This stdudy was performed in order to examine the response to choleretic agents (dehydrocholic acids, glucagon and secretin) and the mechanisms of bile secretion which was normal or not in CBA. Seven children who had undergone so called Suruga II porto-jejunostomy were studied. The effect were calculated as follows; we required the ratio of the mean volume after admistration of choleretic agents to an average volume before its admistration. Bile volume increased by administration of any of the drugs, especially synchronous three drugs medication (1.474±0.350) and glucagon alone (1.416±0.630). Secrected bile bilirubin decreased by almost all choleretic drugs, but secretin alone. So combined medication obstructed bile bilirubin secretion (0.5〜0.6). Secreted bile acid increased by dehydrocholic acid (1.2〜1.75), but glucagon and secretin had no effect on bile acid secretion. These response to choleretic agents suggest that the mechanisms of bile secretion was almost normal in the postoperative patients.

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The characteristic pathophysiology of both cystic and fusiform types in congenital biliary dilatation were studied using 51 cases experienced in our department and the following results were obtained. 1. Sex incidence of female was more frequent in cystic type. 2. Bile flow disturbance was observed more frequently in cystic type. 3. Chyledocal walls were more thick in cystic type. 4. Mucous glands of choledocal wall were prominently seen in cystic type. From these results, the characteristic etiology was not easily determined and the quolitative differences of several factors, such as above factors, may play an important role in making form of either cystic or fusiform type.

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The gallbladder size and its contractile ratio before, during and after oral feeding were examined by ultrasound in 47 normal controls (32 neonates and 15 infants), 3 patients with neonatal hepatitis and 6 patients with congenital biliary atresia. In the control group, the gallbladder size increased its during oral feeding. The muximum size of sonographic gallbladder in two dimensions was 1.14±0.43cm^2 before feeding, 1.59±0.31cm^2 during feeding and 0.76±0.40 cm^2 after feeding. Contractile ratio between maximum and minimum size of gallbladder was 59%. The patients with neonatal hepatitis showed almost the same gallbladder contraction as the control group. However in all 6 patients with biliary atresia we could not identify the gallbladder before, during and after oral feeding. These results suggests that identification of the gallbladder with normal contractile ratio by ultrasound may be applicable to make differential diagnosis of neonatal hepatitis from biliary atresia in infants with jaundice.

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Anorectal manornetry with 4-lumen tube was carried out in 14 normal infants and children, 5 patients with idiopathic constipation, 5 patients with Hirschsprung's disease, 10 patients who had undergone radical operation for Hirschsprung's disease, and 10 patients who had undergone anoplasty for translevator- or intermediate type of anal atresia. Anorectal pressure profiles and recto-anal reflex were studied at 4 different directions (12°, 3°, 6°, and 9°) of the anorectum simultaneously. Measurements of rectal pessure, anal canal pressure, length of anal canal, and basal rhythmic contraction of anal disclosed that there was no significant difference of these values among every directions. Recto-anal reflex was observed in all directions of the anal canal and there was no significant difference of maximum fall of anal canal pressure amang all the directions, when the reflex was present. It was concluded from results of the present study that one can rely on results of anorectal manometry using infused side-opening catheter without considering the direction of side-opening in the anorectum.

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Splenic autotransplantation after splenectomy was successfully performed in two patients (aged two and fifteen) with idiopathic thrombocytopenic purpura (ITP). Following the splenectomy, ten fragments of thinly sliced tissue from a quarter of the spleen was implanted into an omental pouch. The regeneration of implanted splenic tissue was confirmed by radiocolloid localization of Sn Scintiscan. The regenerated spleen did not produce the thrombocytopenia. Furthermore, B-cell lymphocyto reaction to PHA and concanavarin A which were decreased after operation returned to normal after regeneration of splenic tissue. Our results may suggest that splenic autotransplantation after splenectomy could be a new therapeutic method for the patient with severe ITP.

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Since Peden and associates reported the development of hepatic cholestasis and cirrhosis in a premature infant receiving total parenteral nutrition (TPN), the liver damage has been reported bv many authors as the major metabolic complication of XPN in newborn infants. We have experienced cholestasis and abnormal hepatic function in 9 of 17 newborn infants receiving TPN over 1 month. In 6 of the 9 patients, the total bilirubin levels and liver function tests returned to normal after discontinuation of TPN. However the remaining 3 patients receiving TPN more than 6 months died from liver failure. The pathophysiology of the liver disease accompanied by TPN remains obscure. In order to study the mechanism of cholestasis during TPN, we performed immunohistochemical studies using IgA and SC as markers in 4 infants receiving long term TPN. Cholestasis containing IgA and SC limited to the bile canaliculi was seen even when the serum bilirubin levels were still in normal range. Localization of IgA and SC in the bile cananiculi suggested that the cholestasis was responsible for biliary ductal obstruction about the Hering canal. Then the cholestasis developed proximally to the hepatic cells and distally to the bile ductules.

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The new transperineal approach for resection of male vagina is reported. The advantage of this approach is simplicity of exposure of vagino-urethral junction and reliability of closure of urethral side after resection. The damage of urethral sphincter is avoidable by keeping dissection plane adjacent to midline of anterior rectal wall and proceeding sequentially with vaginal wall. Laparotomy should be performed at the same time if the male vagina extend into the abdominal cavity.

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Hemofiltration was used in six critically ill infants including three neonates, with diuretic resistant anuria complicated by severe overhydration with/without hyperkalemia. Severe hypoxia was associated with 3 patients and hypercapnea with 2. All of them were in a state of shock or cardiac arrest, so peritoneal dyalysis was ineffective in these patients. Despite these grave conditions, marked clinical and hemodynamic improvement was noted in three out of the six patients with one surviving to be discharged from the hospital, Hemofiltration is a useful method for controlling body fluids in the overhydrated or hyperkalemic patient in whom peritoneal dialysis is ineffective. Marked improvement in blood gas was noted in hypoxic or hypercapneic patients. But obstruction of dialyzer or root by clotting was noted in 3 and hemorrhagic tendency in one. It may be a serious problem to establish anticoagulant therapy in hemofiltration.

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Double termination of the alimentary tract has two types (Perineal Canal and acquierd type or a kind of periproctic abscess). Congenital type has squamouse epithelium and muscle in submucosa of the fistula. But the differentiated diagnosis of two types by clinical aspect is difficult. We experienced fourteen female infants with double termination of the alimentaly tract. Four cases were congenital type, five cases were acquired type and five cases were of unknown etiology. Surgery is the only treatment for the congenital type, but acquired type can be cured by coservative treatment. Our experience disclosed that four out of five cases of aquired type were cured by conservative treatment. The first choice of treatment is therefore conservative treatment before 6 months after birth. After 6 months if the fistula is not closed, operative treatment must be doned. We think vestibulo-anal pullthrough procedure without colostomy is the best operative methode for this disease.

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Variety of histopathological findings of the thyroglossal duct cysts (TGDC) stimulated us to review the clinical and histopathological findings in 27 cases of TGDC among 37 children having been operated upon for a midline neck mass during the period from 1957 through 1984. The pathologic specimens were reviewed in 23 out of 27 cases. The lining epithelium of TGDC were pseudostratified ciliated and columnar in 6 (26%), stratified squamous in 3 (13%). Two kinds of the epithelium were seen in a cyst in 4 cases (stratified squamous epithelium combined with pseudostratified ciliated epithelium in 3 and stratified squamous epithelium combined with columnar epithelium in one). Unclassified stratified epithelium was found in one case and no epithelial linings could be found in remaining 3 cases. Along with TGDC, thyroid tissue was found microscopically in 7 cases (30.4%) and mucous glands or seromucous glands were seen in one case each. Branching of TGDC was noted in 4 cases (17.4%). Simple excision of XGDC was carried out in 7 cases without recurrence but 2 of 20 cases who had undergone radical excision (Sistrunk's) recurred. The other complications were not experienced throughout the period. To avoid the recurrence, exact knowledge on the embryology and pathology of TGDC as well as the correct preoperative diagnosis and adequate operative procedure are mondatory.

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It has been generally accepted that migratory (retractile) testis requires no treatment because retractibility ceases at puberty and the testis takes up a permanent scrotal position. In some patients, however, migratory testis is difficult to distiguish from true undescended testis. Several authors have also reported that histological changes were observed in migratory testis. Sixty-five children (4 to 9 years of age) with 92 migratory testes underwent orchiopexy. The surgery was indicated when 1) testis was usually located in inguinal area and rarely descended or was difficult to manipulate into scrotum, 2) scrotal position was high, 3) testis was small, or 4) ipsilateral scrotum was underdeveloped. The orchiopexy was easily performed through a scrotal incision. Tunica and cremaster muscle were transected and spermatic cord was elongated. Postoperatively, all the testes were located within the scrotum. It is concluded that a severe form of migratory testis should be treated by orchiopexy rather than being observed till puberty.

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Since July 1981 we have achieved clinical applications of microsurgical techniques in anastomoses of various duct structures (urogenital, digestive tracts and vessels). An 11-year-old boy with bilateral undescended testes underwent microsurgical orchidopexy of the right abdominal testis and standard orchidopexy of the left intra-inguinal canal testis. The right spermatic vessels were anastomosed to the superficial inferior epigastric vessels by microsurgical techniques. The gonad was not perfused or cooled during the revascularization process. The histologic features of testicular biopsies at the time of orchidopexy were more severe atrophic changes in the intra-inguinal canal testis than in the intraabdominal testis. Because of these histologic changes, even if the patient had the unilateral intraabdominal testis, the indication of testicular autotransplantation for the intraabdominal testis should be considered.

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Anorectal electromyography using intraluminal electrodes was carried out on both an aganglionic and a ganglionic segment of the patients with Hirschsprung's disease, The results of present studies were as follows; 1) The spike bursts of ganglionic segment were observed frequently. However, those of aganglionic segment were very rarely observed. 2) The conduction of spike bursts between the anal canal and ganglionic segment was frequently observed. On the contrary, the conduction of spike bursts between anal canal and aganglionic segment was absent in the patients with Hirschsprung's disease. 3) The patterns of the electromyography, which was analysed by signal processor,made spike bursts more clear than those obtained the conventional electromyography. These results suggest that rectal electromyography using intraluminal electrodes is useful for the diagnosis of Hirschsprung's disease.

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This paper reports a 6-month-old boy who suffered septicemia due to osteomyelitis of the lower limbs, subsequently diagnosed as endocarditis. This patient underwent early empiric antibiotic therapy indicated after appropriate culture, and tricuspid valve replacement with Ionescu-Shiley xenograft valve for severe tricuspid insufficiency at the age of 13-month-old. There are only a few reports of infective endocarditis in the infant as a complication of septicemia without the congenital heart disease, although it would be impressed that the these are increasing in young children because of venous drugs abuse and overwhelming infection. We emphasize the peculiarity of the valve replacement in children in whom selection of preferable valve and strict anticoagulant therapy are sometimes difficult.

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A case of intussusceptions in two lesions in a premature infant is described. A male infant was born prematurely with birth weight of 1,090g. He presented with an episode of sepsis and abdominal distension on day 2. Laparotomy on day 6 disclosed intussusceptions in jejunum and ileum and a perforation of the jejunum just oral to the jejunal intussusception. The ileal intussusception was resected and anastomosed, and the jejunal intussusception was resected and the both ends were exteriorized. The postoperative course was complicated with bronchopulmonary dysplasia (BPD) which made ventilatory support imperative. The jejunostomy was closed when he was 3-month-old, and he got weight with oral feeding afterwards. However, he died of progressive BPD and pneumonia on the 196th day after birth. From the world and Japanese literature, forty five cases of neonatal intussusception were collected. Of these, 29 cases were ileo-colic type and 14 cases of them were associated with intestinal duplications. Intussuseption of small intestine was found in 15 cases, three of them were very low birth weight infants and two cases were associated with intestinal atresia. Close relationship between neonatal intussusception and intestinal atresia caused by intrauterine intussusception was considered.

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Congenital urinary umbilical fistula is a rare disorder among anomalies of the urachus. We successfully treated a 5-hour-old male infant who had aplasia of the urachus associated with a hernia into the umbilical cord. The umbilical cord was 4cm in diameter and 3.5cm in height and the intestines were located in the upper half of the umbilical cord as seen through the amniotic membrane. A thumb-sized bladder protruded from the lower half of the umbilical cord. Eleven cases of aplasia of the urachus has been described in Japanese literatures, and had association with hernia into the umbilical cord only 3 caces.

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A rare case of association with lobar emphysema, bronchogenic cyst, funnel chest, and dextrocardia was reported. Lobectomy for lobar emphysema of the left upper lobe and extirpation of cervical tumor (bronchogenic cyst) were perfcr ned successfully at the age of four years. Correction with technique of Peters was performed for funnel chest at the age of ten years and the result was satisfactory. We speculated the correlation of these diseases as follows; the development of lobar emphysema might be promoted by bronchogenic cyst and displacement of pulmonary artery due to dextrocardia (normal type of heart) and the development of funnel chest might be promoted by both lobar emphysema and bronchogenic cyst. Although respective frequency of these diseases was not rare, we were not able to find reports of assocciation with these four diseases such as our case.

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Renal vein thrombosis is a rare lesion seen principally in the neonatal period. We recently encountered an 11-day-old boy with bilateral intra-renal vein thrombosis, who was treated conservatively with anti-coagulant therapy. Such patients, however, require long, carefull follow-up because of possible renal hyper-tension or nephrosis secondary to the successfully treated renal vein thrombosis. In this case report, diagnosis and treatment of renal vein thrombosis are also discussed.

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We experienced a rare cardiac anomaly associated with atrioventricular discordance, double outlet right ventricle (SLD), ventricular septal defect, stenosing supravalvular ring of the left atrium and hypoplasia of the tricuspid valve and right ventricle. The patient did not survive the operation which consisted of resection of the supravalvular ring, Senning procedure and intraventricular rerouting with a conduit. Autopsy findings suggested that hypoplasia of the tricuspid valve and the right ventricle would be related to the patient's death. Recognition of the presence of hypoplasia of the tricuspid valve and the right ventricle is much important for surgical treatment.

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A seven-year-old boy was transferred to our hospital because of traumatic hernia of the abdominal wall. At the operation, the fascia was torn 4cm in length at the left linea semilunaris. The jejunum at 5cm distal to the Treitz's ligament was perforated. The perforated site was closed by direct suture and the abdominal wall hernia was repaired. The postoperative course was uneventful. The analysis of 23 cases of the abdominal trauma in our hospital was presented. The incidence of the traffic accident was most frequent (12 of 23 cases). The injuries of the parenchymal organs were more frequent than that of the hollow organs (27 v.s. 5 organs). Four of the 23 cases died (the mortality rate was 19%). Three of the 4 cases died due to the hepatic ruptures. Traumatic hernia of the abdominal wall is very rare. Though the diagnosis of the traumatic hernia is easy, the diagnosis of the injury of the intraperitoneal organs is difficult. The intraperitoneal organs, especially intestines, should be investigated carefully at the operation.

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Anterior urethral valve and diverticulum are rare lesions and only 21 and 45 pediatric cases, respectively, were reported in Japan. However, pediatric cases may be missed when micturtion is less affected and/or systemic urological examination including the urethra is not performed. The lesions, if untreated, cause renal dysfunction due to secondary hydronephrosis and urinary infection. Therefore, they should be taken into consideration in differential diagnosis of dysuria in children. We recently encountered an infant with anterior urethral valve and diverticulum, who was successfully treated by urethral reconstruction on the 54th day of age following an initial cystostomy in neonatal period. In this case report, symptoms, complications, diagnosis and treatment of the lesions are described.

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A 9-year-old boy had been incontinent following repair of the high type anomaly of anorectal malformation. Therefore, construction of a rectal sphincter using the gluteus maximus muscle was performed on this patient. The result of bowel control after the operation was analyzed from the points of clinical assessment, barium enema study, manometric study, and electromyography. This patient achieved fair fecal continence (3 points of the Kelly's score) 5 months postoperatively. Postoperative barium enema study showed a moderate anorectal angulation. Normal anal resting pressure and adequate voluntary squeezing pressure were found postoperatively. Postoperative electromyography showed a tonic electrical activity of the perianal muscle. These results suggests that fecal continence was achieved following sphincter reconstruction utilizing the gluteus maximus muscle clinically and objectively. However, longterm follow-up should be continued.

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