Immunohistochemical observations were performed by the whole-mount method and by electron microscopy in the congenital aganglionosis rat intestines. The following results were obtained. 1. In the ganglionic segment, the lattices of Auerbach's plexus showed a relatively large irregular pattern, and VIP positive nerve cells and fibers were clearly observed there. 2. In the transitional zone, only nerve fibers were observed and VIP positive fibers distributed to anal side, forming neuro-muscular junctions. 3. The aganglionic ileum had very few nerve fibers and was considered to be virtually denervated.

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The purposes of this study are to the clarify the activation mechanism and dynamics of pancreatic enzymes refluxing into the biliary truct with an anomalous pancreatico-biliary ductal junction. Trypsin, pncreatic-secretory-trypsin-inhibitor (PSTI) , and elastase in bile was measured by RIA, benzoyl-arginine-p-nitroanilide and succinyl-trialanin-p-nitroanilide. All of these enzymes were activated. It seems that enterokinase played an important role in that mechanism. Bile acid, infectious bile and PSTI were not significant.

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Some of the patients with biliary atresia (BA) even after successful operation exhibit various grades of hepatic dysfunction which often lead to serious consequences. Therefore, functional evaluation of the liver becomes very important in the follow up of this disease. However, satisfactory evaluation test has not yet been established. Indocyanine green (ICG) has been widely used to estimate the functional reserve of adult liver. We applied the ICG test to the functional evaluation of liver in BA patients. Nineteen postoperative BA patients were studied. All of them underwent hepatic portojejunostomy. Their age ranged from 2 to 9 years, (8 males, 11 females). Nine were free of jaundice (Group l), 7 were slightly jaundiced (Group II), and 3 were continuously jaundiced (Group III). Forty-four volunteers of comparable age and sex were chosen for controls. After intravenous injection of ICG Imp/kg, plasma concentration of ICG was measured sequentially, and initial plasma clearance rate (ICG-K_1.0) was calculated by microcomputer. The following results were obtained : (1) ICG-K_1.0 of healthy children with ages of 2-5 years old and 5-10 years old were 0.330 ± 0.095 and 0.327 ± 0.092 respectively, therefore normal range of ICG-K_1.0 was determined as 0.19 (mean-1.5SD) or above. (2) ICG-K_1.0 of Groups I, II and III were 0.262±0.073, 0.150±0.060 and 0.054± 0.011, respectively. There were significant differences among those groups. (3) All 9 patients whose ICG-K_1.0 were below normal had esophageal varives, and 7 had a past history of gastrointestinal bleeding, although two of 10 patients whose ICG-K_1.0 were within normal range had esophageal varices and none had a history of GI bleeding. (4) Five of 9 patients whose ICG-K_1.0 were below normal had low serum albumin levels, and all had low prealbumin levels. However, out of the 10 patients whose ICG-K_1.0 were within normal range, none had lower levels of serum albumin and prealbumin. In conclusion, ICG test using K_1.0 was confirmed to be reliable estimator for postoperative liver function in BA patients.

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This is a review of 1350 pediatric patient who under-went surgical procedures at surgical division of Asahi General Hospital during the period from 1977 to 1987. Among those patients, approximately 90% had surgery for either inguinal indirect hernia or acute appendicitis. The remaining 10% includes patients with intussusception, trauma and others. The number of operations in children is gradually increasing at our hospital. Although hospitals without pediatric surgical department cannot handle complicated problems, these data clearly demonstrated the important role of general surgeons in a community hospital in the treatment of pediatric patients.

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Rectovaginal separtion by anorectoperineoplasty (modifield Burrington procedure) was carried out in five girls, which a range of ages from 6 to 15 Years old without temporary colostomy. Four types of perineal skin flap were created and utilized to obtain an adequate degree of separation of the anus and vagina. The long-term functional and aesthetic results after two to three years have been excellent in four patients. However one patient who had poor rectal contience prior to this operation and associated anomalies of the spine, upper limbs and perineum have had the difficulty of bowel control even after the surgery.

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Postoperative courses of eleven splenectomized children who suffered from idiopathic thrombocytopenic purpura, hereditary spherocytosis, hemolytic anemia, Hodgkin's disease and idiopathic portal hypertension were followed up for 1-13 years after splenectomy. Although no death from infectious desease occurred, severe infectious episodes including fulminant hepatitis, pyothorax, DIG or meningitis were experienced by the children who did not get prophylactic antiinfectious treatment during these periods. A case of hereditary spherocytosis encountered streptococcus infection after terminating two year medication of vicilin G. Oral intake of Bacta 1g/day or vicilin G 500,000 unit/day for more than 2 years after splenectomy are considered reasonable as prophylactic medication to avoid severe infection.

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Acute appendicitis in children is often accompanied by perforation, and complication such as wound infection frequentry require prolong hospitalization. We encountered 178 children with acute appendicitis between 1979 and 1988 and have performed delayed wound closure since 1983 in those with gangrenous or perforated appendicitis in danger of wound infection. In children with gangrenous appendicitis. no difference was observed in the incidence of wound infection or the hospitalization period between the group treated by primary wound closure and that treated by delayed closure. In children with perforated appendicitis, the incidence of wound infection was significantly lower in the delayed wound closure group (1/13, 8%) than in the primary closure group (12/26, 46%), and the hospitalization period was slightly shorter in the former group (15.0 days) than in the latter group (21.2 days).

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During the past seventeen years, we have experienced twenty five cases of peptic ulcer disease in neonates at Tokyo Metropolitan Kiyose Childen's Hospital. In eighteen cases (72.0%), initial symptom appeared within forty eight hours after birth, and the majority of babies (72.0%) were healthy fullterm without apparent predisposing factors. The presenting symptoms were hematemesis and melena in fifteen (60.0%), melena in twelve (48.0%), and vomiting in eight (32.0%). Gastric ulcers were multiple, shallow in most of the cases, and the findings were compataible with acute gastric lesions (AGL) or acute gastric mucosal lesions (AGML). Emergency endoscopy was done on 24 cases and was an excellent procedure to define peptic ulcer. Upper gastrointestinal series were performed in 14 cases, but were not valuable for diagnosis except one case. Marked dilatation of the stomach with gastric outlet obstruction was a signifcant radiological finding for suspecting peptic ulcer disease. Feedings following the short period of gastric drainage were well tolerated, and treatment with antacids or H2 antagonist was not necessary if not accompanied with reflux esophagitis.

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A case of intraduodenal choledochal cyst in a 14-year-old male was reported. Intraopertive cholangiogram and pancreaticogram revealed the cystic dilatation of the common channel of pancreaticobiliary ductal system and the fusiform dilatation of the choledochus. Transduodenal resection of the cyst and resection of the gallbladder and the choledochus were achieved. 35 cases in the literature were reviewed.

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A ten-year-old girl was admitted to our hospital because of intestinal obstruction. Laparotomy showed dissemination of recurred yolk-sac carcinoma of the ovary, which had been resected 9 months before. At the second operation, the disseminated tumors were resected but the histological examination revealed only mature teratoma without any malignant cells. The patient had received the chemotherapy of PVB regimen after the first operation. She has been free from disease for 2years now since the second operarion. After dissemination of the relapsed tumor cells, the chemoterapy might have killed only malignant immature cells, leaving benign mature teratoma at the place.

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Three cases of congenital choledochal dilatation in one of monozygotic twins were reported. PTC could not reveal an anomalous pancreatco-biliary ductal junction in Case 1, but Case 2 and Case 3 had the abnormality in the biliary system. The types of choledochal dilatations were varied (Ex.Cylindrical, Cystic, etc.) All cases underwent resection of choledochal Cyst and hepaticoduodenostomy. The other partners of the twins were healthy without choledochal dilatation. Genetic factors may have little importance for the etiology of the disease.

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Copper deficiency in a 2-year-old boy with total intestinal aganglionosis is reported. Proximal jejunostomy was created just below the Treitz ligament. TPN had been performed for prolonged period of time since birth. As for micronutrients, iron, zinc, iodine and copper were administered in TPN solution. At 1 years and 8 months of age, the amount of copper in TPN was reduced from 26μg/kg/day to 13μg/kg/day. After 2 months, progressive anemia, leukopenia and neutropenia developed. His redcell count was 3,400,000/mm3 despite of frequent blood transfusions. White blood cells fell down to 2600/mm3 with neutropenia (880/mm^3) at 2 years and 2 months of age. The serum copper concentration was 61μg/dl (normal 1 70-150μg/dl). The amount of copper in TPN was added to 54μg/kg/day at 2 years and 4 months of age. Subsequently progressive anemia, leukopenia and neutropenia improved. The radiographic findings of metacarpi showed osteoporosis. The amount of copper excretion in jejunal fluid ranged from 50 to 80μg/day even in the copper deficient period. In this case, copper deficiency might have been partly due to excessive excretion of copper from jejunostomy. Therefore frequent monitoring of serum copper concentration could be necessary, particularly in a case with long standing jejunostomy.

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Only 11 cases of bilateral Bochdalek hernia and 13 cases of Bochdalek hernia associated with the thoracic kidney have been reported to date. We recently treated 2 very rare cases of bilateral Bochdalek hernia associated with bilateral thoracic kidneys, and both of them survived. In this report, these cases were described, and the etiologic reletionship between the two conditions was discussed. Thoracic kidneys are classified generally into those above the diaphragm (supradiaphragmatic type) and those interposed by the diaphragm (transdiaphragmatic type). The former is caused by excess elevation of the metanephros during the embryonic development while the latter is considered to be due to elevation of the kidney secondary to hypoplasia of the diaphragm. The thoracic kidneys in both cases were of the transdiaphragmatic type. The diaphragm was bilaterally hypoplastic, and the increase in the intraperitoneal pressure and the decrease in the intrathoracic pressure after repair of hernia on one side are considered to have aggravated the hernia and the thoracic kidney on the other side.

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Fibrous hamartoma of infancy (FHI) is a rare fibro-proliferating lesion arising from the subcutaneous tissue during the first 2 years of life. A case of fibrous hamartoma located in the right shoulder of an 11-month-old girl is reported. A firm, non tender, 2.5 × 2.2 × 1.0cm mass overlying the trapezius was excised. The histological diagnosis was FHI. Thirty one cases of FHI in Japanese literatures are reviewed. The mean age at operation was 13.9 months. Twenty four of 32 cases were male. The common sites of lesions were the axillae (7 cases), upper limbs (6), and lower limbs (6). The tumor was excised in 29 of 32 cases, and recurrence was reported only in a case. In 3 cases of partial resection, the remaining tumor showed no evidence of regrowth. These reports suggest that complete local excision is required as adequate therapy.

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A case of Caroli's disease with a complication of massive gastroesophageal hemorrage is presented. After preoperative management of transfusion and antibiotics, he was operated on. He recovered uneventfully and was discharged on the 44th postoperative day. The definition and conception of this disease are not established firmly. Though it is said that Caroli's disease belongs to congenital dilatation of biliary tracts, we assume that this disorder is one type of congenital hepatic fibrosis from the standpoint of pathology and clinical signs.

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Two neonates with pyriform sinus fistula were presented. The first case was a male with a large cystic mass of the left neck, who was intubated at birth because of respiratory distress. The plain radiograph of the neck revealed that the cyst contained air. Respiratory distress was further deteriorated due to bilateral chyrothorax after starting feeding. The second case was a female with left cervical cyst and hoarseness. These cysts, which were completely excised, had connecting fistulae epithe-lialized with both squamous and columnar cells. Recurrence has not been observed for one year after surgery in each case. In three neonatal cases previously reported, two patients underwent temporary drainage and later excision. However, our experience suggested that primary excision can be safely performed at the neonatal period, which would make patients free from the desease.

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Benign liver tumor in children is a rare entity and mesenchymal hamartoma is one of them. Only 29 cases have been reported in Japanese literature so far. We encountered a 5 months old girl with mesenchymal hamartoma of the liver associated with high serum α-feto protein levels. The tumor arouse from the caudate lobe. An adenomatous solitary lesion of the right hepatic lobe and malrotation were also noted. Japanese 30 cases were also analized.

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Intraarterial injection of anticancer agents with lipiodol has been recently performed widely or hepatoma in adult patients and the remarkable therapeutic effects have been reporeted. An infant with hepatoblastoma was tereated by extended right hepatic lobectomy combined with intraarterial administration of several anticancer agents with lipiodol, pre- and post-operatively. Soon after these treatments he showed severe liver dysfunction, which made the chemotherapy discontinued. Pathohistologically the ballooning degeneration of the hepatic cells was characteristic. Thereafter his liver function has gradually recovered, though associated with severe portal hypertension. He has no obvious sign for tumor reccurence. Intraarterial administration of anticancer agents with lipiodol has been reported to be associated with few major side effect, but in some cases it may cause severe liver damage.

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