for patients without drainage surgery. Bleeding occurred at a mean age of 1.45 years for patients without drainage surgery and 2.98 years for patients with drainage surgery.…”