Introduction: Biliary atresia (BA) is obliterative cholangiopathy with an incidence of 1: 10,000-15,000 live births. Syndromic BA is associated with other congenital anomalies, such as interruption of the inferior cava vein, preduodenal portal vein, abnormal intestinal rotation, complete visceral transposition, cardiac anomalies and polysplenia, this occurs in 10% of patients, it´s believed that there is a relationship between BA and maternal diabetes.
Objective: to present the experience of a patient with syndromatic bile duct atresia and a related living donor liver transplant (LDLT).
Clinical report: Female, with maternal history of type II diabetes, 1 year 6 months old, started suffering from Cholestatic jaudince at 15 days of life, after the work-up for jaundice, the diagnosis of type III BA was made, plus another clinical features malrotation, pre-duodenal portal vein, cardiac anomalie Intraventricular septal defect: mean muscular intraventricular communication of 1.7 mm, shunt from left to right with a gradient of 60 mm without hemodynamic repercussion, patent foramen ovale of 2 mm and dyslipidemia. Despite the kasai, he did not have bile clearance, with little growth with a weight of 7.5 kg an addition to worsening liver failure with a CHILD – PUGH B with 7 pounts and PELD 24 pounts, so it was decided to perform an LDLT. During the surgical event, the following findings were reported: annular pancreas with preduodenal portal vein, single kidney-shaped spleen without polysplenia, agenesis of the retrohepatic cava, agenesis of the celiac trunk, left hepatic artery coming from the left gastric artery, direct gastric artery from the aorta, right hepatic artery coming from the superior mesenteric artery and incomplete intestinal malrotation. The transplant was performed successfully, as well as a prophylactic appendectomy and placement of a biodegradable biliary stent in the roux. In his post-surgical evolution, he presented partial thrombosis of the portal vein in hepatic segment II, which resolved favorably with anticoagulation in the first 2 days. after LT (LT).
Conclusion: The anatomical variants of a syndromatic atresia require great knowledge and vascular control so as not to lose the only possibility of arterial and portal anastomosis that the patient has, in this case the total release of the portal vein without injuring the pancreas or the duodenum represented a challenge. We present our successful experience in the complex surgical approach to a liver transplant with syndromatic atresia.