Operative treatments for esophageal varices in 15 children and its clinical results were discussed. Causes of esophageal varices were congenital extrahepatic portal obstruction (EHPO) in 10, liver cirrhosis or fibrosis in 3, and idiopathic portal hypertension (IPH) in 2. Reasons for surgery on these children were severe bleeding episodes from esophageal varices and risky endoscopic findings or troublesome hypersplenism. Operative procedures were the transthoracic esophageal transection with paraesophageal devascularization (TR) on 2 EHPO patients under 5 years old, TR combined with splenectomy and paragastric devascularization (Sugiura's Ope) on 11 patients, and splenectomy on 2 IPH. Of the 2 patients who underwent TR, 1 needed partial splenic arterial embolization (PSE) 4 years after the initial operation because of gastric bleeding and hypersplenism, and the other needed sclerotherapy due to the bleeding from lower esophageal and gastric varices 7 years postoperatively. The celiac angiography and portography of the 2 patients revealed no development of the hepatopetal collaterals but the hepatofugal shunts toward the inferior vena cava. Sugiura's Ope has been generally effective over 1 to 20 years postoperatively, except 2 patients who underwent the operation under 6 years old. Both the 2 patients suffered from the gastric hemorrhage without esophageal bleeding 10 years after the operation, and underwent selective gastric arterial embolization (GAE) with satis-factory results, because remarkable gastric congestion was shown by the endoscopic mucosal flowmeter. One patient with IPH undergone the splenectomy had dangerous recurred esophageal varces 9 years later, and needed periodic check-ups. We recommend the direct operative procedures, i. e., esophageal transection in case under 6 years old, and one or two staged Sugiura's Ope in case over 7 years old, for the patients of esophageal varices with massive bleeding. Non-surgical treatments such as PSE or GAE and sclerotherapy were also useful as the therapeutic means.

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Fifty-four pediatric patients with teratoma group tumors have been treated at our institute during last 20 years. Differential diagnosis of teratomas by the echography, CT scan and angiography is discussed in this paper The results of operation and chemotherapy for immature and malignant teratomas, especially ovarian, sacrococcygeal and retroperitoneal teratomas are presented. There were 36 patients with benign mature teratoma (Tm) , 8 with immature teratoma and 10 with malignant teratoma. The result of treatment for mature teratoma was good from 1 to 20 years after the removal of tumors. Fourteen of 18 patients with immature and malignant teratoma are doing well from 10 months to 17 years after the removal of tumors with/without the chemotherapy and/or radiation. The histologic type was : immature teratoma (Ti) 8, dysgerminoma (Dys) 4, embryonal carcinoma (EC) 3 and yolk sack tumor (YST) 3. The primary sites were : ovarian 8, sacrococcygeal 7, retroperitoneal 1, mediastin al 1 and testicular 1. Ovarian immature and malignant teratoma : Good re sponses were obtained by VAC therapy for stage III Ti high dose cyclophosphamide (HD-CPA) therapy for stage III Dys, HD-CPA and radiation for stage IV Dys, radiation for stage III Dys, low dose CPA therapy for stage I Dys, T2 protocol for stage I EC, after salpingo-oophorectomy. Sacrococcygeal : Three patients with Ti survive after the complete reseciton without chemotherapy, but 2 patients have had problems related to the urinary system. Preoperative PVB therapy and second look operation for stage IV YST have been successful. Retroperitoneal : One stage II Ti case is uneventful after the complete removal of tumor without chemotherapy. One Tm patient had the recurrence of the mature neural component 14 years later at the same site as the previous lesion with several peritoneal disseminative nodules. The patient has been on a good condition without chemotherapy after the second resection.

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During the last 4 years, 7 infants (two boys and five girls with ages ranging from 5 days to 10 months with a mean of 3.9 months) were experienced. Four cases presented with severe infection, two with abdominal swelling, one with prenatal hydronephrosis. All were ectopic ureterocele (bilateral 2 and unilateral 5) and associated with the duplex system, except for one with unilateral single ectopic ureterocele. Voiding disturabance was observed in 4 cases including 2 with cecoureterocele. Eversion of the ureterocele was noted in 5 cases on voiding cystourethrography and/or endoscopy. Transurethral incision (TUI) was primarily performed in 6 (8 units) at the time of endoscopy. Vesicoureteral reflux was appeared in all units after TUI, but resolved spontaneously in 3 units. The TUI for free drainage resulted in the easy control of infection with no further episode of severe infection. Assessment of renal function by <99m>^Tc-DTPA scintigraphy showed that 2 units apparently improved after TUI, though one unit in bilateral case deteriorated. Because of amelioration of the renal function and control of infection, definitve surgery such as heminephroureterectomy or ureteroneocystostomy, could be delayed. Despite of the risk of reflux, TUI of the ureterocele in infants, including everting one, is an effective initial modality for the better drainage and preserving the function of the adjoining unit.

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To clarify how many cases of surgical neonates there were in the perinatal deaths who were not referred to pediatric surgeons, and why they were not consulted, we checked up the autopsy records of the perinatal death in our perinatal center during the past ten years. We analyzed autopsy records and clinical records of mothers and babies who were not registered to our surgical clinic although whose diagnosis was established as abdominal wall defect, congenital diaphragmatic hernia or tracheoa-esophageal fistula. As corresponding cases, we found 13 cases of omphalocele, 8 cases of diaphragmatic hernia and 3 cases of esophageal atresia with tracheo-esophageal fistula. Total 24 corresponding cases were equal to 4% of 593 registered surgical neonates during the same peroid. Only 4 fetus were died in the early neonatal period and the reminder were still-birth. The reason of still-birth were intra-uterine fetal death in 6 cases and the reminder were terminated because of associated chromosomal anomaly (18 trisomy) and/or multiple severe anomalies (limb-body wall complex etc). So it seemed that few fetus in our autopsied surgical neonates who were not referred to surgical clinic, were expected to survive according to the improve of prenatal diagnosis or perinatal cases.

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N-myc protein and ras p21 expression were analyzed in 76 primary neuroblastomas by immunohistochemical techniques using formalin-fixed paraffin sections. N-myc protein expression was detected in 17 of 76 cases (22.4%). Five-year survival rate (5YSR) of the N-myc protein expression positive patients was significantly low (17.3%) compared with that of the negative ones (69.8%) (p<0.00l). ras p21 expression was detected in 35 of 76 cases (46.1%). 5YSR of the ras p21 expression positive patients was significantly high (84.2%) compared with that of the negative ones (40.7%) (p<0.0l) Both N-myc protein and ras p21 expression correlated with patients' age, clinical staging, mass screening and DNA ploidy, but not with tumor histology. The patient with N-myc positive expression showed poor prognosis. In contrast, N-myc negative expression did not necessarily suggest a good prognosis. In these cases, ras-p21 expression suggested a good prognosis. It is concluded that the immunohistochemical analysis of both N-myc and ras p21 expression are useful for predicting prognosis in neuroblastoma patients.

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One hundred eighty four cases of congenital abdominal wall defects during the past 10 years were reviewed by the questionnaire method at 22nd Kyushu Pediatric Surgery Study Meeting. There were 66 cases of omphalocele, 49 cases of hernia into the umbilical cord and 70 cases of gastroschisis. Survival rate of the 185 newborn infants were 69.7% (omphalocele : 69.1%, hernia into the umbilical cord : 75.5%, gastroschisis : 75.7%). Survival rates of each type were correlated with birth weights and gestational ages of these neonates. Combined malformations of newborn infants with gastroschisis were less frequent when compared with other types of the abdominal wall defects. Although survival rates of the neonates with hernia into the umbilical cord and with gastroschisis have improved recently, one with omphalocele has not improved. The survival rates seem to be greatly influenced by combined anomalies and their severities. Cases of intrauterine diagnosis of the abdominal wall defects have increased during the past 5 years : its merits and demerits were discussed. Several operative procedures were selected according to the associated malformations and the size of the abdominal wall defects. The dry frozen dura matter was reported to be useful covering the abdominal wall defects.

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A five-month-old girl who had undergone a relief of vascular ring from double aortic arch was suffered from respiratory distress in the postoperative period. She had a tracheal stenosis due to the vascular ring and was undertaken two trials of balloon dilation for tracheal stneotic segment at the 4th and 21st postoperative days. After these procedures she could wean from the ventilator on the 13th day after the second dilation. At present, six years after the operation, she is asymptomatic and has developed normally. We believe that the balloon dilatation method is suit-able to relieve a tracheal or bronchial stenosis whether it has congenital or acquired origin. Tracheo- or bronchomalasia, however, is not indicated for the balloon dilatation therapy because a weak cartilage can not support a lumen which is periodically obstructive during inspiration.

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A 1-day-old boy with complete type of caudal regression syndrome is reported. This patient showed flexion constructure of hip and knee joints, paresthesia at lower legs, urinary and fecal incontinence. Agenesis of lumbosacral vertebrae was recognized with abdominal X-ray. His mother had 1. c. iabetes mellitus. His motor and sensorynerves were disturbed at L2 and L4 level, respectively. On the other hand, his autonomic nerves were recognized not to be disturbed with anorectal manometry. The disturbance-level of motor and sensory nerves had dissociation in this case as well as the previously reported cases. The MRI findings showed that the spinal cord ended at Th-8 level. These findings suggested that the nerve roots branched off from the upper level compared with normal case.

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Renal oncocytoma is a rare tumor of the kidney characterized with eosinophilic granular cytoplasm, containing numerous mitochondria. A 9-year-old girl came our hospital because of macroscopic hematuria. US showed a large right renal solid tumor occupying most part of right abdomen. Metastasis was not found with bone scan and chestX-ray film. Under the general anesthesia, right nephrectomy was performed. The size of the tumor was 148X108X60mm and the weight was 390g. The tumor was well encapsuled. Large amount of eosinophilic granular cytoplasm were found microscopically, and under the electron-microscope, there were numerous mitochondria identified. Pathological diagnosis was oncocytoma with malignant potencial because vascular invasion was found at a part of the tumor. It is two years after the operation now, there has been no evidence of recurrence nor metastasis with no chemotherapy.

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Two cases of the impending rupture of the stomach associated with massive gastric hemorrhage in infants were reported. Case 1 : One-day-old female having meconium disease had ileostomy performed. She began to have massive bloody vomiting at 3 days after the operation. She died of MOF at the 13th postoperative day. A seromuscular tear at the greater curvature of the posterior wall of the stomach with the herniation of mucosa was demonstrated during autopsy. Impending rupture of the stomach with massive gastric hemorrhage was the diagnosis. Case 2 : Two-day-old male presented with massive bloody vomiting and abdominal distension beginning twenty hours after normal birth. During emergency operation a seromuscular tear at the greater curvature of the anterior wall of the stomach with herniation of mucosa due to massive gastric hemorrhage was found. Postoperative course was uneventful. It seems that the cause of impending gastric rupture in these two cases might be not only an elevation of intragastric pressure but also gastric ischemia due to diving relfex.

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Although it has been described that various anomalies are associated with Bochdalek hernia, few cases have been reported concerning infantile hypertrophic ryloric stenosis (PS) and/or esophageal hiatus hernia after the repair of Bochdalek hernia. The author treated such a case, as described below. A newborn baby suffering respiratory distress was diagnosed as having Bochdalek hernia. The left-side Bochdalek hernia with sac was repaired within the first 24 hours. Two days following the operation, vomiting occured. Contrast study revealed PS with gastroesophageal reflux. Laparotomy at 20 days after the diaphragmatic repair confirmed the presence of a moderate-sized pyloric hypertrophy, for which pyloromyotomy was performed. Subsequently the patient showed severe vomiting. Contrast study revealed severe gastroesophageal reflux due to esophageal hiatus hernia. At 128 days, antireflux procedure was performed using Dor-Nissen method. Now the patient is free of symptoms.

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A male patient with omphalocele and thoracoabdominal ectopia cordis was referred to our hospital immediately after birth. Reduction of the omphalocele and ectopia corids was successfully performed using a dura mater prosthesis (Lyodura^<[○!R]>) at 2 hours after birth. Surgical-procedures were 1) to reduce the heart into the thoracoabdominal cavity by making a pocket usuing Lyodura^<[○!R]>, 2) to reconstruct the new pericardial sac by covering the heart using EPTFE sheet, 3) to reconstruct the anterior chest wall using Marlex^<[○!R]> Mesh. Unfortunately on the 74th postoperative day he died of heart failure after cardiac catheterization. Our new surgical repair technique is a method to be satisfied for reduction of the thoracoabdominal ectopia cordis.

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Ano-rectal stenosis is a rara malformation. How to treat this malformation is still controversial and several operative procedure were performed in the past. A 4-mo-old boy was admitted to Osaka University hospital with the complaint of constipation and vomiting. Rectal examination and barium enema demonstrated anorectal stenosis at the level of the pubo-coccygeal line. Posterior sagittal anorectoplasty was performed for this patient. The stenotic portion of the rectum was resected and a simple end-to-end anastomosis is carried out. This patient is now free from the constipation without any enema and doing well for 6 months after the operation.

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We reported a rare case of omental cyst in a 15/6-year-old girl, which showed an acute onset of massive hemorrhage into the cyst. This patient showed marked abdominal distention and progressive anemia following slight fever, abdominal pain, and vomiting. From the fidings of abdominal computed tomography and ultrasonography, massive homorrhage into an intraabdominal cystic mass was suspected preoperatively. A 1,375g-weighed omental lymphangioma, which was full filled with bloody fluid, was excised.

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A three year old boy was found an infiltration in the left lower lung field on the chest X-ray film after an respiratory infection. The bronchogram showed a normal bronchial tree, and the arteriography revealed an abnormal arterial supply from the inferior phrenic artery to the left lower lobe and systemic artery pulmonary artery anastomosis. Preoperative diagnosis was bronchopulmonary sequestration (Pryce type I) , and the left lower lobectomy was performed. But in a month, systemic artery-pulmonary artery anastomsis recurred between the left inferior phrenic artery and the left upper pulmonary lobe. A systemic pulmonary shunt which occurs in lung disease characterized by chronic inflammation can presrnt a diagnostic challenge. This disease should be considered as one of differetial diagnoses, even though angiographic findings are compatible with bronchopulmonary sequestration.

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We report a case of gastroschisis with gastroesophageal rupture, a rare complication of vaginal delivery. A routine ultrasound at 34 weeks' gestation demonstrated intestinal loops protruding from the anterior abdominal wall. After vaginal delivery at 36 weeks, the male baby weighing 2,240g was found to have not only gastroschisis but also gastroesophageal rupture along the lesser curvature of the stomach extending upto the infradiaphragmatic esophagus. Within two hours of life, he underwent a repair of the rupture, a gastrostomy, and a successful primary closure of the abdominal wall. The postoperative course was uneventful, and he was discharged in good condition 44 days after surgery. This report does not necessarily support an elective cesarean section for gastroschisis, because visceral injury from the vaginal delivery process is rare. It remains to be clarified whether more accurate antenatal diagnosis will identify subgroups of infants who would benifit by elective cesarean section.

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