In order to evaluate whether or not antenatal diagnosis has made contributions to the clinical profile of hydronephrosis due to pelviureteric junction stenosis (PUJS), we examined the distance of the central echo complex (CEC) of the fetal kidney and the renal function of the infant. Methods : 18 fetuses with a disteded renal pelvis in past 5 years were retrospectively reviewed. For antenatal evaluation, CEC of fetal kindney was measured in the third trimester of pregnacy. Ccr, DMSA uptake, U-beta 2 microglobulin index and U-NAG index were measured as indicators of the renal function. Results : In all cases glomerular function remained normal. In seven of 18 cases, CEC distance was more than 15 mm. Six of these seven cases had tubular dysfunction. There was a significant correlation between U-beta 2 microglobulin index, NAG index and CEC. (p < 0.0l) Conclusion : Cases with CEC distance more than 15 mm may have tubular dysfunction, we should carefully follow up such patients.

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A case of chest wall hamartoma in a 5-month-old male infant with respiratory distress is reported. A chest roentgenogram showed a tumor with calcification occupying almost all of the left hemithorax. Computed tomography revealed heterogeneous mass arising from the posterior part of the left 3rd and 4th ribs. Laboratory findings were within normal range. En bloc resection of the capsulated 6.5 cm tumor and the posterior portions of the 3rd and 4th ribs was performed. Prosthetic material was not used to close the chest wall defect Histological examination revealed a granuloma-like fibrous tissue, a number of blood-filled aneurysmal bone cyst-like spaces, hyaline cartilage and bone tissue. The diagnosis was hamartoma. He is now free from recurrence and scoliosis 8 years after operation although sugery for correction of pectus carinatum was performed. There are only 41 cases of chest wall hamartoma in a review of the literature. If the patient prsesents respiratory distress etc, resection of the tumor including the involved ribs should be required. However, if the patient has no symptoms, resection can be postponed, because postoperative scoliosis may occur.

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We reported here a case of congenital mesoblastic nephroma. An abdominal mass was noted on fetal echogram and MRI imaging was carried out on 26 weeks of pregnancy because of polyhydramnios of the mother. A female baby, weighing 2176 gm, born by caeserian section presented with an abdominal mass of 9×11 cm in size and dyspnea. A laparotomy, done 20 hours after birth, disclosed a mass within the right kidney. A right nephrectomy was performed. The mass was 7×6×5 cm in size and weighed HOgm. The cut surface showed trabecular structure. Histo-pathologic diagnosis was congenital mesoblastic nephroma of fibromatous type and DNA flow cytometry showed a diploid pattern. Postoperative course was uneventful but moderate liver dysfunction. Neither postoperative radiation nor adjuvant chemotherapy was given and no sign of recurrence has been seen for 6 months since the operation.

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A case of neonatal ileal stenosis caused by a protruding intraluminal tumor of the vascular origin was reported. Thirty-day-old male infant was admitted to our hospital because of mild abdominal distention and bilious vomiting. On admission, abdominal scout film revealed markedly distended small bowel loops with air-fluid levels, compatible with small bowel obstruction. Using a long intestinal tube, the intestinal fluid and gas were evacuated until the symptoms and signs were relieved. The patient underwent laparotomy, tentatively diagnosed as small bowel stenosis of unknown origin. At operation, there was a marked proliferation of the small vessels in the mesentery involving ileal wall located at the site of 130cm from the Treitz's ligament The mesentery and the ileum at this lesion was resected. Histopathological study revealed a vascular origin tumor which was composed of capillary and cavernous hemangiomas and hemangiopericytoma. His postoperative course is uneventful.

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Two children with ileo-ileo-cecal intussusception caused by enlarged heterotopic gastric mucosa, mimicking hypertrophic Peyer's patch, are reported. Case 1 was a 6 year-old girl having previous 3 episode of intussusception. A barium enema reduction revealed a shadow defect at the terminal ileum. Case 2 was a 6 year-old girl with right lower abdominal pain due to suspected intussusception. Both were operated on and found to have protruding disc-like lesions at the antimesenteric part of the ileum. Although no diverticular projection of the ileum was observed in 2 children, vitello-intestinal remnant was strongly indicated by attached mesodiverticular artery. Seven patients with intussusception due to the heterotopic gastric mucosa were reported in Japan, and 3 of them were misdiagnosed as having hypertrophied Peyer's patches.

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The authors present the case of a 13-year-old female adolescent with tiny parotid adenocarcinoma which was manifested as cervical lymph node metastasis. The patient had noticed a walnut-sized lymph node on the left side of her neck for several months. Since its consistency was too hard for a reactive lymph-adenopathy, incisional biopsy was performed. Histological study revealed metastatic adenocarcinoma in the lymphnode. Ga-scintigram showed RI accumulation at the subparotid region identical to the residual lymphonode. MRI delineated the residual lesion adjacent to the lower pole of the left parotid. Other morphological studies including a sialography of the left parotid failed to detect the primary lesion. Considering the location of the lymphnode and the direction of the lymphatic drainage, parotid adenocarcinoma was highly suspected as the primary lesion, thus a total parotidectomy with radical neck dissection was performed. Histological study revealed a tiny adenocarcinoma of parotid at the lower pole of the superficial lobe and lymph node metastasis adjacent to it. Adenocarcinoma in children and young adolescents is rare, only four cases have been reported in the literature in Japan. This is the first reported case that was discovered through lymphnode metastasis.

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A 5-day-old male neonate was admitted to our hospital because of abdominal distension. Plain abdominal X-ray examination revealed dilatation of the sigmoid colon and the presence of free air below the diaphragm. Under the diagnosis of Hirschsprung's disease complicated by neonatal gastrointestinal perforation and generalized peritonitis, urgent laparotomy was performed. At laparotomy, a transitional region was apparently found in the upper rectum, and a perforation at the splenic flexure was revealed. The perforated site was exteriorized as a colostomy. Postoperative course was uneventful. After the operation, pathological, examination was performed, and he was diagnosed as short segment type aganglionosis. A modified Duhamel's procedure was performed at the age of 6 months. Now he is 5 years old, and doing well.

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A 6-month-old boy was admitted to our university hospital because of abdominal distension persisted for one month. Ultrasonography and Magnetic Resonance Imaging revealed a cystic tumor in the abdomen. Two days after admission, emargency operation was performed because he developed a respiratory disturbance. Laparotomy revealed a mass arising from the right lobe of the liver. Extended right hepatic lobectomy was performed. The tumor was 16×15×8cm in size and weighed 1080 gm. The tumor was mainly composed of cystic mass containing clear yellowish fluid: Histological examination revealed the cystic wall mainly consisting of the collagenized fibrous tissue and proliferation of the bile ducts and liver tissue was found. Malignant tissue was not observed. The diagnosis was mesenchymal hamartoma. The patient had an uneventful postoperative course without a recurrence for 9 months after operation. Mesenchymal hamartoma of the liver is a rare benign tumor. This case corresponds to the 43rd reported case in Japan.

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A 2-day-old boy of buccal hemangioma associated with Kasabach-Merritt syndrome was treated with steroids, radiotherapy, and ligation of the external jugular artery but the results were unsuccessful. 2 months later, subcutaneous injection of α-interferon (IFN-α) was started with a dose of 3,000,000 unit/m^2/day for 5 months. Supplement of blood products was not subsequently needed. Visible regression of the hemangioma was noted. Then, the platelet count, fibrinogen, APTT, and PT became normal, and IFN-α was decreased to a dose of 3,000,000 unit/m^2 every other day. After 7.5 months of treatment IFN-α was finished. This treatment initially caused a high fever and neutropenia, which resolved spontaneously. A few months later, liver dysfunction occurred but it was improved by tapering the dose of IFN-a. Repeated CT scans showed a resolution of the mass. IFN-α therapy was quite effective for Kasabach-Merritt syndrome with minimal side effects and it should be considered as the first agent of treatment for complex hemangiomas of infants.

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A male neonate presented a soft mass at the anterior left side of the neck and developed a cyanosis soon after his first bathing. Chest X-rays revealed pneumothorax in the left side. A CT scan of the neck showed that a huge cyst contained air and his trachea was dislocated by compression of the mass. Symptoms of airway obstruction spontaneously improved with oxygen supplement in a incubator. After reduction of local inflammation by IV antibiotics, he was referred to us at three months of age. A barium swallow test showed a communication between the left piriform sinus and the cyst through a draining fistula. Diagnosis was confirmed as a cervical cyst communicating a piriform sinus fistula. One month later we excised the cyst and fistula. A number of reported neonatal cases of a piriform sinus fistula associated with airway obstruction are increasing. But this is the first case associated with nneumothorax in the same side.

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[in Japanese]

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