A 3-month-old girl was referred for jaundice, hepatomegaly, acholic stools, and dark urine. At blood testing, conjugated hyperbilirubinemia, thrombocytosis, and raised liver enzymes were found, and triangular cord sign was observed at liver ultrasound. Cholangiography confirmed BA, and KPE was performed at the age of 94 days. At surgical exploration, the liver was fully mobilized and appeared enlarged and greenish-brown in color with increased consistency and macronodular surface. Hepatic histological evaluation revealed severe fibrosis (METAVIR F3-F4). During the following 4 months, the girl was in good clinical conditions, and bile flow was completely restored within 1 month after KPE with total bilirubin serum levels falling under 1 mg/dl. Unfortunately, she developed refractory ascites requiring chronic diuretic treatment with furosemide (1 mg/kg/day) and spironolactone (3 mg/kg/day). At the age of 7 months, the patient experienced a sudden respiratory distress. On admission, she was apyretic with subcostal and jugular retractions. Thoracic auscultation revealed decreased airflow in the right basal region, with oxygen saturation at room air 94%. Laboratory data showed normal liver function tests (albumin 3.2 g/dl; prothrombin time international normalized ratio 1.2) with normal values of aspartate aminotransferase, alanine aminotransferase, and gamma-glutamyltransferase; conjugated serum bilirubin was 0.6 mg/dl. Blood count was normal (platelet count 230,000/mul), and C-reactive protein was negative. Echocardiogram revealed normal left ventricular wall motion and contractility. Chest X-ray and computed tomography (CT) scan showed pleural effusion with an almost complete opacification of the right hemithorax (Figure 1). Rapid deterioration of pulmonary function led to an emergency intubation, and thoracentesis drained about 250 ml of transudate (proteins = 1.3 g/dl, 1 leukocytes/mm3, sterile).
Subsequently, in combination with diuretic therapy, non-invasive ventilation (NIV) with full-face mask to high-flow nasal cannula oxygen (HNFC) was started. Infections (including tuberculosis) and inflammatory, renal, and neoplastic diseases were also excluded. Esophagogastroduodenoscopy showed absence of esophageal varices. After discharge, pleural effusion recurred three times despite aggressive diuretic therapy; in one circumstance, imaging revealed only minimal ascitic effusion. In the following months, serial ultrasound evaluations confirmed persistent perihepatic and perisplenic ascites with fluid between intestinal loops and showed signs of PHT with hepatofugal venous flow.
At 10 months of age, despite a low pediatric end-stage liver disease (PELD) score (equal to 3), the infant was listed for LT due to repeated episodes of respiratory distress, the need for multiple thoracentesis, and frequent hospitalizations. At the age of 11 months, the patient underwent deceased donor split LT. During surgery, no vascular, diaphragm, or azygous vein changes were identified. Intraoperatively, PHT was advanced, and the native liver appeared enlarged and with a multinodular cirrhotic aspect. Histological evaluation showed signs of biliary cirrhosis due to BA. In the following 12 months after transplantation, the infant showed good liver function and no further HH relapse.