Liver transplantation for syndromic biliary atresia with a pedunculated accessory hepatic lobe

Abstract

Extrahepatic biliary atresia (EHBA) is the most common indication for liver transplantation in infants and children. Congenital malformations associated with EHBA are common and present in about 9%-37%. We describe a case of a child who received a left lobe liver transplant for syndromic EHBA.

Accessory lobes of the liver: A report of 3 cases and review of the literature

“…During the 7th and the 8th week of embryo development, which is when the muscular layer of the abdominal wall is formed, development of an ALL in the embryo may obstruct the closing of the umbilical ring (3,10), which is why most ALLs are associated with acromphalus. ALLs are also associated with congenital biliary atresia (22), congenital diaphragmatic defects, and angiocavernoma (8). Most reported cases of an ALL involve females ranging in age from newborns to 75 years.…”

“…In order to make an accurate diagnosis, factors such as size, shape, position, whether complications are present or not, and the type of complication should be considered. In terms of position, (i) an ALL in the thorax should be differentiated from a tumor of the pleura, lungs, chest wall, or diaphragm (1,4,24,26) while (ii) an ALL in the pelvic cavity should be differentiated from a benign or malignant tumor of the pelvic organs (33) and (iii) an ALL on the surface of the liver or abdominal organs should be differentiated from pathological changes in the liver, gall bladder, pancreas, spleen, or adrenal glands (13,22). Most patients with an ALL have no clinical manifestations and the ALL may be large enough to jostle surrounding organs.…”

“…Whether complications are present or not and the type of complication relate to the patient's age and the type, size, and position of the ALL. Most infants with an ALL also have congenital acromphalus, congenital biliary atresia, or gallbladder torsion (22,41). A pedunculated ALL carries a higher incidence of reversion than the other types of ALLs.…”

Right Iliac fossa pain due to torsion and ischaemia of accessory liver lobe

“…It may be caused by strenuous exercise, as in our patient, or trauma [8]. Associated umbilical hernia or bile cysts increases the suspicion of torsion ALL [9]. ALL may also revert, infarct, rupture, fracture or bleed.…”

Ectopic Hepatocellular Carcinoma

“…Liver ectopia outside the bile ducts and the gallbladder is mostly an isolated phenomenon, but it can also occur in conjunction with other congenital abnormalities, including biliary atresia, caudate lobe agenesis, omphalocele, ductal plate malformations, and laparoschisis, Parts of liver tissue nodules sometimes identified as ectopic liver are connected to the orthotopic organ through a vascularized pedicle and are, therefore, not true ectopias but may rather be related to accessory and abortive liver lobes (hepar succenturiatum;Algin et al 2008;Wang et al 2012). In comparison with true liver ectopia, accessory liver lobes have been described numerous times (Levi et al 1969;Pujari and Deodhare 1976;Go et al 1978;Kuroiwa et al 1984;Tomooka et al 1988;Fogh et al 1989;Massaro et al 2007;Stattaus et al 2008;Faraj et al 2010;Wang et al 2010;Kostov and Kobakov 2011). Accessory livers may have their own mesentery and, depending on their location, can drain into the biliary system or have no drainage system.…”