Congenital biliary dilatation (CBD) is more in some Asian region as than in the Western countries, including United States, and some Asian populations have an incidence as high as 1 in 1,000 births (1–5). In contrast, some Asian populations have an incidence as high as 1 in 1,000 births. There is a marked sex difference, with the rate in females being 3–4 times higher than that in males (6–8). Todani et al. classified the extrahepatic bile duct into four types and the intrahepatic bile duct into one type (9). Type V is treated synonymously with Caroli disease, one of the so-called fibropolycystic diseases, but differs from congenital hepatic fibrosis in the thickness of the bile ducts that are damaged (10, 11). Almost 80% of choledochal cysts (CCs) are now discovered prenatally or in infancy (12), but it has been reported that biliary cirrhosis rapidly progresses from the neonatal period to the infantile period in some cases of CBD (13). We herein report an infantile case of CBD that showed severe biliary cirrhosis at the diagnosis, which was treated by laparoscopic surgery.

The primary goal of CBD treatment is of resection the extrahepatic bile duct and reconstruction of the bile flow route to prevent the potential occurrence of malignant tumors of the bile duct. However, the possibility of liver fibrosis progressing—leading to end-stage cirrhosis requiring liver transplantation—cannot be ruled out. In Todani type IV, such as our case, the CBD shows intrahepatic bile duct dilatation. CBD is mainly represented by Todani types I and IV. The surgical treatment for CBD is resection of the dilated bile duct and biliary reconstruction. Biliary reconstruction can be performed by means of Roux-en-Y hepaticojejunostomy, hepaticoduodenostomy, or jejunal interposition heptaticoduodenostomy (5). In the case of type I, there is a possibility of malignancy, the incidence of which different differs between Asian and Western countries. However, due to the lack of long-term patient information, the subset with a low risk of developing malignant tumors is unknown. Thus, resection of the extrahepatic bile duct seems to be recommended worldwide as a basic treatment strategy, as it is for type IVa (3).

Liver fibrosis in patients with CBD has been shown to worsen with age, and there are reports recommending surgery in early infancy (15). Obstructive bile ducts with impaired duodenal outflow, such as in our case, may progress rapidly from liver fibrosis to cirrhosis. Suita et al. reported that obstructive cholangiopathy is the main pathology in the neonatal period, causing acute and chronic cholangitis due to reflux of pancreatic juice. It is speculated that exacerbation of cholestatis due to this inflammation is the underlying cause of liver fibrosis (16). Type IVa CBD is likely to be associated with the progression of liver fibrosis, and prompt surgical intervention is recommended, especially for neonatal and infantile cases with type IVa because irreversible liver cirrhosis can occur, even in the infantile period (17). We think that the process leading to hepatic fibrosis is due to the degree of cholestasis. Our case was a type IV CC with no prenatal diagnosis and was not diagnosed until it became symptomatic, and it took a long time to be diagnosed. The cholestasis, in our case, may be mild in the early postnatal period. Reflux of pancreatic juice causes inflammation and exacerbates cholestasis, and the liver began with mild inflammation, and after a prolonged course, chronic inflammation accumulated and caused liver fibrosis. It is possible that the interrelationship with the varying with or without congenital cyst, cholestasis, and refluxed pancreatic secretions into the bile may have contributed to the differences in the course of the disease. There is no need to rush the timing of surgery, as long as it is possible to prevent malignancy; however, in symptomatic cases it is necessary to assess the condition of the liver and decide a therapeutic strategy.

Our search of the PubMed database using the keywords [congenital biliary dilation, liver transplantation], yielded 46 reports. Most of the reports describing cases in which CBD led to liver transplantation involved patients with Caroli disease, which is a rare inherited disorder characterized by cystic dilatation of the bile ducts within the liver. On the other hand, type I–IVa CBD was reported in only 6 patients by Hori et al. (13) and 1 patient by Wang et al. (18). The classifications of these 7 cases included type Ia (n = 1), type Ic (n = 2) and type IVa (n = 4), and their ages ranged from 0.7 to 37.3 years. The grade of liver fibrosis ranges from F2 to F4. The causes leading to liver transplantation were postoperative portal vein thrombosis, postoperative anastomotic stenosis, repeated shunt surgery, recurrent cholangitis and pancreatitis, and slowly progressive postoperative cirrhosis. Many of these causes underwent liver transplantation due to the worsening clinical course after extrahepatic bile duct resection and hepaticoenterostomy although these CC patients are not indicated for liver transplantation at the time of initial surgery. We evaluate that our case was not indication for liver transplantation at the time of diagnosis. Liver transplantation may be avoided with appropriate therapeutic intervention in many of the liver fibrosis patients with CC. However, there are various reports of liver cirrhosis in the neonatal period and reports of the progression of postoperative liver fibrosis (13); thus, careful postoperative follow-up is required. This patient's condition at the time of admission suggested liver fibrosis, and there was concern about the progression of the disease in the perioperative period; thus, hepatoprotective therapy with minimally invasive drainage was performed first. In our case, hepatic deviating enzymes were improved by blood test, but fibrosis of F2–3 remained histologically, and ammonia was not completely improved. This may suggest that normalization of hepatic deviant enzymes does not necessarily indicate complete recovery of liver function. Ammonia was thought to have a stronger correlation with histological evaluation than hepatic enzymes, and it was thought that ammonia should be measured during follow-up. Ishimaru et al. reported a case with postoperative liver cirrhosis that improved with standard surgical strategies (19). Some CBD patients with liver cirrhosis at surgery may also be able to avoid surgery with standard surgical treatment, but malnutrition—represented by hypoalbuminemia and a decreased liver function—may affect the progression of postoperative liver cirrhosis and outcomes in postoperative period as well as the perioperative period (20). Oral nutrition combined with branched-chain amino acids was given for liver cirrhosis. Recovery of liver function and improvement of nutritional status associated with appropriate amino acid supplementation may have had a positive impact on postoperative uneventful outcomes. Immnonutrition is useful for improving postoperative outcomes in adult surgical diseases (21), but it seems that appropriate perioperative nutritional management and determination of surgical timing are also important in the pediatric field. Laparoscopic cholecystostomy followed by laparoscopic cyst excision is a useful and safe procedure for the treatment of CBD patients whose symptoms do not improve (22). A hepatoprotective treatment strategy, such as two-stage surgery for CBD patients with liver cirrhosis—as in our case—may also help reduce postoperative complications and improve postoperative outcomes in comparison to initial radical surgery.