The isolated bowel segment (IBS) is a loop of bowel which is totally free of its mesenteric attachment, but with its viability preserved. The IBS is created by two staged procedures ; initial coaption of the IBS to certain host organs, and secondary IBS mesenteric division several weeks later. During the interval between these procedures, vascular collaterals are formed between the IBS and host organs, which preserves viability of the IBS. The IBSs can be attached to host organs such as abdoninal wall muscle, liver and adjacent bowel, which we have termed Iowa Models I, II and III, respectively. In previous studies, motility and absorption in the IBS were observed in experimental animals. We conclude from these studies that a loop of bowel can maintain its normal physiologic functions without extrinsic innervation. This paper describes the techniques used to create the IBS, and a newly designed bowel elongation procedure which incorporates the principle of the IBS.

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The purpose of this paper is, 1) to clarify why there is no pathological dilatation of the bile duct in gouts in spite of their very long common channel, 2) to produce a model of congenital biliary dilatation in the gout, and 3) to clarify the etiology and the pathogenesis of congenital biliary dilatation. In the biochemical examination on the pancreatic and bile juice of man and gout, a marked difference was found in the composition of pancreatic juice between man and gout, and especially activity of the pancreatic enzymes was extremely lower in the gout. Therefore, administration of human pancreatic juice into the gallbladder of gout was performed using tube-cholecystostomy. Infant gouts within 1 month of age definitely showed cylindrical or spindle-shaped dilatation of the bile duct after infusion for about 4 weeks. But adult gouts showed no or only slight dilatation of the bile duct in the same period. As the pathogenesis of congenital dilatation of the bile duct, the effect of reflux of pancreatic juice into the bile duct was strongly suggested. Also it was suggested that the degree of dilatation of the bile duct may be determined at the intrauterine life or the early stage of life.

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It was difficult to obtain tumor resistance against murine C1300 neuroblastoma (C1300) in syngeneic hosts. We attempted to immunize A/J mice by weekly intraperitoneal injection of 2x10^6 Mitomycin Ctreated tumor cells as well as 200μg formalin-killed Corynebacterium parvum for 3 weeks. Approximately 70% of these mice were resistant to subsequent live tumor challenge of 10^5 cells. We used the spleen cells of these tumor resistant mice in Winn assay, adoptive chemoimmunotherapy and in vitro <51>^Cr-release assay. Winn assay demonstrated that immune spleen T cells had the capacity to reject the tumor. This cytotoxicity was partially eliminated by the treatment with ether anti-Lyt-1 or anti-Lyt-2 monoclonal antibody and complement. Administration of cyclophosphamide (I50mg/kg) and 5×10^7immune spleen cells into tumor-bearing hosts prolonged mean survival time. In 4-hour <51>^Cr-release assay, cytotoxic cells were detected in the immune spleen cells in 5-day Mixed lymphocyte tumor cell culture. Percent specific lysis of C1300 was over 40% (E : T = 40 : 1). These cells had no cytotoxicity against human neuroblastoma cells. These cytotoxic cells contained Thy-1^+ , Lyt-2^+ cells and Thy-1^+ , Lyt-1^- , Lyt-2^- cells. Anti-asialo-GM_1 antibody and complement could not eliminate cytotoxicity of these cells. Thy-1^+, Lyt-2^+ cells had specific cytotoxicity to C1300, and Thy-l^+, Lyt-1^-, Lyt-2^- cells had nonspecific killing ability. These data suggest the possibility of specific and nonspecific antitumor immunotherapy to neuroblastomas.

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Eight infants weighing less than 1500g underwent primary anastomosis for localized intestinal perforation due to necrotizing enterocolitis in 7 infants and meconium disease in one. The mean body weight was 1016g, the mean gestational age was 28 weeks, and the mean age at the time of operation was 11 days. All patients were diagnosed by free air on the abdominal roentgenograms. Intestinal perforation was observed in the terminal ileum in 7, and in the jejunum in one. Two babies died of postoperative sepsis without anastomotic leakage, and five have survived with adequate body weight gains due to early enteral feedings which started within 2 weeks after the operations. A recent case who is now one monthe after the operation has not yet observed enough body weight gain. It is easier and safer than was previously thought to achieve successful primary anastomosis for intestinal perforation in very low birth weight infants.

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Our recent experiences in transferring patients with biliary atresia (BA) to an overseas transplantation center which had required our own clinical judgment with regard to the indications for orthotopic liver transplantation (OLT) prompted us to revise our paper previously written on this same issue. Sixty-four patients who had undergone the Kasai procedure for BA at the Kobe Children's Hospital during the period from 1971 through 1987 were subjected to this study. These 64 patients were classified into 3 groups according to their degree of bile drainage. Group I (n = 31) : bile fully excreted and their serum bilirubin was below 1.0 mg/dl. Group II (n = 21) : bile inadequately excreted, with serum bilirubin over 1.0 mg/dl. Group III (n = 12) : no bile flow achieved. Group I : 3 pts died due to rupture of esophageal varices despite the normal bilirubin level. Liver transplantation was indicated for these pts. Group II : 17 pts died due to progressive cholestatic liver disease. Group III : all died of rapidly progressive liver disease. All these pts required OLT, eventually in Group II and urgently in group III. Unfavorable factors for successful liver transplantation, such as portal vein thrombosis, intrapulmonary shunt or malnutrition should be carefully considered, when trying to decide the timing of OLT regardless ol the serum bilirubin level.

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The subjects were 119 patients (149 air reductions) with intussusception whom we observed from April 1981 to December 1990 and in whom we observed the obvious presentaions under fluoroscopy during air reduction. These subjects were aged 2 months to 9 years. Of 149 air reductions (including recurrences), 140 were successful with a reduction rate of 94%. Air pressure during reduction were <90, 90〜120, 120〜150 and 150〜180mmHg for 33, 47, 24 and 6 cases, respectively. Nine cases including 3 with reduction perforations were operated. Of these 9 cases, 5 including the above 3 cases underwent enterectomy, the remaining 4 required only manual reductions for ileal intussusceptions. The above 3 cases of reduction perforations occured at reduction air pressures of 130, 140 and 180mmHg, respectively. They showed neither symptomatic aggravation nor intracavitary intestinal contents upon labarotomy, thanks to quick intracavitary de-airing just after perforation leading to favorable postoperative courses. Recurrence was noted in 16 cases (13.4%) at a frequency of 1〜6, but air reduction was successful in all cases. Those patients who had at least 2 recurrences received a contrast enema or colic fiberscopy, but none of them showed any organic disease other than lymphoid hyperplasia. The analytical results of reduction air pressure and reduction perforation in these cases, as well as in operated and recurrent cases are reported in this study, together with additional consideration of the related literature.

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Whole gut lavage was applied in 19 pediatric patients using polyethylene glycol-electrolyte solution, The indications for use of this procedure were the following- ;preoperative bowel prepartion for colonic surgery : 12, preoperative emptying of the bowel contents for abdominal wall closure in a patient with an omphalocele : 1, bowel preparation for colonofiberoscopy : 3, bowel prepartion before intensive chemotherapy in patients with malignant diseases : 2, whole gut lavage in a patient in hepatic coma : 1. There were two patients who could not tolerate this procedure, but no severe complication was observed during or after its use. Older patients had some difficulties in the use of this procedure because they could not take enough lavage solution by mouth or they could not tolerate the unpleasantness of inserting the nasogastric tube. We emphasize the effectiveness and safety of whole gut lavage using a polyethylene glycol-electrolyte solution in pediatric patients.

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Apley's widley accepted criteria for recurrent abdominal pain are : (1) at least three episodes of pain, (2) pain severe enough to affect activities, (3) attacks occuring over a period of 3 months, and (4) no known organic cause. Fourteen children meeting Apley's criteria for recurrent right lower quadrant abdominal pain were selected from among 23 patients diagnosed with lympoid hyperplasia of the appendix based on the results a barium enema. All patients underwent appendectomy. There were 4 males and 10 females, ranging in age from 6 to 15 years old (mean, 10.5 years). Follow-up periods ranged from one month to 3 years. Eleven of 14 children (78.6%) showed no recurrence of recurrent abdominal pain during the follow-up period. Three patients continued to experience abdominal pain after appendectomy. It was determined that the continued abdominal pain in these 3 children was psychogenic, i. e., there were problems at school and at home. These 3 children received counseling from a case worker and their pain subsquently disappeared.

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In 142 patients with biliary atresia in whom jaundice disappeared after successful treatment, 76 patients had recurrence of jaundice or fatal complications. Only 29 patients recovered from these conditions, but 18 patients still have jaundice and another 29 died. We analyzed the data of these 142 patients and following results were obtained ; 1) Type II obstruction (obstruction at hepatic duct) showed worst results; 2) High incidence of complication was seen in patients with subtype a obstruction (obstruciton of hepatic port or hepatic duct, but patent common bile duct) ; 3) Poor prognosis was observed in subgroup β (obstruction at common bile duct with patent but very narrow hepatic duct) if complication occurred; 4) Rate of recovery after treatment for complications were better in patients having the initial operation at younger age; 5 ) More than four-fifth of the patients with a history of cholangitis showed severe complications; 6) There are no differences among surgical procedures; 7) Fifty seven percent of the patines had complications within one year after the primary operaiton.

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A total of 36 patients aged under 11 months (4 weeks-11 months) with pulmonary hypertension underwent open-heart surgery between April, 1986 and March, 1990. We assessed the recovery of A-aDO_2 until 48 hours after surgery and the improvement of pulmonary artery systolic pressur (PAPs) at 2 weeks and 1 year after surgery. Anomalies of those patients were : ventricular septal defect (VSD) in 12, VSD with atrial septal defect (ASD) in 9, VSD with coarctation of aorta in 2 (Group VSD, n = 22), ASD in 4 (Group ASD), total anomalous pulmonary venous return (TAPVR) in 9 (Group TPR). And VSD and TAPVR groups were divided into each two subgroups : VSD associated with Down syndrome (Group V-D), or not (Group V-N) ;TAPVR aged under 8 weeks (Group T-1), over 9 weeks (Group T-2). In A-aDO_2, group V-D and T-2 showed a significant delay of the recovery though all groups recoverd to normal levels before 48 hours. In PAPs at 2 weeks after surgery, group V-D and T-2 showed high levels (43.5 ±12.6, 47.7 ± 13.6mmHg, respectively) though all groups had a significant decrease compared to preoperative levels. At 1 year, the PAPs in group V-D remained at high levels (56.6±9.0mmHg), and in group T-2 decreased gradually (34.6±3.0mmHg) We concluded that corrective surgery for VSD with Down syndrome and TAPVR should be performed in the early stage because the pulmonary vascular disease in those patients might be produced in early infancy.

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Ureteral ectopy is the result of faulty development of the terminal wolffian duct, and the association with a double ureter and pyelocalyceal system is not uncommon. Although single ectopia accounts for about 70 to 80 per cent of total in Japanese literature, bilateral single ectopic ureter or single ectopic ureter in a solitary kidney is a rare occurence. During the last 17 years we treated 6 children (3 males and 3 females) with bilateral single ectopia and 3 (1 male and 2 females) with single ectopia in a solitary kidney. Age at presentation ranged from 4 days to 3 years. Main clinical presentations included fever from UTI and hematuria. In 5 of 6 children with bilateral ectopia, either one or both of the ureteral openings were found at the bladder neck. In the last case both ureters opened into the vestibulum and the bladder was severely hypoplastic with its capacity less than 5 ml. Among 3 children with solitary kidney, the ureter opened at the bladder neck in one and in the urethra in two. One of the children whose ureter opened in the urethra presented severely hypoplastic bladder of about 3-4ml in capacity. Dilatation of the upper urinary tract was seen in all cases. Ureteral reflux was evident in about half of the ureters. Antireflux ureterovesical anastomosis was done in 9 ureters of 7 children. In two children with severe bladder hypoplasia, we performed staged procedure, first to increase the bladder capacity and second to prevent vesicoureteral reflux. In the first procedure, side-to-side anastomosis of the dilated ureter and lateral wall of the hypoplastic bladder was done using operative loupe. One neonate required temporary diversion of the upper urinary tract prior to the first procedure, since she presented acute renal failure from the obstruction of bilateral ureteral openings. Six to seven months after the first operation the bladder capacity reached about 40 to 70 ml. In the girl with solitary kidney, the second operation of antireflux ureterocy-stoneostomy was performed 9 months after the first procedure with satisfactory result. The second child is now waiting for the second operation.

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This study evaluated infant formula thickened with corn starch in the treatment of gastroesophageal reflux. Since we consider that the effect of thickened feedings depends chiefly on viscosity, the determination of suitable and stable viscosity is essential for clinical application. The study suggested that : (1) Constant viscosity can be achieved using our original method of preparing feedings using high quality materials, (2) stability of thickened feedings was demonstrated for 24 hours, and(3)a suitable method of feeding should be selected depending on difference of viscosities.

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Ohe patients who had biliary atresia and in whom intrahepatic cholelithiasis was recognized 19 years after hepatico-jejunostomy is presented. This patient underwent lithotomy as well as intraoperative endoscopic study of the biliary tract. Eight cases of biliary atresia with postoperative, intrahepatic cholelithiasis are reviewed. The age of these subjects ranged from 2 months through 26 years. Of the 8 subjects, 4 were diagnosed as having bile stone at autopsy and the surviving 4 underwent surgery successfully.

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Two children with anomalous conjugation of the pancreatico-biliary tract with protein plug formation are presented. A 10-year-old girl was admitted to our hospiatl with severe pancreatitis and cholangitis and a 3-year-old girl was admitted with peritonitis due to perforation of the bule duct. The type of anomalous conjugation in both cases was type III-B according to the Ohi classification. Histologically, we found remarkable infiltration of inflammatory cells in their resected bile ducts. Protein plugs, which might have been formed by stasis of bile and pancreatic juice, were found in accessory pancreatic ducts near the accessory papilla. From these findings, it is suggested that when protein plugs entered into accessory papillae, acute massive reflux of bile and pancreatic juice brought on severe complications such as severe pancreatitis, cholangitis, and perforation of the bile duct.

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The patient was a Japanese male newborn infant whose delivery was at term and uneventful, but who had severe hypoxia at birth. X-ray examination revealed a left diaphragmatic hernia. Peromelia and micrognathia were not recognized. At operation, a posterolateral defect in the left diaphragm was repaired and we found an incidental splenic anomaly. A splenic elongation arised from the normal spleen, and tapared downward to the left intra-abdominal testis. He expired four hours after surgery because of cardiopulmonary insufficiency. An autopsy confirmed the dignosis of splenogonadal fusion associated with congenital diaphragmatic hernia, left ventricular hypoplasia of the heart and male pseudohermaphroditism. Splenogonadal fusion (SGF) is a rare but benign anomaly. To our knowledge, 38 cases of SGF in infancy and children have been reported in the world literature during the past twenty years. In summarizing of the literature, we found only two cases of SGF associated with congenital diaphragmatic hernia. Our case is the first report of the continuous type of neonatal SGF in Japan.

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Gastric teratoma is a very rare benign tumor. Only 36 cases have previously been reported in the Japanese liturature. Here we reported a case of 47 day-old male infant with a huge gastric teratoma causing acute respiratory distress. Upon surgery, an intraabdominal huge capsulated mass arising from the greater curvature of posterior gastric wall was found. The tumor which was exstirpated combined with gastrectomy (tubular resection by Connell technique) was measured 17 X12 X 12cm and weighed 900g. Histological diagnosis was benign teratoma including immature neural tissue cells. Two years after surgery, he was healthy and symptomless. Clinical presentation, diagnosis and surgical treatment of the gastric teratoma were discussed briefly with relevant literatures.

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Mortality from pulmonary agenesis is still high because of associated anomalies and respiratory complications during the neonatal period. In this report, a rare case of pulmonary agenesis in a neonate who was treated with ECMO is described. A full term baby boy was admitted to the hospital with the diagnosis of anorectal anomaly on the day of birth. The patient suddenly developed progressive respiratory distress on admission. A chest radiograph showed opacity of the right hemithorax with a shift of the mediastinum to the right. The patient fell into serious cardiopulmonary failure due to left pneumothorax following tracheal intubation. ECMO was started 20 hours after birth. The patient improved and was weaned from ECMO after 10 days. Further examination revealed total anomalous pulmonary venous drainage (TAPVD) and duodenal atresia. Although the patient died of heart failure after cardiac operation on the 29th day of age, ECMO was found to be a life-saving measure in pulmonary agenesis with respiratory complications in the early days of life.

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This is a case of a 10-year-old boy with an extraskeletal myxoid chondrosarcoma. A solid and painless mass was discovered in the subject's left shoulder region. A local excision was performed and histological examination revealed that it was an extraskeletal myxoid chondrosarcoma. Two weeks later, a wide excision was performed, and subsequently chemotherapy was applied for three months. The child has been doing well, with no evidence of recurrence, ten months after the second excision. Extraskeletal Myxoid Chondrosarcoma is a rare tumor that usually appears in the soft tissue of the extremities. In children, this tumor is extremely rare, and only nine cases have been reported to date. In general, extraskeletal myxoid chondrosarcoma has been considered to be only slightly aggressive. Of these nine children, however, six died of the disease within several months after the diagnosis. Seemingly this tumor is more aggressive in children than in adults. It, therefore, requires a careful treatment coupled with close observation and a long-term follow-up.

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Splenic cyst in children is a relatively uncommon disease, which is usually treated by splenectomy, although it is important to consider the possibility of postperative infection. A 14 years old female case was treated by cystectomy using an Infrared Contact Coagulator (I. C. C.) preserving the spleen. She had abdominal pain, but no other complaint or contributory history. The preoperative diagnosis was established on the basis of characteristic radiographic findings. In particular echogram, CTscan and MRI were useful. Cystectomy without splenectomy using the I.C.C. was effective to control splenic bleeding. This useful instrument will probably be used increasingly in pediatric and general surgery.

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We experienced 9 children with rectal prolapse. 8 -of them were successfully treated with the consevative therapy. A 9-year-old girl with persisting rectal prolapse was treated with so called biofeedback training using an anorectal manometric technique. She achieved an excellent clinical response to the training. Manometrically, active anorectal contraction improved significantly and low rectal compliance reached the normal range. We believe that biofeedback training can be a choice of treatment for persisting or recurrent rectal prolapse in childhood.

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