An Unexpected Hepatic Hydrothorax After a Successful Kasai Portoenterostomy: A Case Report

Hepatic hydrothorax (HH) represents a rare complication of portal hypertension among adult cirrhotic patients. Here, we describe a pediatric case of HH, observed in a biliary atresia infant. The child presented with recurrent right-sided pleural effusion, after a successful Kasai portoenterostomy with restoration of bile flow and without overt signs of hepatic failure. Recurrence of HH led the patient to liver transplant despite a low pediatric end-stage liver disease value. Although rare, HH can also occur in children and should be suspected in patients with portal hypertension and respiratory distress. HH may be an indication for liver transplantation.

Analysis of the odds ratio of developmental delay in children with biliary atresia 12 months after liver transplantation

Background. Liver cirrhosis occurring before 1 year of age can affect a child’s development. Liver transplantation is the only radical treatment for decompensated cirrhosis. In biliary atresia, cirrhosis develops during the first months of life. The duration of cirrhosis in biliary atresia may vary from palliative Kasai portoenterostomy (PE) to liver transplantation. Developmental abnormalities in children with biliary atresia have been shown to occur both before and after liver transplantation. Association between duration of liver cirrhosis and psychomotor development of children has been underestimated.Objective: to determine the chances of developmental delay in children depending on the cirrhosis persistence duration.Materials and methods. The study enrolled 83 children with biliary atresia (47 children underwent palliative Kasai PE, 36 children with liver transplantation did not undergo Kasai PE). All children had their psychomotor development assessed before PE and 12 months after PE using the Griffiths psychomotor developmental scale (translation and adaptation by E.S. Keshishian) for children up to 24 months of age. Statistical analysis was performed by calculating odds ratios with 95% confidence intervals.Results. Comparative analysis showed that in the subgroup of children who underwent Kasai PE, cirrhosis persistence before transplantation was 2.6 months longer than in children without Kasai PE (p = 0.011). The odds of developmental delay in preparation for liver transplantation were 3.3 times higher in the subgroup of children who underwent Kasai palliative PE compared to children without palliative (95%, CI 1.35–8.31). The odds of developmental delay 12 months after liver transplantation were 4.4 times higher in the subgroup of children who underwent palliative Kasai PE than in children without the palliative care (95% CI 1.54–12.5).Conclusion. Children who underwent liver transplantation after palliative surgical treatment had lower levels of psychomotor development than children without palliative Kasai PE both before and 12 months after liver transplantation (p = 0.0018, p = 0.01 respectively).

Extended Adhesion-Sparing Liver Eversion during Kasai Portoenterostomy for Infants with Biliary Atresia

Background: Despite the fact that Kasai portoenterostomy (KPE) is the primary treatment for biliary atresia (BA), liver transplantation (LT) remains the ultimate surgery for two-thirds of these patients. Their true survival rate with the native liver reflects the original KPE and the burden of post-operative complications. We report an original modification of the adhesion-sparing liver eversion (ASLE) technique during KPE that facilitates the total native hepatectomy at time of transplantation. Methods: All consecutive patients with BA who underwent KPE at our department and subsequent LT at Paediatric Liver Transplant Centre at Papa Giovanni XXIII Hospital between 2010–2018 were retrospectively enrolled. All patients underwent ASLE during KPE. Patients’ demographic data, type of KPE, total transplant time (TTT), hepatectomy time (HT), intra-operative packed red blood cells and plasma transfusions, intra- and post-operative complications were noted. Results: 44 patients were enrolled. Median TTT and HT were 337 and 57 min, respectively. The median volume of packed red blood cell transfusion was 95 mL. No patients presented bowel perforation during the procedure or in the short post-operative course. No mortality after LT was recorded. Conclusions: In addition to the well-known advantages of the standard liver eversion technique, ASLE reduces the formation of intra-abdominal adhesions, lowering significantly the risk of bowel perforation and bleeding when liver transplantation is performed for failure of KPE.