Liver transplant in syndromatic biliary atresia

Introduction and Objectives

Biliary atresia (BA) is an obliterative cholangiopathy, there are at least two phenotypes, one of them is the syndromic form, which occurs in 10-15% of cases, it is associated with polysplenia, heart disease, heterotaxy and malrotation intestinal. The objective of this review is to present the experience when performing liver transplantation

Materials and Patients

Female patient daughter of a diabetic mother, carrier of BA type III who underwent Kasai surgery at 89 days of life, carrier of intestinal malrotation, preduodenal portal vein, intraventricular communication of 1.7 mm without hemodynamic repercussion and dyslipidemia, without biliary clearance and pondostatural arrest, worthy of performing a liver transplant from an unrelated living donor, due to complications of cirrhosis such as ascites, malnutrition and cholesasis, at the time of surgery, annular pancreas, were found as additional findings to those described agenesis of cava, agenesis of the celiac trunk, presented early partial thrombosis of the portal vein, meriting anticoagulant and antithrombotic treatment, with resolution of the condition, without requiring surgical intervention. Currently, after one year of follow-up with adequate evolution, without cholestasis or transaminasemia, adequate growth, immunosuppression with a calcineurin inhibitor, the dyslipidemia resolved. Our patient does not have polysplenia.

Results

The clinical case of syndromic BA is presented, although BA is rare, the syndromic presentation is even more, so we present a successful case, with complex vascular malformations combined with extrahepatic malformations, mainly cardiac, with which mortality at time of performing the transplant is high, greater than 90%. Our patient had a satisfactory surgical and clinical evolution.

Conclusions

BA is the main cause of liver transplantation in pediatrics; reported cases of the syndromatic type are rare. The complete evaluation and planning of possible vascular malformations associated at the time of transplantation should alert the medical and surgical team.

Ethics statement: No patient-identifying data is used in this presentation.

Declaration of interests: None.

Funding: This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.