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Biomedical subjects

T Todani

Publications and source records attributed to T Todani.

At least 55 records · Page 3Linked to original sources

Wilms' tumor associated with Moyamoya disease: a case report.

A high incidence of congenital malformations with Wilms' tumor had been recognized lately. Aniridia, hemihypertrophy, chromosome deletion, urinary tract anomalies, and Wiedemann-Beckwith syndrome have often been observed in patients with Wilms' tumor. However, Wilms' tumor associated with Moyamoya disease, which refers to a stenosis of the distal portion of the carotid artery with an abnormal network in the brain base, has never been reported. In this report, a male child aged 28 mo. with Wilms' tumor associated with Moyamoya disease is presented.

Arterial Occlusive Diseases↗

Cylindrical dilatation of the choledochus: a special type of congenital bile duct dilatation.

Cylindrical dilatation of the choledochus develops in 20% of patients with congenital bile duct dilatation and usually has acute-angled unions of the pancreatobiliary ductal system. Symptoms generally develop in patients over 1 year of age. The patients frequently complain of abdominal pain, vomiting, and fever as in those with acute pancreatitis. Ultrasonography and infusion cholangiography are the most useful tools in making a correct diagnosis. A high amylase level in the bile caused by the refluxing of pancreatic juice through anomalous ductal unions is commonly observed. This is responsible for biliary perforation in infancy and possibly carcinoma arising in the bile duct. The amylase concentration in the serum at the time of epigastric pain often is high, which leads to the diagnosis of acute pancreatitis. However, evidence of pancreatic inflammation is seldom noted. Accordingly, amylase in the bile may enter the circulating blood through the denuded epithelium or sinusoids of the liver. Excision of the whole extrahepatic duct along with hepaticoenterostomy would be essential for the treatment of cylindrical dilatation of the bile duct, especially when an anomalous ductal union is present.

Adolescent↗

Congenital choledochal cyst with intrahepatic involvement.

We reviewed congenital choledochal cyst with intrahepatic involvement in 38 patients aged 52 days to 29 years. Dilatations were classified into the following three forms, based on the shape of the extrahepatic and intrahepatic bile duct: cystic-cystic, cystic-cylindrical, and cylindrical-cylindrical. Stenosis of the hepatic duct near the hilum probably does not cause dilatation of the intrahepatic duct. The anomalous arrangement of the pancreatobiliary union seems to determine only the form of extrahepatic dilatation, and cystic dilatation of the intrahepatic duct may be caused by some other factors. Total excision of the extrahepatic bile duct with the creation of a wide anastomotic stoma apparently is the treatment of choice. Hepatectomy would be recommended for some patients with the cystic-cystic form.

Adolescent↗

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.

Adolescent↗

Congenital oesophageal stenosis due to fibromuscular thickening.

Three patients with fibromuscular oesophageal stenosis are reported. The onset was usually before 6 months of age; earlier than that of stenosis due to tracheobronchial remnants. The localization of the stenotic area varied from the distal to the mid-oesophagus. Extramucosal myotomy seemed to be the treatment of choice for this lesion, particularly in the mid-oesophagus involved diffusely, because of its good effectiveness, less invasiveness, and simplicity.

Child↗

Glucagon administration for the treatment of postoperative cholangitis after Kasai's operation--a preliminary report.

Clinical application of glucagon in postoperative ascending cholangitis after Kasai's operation for biliary atresia is reported. Therapeutic use of glucagon in two patients succeeded in treating ascending cholangitis, but failed to prevent progression to hepatic fibrosis. Prophylactic use in two patients prevented development of ascending cholangitis. Prophylactic glucagon administration appears to improve the prognosis of patients with biliary atresia who have undergone a Kasai's operation.

Bile Ducts↗

Inflammatory stenosis of the whole extrahepatic bile duct in a child: a case report.

A female child, 5 years and 5 months of age, who had had longstanding obstructive jaundice with hepatic dysfunction from the age of 6 months, underwent Y-hepaticojejunostomy according to Roux after dissection of the entire extrahepatic duct with severe periductal fibrosis. At that time, liver biopsy already showed progress to biliary cirrhosis with prominent ductal proliferation in Glisson's triad. Although good bile drainage to the intestine was obtained early after the operation, 2 years elapsed until disappearance of jaundice and ascites with improvement of liver function. The patient is now 13 years old and doing well with moderately impaired liver function. No oesophageal varices have been seen by endoscopy lately. The clinical course and data are suggestive of an abortive form of biliary atresia.

Child, Preschool↗

Effects of hepatic periarterial neurectomy (HPN) on the treatment of biliary atresia.

Thirty one patients with biliary atresia have undergone Kasai's operation in our clinic during the past 10 yr. Three of the patients died from postoperative complications. Of 12 patients who underwent Kasai's operation only five (41.7%) showed good excretion of bile. Two of them, 16.7%, have survived without jaundice for more than 8 yr. Of 16 patients who underwent hepatic periarterial neurectomy (HPN) combined with hepatic portoduodenostomy, 11 (68.8%) showed good bile excretion. Seven of them, 43.8% are alive and well for period between 6 mo and 5 yr 6 mo postoperatively. Although improvement in the technique for boring the porta hepatis may have contributed to these good results, we think that the addition of hepatic periarterial neurectomy to Kasai's operation gives even better results. According to our experimental studies, hepatic periarterial neurectomy resulted in increased bile flow in the early postoperative period and thus seemed to be of value in preventing postoperative ascending cholangitis.

Bile↗

Rectal carcinoma with a large amount of mucin production in childhood--report of a case.

A 10-year-old boy with rectal carcinoma underwent an abdominoperineal resection with postoperative radiotherapy and chemotherapy. The tumor showed a moderately differentiated adenocarcinoma with a large amount of mucin production. Five years later, obstructive hydronephrosis due to postoperative radiotherapy developed and uretero-ileo-cysto-neostomy was performed after transient bilateral nephrostomies. He is now 17 years of age, 7 years after the initial operation, and doing well with nearly normal renal function. Colorectal carcinoma in childhood is very rare and has a poor prognosis, which seems due to the high incidence of mucinous adenocarcinoma and delay in early diagnosis. Besides polyp carcinoma with low grade malignancy, an aggressive abdominoperineal resection with sigmoid colostomy is the treatment of choice as in adults. Postoperative radiotherapy under a total of 3000 rads is also recommended for a better chance of cure. The effectiveness of pre- and postoperative chemotherapy is still doubtful.

Adenocarcinoma↗

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.

Adolescent↗

Portal hypertension after successful Kasai's operation for biliary atresia--special reference to esophageal varices.

Thirty-four patients with biliary atresia have undergone Kasai's operation in our clinic during the past 12 years. Of 21 patients who showed good bile excretion postoperatively, 8 are surviving more than 2 years later. 4 of these survivors and 2 who died of hepatic failure after operation which succeeded in achieving bile excretion, developed portal hypertension with esophageal varices. More than 30 similar patients have been reported in the literature. Esophageal varices after a successful Kasai's operation developed in one fourth of the patients who were expected to be cured. Half of these went on the hemorrhage. Portal hypertension due to biliary hepatic fibrosis could be divided into acute and chronic types. The acute type had a poor prognosis due to concomitant severe postoperative cholangitis, whereas, almost all patients with the chronic type survived, if the esophageal varices were well controlled conservatively or operatively. Transthoracic esophageal transection with paraesophagogastric devascularization seems to be the treatment of choice, and splenectomy may be added through the diaphragm only in patients with hypersplenism. Major or minor shunting procedures should be avoided because they decrease the blood flow to the liver.

Biliary Tract↗

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 extra-hepatic 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.

Adenocarcinoma↗

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 frequent 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.

Adenocarcinoma↗

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.

Adolescent↗