The purpose of this study is to demonstrate possibilities to transplant the small intestine of newborn rat as a free graft without any vascular anastomoses. We evaluated optimal timing and site of the transplantation. In the first experiments, the small intestine was obtained from a newborn of Lewis stain rats. The newborn was divided into four groups according to their ages ; 0-day-old, 3-day-old, 5-day-old and 7-day-old. The donor jejunum, about 4cm in length, was implanted as a graft into the abdominal wall of syngeneic Lewis rats. More than 80% of the grafts were accepted and grew in the 0-day- and 3-day-old groups. In these groups, the majority of the grafts were histologically normal. However, the grafts from the 5-day- and 7-day-old groups disappeared at the recipient's abdominal wall in three weeks. In the second experiments, the grafts from the 3-day-old rats were transplanted into the three different sites syngeneically; the abdominal wall, the retroperitoneum and the omentum. Eighty-six percent of the grafts transplanted into the abdominal wall survived, while the survival rates of the grafts transplanted into the other sites were below 20%. From these results, we conclude that : 1) the small intestine of the newborn rats can be transplanted as a free graft into a syngeneic adult host, 2) the time limit of the newborn grafts for survival may be between 3 and 5 days of age in this rat model, and 3) the abdominal wall is one of the most suitable sites for transplantation of the intestine.

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We have evaluated the effect of immunosuppression on the graft of the newborn small intestine transplanted without any vascular anastomoses in a rat model. We have also examined the function of the transplanted intestine which was interposed between the divided ends of the host intestine 4 weeks after transplantation. In the first experiments, the grafts from 3-day-old Lewis newborn rats were transplanted into the abdominal wall of allogeneic Wistar strain rats. The rats were divided into three groups as follows; IS (-) group (without immunosuppression), FK group (with FK 506; 0.2mg/kg/day for 2 weeks) and CyA group (with cyclosporine-A; 20mg/kg/day for 2 weeks). Seven weeks after transplantation, all grafts were harvested and examined histologically. In allogeneic transplantation, only FK-group-rats survived with growth of the grafts. In the other groups, the grafts were rejected and disappeared. In syngeneic transplantation, evaluated in the same experimental protocol, immunosuppressant, especially FK, was considered to promote growth and survival of the grafts. In the second experiments, after successful syngeneic transplantation of the newborn intestine into the abdominal wall, the graft was anastomosed in end-to-end fashion to the divided jejunum of the host. Five of 15 rats survived after anastomosis. Fluoroscopic examination of the transplanted intestine demonstrated good peristalsis of the graft without stenosis in 3 of the 5 rats. From these results, it is strongly anticipated that the graft of newborn intestine transplanted without any vascular anastomoses may be useful as an alternative to the previous conventional transplantation of the intestine.

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Hepatic portoenterostomy (Kasai procedure) has shown remarkable improvement in the treatment of biliary atresia. However, because there are not a few patient with unsuccessful portoenterostomies, a liver transplant is the last treatment to save them. One hundred and two patients with biliary atresia were treated by Suruga II modifications from 1977 to 1989 in our institute. In this paper we chose 17 patients with good prognosis (group A) and 27 patients with progressive liver failure (group B) at random from all cases, and retrospectively analysed laboratory data including weight for age as a general index. We concluded as follows : (1) Serum total bilirubin, albumin, and cholinesterase were valuable index to show the timing of liver transplant for biliary atresia. (2) When the following conditions continue for more than 6 months, the patients will possibly die within 6 months, the serum total bilirubin is above 10mg%, albumin is below 3.5g%, cholinesterase reaches below 500IU/1 (normal limit 700-1400). (3) In group B, relative weight gain for age was smaller at 6 months before death than at 12 months before death. When patients with biliary atresia after portoenterostomy reach these conditions, they need a liver transplant urgently.

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Without complete excision, primary liver carcinoma in children is fatal. Six children with initially unresectable liver tumors were treated with chemotherapy in an attempt to decrease the size of the tumor. Cisplatin (CDDP) was added to the regular regimen of Adriamycin (ADR), Vincristine (VCR), and/or Cyclophosphamide in 4 cases. There was a marked reduction in tumor size in three cases. Delayed resections of the primary lesions were successfully perfomed in these 3 cases. These 3 patients are alive and well, and off therapy. The other 3 patients did not respond and died of progressive disease. Cisplatin with Adriamycin, or in combination with other agents, has proven effective in hepatoblastoma. There is a large difference in chemotherapeutic effect between histologic types, with a dramatic response in mixed mesenchymal hepatoblastoma, and a poor response in the adult type. This preoperative chemotherapy regimen permits resection of previously unresectable hepatoblastomas at a "second look" operation and reduces the morbidity and mortality of an otherwise extensive operation.

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The case histories of fifteen adult patients diagnosed at male infertility clinics with vasal obstruction due to inguinal herniorrhaphy were reviewed. Seven had bilateral obstruction after bilateral herniorrhaphy while eight had unilateral obstruction. All patients but one underwent inguinal herniorrhaphy more than 20 years ago, at the mean age of 4 years. Microscopic vasovasostomy was done in all patients resulting in a patency rate of 83%. About half of the vasa deferentia were obstructed at the internal inguinal ring and a length of more than 5cm. of the vas was resected in 24% of the obstructed vasa. The incidence of vas deferens obstruction was studied in a group with a history of inguinal herniorrhaphy in infancy out of the 347 patients attending the male infertility clinic of Kyoto University Hospital from July 1987 to June 1989. We found that nine vasa in 33 herniorrhaphies were obstructed and that the incidence of vasal obstruction was 27%. Because the unilateral obstruction of a vas deferens does not always bring about male infertility, there must be many males with unilateral obstruction and no symptoms. Obstruction of the vas deferens is the most frequent complication of inguinal herniorrhaphy in infancy. Surgeons must be careful of the vas deferens during the operation, especially when ligating a hernia sac at the internal inguinal ring.

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Therapeutic endoscopy has an important role in congenrital pediatric surgical disease. Endoscopic balloon dilatation was performed in 8 cases from 2 months to 6 years old, who had the severe stenosis and stricture in upper digestive tract, during the period from February, 1987 to June, 1989. Anastomotic strictures after the surgical repair of congenital esophageal atresia with tracheo-esophageal fistula occarred in five cases. Congenital esophageal stenosis, postoperative stricture of Heller's procedure for achalasia and secondary stenosis caused by esophagitis due to gastroesophageal reflux were seen in one case each. All cases underwent endoscopic balloon dilatation with the Rigiflex TTS balloon dilatation system under genieral anesthesia. The esophageal strictures and stenosis were effectively released without complications in six cases. The efficacy and safety of the endoscopic Rigiflex TTS balloon dilatation system was evaluated in pediatric cases with upper digestive tract stenosis and stricture.

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The therapeutic effect of cisapride was ivestigated in four patients with chronic idiopathic intestinal pseudo-obstruction (CIIP). Patients were evaluated at the beginning and at the end of the study by scintigraphic evaluation of gastric emptying of liquids and scoring of the following symptoms : abdominal pain, vomiting and distension. Cisapride resulted in an increase in gastric emptying in three of four patients. An improvement in total symptom scores was also shown in these three paients. An oral dose of 0.2mg/kg or 10mg of cisapride was needed to improve their symptoms. It is likely that the therapeutic effect is dependent on the dosage. This study suggests that cisapride may be useful in the treatment of CIIP.

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This report summarizes our experience of a rare type of omphalocele seen in a full-term male neonate, in which the gallbladder was located on the accessory liver lobe attached inside of the omphalocelic sac. An embryological implications of this case is interesting with regard to the determination of the site of the gallbladder. The gallbladder and the accessory liver lobe were inadvertently excised and a primary closure was done in this case, but the best approach whether to excise the gallbladder or not is still open for discussion.

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A newborn baby with a patent omphalomesenteric duct was successfully operated on by us. This disease is rare and has a reported incidence of about two out of 30, 000 live births (0.0067%) in Europe and America. There have been reports on 63 cases in Japan from 1925 to 1988. We tried to analyze the clinical features of 64 cases, including ours. Males predominate with a sex ratio of 2.3 : 1. Ileal prolapse is the most serious complication, and occurred in 24 of 60 cases (40%). Surgery was carried out on 60 cases (94%), and 8 cases of them (13%) died. Surgical treatment should be undertaken as a semi-emergency, once the diagnosis is made.

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A case of Crohn's disease in a 14-year-old boy with situs inversus is reported. At the age of 12 years he was operated upon with a diagnosis of appendicitis, but appendectomy failed through right lower quadrant incision because his situs of viscerum was inversus. Since then, his abdominal pain had been gradually increasing and he had been suffered from diarrhea associated with mucus and blood. He had lost 20kg of body weight in these two years. In August, 1986, he was admitted with the diagnosis of Crohn's disease and situs inversus. The lesions were scattered from the terminal ileum to the right transeverse colon, with strictured lesions of the ileocecum and right transeverse colon. For bowel obstruction, the strictured bowel (ileo-cecum and right transeverse colon) was partially resected. Although this surgery is palliative, he has been doing well since surgery with normal growth and weight gain in the last three and a half years. This may be the first report of Crohn's disease in a person with situs inversus.

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Gastric leiomyoma in childhood is extremely rare. A case of a gastric leiomyoma in a two month old boy is reported. He was admitted to our hospital for vomiting and loss of body weight at one month of age. Physical examination revealed no palpable masses in the abdomen. Upper gastro-intestinal study did not seem to indicate typical hypertrophic pyloric stenosis. Conservative therapy and observation were continued for one month, but the symptoms became worse. A second upper gastrointestinal study made at two month of age found an intraluminal mass with a smooth surface on the prepyloric site of the stomach. Partial gastrectomy was undertaken to determine whether the tumor was a leiomyoma or sarcoma. Microscopic examination of the specimen showed the tumor to be a leiomyoma. The patient made an uneventful recovery and was discharged 37 days after operation. A review of seven cases in the Japanese literature revealed no predilection for a specific location in children compared with adults. A correct preoperative diagnosis was made in only two of seven cases. Histologic differentiation between benign and malignant tumors has often proved difficult.

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A case of intestinal malrotation with idiopathic pneumoperitoneum and chylous ascites was reported. A 6-day-old male neonate was admitted because of frequent vomiting. He had hyperbilirubinemia and the vomitus was bile-stained. Plain X-ray film on admission revealed pneumoperitoneum. Since there were no clinical signs of peritonitis, conservative therapy was initiated. The pneumoperitoneum was disappeared after 7 days and tube feeling was started. The patient gained weight constantly, but vomited once in every three days. The diagnosis of intestinal malrotation was made by G-I tract study on the 25th day. Massive chylous ascites were noted at laparotomy. However, abnormal adhesion, which is usually the result of intestinal perforation, was not formed. Ladds operation was performed and the post operative course was uneventful. Idiopathic pneumoperitoneum in this case seems to have been induced by intermittent volvulus of the small intestine. It should be noted that pneumoperitoneum itself is not an indication for urgent operation, especially in newborn infants. Some cases recover with conservative therapy only. This is the third reported case with chylous ascites associated with intestinal malrotation in Japan. Ladds operation seems to be effective.

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We have experienced intussusception in a three-month-old boy of who has an aberrant pancreas in the ileum. We suspected the diagnosis of intussusception from clinical symptoms. In barium enema examination the result revealed ileo-ileo-colic intussusception, but closed reduction was incomplete. The emergency laparotomy was performed, but the intussuscepted intestine could not be relieved through maneuver. Finally, the ileum was resected. A 1.5×1.0cm sized polypoid lesion found in the resected ileum, histologically evidenced an aberrant pancreas, which was classified as Heinrich III-type. In Japanese literatures, 36 patients with intussusception caused from an aberrant pancreas have been reported. Clinical and pathological findings in these patients are reviewed.

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A retroperitoneal enteric cyst and tubular ileal duplication in 10-year-old girl was reported. The patient had recurrent episodes of abdominal pain and vomiting during the past 15 months. On abdominal computed tomography a retroperitoneal cyst had multilocular form and massive calcification in the cyst wall. No abnormality was found in the thoracic region and in the lumber spines. Through celiotomy two abnormalities were disclosed. The first was retroperitoneal cyst at the left side of the ligament of Treitz. The cyst measured 4×2cm did not cummunicate with either the gastrointestinal tract or the vertebral column. The second, 15cm-long tubular duplication protruded from the lateral wall of the ileum located 10cm proximal to the ileocecal valve. The duplication communicated with the bowel at the junction. Both lesions were completely eliminated. On microscopic examination, the cyst has epithelium of small intestine and two muscle layers. The diagnosis was made as enteric cyst. In both lesions, neither gastric mucosa nor pancreatic tissue could be demonstrated. There was a cluster of normal ganglion cells in the plexus of Auerbach and Meissner in both lesions. These morphological findings suggest that the split notochord theory, which has been widely accepted as hypothesis of developing duplicaions, is less supportable in this case.

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A 632-gram male infant was born at 26 weeks and 4 days of gestation, the mother was complicated by an intrauterine fungal infection from the 25th week of gestion. Delivery was performed with emergency Caesarean section due to fetal distress. The baby showed NEC symptoms from the fifth day of his life. On the 10th day of life, an abdominal x-ray showed free peritoneal air. At laparotomy an ileum perforated, and an ileostomy was done. In a second laparotomy, on the 15th day of life, the ileum and jejunum completely necrotized. Post-operatively, the patient had deteriorated his condition until his death at 18th day of life. Specimen of the ileum examined histologically, disclosed necrotizing inflammatory and ischemic process with abundant colonies of yeast in the intestinal wall. The antifungal therapy initiated promptly postnatal may decrease the chance of lethal NEC when mother is complicated with intrauterine fungal infection.

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Gonadoblastoma occurring in a 6-year-old phenotypically girl, the 19th case in the Japanese literatures, is reported. She was admitted because of lower abdominal painful mass. On physical and radiological examinations the findings suggested twisted ovarian tumor. In laparotomy fist-sized, well-capsulated tumor of the left ovary with torsion of pedicle was found, and the left salpingoophorectomy was carried out. Chromosomal karyotype was 46, XY. Histologically, the specimen demonstrated gonadoblastoma with dysgerminoma. The other gonadoblastoma was identified through the right salpingoophorectomy three months later. Karyotype shoud be examined in female with primary amenorrhea or virilism such as hirsutism or clitoromegaly. If she has Y-chromosome, exploratory laparotomy should be indicated. Early bilateral gonadectomy is necessary for those with gonadoblastoma because of malignancy highly combined.

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Colorectal carcinomas in childhood is very rare and its prognosis is poor in the literature. A 12-year-old boy was admitted to the University hospital with left lower abdominal pain, bloody feces and anemia in these 3 month. In rectal examination, a firm and fixed mass was papated. In barium enema the rectum demonstrated obstruction and irregular mucosal relief. A lower anterior resection of rectosigmoid colon was performed. Macroscopically the resected colon revealed two distinct lesions in a distance of 4cm length. Three polyps were noted near by. Histopathologic findings revealed a poorly differentiated adenocarcinoma of the sigmoid colon a well differentiated adenocarcinoma of the rectum and three adenomatous polyps, Only one specimen of paracolic lymphnodes showed pathologic changes of metastasis. The disease was at Dukes C stage. In 5 years after resection of the tumors, he is doing well and free of recurrence.

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A patient with tracheal agenesis, type 1 of Floyd, surviving longer than two and a half year was reported. Initial surgical treatment consisting of a gastrostomy, a double-barreled cervical esophagostomy and a banding of distal esophagus was effective for primary care. A sudden respiratory failure due to accidental extubation caused irreversible damage on central nervous system at the age of 9 months. The initial treatment at birth and delayed radical correction should be essential for such a life-threatening disease.

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A 15-year-old boy with severe anemia and fever of unknown origin was admitted to our hospital. A mass was found in the left upper abdomen. Swelling lymph nodes at the base of the mesenterium of the transverse colon was resected. Histological findings of the lymph nodes were compatible to a type of Castleman's lymphoma, but they were not a typical one. The patient showed a-marked improvement of anemia postoperatively.

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An Infant with congenital tracheal stenosis and subglottic stenosis was successfully simultaneously oeprated on. This 1-year-old boy with Down syndrome had progressive stridor with dyspnea and repeated cyanotic attack since 5 months old. These symptoms had been suspcted from Tetralogy of Fallot. The surgical repair for TOF was scheduled at 14 months of age. In the operating room, when an anesthesiologist tried to intubate the trachea, he noticed an obstruction distal to the larynx. The operation was postponed, then he was transferred to our hospital with suspected tracheal stenosis. The diagnosis was correctly made through bronchoscopy and bronchography. Both lesions were so severe that we planned surgical repair for them at the same time. In the surgical repair, the tracheal stenosis was circularly resected 1.7cm in length and the subglottic lesion was enlarged with a costal cartilage. He had been treated with postoperative endotracheal stent and ventilatory assist for two weeks, being extubated successfully. His symptoms have dramatic and immediately improved.

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A 9-month-old infant was admitted because of hypoglycemic convulsion. Although about 85% of pancreas was resected under the diagnosis of hyperinsulinemic hypoglycemia, hypoglycemic attack that was resistant to medication recurred. The specimen histologically proved to be nesidioblastosis. Two months later, approximately 98% of pancreas was resected while small amount of pancreatic tissue between duodenum and common bile duct left intact. Two months postoperatively, the level of blood sugar was stabilized at normal range without administration of insulin and gradual psychomotor development was obtained. It is controversial about the adequate range of pancreatectomy for nesidioblastosis. The review of Japanese literature including our case provided the following results : In the patients who received pancreatectomy less than 90% for nesidioblastosis, the rate of recurrence of hypoglycemia was 53% and the rate of additional operation was 35%, whereas in the patients with pancreatectomy more than 90%, the rate of recurrence and reoperation was 17% respectively. In conclusion, we recommend that near total, over 95%, pancreas should be initially resected for nesidioblastosis.

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Foreign body aspiration is one of the major cause of sudden infant death. The most commonly aspirated items are peanuts and beans. Because of its round shape and fragility, removal of the peanut is a hazardous procedure. In most of the cases the rigid bronchoscope have been employed. We extracted a peanut from left main bronchus with the use of bronchofiberscope and Fogarty balloon catheter under endotacheal anesthesia in 1 year 9 month old infant. The bronchofiberscope have some advantages over rigid bronchoscope. Foreign bodies lodged in more distal portion of the airway can be extracted, and tissue trauma from instrumentation is less likely.

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