Pancreaticobiliary maljunction: retrospective and nationwide survey in Japan.

Pancreaticobiliary maljunction (PBM) is a congenital anomaly defined as a union of the pancreatic and biliary duct that is located outside the duodenal wall. The Japanese Study Group on Pancreaticobiliary Maljunction and the Committee for Registration enrolled and analyzed 1627 patients with PBM who had been diagnosed and treated from January 1, 1990 to December 31, 1999 at 141 hospitals throughout the country. There were 1239 patients with dilatation of the bile duct (group A) and 388 patients without dilatation (group B). The average age was 24 years in group A and 47 years in group B; the age was significantly higher in group B. The type of confluence between the terminal choledochus and the pancreatic duct has been classified into three types (type a, right-angle type; type b, acute-angle type; and type c, complex type). In group A, type a accounted for 57.9% and was significantly more frequent compared with the other types (type b, 32.4%; type c, 5.6%). In group B, type b accounted for 60.8%, being significantly more frequent compared with the other types (type a, 29.4%; type c, 7.2%). Subjective symptoms, preoperative complications (e.g., liver dysfunction and acute pancreatitis), pancreatic stone, and pancreatic duct morphological abnormality were significantly more frequent in group A. However, the amylase levels in the bile and gallbladder were significantly higher in group B, and the presence of gallstone and morphological abnormality of the gallbladder was significantly more frequent in group B. The occurrence rate of cancer in the biliary tract was 10.6% in group A and 37.9% in group B, being significantly higher in group B. In group A, cancer of the extrahepatic bile duct was seen in 33.6% and cancer of the gallbladder was seen in 64.9%, but gallbladder cancer was present significantly more frequently in the patients with diffuse or cylindrical dilatation, and bile duct cancer was present significantly more frequently in the patients with cystic dilatation. In group B, 93.2% of the patients had gallbladder cancer, and bile duct cancer was found in as few as 6.8%. Against this background Japanese surgeons regard cholecystectomy, resection of the extrahepatic bile duct, and hepaticojejunostomy as standard operations for PBM with dilatation of the bile duct. However, opinion on whether or not the bile duct should be removed in the treatment of PBM without dilatation of the bile duct has been divided among Japanese surgeons. A randomized controlled trial is necessary.

Smallest Todani's type II choledochal cyst.

A choledochal cyst is defined as an isolated or combined congenital dilation of the extra hepatic or intrahepatic biliary tree. Todani and colleagues proposed the five types of congenital choledochal cysts which have gained widespread acceptance. Type II choledochal cyst, a diverticulum of common bile duct, is rarest, and most reported cases of Type II were as large as several centimeters in size. We herein report the case of a small Type II choledochal cyst which was resected at pancreatoduodenectomy for carcinoma of the papilla of Vater. A 58-year-old Japanese male was referred to our hospital for the evaluation of jaundice. Preoperative cholangiogram via the percutaneous transhepatic biliary drainage tube revealed a complete obstruction at the narrow terminal segment. Furthermore, a small diverticular protrusion was demonstrated on the lower part of the common bile duct. The resected specimen showed a 2.2 x 1.7 x 1.2 cm carcinoma of the major papilla, and a deep, 2 mm in diameter and 5 mm in depth, depression on the posterior wall of the common bile duct. The anomalous pancreatobiliary duct was not seen. The deep depression was confirmed microscopically to penetrate the fibromuscular layer of the common bile duct and diagnosed as a Todani's Type II choledochal cyst. To our knowledge, the current case is the smallest Type II choledochal cyst which was completely resected.

Bile duct cancer developed after cyst excision for choledochal cyst.

Oncogenesis after cyst excision for choledochal cyst and suitable surgical procedures for this operation are discussed. The clinical data of 23 patients with cancer of the biliary tree after excision of choledochal cyst reported in the English-language and Japanese literature were reviewed, and data for 1353 Japanese patients with choledochal cyst and/or pancreaticobiliary malunion were analyzed. In the 23 patients reported in the literature, age at cyst excision ranged from 1 to 55 years (average, 23.0 +/- 13.7 years), and cancers were detected at age 18-60 years (average, 32.1 +/- 12.2 years), with intervals between cyst excision and cancer detection of 1-19 years (average, 9.0 +/- 5.5 years). Sites of cancer development were: intrahepatic, six; anastomotic, eight; hepatic side residual cyst, three; and the intrapancreatic duct, six. In the Japanese patients with choledochal cyst and/or pancreaticobiliary malunion, the incidence of cancer associated with primary choledochal cyst and/or pancreaticobiliary malunion was 16.2% (219/1353). The incidence of cancer development after cyst excision in this population, of whom 1291/1353 underwent surgery, was assumed to be 0. 7%. Nearly half of the 23 patients in the literature had undergone inadequate cyst excision. Oncogenesis of cancers after cyst excision is possibly different from that of choledochal cysts.

Co-existing biliary anomalies and anatomical variants in choledochal cyst.

BACKGROUND: Excision is the treatment of choice for choledochal cyst, and free bile drainage is essential to avoid ascending cholangitis. However, anastomosis between the relatively narrow common hepatic duct and bowel (conventional anastomosis) in case of type IV-A cyst, co-existing biliary anomalies and anatomical variations may cause ascending cholangitis resulting from insufficient biliary decompression. METHODS: One hundred and four patients with choledochal cyst were treated by cyst excision. Conventional anastomosis was performed in 22 patients and hilar anastomosis in 82. RESULTS: An anastomotic stricture developed in nine of the 22 conventional anastomosis cases and all required reoperation. Of the 82 hilar anastomosis cases, only one required reoperation for a primary stricture. Co-existing biliary anomalies and anatomical variants were seen in 26 patients: (1) primary stricture in 18; (2) aberrant posterior duct in two; (3) low confluence of the hepatic ducts in two; (4) aberrant hepatic artery in two; and (5) very small bile duct in two. All 26 patients underwent widening of the ductal lumen (ductoplasty) or additional procedures. CONCLUSION: Complete removal of the extrahepatic bile duct and a wide hilar anastomosis is essential to prevent ascending cholangitis. Ductoplasty or additional procedure can be performed only at the hilum.

Conservative management of fistula in ano in infants.

We analyzed the natural course of fistula in ano (FIA) and/or perianal abscess (PAA) in 97 children (male: 90, female: 7) and recommend conservative management in infants. Nearly one-half of these children had no recurrent episodes, and 80% of the infants recovered spontaneously within their 1st year of life. A radical operation was required in only 6 children after 2 years of age. Two-thirds of the children with a PAA did not develop fistula. Of the children who developed FIA, 42% had no recurrence. Although the duration of FIA was relatively longer than that of PAA, 55% of the children were cured within 1 year. PAA and/or FIA in infants is likely to be a period-limited disorder that occurs mainly in infancy and spontaneously resolves within 1 year of life. Fistulotomy or fistulectomy should be avoided in infants.

Esophageal hiatal hernia after omphalocele repair.

Esophageal hiatal hernias (EHH) are probably caused by congenital, traumatic, or iatrogenic factors, although the etiology remains unknown. EHHs may develop after abdominal wall closure for omphalocele or gastroschisis due to the increased intra-abdominal pressure, however, there have been few reports in the literature. We present a case of EHH developing after abdominal wall closure.

The effects of adenosine on the energy metabolism of the reperfused intestine in rats.

The effects of adenosine on energy metabolism in the intestine during reperfusion after intestinal ischemia were examined in rats subjected to intestinal ischemia for 60 min by clamping the superior mesenteric artery, followed by 20 min reperfusion with tested agents. The rats were divided into a control group, a 200 micrograms adenosine group, a 500 micrograms adenosine group, and a 500 micrograms adenine group. Jejunal tissues were taken preischemia, 30 and 60 min post-ischemia, and 20 min after starting reperfusion. Adenosine triphosphate, -diphosphate, -monophosphate, and thiobarbituric acid reactive substances (TARS) of lipid peroxidation were measured by high-performance liquid chromatography or spectrophotometry. The ATP levels in the jejunal tissues decreased extensively 30 min after ischemia, but no further decrease was observed 60 min after ischemia. These levels recovered slowly 20 min after starting reperfusion in the control group, but they recovered significantly in the 500 micrograms adenosine group and moderately in the adenine group, with no significant difference between the 200 micrograms adenosine and control groups. Thus, the effect of adenosine on energy metabolism appears to be dose-dependent. The TARS levels increased significantly during ischemia and reperfusion, but no significant difference was observed between the control and 500 micrograms adenosine groups. In conclusion, adenosine promotes the rapid resumption of ATP levels during reperfusion, but adenine is less effective. Adenosine does not affect lipid peroxidation mediated by free radicals.

Germ-cell degeneration in experimental unilateral cryptorchidism: role of apoptosis.

We investigated the possible involvement of apoptosis in the increased germ-cell degeneration in undescended testes (UDT). Experimental unilateral cryptorchidism was induced in 21-day-old rats, and both testes were removed for in-situ TUNEL staining of apoptotic cells at 1, 3, 7, 10, and 14 days postoperation. A gradual increase in the incidence of apoptosis was seen at 21-28 days of age in the control testes, followed by a decrease thereafter. After 10 days postoperation, the weight of the UDT was significantly lower than that of the contralateral descended testis (CDT) and the controls. However, the weight of scrotal testes in each group was similar. UDTs demonstrated a markedly increased incidence of apoptosis. By 7 days postoperation, the percentage of seminiferous tubules containing apoptotic germ cells significantly increased in UDTs compared with that in CDTs and controls (P < 0.001). Moreover, there was a significant difference in the percentage of seminiferous tubules containing apoptotic germ cells between CDTs and controls (P < 0.01). In addition, an increased incidence of seminiferous tubules containing 8-10 and >10 apoptotic germ cells from 7, 10, and 14 days postoperation in UDTs was detected. In-situ TUNEL analysis demonstrated spermatocytes to be the main type of germ cells affected in all groups. These findings suggest that spermatogenesis decreases not only in the UDT, but also in the CDT, and that the germ-cell degeneration in cryptorchidism took the form of apoptosis.

Carcinoma of the colon in childhood; report of 2 cases expressing p53 without K-ras mutation.

We report on 2 children with colonic carcinoma and also review 62 cases of Japanese children with colonic carcinoma including ours. Although the dismal prognosis in colonic cancer in children is possibly due to the predominance of poorly differentiated carcinoma, there is no significant difference in the 5-year survival rates among well, moderately and poorly differentiated carcinomas in children. Positive staining with p53 in tumor cells was observed in each, but K-ras mutations were not detected in any. Therefore, these carcinomas possibly developed from de-novo carcinoma. The development pathway of colonic carcinoma may relate to the prognosis in children, and be different from that in adults.

Spiral computed tomography with 3-dimensional reconstruction for the diagnosis of tracheobronchial stenosis.

We report on the usefulness of spiral computed tomography (CT) with 3-dimensional (3D) reconstruction in the diagnosis of tracheobronchial abnormalities based on three cases of children with congenital tracheobronchial strictures. Images were reconstructed using a curved planar reformat and shaded surface display. The images obtained from our three cases of tracheal stenosis, subglottic stenosis and tracheobronchial strictures were extremely clear. Spiral CT with 3D reconstruction provides excellent anatomic delineation of the tracheobronchial airway, and is safe and less invasive than tracheobronchography.

Anti-asthma effect of an antiviral drug, acyclovir: a clinical case and experimental study.

BACKGROUND: Although acyclovir (9-(2-hydroxyethoxymethyl) guanine) is an antiviral drug that inhibits DNA polymerase of herpes virus, we have had the experience of an asthmatic patient's peak flow rate being improved by oral administration of acyclovir. OBJECTIVE: The aim of this experiment is whether acyclovir has anti-asthma effects using an asthma model in guinea-pigs. METHODS: The airway response was induced by a single inhalation of calcium ionophore A23187 (2 mg/mL). The airway obstruction was estimated by the ratio of expiration to inspiration time (E/I). The peribronchial eosinophil infiltration and eosinophil influx into bronchoalveolar lavage (BAL) fluid 7 h after the inhalation were also examined. To assess the effects of acyclovir (1, 10, and 100 mg/kg), aminophylline (20 mg/kg) and pemirolast potassium (TBX, 20 mg/kg) on A23187-induced asthmatic response, the drugs were intraperitoneally administered before the inhalation. RESULTS: The immediate airway obstruction was significantly suppressed by acyclovir (10 mg/kg) and aminophylline, whereas different doses of acyclovir (1 and 100 mg/kg) and TBX showed only a small inhibitory effect on the airway obstruction. On the other hand, the peribronchial eosinophilia was most successfully inhibited by TBX. Acyclovir (10 mg/kg) and aminophylline also suppressed the eosinophilia significantly. Furthermore, acyclovir significantly suppressed eosinophil influx into BAL fluid, whereas aminophylline and TBX weakly suppressed the influx. CONCLUSION: These results suggest that acyclovir exhibits not only antiviral but also antiasthma activity.

Liver volume in children measured by computed tomography.

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.

Congenital right diaphragmatic hernias through posterolateral and anterolateral defects associated with extralobar pulmonary sequestration: a case report.

Multiple diaphragmatic hernias in the unilateral diaphragm are extremely rare. The authors report a neonate with diaphragmatic hernias through two defects in the right diaphragm: a posterolateral defect without a hernia sac and an anterolateral defect with one. After excision of the anterolateral hernia sac, each defect was closed. Histology studies showed extralobar pulmonary sequestration in the removed hernia sac. The presence of sequestrated pulmonary tissue indicates the possibility of interference with the closure of the pleuroperitoneal canal and muscularization in the diaphragm, which may result in multiple defects.

Changes of hepatic volume after successful Kasai operation.

The number of long term survivors who have undergone Kasai operation for biliary atresia is increasing, but some have a hepatic dysfunction likely to require liver transplantation in the near future. Hepatic volume possibly reflects whole liver function, and our objective was to assess the changes of hepatic volume after Kasai operation. Ten patients were studied. Ages ranged from 3 to 27 years. They underwent Kasai operation at ages ranging from 50 to 80 days. Liver areas (cm2) on CT images were measured with an image processing and analysis program (NIH Image 1.57). Hepatic volume (cm3) was calculated by summing up the areas of each image and multiplying by slice thickness (cm). After Kasai operation, the size of the liver increased to 1.7-1.9 times the standard volume, and then reduced to normal size around 5 years of age. In the teens, hepatic volume decreased below the standard volume. Segmental hypertrophy accompanying atrophy of other hepatic segments was observed in 9 out of 10 patients; right lobe hypertrophy: 6, medial segment: 2, and lateral segment: 1. Therefore, progressive hepatic atrophy begins in the teens, but is compensated for by segmental hypertrophy.

Bone mineral analysis in patients with biliary atresia after successful Kasai procedure.

Metabolism is probably disturbed in patients with abnormal liver function who have undergone a successful Kasai procedure. We examined bone mineral metabolism in patients who have successfully undergone Kasai procedure. Bone mineral metabolism was examined in 8 patients with biliary atresia after a successful Kasai procedure. Five patients were female and 3 were male. The ages at Kasai procedure ranged from 50 to 80 days, and the follow-up periods ranged from 3 to 27 years after the operation. All patients eat a normal oral diet. We examined plasma levels of 25-OH-D3, 1, 25-(OH)2-D3, Ca and phosphorus (P). Bone mineral content of the lumbar spine (L2-L4) was assessed by dual energy x-ray absorptiometry (DEXA), and the data were expressed as a bone mineral density (BMD). Two patients showed abnormal levels on hepatic function tests. Plasma levels of 1, 25-(OH)2-D3, Ca, and P were normal in all patients. The level of 25-OH-D3 was normal in 7 patients. BMD levels were normal in 6 patients, but low in 2 who had undergone partial splenic embolization and splenectomy, respectively, due to hypersplenism. In long-term survivors of Kasai procedure, measurement of BMD may detect bone mineral deficiency earlier than measurements of serum levels of 25-OH-D, 1, 25-(OH)2-D, Ca and P.

Standard splenic volume in children and young adults measured from CT images.

The number of children and adolescents with hypersplenism is increasing as the number of long-term survivors undergoing successful Kasai operation for biliary atresia increases. The aim of this study was to determine the standard splenic volume in normal children and adolescents. We measured the splenic volumes with computed tomography (CT) images obtained from 49 Japanese children, adolescents, and young adults, ranging from 9 days to 25 years of age, and from 3.0 kg to 89.0 kg of body weight. The best correlation was observed between splenic volume (SV: cm3) and body weight (BW: kg) with a power curve regression; SV = 6.516 BW0.797, r = 0.924. The splenic volume and age (AG: years) also correlated well on a power curve; SV = 41.879 AG0.411, r = 0.897. The ratio of the splenic volume to the body weight (SV/BW: cm3/kg) decreased with age; from 4.5 cm3/kg at 1 month to 2.4 cm3/kg at 25 years of age, according to an exponential curve; SV/BW = 4.473e-0.026AG, r = 0.593. The above formulas are thus considered to be clinically useful, especially in the assessment of splenic size in children with hypersplenism both before and after partial splenic embolization.

Ductoplasty for an aberrant hepatic duct in a choledochal cyst.

The confluence of the right and left hepatic ducts at the hepatic hilum frequently shows normal anatomic variations. Choledochal cysts (CC) are also accompanied by similar variations, and devices for free drainage of bile are occasionally required in biliary reconstruction. We present a CC that had an aberrant posterior branch of the right hepatic duct draining into the distal common hepatic duct. A capacious hepaticoduodenostomy at the hilum was performed after joining the hilar and aberrant ducts.

[Cancer arising in choledochal cyst and management].

Biliary cancer develops in 20-30% of the patients with choledochal cyst and pancreatobiliary malunion. Some bile acid fractions and refluxed pancreatic enxymes into the bile duct is probably responsible for carcinogenesis. Cancer often develops in the extrahepatic bile duct and gallbladder, and rarely in the intrahepatic duct. In cystic dilatation, cancer often occurs in the common bile duct, while in diffuse or non-dilated type it occurs in the gallbladder. Cancer usually occurs in younger patients than does biliary cancer in general population, and the average age is in the 40s. The risk of malignancy in cysts with internal drainage is higher than that in primary cysts, and early removal of the retained cyst should be performed as quickly as possible. Although the prognosis of biliary cancer is usually dismal, aggressive procedures are recently gaining better results than that by conventional methods. The prevention of cancer is the procedure of choice by early excision. Removal of the whole extrahepatic bile duct is necessary, even in case of malunion with no biliary dilatation. Cancer rarely arises in the intrahepatic duct after excisional surgery, due to long standing biliary stricture. Wide anastomosis with ductoplasty should be essential. Cancer also occurs in the remnant duct. Excision of the distal duct in the pancreas is also necessary.