Endopyeloureterotomy and transluminal balloon dilation were performed on 18 children with primary or secondary ureteropelvic junction obstruction (UPJO) or ureteral stricture. For postoperative evaluation, an excretory urogram was obtained 3 to 6 months after removal of the stent. The clinical success rates by the procedure were as follows : endopyelotomy on 12 patients with UPJO 9/12(75%), balloon dilation on 2 patients with UPJO 0/2(0%), endoureterotomy on 2 patients with ureteral stricture 2/2(100%), and balloon dilation on 2 patients with ureteral stricture 1/2(50%). One pyelotomized patient whose hydronephrosis had progressed was treated surgically 2 months after removal of the stent. Complications were observed only in pyelotomized patients (pyelonephritis 2, transient ureteral edema 2, and blood transfusion 1). Endopyeloureterotomy is an effective option for the treatment of primary or secondary UPJO and other ureteral strictures in children

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Forty patients with stage III and IV (excluding IVS) neuroblastoma were treated at Gunma Children's Medical Center and Gunma University Hospital between 1960 and July, 1992. Before 1985, resection of the primary tumor was done initially at diagnosis in 8 and after chemotherapy in 2. Complete resection was achieved in 7 of 16 patients (44%). On the other hand, out of 24 patients treated after 1985, 7 underwent initial resection and 16 delayed primary operation, resulting in total or subtotal resection in 18 (88%). At the time of evaluation, July, 1993, while 3 patients treated before 1985 remained in complete remission with a follow-up time of from 151 to 203 months, 18 patients treated after 1985 survived for from 12 to 96 months. In 15 of 18 patients whose local tumor was resected by delayed procedures, preoperative treatment and its effect were reviewed. Resection was done between 9 to 19 weeks (mean 12.9 weeks) after the initiation of treatment in 12 and was delayed in 3 until 29, 38 and 40 weeks, respectively. Preoperative treatment reduced the size of the tumor by 2.4-92.7% (mean 60.1%). The degree of reduction was not correlated with the total dose or dose intensity (Dl) of each chemotherapeutic agent. The percentage of projected DI (PPDI) before, at and after operation was 0.89 ± 0.15, 0.62±0.13 and 0.81±0.11, respectively. PPDI at operation was significantly lower than that of others (n=11, p<0.0l). From these results, it is concluded that both resectability and survival of the patients have been improved by combination chemotherapy and delayed primary operation. As for the timing of the operation, resection should be delayed so as not to decrease PPDI during initial treatment.

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Experimental orthotopic small bowel transplantation had been successfully performed in rats. Recipients showed normal absorption through transplanted bowel soon after surgery. A life-threatening malnutrition, however, was occasionally observed and those of status was. persisted during the early postoperative periods. It was suspected that "protein losing enteropathy" might developed in the transplanted small bowel. We measured fecal alpha 1-antitrypsin (FA1AT), serum total protein (Tp) and albumin (Alb) in the experimental study, and evaluate the possible enteropathy. Ten out of 15 rats showed high FA1AT excretion. A maximum of excretion was observed at 15 th postoperative days. A high excretion group was noted to develop severe weight loss with low serum albumin level comparing with control group (p<0.05). The FA1AT levels in 6 out of 11 rats returned to normal levels at 30-51 postoperative days. Although the body weight of this group showed gradual increase, four of them died with malnutrition eventually. There were no significant difference in weight change or Tp and Alb levels between normal FA1AT excretion group and control group during consecutive postoperative days. All of experimental animals survived more than 30 days after surgery. The results is as follows : 1) The malnutritional status due to "protein losing enteropathy" occurred in the experimentally transplanted bowel at the early post operative period. 2) This status showed a considerable fatal outcome. 3) We suggest this status might occur at clinical application of the bowel transplantation.

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Laparoscopic cholecystectomy (LC) was successfully performed in a 8-year-old girl (weight 29 kg) with severe acalcurous cholecystitis following burn. This report illustrates technical cinsiderations comparing with adult cases. 1) More attention should be payed in insertion of insufflation needle and trocars than in adults because of the elastic abdominal wall of children. 2) To obtain a good exposure of cystic artery and cystic duct, blunt dissection using gauze tampon (Tupfer's gauze) was very effective especially in such a case with adhesive cholecystitis. After LC the patient was able to resume normal diet and activity quickly as in adults. With a few technical modifications, we found that LC was safe and effective in chilren.

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An 11-month-old girl with malignant rhabdoid tumor of the kidney underwent left nephrectomy with dissection of paraaortic lymph nodes followed by postoperative chemoradiotherapy. The patient did not respond to the treatment, and died of tumor 14 months later. At autopsy. metastase were found in the lungs, chest wall, diaphragm and liver. Lymph node metastase were also seen around the left bronchus, in mediastinum, at paracardiac region and at the head of pancreas. Immunohistochemical studies demonstrated that tumor cells stained positively for vimentin, α_1-antichymotrypsin and keratin. Only 3 survivors of such kidney tumors have been reported among 17 reported cases in Japan.

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A nineteen years old patient with esophageal achalasia and acquired hypoganglionosis is reported. Histological study of the resected colon using anti-neuron specific enolase (NSE) and anti-S-100 protein immunostaining was performed. NSE positive ganglin cells and S-100 positive satellite cells were identified. The myenteric and submucosal plexuses were decreased in total colon. The size of intramural plexuses was normal but the ganglion cells were decreased and degenerated. There were many glial cells in the plexuses which replaced ganglion cells. These findings strongly suggested this case should be secondary hypoganglionosis. Immunohistochemical staining was useful for study in disorders of intestinal plex-uses.

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In high jejunal atresia, the functional intestinal obstruction occurs. We describe here a good technique of tapering duodenojejunoplasty, special anastomosis and colon mobilization. At surgery, on the first day of life, the bowel proximal to the obstruction became greatly dilated. A tapering duodenojejunoplasty of the proximally dilated segment was accomplished. In the distal part of the dilated intestine, the tapering jejunoplasty was not achieved so that the suture line was not involved in the following anastomosis. An end-to-end anastomosis was then performed. The right colon was mobilized, the ligament of Treitz was divided, and the retrocolic portion of the duodenum and jejunum were brought to the right of the superior mesenteric vessels. The patient has been doing well without functional intestinal obstruction. We can therefore recommend this procedure.

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Three cases of atypical congenital diaphragmatic hernia including an extremely rare case of multiple diaphragmatic hernia were reported. Case 1 was a 10-month-old boy who showed two round densities at the base of the left hemithorax on preoperative chest roentgenography, CT, and MRI before the radical repair of imperforate anus. Laparotomy for closure of colostomy revealed two protrusions of the diaphragm into the left hemithorax (3.5 cm and 4 cm in diameter, respectively), which contained part of the left lobe of the liver and part of the spleen each. Both diaphragmatic defects were closed. Histological examination of the biopsied hernial sac revealed a scarcity of muscle fibers. Case 2 was an 11-month-old boy in whom mass was detected at the base of the right hemithorax before the radical surgery for Hirschsprung's disease. This lesion was not present in the neonatal period. Thoracotomy revealed a protrusion of the liver through a defect 3-cm diameter located at the center of the diaphragm. The liver tissue was excised and the defect was closed. Case 3 was a 25-day-old boy under treatment for sepsis at NICU, in whom a roud mass was detected incidentally at the top of the right hemidiaphragm. Laparotomy revealed bulging of the right lobe of the liver into the hemithorax with a hernial sac of 3 cm in diameter. The defect was closed. All three patients did well after surgery. The characteristics of this type of congenital diaphragmatic hernia were discussed.

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We experienced a case of heterotopic gastric mucosa of the ileum without Meckel's diverticulm. The patient suffered from abdominal pain and vomiting at 11 year-old. Under the diagnosis of intestinal obstruction, laparotomy was performed. Subserosal hematoma of the ileum was noticed, suggesting the diagnosis of Schoenlein-Henoch's purpura. Histopathological study of resected appendix revealed angitis, which was compatible with Schoenlein-Henoch's purpura. After the operation, she had severe abdominal pain twice for two years. Each time, steroid therapy was effective. At 13 year old, she visited our clinic with severe abdominal pain for the forth episode. Ultrasonographic examination showed intussusception. Hydrostatic reduction was not successful and laparotomy was done. At operation, a mucosal tumor on the antimesenteric side of the ileum, 80cm from the ileocecal valve, was resected. Microscopic examination revealed gastric mucosa. To our knowledge, this is the seventh reported case of heterotopic gastric mucosa in the ileum without Meckel's diverticulum in Japan.

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This is case report of multicentric neuroblastoma, which is thought to maturate to ganglioneuroma with chemotherapy. 1 year 7 month old girl was admitted to our hospital due to mediastinal and retroperitoneal tumor. Labolatory data showed the elevation of serum LDH and urinary VMA, HVA level. CT scan showes mediastinal and retroperitoneal tumor, and bone scintigram showed positive uptake in both field. Chemotherapy was started in the diagnosis of multicentric neuroblastoma. During chemotherapy, CT showed no change of the size in both tumors, but LDH, VMA, HVA level were gradually decreased. Three years later, operation has done and pathological finding showed ganglioneuroma of both tumors.

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Three cases of intestinal duplication were experienced. One was duodenal duplication in a newborn and the other two were ileal duplications in a child and an infant. Case 1 : An 8-year-old female presented with intussusception was operated on because of intestinal obstruction. Operation revealed an induration measuring 1×1.5cm inside of the ileum 50cm proximal to the ileocecal junction. The ileum with the induration was resected. Case 2 : An 8-day-old male was operated on because of a cystic mass in the right upper abdomen. Operation revealed an cystic lesion measuring 5cm in diameter and attached to the duodenum. The cyst was unroofed and the remaining mucosal wall attached to the duodenum was stripped off. Case 3 : A 9-month-old female with a filling defect in cecum demonstrated by barium enema was operated on because of intestinal obstruction. Operation revealed an induration measuring 4 X 3cm inside of the terminal ileum. The ileocecum was resected. In all cases, duplication was demonstrated histologically. Because ectopic gastric tissue, pancreatic tissue and malignancy have been reported in the intestinal duplication, internal drainage alone is not recommended. The procedures of complete removal of the duplication, resection of duplicated intestine or excision of the free wall of the duplication and stripping off of the remaining mucosal wall should be selected.

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Neutropenic enterocolitis is a necrotizing inflammatory disease of the right colon that develops in the setting of severe neutropenia. We experienced a case of neutropenic enterocolitis with intermittent neutropenia in a 9-year-old boy. Althouth intermittent neutropenia has no regular cycles, it may be a subtype of cyclic neutropenia which is a rare hematologic disorder characterized by regular cycles of severe neutropenia occuring approximately every 3 weeks, and neutropenic enterocolitis is a life threatening complication of cyclic neutropenia. Although the clinical presentation and findings were resembling those of acute appendicitis, ultrasonography and CT showed diffuse thickening of the cecum and ascending colon wall. The treatment of neutropenic enterocolitis is not uniform as it represents a spectrum of disease ranging from mild, selflimited inflamation to fulminating necrosis and perforation. The indications for surgery in neutropenic patients should be the same as those in nonneutropenic patients. Pre- and postoperative granulocyte transfusions and administraiton of G-CSF should be performed because the return of adeqate numbers of functional neutrophils will determine the outcome in most cases.

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This case is with regard to a male infant at the age of 40 days after birth. He was hospitalized because his abdominal mass was indicated during his 1 month infant general chekup. From the X-ray examination of the abdomen, ultrasound, CT scan, magnetic resonance imaging (MRl), and the angiogaraphy, we were able to give his diagnose as a solitary tumor of the liver or retroperitoneum and enforced the removal operation. All of the tumor was removed by a partial resection because we did not notice any malignant remarks of the revealed hamartoma of the liver during the biopsy of the operation. The histopathological diagnosis was the hemangioendothelioma of the liver. The hemangioendothelioma of the liver for infants are relatively rare. There are various reports about the imaging diagnosis and the histopathological diagnosis but only few reports exists for the MRI examination and the electron microscopic examination. This has been reported with additional reference to other studies including these remarks.

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A three year-six month-old-boy weas transfered to our hospital with chief complaints of respiratory distress and abdominal distention. After chemotherapy for one year, laparotomy was performed. In surgery, inferior vena cava (IVC) and abdominal aorta was not visible due to the huge tumor. The tumor was removed almost completely by resecting all the length of IVC with tumor emboli. Even with 48 Gy irradiation, recurrence was observed twice and all of tumors were resected each time. After two years, the patient is still hospitalized for chemotherapy of VAC protocol.

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We reported a rare case of extralobar pulmonary sequestration (EPS) with hydrothorax. At 34 weeks gestation, EPS was recognized as a homogeneous mass above the left diaphragm, and coexisting remarkable pleural effusion was clarified by ultrasonography. Immediately after delivery (35 weeks and 1 day gestation), an endotracheal tube was inserted because of severe respiratory distress, and close thoracotomy was performed to drain pleural effusion in the left chest cavity. Approximately 75 ml of pleural effusion was drained daily. After 3 weeks of respiratory intensive care the patient's condition was stabilized. Then a left thoracotomy was performed, when EPS was found. The pleural effusion disappeared soon after the resection of EPS and the postoperative course was uneventful. Association of EPS and pleural effusion is rare, as we could find only 20 reported cases whose prognosis was poor. All the patients having hydrops fetalis at birth could not survive. And coexisting hypoplasia of the lung, which might be induced by direct compression of pleural effusion, was the most frequent cause of the death in the perinatal period. When these risk facters were evaluated in this case, our patient survived because he had neither hydrops fetalis nor hypoplasia of the lung at birth.

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A female infant suffering from severe diarrfea after the long term administration of antibiotics was treated with twice rectal instillation of homologous faeces diluted in sterile saline. The first faecal enema carried out for the infant consisted of 7.5 gram fresh stool of her mother which was mixed in 30ml saline. The second faecal enema was done consisted of 6 gram stool. The diarrhea stopped and the volume of stool decreased to normal level within several days after the second faecal enema. She recovered well. Many kinds of anaerobic bacterias which had not been seen in her pretreatment stool were seen in her posttreatment stool.

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Complete excision is essential for the sacrococcygeal teratoma. Since these tumors adhere to the coccyx, the coccyx should be removed in continuity with the tumor. Nevertheless, recurrence of this benign tumors occurs infrequently. We experienced 18 year-old girl with a recurrenced huge intrapelvic sacrococcygeal teratoma 18 years after the resection. She visited our hospital complaining severe pain of the buttocks. Large mass was palpated in the right lower quadrant of the abdomen. Clinical investigations by CT and MRI revealed a recurrent sactococcygeal teratoma. Excised tumor was a benign cystic mass, 25X15X15cm, with a polyp, 7X3cm, on the inner surface. Now she has been free from the disease for 23 months since the second operation.

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