Takuji Todani

Congenital bile duct cysts: Classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst

Congenital bile duct cysts are observed in any part of the bile duct from the liver to the duodenum. Reports of cases of cancer arising from it are increasing. Excision of the choledochal cyst seems to be the treatment of choice and partial resection of the intrahepatic cyst followed by intrahepatic cystoenterostomy at the porta hepatis is necessary for type IV-A cysts.

Carcinoma arising in the wall of congenital bile duct cysts

The incidence of carcinoma arising in the wall of the congenital bile duct cysts is much higher than previously assumed. The authors report 4 such cases of primary and secondary carcinomas and review their clinical features through the similar 59 cases in the literature. Of the 63 cases, the average age was much younger, at least several decades, compared with cases of extrahepatic carcinoma without bile duct cysts. The female‐male ratio was 2.5:1. Racial preponderance was also observed, namely, the majority were Japanese. Additionally, many patients previously received various internal drainage procedures, especially choledochocystoduodenostomy. As the treatment, primary excision of the extrahepatic bile duct cyst seems to give the best results because it can avoid ascending cholangitis and prevent development of carcinoma. However, carcinoma still can arise in the intrahepatic bile duct cyst, which cannot be removed at the present time.

Anomalous arrangement of the pancreatobiliary ductal system in patients with a choledochal cyst

An anomalous arrangement of the pancreatobiliary ductal system is usually observed in patients with a congenital choledochal cyst and is represented by a long common channel distal to the pancreatobiliary junction. According to the angle of the pancreatobiliary junction, anomalous junctions can be classified into four groups: right angle, acute angle, complex union, and normal union (presented here in the order of their incidence). The right-angle type tends to have cystic dilatation of the choledochus, whereas the acute-angle type generally has cylindrical dilatation. However, the grade and length of the stenosis on the distal part of the choledochus tend to determine the type of choledochal cyst. Cystic dilatation is usually observed in patients with high-grade and long stenosis, whereas cylindrical dilatation is seen in those with low-grade and short stenosis. Patients with an acute angle junction seem to only suffer from abdominal pain, and those in the complex union group tend to have jaundice and an abdominal tumor. A high amylase level in the bile within the cyst is observed frequently. In patients with obstructive jaundice, however, the amylase level is usually not elevated, because pancreatic juice cannot enter the choledochus.

Management of congenital choledochal cyst with intrahepatic involvement.

The clinical features and surgical treatment of 75 patients with choledochal cysts with intrahepatic involvement (Type IV-A) were evaluated. Seventeen were in this series and 58 were reviewed from the literature. It was of interest that the incidence of Type IV-A choledochal cysts was nearly 30%, considerably higher than had been previously assumed. This type is commonly found in older children and young adults. Abdominal pain and fever indicative of biliary tract infection are more frequently seen than in patients with Type I, and a mass is not commonly palpable. Surgical treatment of Type IV-A must achieve bile drainage from the intrahepatic cysts as well as from the choledochal cyst. For this reason, as well as the prevention of later development of cancer in the wall of the cyst, hepaticoenterostomy at the porta hepatis with partial resection of the wall of the intrahepatic cyst (or partial hepatectomy if possible) combined with excision of the intrahepatic cyst is recommended as the procedure of choice for type IV-A cysts.

Reoperation for congenital choledochal cyst

A reoperation after excisional procedure was carried out in seven cases due to early or late postoperative complications. Of the 12 patients with early complications, four underwent relaparotomy due to anastomotic leakage and bleeding. Late complications were seen in nine patients with recurrent cholangitis caused by an anastomotic stricture, and three patients with intrahepatic involvement required a reoperation several years after the initial surgery. Recurrent cholangitis after biliary reconstruction mainly occurs due to an anastomotic stricture of the hepaticoenterostomy. There was no significant difference in the results between hepaticoduodenostomy and hepaticoje-junostomy over a long follow-up period. A wide anastomotic stoma that permits free drainage of bile into the intestine is imperative to the prevention of cholangitis, and can be created by an incision extending along the lateral wall of both the hepatic ducts with a hepaticoenterostomy at the hilum. This procedure is obviously necessary in all patients with or without intrahepatic involvement. Carcinoma of the intrahepatic ducts and the retained distal choledochus have rarely developed in patients undergoing cyst excision followed by biliary reconstruction. Complete excision of the whole extrahepatic bile duct could prevent carcinoma arising in the distal choledochus, although it could not prevent carcinoma arising from the intrahepatic ducts. However, patients with carcinoma of the intrahepatic duct were reported to have had symptoms of biliary stricture for a long time since the cyst excision. Bile stagnation in the intrahepatic ducts is possibly responsible for the development of carcinoma. A wide anastomosis resulting in free drainage of bile appears to be essential to the prevention of carcinoma arising in the intrahepatic ducts after cyst excision.

Biliary complications after excisional procedure for choledochal cyst

During the last 25 years, from 1969 to 1994, the authors treated 97 choledochal cysts by surgical excision. Biliary reconstruction consisted of 67 hepaticoduodenostomies and 30 hepaticojejunostomies. The common hepatic duct was the site of anastomosis in 9 of the duodenostomies and 13 of the jejunostomies and of the bifurcation of the hepatic ducts in 58 duodenostomies and 17 jejunostomies. Reoperation was required in 10 cases because of recurrent cholangitis with intrahepatic gallstones. Biliary strictures were responsible for the cholangitis in 9 children with anastomoses at the level of the common hepatic duct and in 1 with an anastomosis at the level of the bifurcation. These results suggest that biliary complications develop because of anastomotic stricture or primary ductal stricture, and may be minimised by the creation of a wide anastomosis, which is best accomplished at the hepatic hilum.

Frantz's tumor: A papillary and cystic tumor of the pancreas in girls

Four girls with Frantzs tumor, a papillary and cystic tumor of the pancreas, are studied and discussed in comparison with 112 cases in the literature, including 58 Japanese cases. The neoplasms occur predominantly in girls and young women. Up to recently, the tumors have possibly been misclassified as nonfunctioning islet cell tumor or carcinoma, acinar cell carcinoma, papillary cystadenocarcinoma, or pancreatoblastoma according to microscopic findings and frequently have been managed with aggressive surgery such as pancreatoduodenectomy. The tumors are well encapsulated and the cut surfaces show characteristically solid and hemorrhagic-necrotic patterns. Ultrasonography and CT scan are the most useful tools for the diagnosis. The neoplasms usually behave like a very low-grade malignancy, so that complete removal is the treatment of choice for the tumor arising anywhere in the pancreas. Immunochemical and electron microscopic studies can differentiate Frantzs tumor from other neoplasms and also suggest that the tumors originate from primordial cells or multipotential stem cells capable of differentiating into both exocrine and endocrine lines.

Hepaticoduodenostomy at the hepatic hilum after excision of choledochal cyst

During the past 11 years, 30 patients, aged 27 days to 25 years, underwent excision of choledochal cyst. Reconstruction of the biliary tract was performed by two techniques: hepaticoduodenostomy in 19 patients and Roux-Y hepaticojejunostomy in 11. The technique of hepaticoduodenostomy consisted of transection of the common hepatic duct at the hilum with an incision extending approximately 5 mm along the lateral wall of both the hepatic ducts to permit a wide anastomotic stoma. There was no mortality. Postoperative cholangitis occurred in five patients, four of whom were in the hepaticoduodenostomy group. All episodes except one responded to antibiotics and have resulted in no demonstrable hepatic dysfunction. Intestinal bleeding occurred in one Roux-Y patient and postoperative intestinal obstruction in another. There was no significant difference in the results of these two procedures in the follow-up period (average length 4 years, 3 months). The hepaticoduodenostomy with a wide stoma at the hilum is advocated because (1) it has significant capability of preventing cholangitis, which has been thought to be the primary objection so far, (2) it creates a better physiologic state, and (3) it may be associated with fewer postoperative complications.

Liver volume in children measured by computed tomography

Abstract Liver volume was measured by computed tomography in 54 children and young adults with no history of liver disease. Their ages ranged from 10 days to 22 years. The volume was calculated as follows: (1) the edges of the liver were traced on each scan image and the area was calculated by computer; (2) the areas were summed and multiplied by the scan interval in centimeters. The mean liver volume (± SD) was 178.2 ± 81.9 cm3 in infants (less than 12 months old) and 1114.3 ± 192.9 cm3 in adolescents (more than 16 years old). The mean liver volume in relation to body weight (± SD) was 34.1 ± 5.5 cm3/kg in infants and 20.2 ± 3.1 cm3/kg in adolescents. In general, liver volume increases rapidly in infants, gradually in schoolchildren, and not at all in adolescents. Volumetry might be clinically useful for evaluating the liver function in children and determining the graft size in liver transplantation.

Intestinal atresia due to intrauterine intussusception: Analysis of 24 cases in Japan

Abstract We have presented two cases of intestinal atresia due to intrauterine intussusception and reviewed 22 Japanese cases collected through personal communication. Most of the infants were full-term and without other malformations; they passed meconium, even though the abdominal scout film suggested an ileal atresia. On barium enema, a normal size colon was frequently observed. At operation, the atresia in all cases was single, and cord or gap type, and their prognoses were excellent. A necrotic intussusceptum or polypoid protrusion was seen in the distal bowel. These facts strongly suggest that intrauterine intussusception may occur in a late stage of pregnancy, and cause the impairment of blood supply to the intestine. The presence of occult blood in the meconium is indispensable for the diagnosis of these types of intestinal atresias.