Eleven post-operative newborns and babies who had poor weight gain in spite of full milk feeding had a supplementation of polymeric diet to breast milk or powdered milk. The polymeric diet included dextrin, rice oil, casein, minerals and vitamins which are more easily absorbable than milk by alternative pathway for absorption of saccharides. They achieved accelerated weight gain significantly while the polymeric diet was given (from 13.5±8.4g/day to 32.7±18.2g/day; n = 14) and they had less weight gain after it was discontinued in spite of minimal change in total caloric intake (from 42.0±18.9g/day to 9.5±10.1g/day; n = 7). The transaminase increased in 1 patient and the diarrhea became worse in another who had ileostomy while the polymeric diet was supplemented over about 40% of total calory intake. The supplementation of the polymeric diet, therefore, is recommended to be limited less than 30-40% of the total caloric intake.

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Most patients with neuroblastoma (NB) achieve some response to aggressive chemotherapy. However, some of them have relapse and the tumors subsequently become refractory to drugs. The development of resistance to chemotherapy is a major problem in the treament of NB. We have examined the development of resitance to a high dose of Cyclophosphamide (CPM) in human NB growing as a xenograft (HNX) in nude mice. Three types of HNX (CH-NBI, CH-N-1, CH-NB-4) were treated in consecutive passages with a single dose of 250mg/kg CPM. At each passage the first treated tumor to regrow was transplanted into 10 fresh mice. Of these mice half were treated and half served as controls. This procedure was repeated six times. Growth curves were plotted and the time taken for treated and control groups to double in volume was obtained. Tumor growth delay (TGD) was calculated as the difference between these values. Various changes in TGD produced by repeated CPM treatments were observed in three HNX. TGD dropped from 10.7 to 7.1 days in CH-NB-4 (p < 0.05) at the 2nd transplant and from 9.6 to 7.5 days in CH-N-1 at the 5th transplant and no further decrease was observed by additional treatment in any tumor. TGD was 5.3 days in CH-NB-I at the 1st transplant and no decrease was observed by additional treatment. Treatment with CPM resulted in a rapid reduction in responsiveness to CPM in two HNX but no reduction was observed in one HNX which was considered to have primary resistance to CPM.

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PURPOSE : To our knowledge, there are few published data concerning hemodynamics of abdominal organs in children. Therefore, an analysis of hepatic and splenic hemodynamics in normal children using color Doppler ultrasonography (CDUS) was undertaken. MATERIALS AND METHODS : Hundred and six normal children were divided into four age groups (neonatal, infantile, preschool, and school period) and were studied. Mean blood flow velocity (Vmean), blood flow volume (Q), blood flow volume per body weight (Q/BW) in portal vein, splenic vein, proper hepatic artery, and splenie artery were measured using Aloka SSD-870. RESULTS : 1. Vmean in portal vein was 15.4±3.5cm/second, that in splenic vein was 11.3±2.3, and there were no significant differences between the groups. But Vmean in arteries was significantly larger in the elder age groups. 2. In all the studied vessels, Q was significantly larger in the elder age groups. But Q/BW was significantly larger in the younger age groups. 3. Total liver blood flow volume seemed to reflect liver weight per body weight ratio. 4. Usefulness of CDUS in analizing hepatic and splenic hemodynamics in children was proved. CONCLUSION : We are intending to analyze hepatic and splenic hemodynamics in digestive diseases in children using data provided by this study.

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To investigate injury of arterial wall with canulation technique, an experimental study was performed with a rabbit model of canulation to right common carotid artery (RCCA). After 3-7 days, segments of the RCCA were excised and examined histologically. Arteriotomy sites in the segments of the RCCA were compared with more proximal parts. Histrogical findings revealed that arterial walls were more severely injured in the arteriotomy sites than in the proximal parts. Therfore, end-to-end anastomosis after excision of arteriotomy sites was proposed for reconstruction of the RCCA. From this experimental study, we learned to make reconstruction of the RCCA in 4 neonates treated with extracorporeal membrane oxygenation (ECMO). Repaired RCCAs with end-to-end anastomosis were patent without significant stenosis, and no signs of unilateral brain injury appeared. These findings suggested that reconstruction of the carotid artery could be performed safely.

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A 0-day-old boy with congenital cystic adenomatoid malrformation (CCAM) of the lung is reported. Fetal ultrasonography showd multiple small cysts of the left lung, at the 20th gestational week. Sequnetial examinations revealed change of the characteristics of the lesion at 31st and 38th weeks in gestation. Agter birth, he developed no symptom. A large single cyst in the middle portion of the right lung, and multiple small cysts in the middle nad lower portion of the left lung were identified on plain chest X-ray and CT scans. The large cyst of the right lung and multiple small cysts in the middle portion of the left lung disappeared spontaneously on the 7th day of life. The multiple small cysts of the lower portion of the left lung got smaller, but were persisted. The scintigraphy revealed no function of the left lower portion. The left lower lobectomy of the lung was performedat the age of 17 days. The histological findings were consistent with those of CCAM Stocker type2. Seventeen cases of CCAM including our case have been reported, in which the antenatal findings of CCAM were compared with those of postanatal ones. Seven out of 17cases showed the taransformation during perinatal period. This fact is important to decide the timing of the surgery because the pulmonary lung diseases including CCAM and emphysematous diseases may decrease in size or disappear in the first fewmonths after birth.

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A 23-year-old female undergone a pull-through for cloacal malformation at 1-year-old, complained of a huge rectal prolapse and menstruous disturbance. She received vaginal skin-flap plasty for vaginal orifice deformity and Gant-Miwa's procedure for rectal prolapse. Because of recurrence of rectal prolapse, she received Gant-Miwa's procedure again and Thiersch repair additionally, but failed again. Barium enema, manometry, MRI showed that the cause of rectal prolapse was due to poor fixation of rectum to the pelvic wall. We performed Ripstein operation for her via a transabdominal approach. She got off the prolapse completely without any discomfort after Ripstein operation. Ripstein operation is a proper procedure for the prolapse after pull-through of imperforate anus, due to poor fixation of rectum to the pelvic wall.

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We tried to treat a 2-year-old girl with congenital duodenal stenosis in the second portion with the endoscopy. We could not perform endoscopic resection of the diaphragm, because we could not observe the papilla of Vater. We discuss the indications for endoscopic treatment to congenital duodenal stenosis in an infant. Endoscopic treatment is useful in some cases of congenital duodenal stenosis and therefore should be performed carefully and actively before surgical treatment.

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Two cases of neonatal spontaneous duodenal perforation are reported. Case 1: A 8-day-old girl was admitted with melena. She was found at surgery to have a rupture at the bulbus of the duodenum. A simple closure was performed. She was put on an anti-acid therapy and, her postoperative clinical course was uneventful. Case 2: A 5-day-old girl was referred as a diagnosis of gastrointestinal perforation. An emergency laparotomy revealed a perforation measuring 2×2 mm at the bulbus of the duodenum. A review of the Japanese literatures regarding this disease published between 1980 and 1993 revealed 27 cases. Girls were predominated. Surgery was done most frequently at 3-day-old. The duodenal bulbus was the most common perforation site and a small punched-out lesion was found frequently. A favorable prognosis was obtained by a simple closure of the perforation. The pathogenesis of the disease has been reported to be an acute peptic ulcer due to a perinatal stress. The most suggestive treatment would be a simple closure of the perforation, and postoperative anti-acid therapy would be recommended.

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We experienced 2 cases of atrio-visceral heterotaxy syndrome (asplenia syndrome) associated with esophageal hiatus hernia. Case 1 presented vomiting and malnutrition due to GER caused by esophageal hiatus hernia and then underwent the plication of esophageal hiatus alone. Case 2 presented prominent cardiac symptons such as cyanosis, dyspnea due to complicated cardiac anomaly, however didn't showed any digestive symptons related with large sliding hiatus hernia, and then underwent cardiac operation. Many cases of asplenia syndrome associated with esophageal hiatus hernia demonstrated poor prognosis because most of them died of cardiac failure, respiratory insufficiency and systemic infection after the operation of esophageal hiatus hernia. Therefore invasive surgical procedure should be avoided. Less invasive appropriate treatment should be chosen according to patient's general conditions and clinical symptons in consideration of concomitant complicated cardiac anomaly and immature immune system.

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A case of a relatively rare form of congenital coronary arterial fistula (CAF) was reported. A one year and 9 months old boy was taken to our hospital for evaluation of a grade 3 systolic murmur and a grade 2 early diastolic murmur in the aortic area. The coronary cineangiogram showed the remarkably dilated circumflex (Cx) and the CAF originating from the Cx and draining to the left ventricle (LV). We operated using a cardio-pulmonary bypass and closed the CAF by a transarterial approach (Symbas method). Heart murmur wad disappeared postoperatively, and no evidence of myocardial ischemia in ECG was noted during the postoperative course so far. The postoperative coronary cineangiogram revealed that : 1) the Cx was occluded at just the portion of taking off the left anterior descending branch. 2) the CAF was closed completely. 3) the posterior wall was perfused by collateral flow from the right coronary artery. A congenital CAF is not a rare malformation. A CAF, however, originating from the Cx and draining to the LV is a relatively rare form. We consider that Symbas method is a most reliable method for CAF closure. A post operative anti-coagulant therapy is mandatory even for an infantile case.

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Recently the effective chemotherapy against hepatoblastoma have been reported by American and European group studies. In this paper three patients with unresectable hepatoblastoma on admission were reported. They were treated by the protocol of Japnaese Study Group for Pediatric Liver Tumor (J ・ PLT), including THP-Adriamycin and Cisplatin. Following either 3 or 4 courses of chemotherapy, tumor sizes, evaluated by magnetic resonance imaging (MRI) , reduced to 64, 68 and 46%, respectively. In addition, serum levels of α-fetoprotein decreased to 0.1, 0.6 and 1.6%. Tumors were totally resected by the second look operation. At present (April, 1994), theree patients are in tumor free conditions for 26, 20 and 16 months after their operations. Although the observation periods were not long enough, J ・ PLT protocol is indicated to be effective to patients with hepatoblastoma.

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Two cases of piriform sinus fistula were presented. [Case 1] An 11-year-old female had repeated episode of swelling in her anterior neck since infancy. Fistulography under endoscopy demonstrated right piriform sinus fistula. Fistulectomy was performed by dye injection into the fistula under endoscopy. [Case 2] A 1-year 9-month-old male was noted to have swelling in his anterior region of neck. Left piriform sinus fistula was diagnosed with fistulography. In this case, during operation the side wall of left piriform sinus was opened with the help of endoscopy and the bottom part of the mucosa of the sinus was transected with the internal orfice of the fistula. With an aid of endoscopy, correct diagnosis and treatment of piriform sinus fistula were made in these cases.

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This is a report of two cases of congenital vesical diverticula in infants. Case 1 is a 2-month-old boy with acute urinary retention and consequent acute renal failure, and case 2 is a 5-month-old boy with urinary tract infection due to left vesicoureteral reflux. Thirtyeight other cases of infants and children in the Japanese literature are reviewed. Congenital vesical diverticula are more frequent in male (38 of 40 cases), and mainly located around the ureteral hiatus (32 of 40 cases). Acute urinary retention and urinary tract infection are the main presenting symptoms of vesical diverticula.

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A 2-year-old female of anal canal duplication is reported. Anal canal duplication is a rare abnormality. Only 7 cases including this case have been reported in Japan. These all 7 cases are female. Abnormal anal pittings are located just distal position of the dentate line behind the anal canal. They are covered transitional epithelium and have anal glands and no ganglion cells. We think the etiology of the anal canal duplication is a duplication of the dorsal cloaca. No complication of the anal canal dulication is reported at present. We think, however, there is a possibility that it may cause periproctitis or anal carcinoma. And it's removal is not difficult by the penial approach and the postoperative course must be uneventful, we recomend the anal canal duplication should be removed in early period of life.

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