Hiroo Takehara

Relation of patient age to premalignant alterations in choledochal cyst epithelium: Histochemical and immunohistochemical studies

Forty patients with choledochal cysts, 17 children and 23 adults, were treated by cyst excision in this institution (1972 to 1982). Histochemical and immunohistochemical studies were performed on tissue from the walls of these 40 cysts. The type and degree of histologic changes observed were found to correlate with patient age at time of cyst excision. Specimens from four patients less than two years of age at time of excision revealed epithelial desquamation, fibrosis, and minimal evidence of inflammation. Among specimens from 13 patients, 4 to 15 years of age, only two had an intact epithelial lining. Marked inflammation and intramural glandular structures were observed. Specimens from 23 patients, more than 15 years of age at the time of cyst excision, showed marked acute or chronic inflammation and destruction of the epithelial lining. In this age group, intramural glandular structures with goblet and argyrophil cells were regularly observed within the cyst walls, and gastrin and somatostatin-like immunoreactivity was detected in cells of these metaplastic glands. In association with these changes, in five of the 23 patients in the oldest age group, adenocarcinomas were observed in the cyst walls. These studies establish the increasing rate of epithelial metaplasia in the walls of choledochal cysts with advancing age, and suggest that these changes are a continuing process with malignancy one of the end results.

Histochemical and immunohistochemical studies on development of biliary carcinoma in forty-seven patients with choledochal cyst—Special reference to intestinal metaplasia in the biliary duct—

Histochemical and immunohistochemical studies on 47 consecutive specimens excised for choledochal cyst were performed to clarify possible metaplastic changes of the biliary duct in relation to carcinogenesis. An anomalous arrangement of the pancreaticobiliary ductal system was observed in all 39 cases examined. Among the 47 patients, 5 (10.6 per cent) had biliary carcinoma. 27.3 per cent mucous gland, 13.0 per cent goblet cell and 9.5 per cent argyrophil cell in 23 children. On the other hand, 81.8 per cent exhibited mucous gland, 41.7 per cent goblet cell and 27.3 per cent argyrophil cell in 24 adults. These metaplastic changes seemed to be an intestinal metaplasia and increased with age. Immunoreactive-gastrin or-somatostatin were evident immunohistochemically in 4 adults. These findings confirmed that intesinal metaplasia may develop in the biliary duct in cases of choledochal cyst. Although direct evidence between intestinal metaplasia and the development of biliary carcinoma was not found, reflux and stasis of pancreatic enzymes in the biliary duct may relate to the development of intestinal metaplasia and be an important factor related to the carcinogenesis of choledochal cyst.

Choledochal cyst: Anomalous arrangement of the pancreaticobiliary ductal system and biliary malignancy

Congenital biliary dilatation (CBD) or choledochal cyst has previously been considered a rarity; however, the number of cases reported in the literature has been increasing. It is generally accepted that there is a predominance in Japanese, as more than one‐third of the cases are from Japanese literature.

Report on the first annual survey of home parenteral nutrition in Japan

An annual survey of the current national status of home parenteral nutrition (HPN) in Japan was begun in 1991, with a total of 231 registered patients from 142 institutions providing adequate data for evaluation and analysis. HPN was given for an average of 683±764 days to 93 patients with malignant diseases and 138 with benign disease, including 53 with inflammatory bowel disease and 79 with short bowel syndrome, 107 (46.3%) of whom were successfully rehabilitated. By the end of 1990, 61% of the patients investigated were still on HPN, 7% had already completed HPN, and 26% had died, the deceased accounting for 54% of the patients with malignant disease and 7% of those with benign diseases. A total of 321 catheters had been used by all 231 patients, the most common being the subcutaneously implanted type, accounting for 33% of all catheters. By the end of 1990, 32% of these 321 catheters were still in place, 18% had been removed on the termination of HPN and 44% had been removed due to complications of total parenteral nutrition, including 20% for catheter fever. Rehospitalization was required in 62% of the patients, the cause being HPN-related in 21% of the total patients. Metabolic complications were experienced by 60% of the patients, but none of these were severe although 6% required hospitalization. Thus, the total population of HPN patients and the success rate of rehabilitation in Japan were close to those reported in Europe, while the indications for HPN and its outcome were similar to those documented in the USA OASIS report, except that the incidence of rehospitalization from HPN-related causes in the Japanese survey was lower.

Left diaphragmatic hernia associated with lower esophageal atresia

A case of combined left diaphragmatic hernia and lower esophageal atresia, which is an unusual occurrence, is described. The preoperative diagnosis was difficult because of the presence of an abnormal air bubble, thought to be the stomach, in the chest. The surgical management is discussed.

Presumed hypoplastic intrahepatic portal system due to patent ductus venosus: Importance of direct occlusion test of ductus venosus under open laparotomy.

Continuous galactosemia in the absence of enzyme deficiency is usually due to congenital dysplasia of the portal system. In this study, we report on a 1-year-old boy with presumed severe hypoplasia of the intrahepatic portal system due to a patent ductus venosus (PDV) presenting with hypergalactosemia, hyperammonemia, and markedly elevated serum levels of total bile acids (TBA). The patient underwent surgical ligation of the PDV following the direct occlusion test under open laparotomy, and his clinical condition was dramatically improved after surgery.

A case of biliary stricture caused by cavernous transformation of the portal vein

A case is reported of extrahepatic obstruction of the portal vein with the unusual complication of obstructive jaundice that resulted from compression of the common bile duct caused by cavernous transformation of the varicose network. It is surgically impossible to repair choledochal stenosis resulting from enlarged varices in the vicinity of the common bile duct. In this case, internal drainage from the biliary duct to the duodenum using a silastic tube and a reservoir implanted in the subcutaneous space successfully eliminated the jaundice.

Congenital segmental dilatation of the colon: report of a case and review of the literature

Segmental dilatation of the colon is a rare lesion. A patient with this lesion complicated by chronic constipation from birth is described. Although the clinical picture and radiographic studies are indistinguishable from Hirschsprungs disease, anorectal manometric studies and a rectal mucosal biopsy are of paramount importance for the diagnosis of this condition. This disease can be cured by resection of the dilated segment.

Intra-operative total enteroscopy for the management of Peutz-Jegher's syndrome.

We present a case of Peutz‐Jeghers syndrome in an 18 year old female who was followed for gastrointestinal polyps for 13 years from the age of 5 years. The patient was treated four times with surgical or endoscopic polypectomy for gastrointestinal polyps. At the age of 14 years, a combined surgical and endoscopic approach for the management of Peutz‐Jeghers syndrome was carried out. A large polyp of the ileum required enterotomy for its removal, and another smaller polyp of the upper jejunum was identified and removed by intra‐operative total enteroscopy via the anus. Intra‐operative enteroscopy allows one to identify polyps that would previously have been missed. A more complete polypectomy can be performed using this technique, allowing the patient with Peutz‐Jeghers syndrome a longer interval between laparotomies and a reduction in symptoms attributed to polyps.

Refractory pediatric nonrhabdomyosarcoma soft tissue sarcoma associated with ectopic production of beta hCG and hypercalcemia induced by PTHrP.

A 3‐month‐old male with a mass on the right side of his back was admitted to our hospital. The tumor was a pathologically high‐grade nonrhabdomyosarcoma soft tissue sarcoma (NRSTS). Treatment included subtotal tumor resection followed by chemotherapy. Elevation of serum beta hCG and hypercalcemia with detection of PTHrP was associated with tumor progression. The tumor was refractory to multiagent chemotherapy, and the patient died of the disease at 22 months of age. This case is novel in demonstrating a beta hCG secreting refractory NRSTS. Pediatr Blood Cancer 2011; 57: 1244–1246.