Technical Considerations in Liver Transplantation for Biliary Atresia With Situs Inversus

Abstract

In liver transplantation (LT) for biliary atresia (BA) with situs inversus (SI), the surgical procedure is technically challenging due to multiple anatomical variations. We evaluated the surgical procedures and the outcomes in our patients and in the previously reported patients undergoing LT for BA with SI. Between November 2005 and October 2018, 235 children underwent LT with an overall 10‐year graft survival of 94.7%. Of these, 6 (2.6%) patients received LT for BA with SI. Living donor liver transplantation (LDLT) was applied in all patients. Vascular anomalies at our center included the absence of the inferior vena cava (IVC; n = 5), a preduodenal portal vein (PV; n = 4), and an aberrant hepatic artery (HA; n = 1). Hepatic vein (HV) outflow obstruction occurred in 1 patient with an absent IVC and necessitated repositioning of the graft immediately after LDLT. PV stenosis occurred in 1 patient with a preduodenal PV at 5 months after LDLT. The overall survival of our patients was 80.0% at 3 years. In the literature, 49 patients with LT for BA with SI were reviewed. The survival rate was 91.8%, and the vascular complications included HV outflow obstruction (2.0%), PV complications (10.2%), and HA thrombosis (4.1%). All PV complications occurred in LDLT patients with a preduodenal PV (5/23; 21.7%). In conclusion, the detailed assessment of the surgical procedures and the systematic literature review demonstrated excellent outcomes after LT for BA with SI. Liver recipients with a preduodenal PV are exposed to a higher risk for vascular complications after a very challenging LT procedure with overall good outcomes.

Transplante hepático em paciente pediátrico com situs ambiguus: um relato de caso

“…Especial atenção deve ser dada à dissecção cirúrgica do sítio receptor, devido às alterações anatômicas decorrentes da apresentação em espelho, notadamente: risco de paralisia no nervo frênico, a posição da veia porta em relação ao duodeno, origem da artéria hepática, ausência de veia cava inferior e drenagem venosa do fígado 5 . 5 .…”

“…Especial atenção deve ser dada à dissecção cirúrgica do sítio receptor, devido às alterações anatômicas decorrentes da apresentação em espelho, notadamente: risco de paralisia no nervo frênico, a posição da veia porta em relação ao duodeno, origem da artéria hepática, ausência de veia cava inferior e drenagem venosa do fígado 5 . 5 . No caso relatado neste artigo, não foram necessários enxertos vasculares e não houveram complicações pós-operatórias relacionadas diretamente a anatomia ou técnica operatória.…”

A rare hepatic artery variant reporting and a new classification

“…Interestingly, normal patterns occur only in 42%-75.7% of cases (1,2). Aberrant arteries may be accidentally injured during surgical procedures resulting in severe haemorrhage and other fatal complications (3)(4)(5)(6)(7)(8)(9)(10)(11). A thorough knowledge of possible variations in branching, courses, and distribution of the vessels supplying the liver and gallbladder is essential.…”