Vitamin A status was assessed in 19 patients with biliary atresia treated by hepatic portoenterostomy. Plasma vitamin A levels were almost within normal range in patients without jaundice (T.Bil2mg/dl), but significantly low both in patients with slight jaundice (2≦T.Bil<5mg/dl) and severe jaundice (T.Bil≧5mg/dl). Intestinal absorption of vitamin A was maintained in patients without jaundice and with slight jaundice, whereas significant malabsorption was suggested in patients with severe jaundice. Because liver vitamin A levels revealed over 20μg/g wet tissue in each group, hepatic reserve was considered to be adequate. After oral administration of vitamin A, the increase in RBP was smaller in patients with low plasma vitamin A than those with normal vitamin A. These results suggest that vitamin A status in biliary atresia mainly depends upon hepatic RBP production.

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Since 1985, anterior sagittal anorectoplasty (ASARP) as a re-do operation has been performed in 10 postoperative patients with imperforate anus presenting severe to moderate fecal incontinence. The indication of this operation was defined as the presence of fecal incontinence and an anal orifice shifted anterior to the center of contraction of external sphincter muscle following electrical stimulation. The postoperative assessment of anorectal functions was based on a clinical as well as manometric and radiographical evaluations. The clinical score, based on the scale proposed by the Japanese Study Group of Anorectal Anomaly, showed that the postoperative anal function had improved significantly. The average total score was 6.1 postoperatively, a significant increase from a 3.4 preoperative score. Preoperative and postoperative incontinence scores were 1.9 and 3.4, respectively, and soiling scores were 0.4 and 1.4, respectively. Also, the improvement in the clinical score was progressive after re-do operation. In manometric studies, maximum anal pressure increased significantly from 18.2 ± 5.6 mmHg to 24.0 ± 6.0 mmHg postoperatively. Three patients of six who had a preoperative barium enema study, showed poor or fair anterior angulation, and the leakage of contrast medium from the anus was seen in three of these cases before operation. These radiographic findings were improved in all patients postoperatively.These results suggest that anterior sagittal anorectoplasty is a valuable procedure as a re-do operation for patients with fecal incontinence following a primary operation for congenital anorectal malformations.

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Recently available totally implantable venous access system (TIVAS) facilitates administration of hyperalimentation, chemotherapy and blood products and taking blood samples. Furthermore TIVAS enables patients to care at home and to bathe. We have used TIVAS in 33 children with cancer (Port-A-Cath in 15, Infuse-A-Port 18) between August 1988 and March 1990. The mean age of the patients was 4 years 10 months (range, 3 months to 14 years 10 months), and the mean functioning time was 207 days (range, 13 days to 572 days). Major complications were regarded as infection (4 patients, 12.1%) and occlusion (7 patients, 21.2%). Catheters were removed in 2 patients due to infection. Six out of 7 patients with occlusion were treated by changing the site of the catheter tip, but in almost all cases the catheters were imcompletely occluded. TIVAS is a useful armamenterium in the management of infants and children with cancer.

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Ureterocystoneostomy (UCN) by submucosal tunnel method is most reliable procedure in the surgical management of vesicoureteral reflux (VUR). We reviewed the clinical data of 361 children who underwent antireflux surgery between July 1973 and December 1988 and followed for more than 1 year after the operation. The incidence of patients undergoing UCN was 40 per cent among 901 children with primary VUR treated during the same period. Surgery was indicated in 65 per cent of ureters with grade VI reflux and 74% with grade V reflux, while 25% of grade III and only 19% of grade I-II underwent operation. Renal scarring was detected at the first examination in 36% of kidneys in the surgical group. Among 63 kidneys which showed progression of renal scarring, only 17 kidneys revealed the pathological change at later than 2 years after surgery. The prevalence of a small kidney was 18%, and accelerated growth after successful surgery was only exceptional. The complications of antireflux surgery consisted of VUR, obstruction and bladder diverticulum. Persistent VUR was observed in 12 ureters (2.0%), and 3 of the ureters required reoperation. Contralateral reflux following unilateral UCN was detected in 17 ureters (14%) on the first postoperative evaluation, and in 10 of the ureters reflux stopped spontaneously. No ureter required surgical correction. Persistent obstruction was seen in 11 ureters (1.8%), and 4 of the ureters required reoperation. Bladder diverticulum with significant amount of residual urine was observed in 6 patients (1.7%). Surgical correction was needed in one patient associated with persistent VUR. In total the incidence of complications was 5.0% in our series. Lower urinary tract infection was seen in 10 per cent of patients after UCN, and females were 2 times more frequently affected than males. The postoperative incidence of pyelonephritis was 6.6% which was far lower compared to the preoperative incidence. Antireflux surgery is highly reliable and protects renal scarring. The majority of complications are avoidable when careful patient selection and meticulous technique are carried out.

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Intragastric pressure in the patients with hypertrophic pyloric stenosis and normal children was measured using an open tip manometer with a 3-lumen tube. In the stomachs of 13 normal children, 1.79 peristaltic waves per minute with strength of 10.5 cmH_20 and duration of 10.5 seconds were observed. The pressure at the lower esophageal sphincter (LES) was 22.3 cmH_20. In 31 patients with hypertrophic pyloric stenosis, the peristaltic waves showed 21.7 cmH_2O in pressure and 21.1 seconds in duration. Its frequency was 1.11 contractions per minute. The LES pressure in the children with pyloric stenosis was 28.0 cmH_20. In one week after pyloromyotomy, the pressure of the peristaltic waves decreased to 8.4 cmH_20 with duration of 17.8 seconds, and frequency of 0.63 contractions per minute. All these values reduced from those before pyloromyotomy. The LES pressure also reduced to 21.8 cmH_20.

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Distal esophageal pH was monitored in 8 infants without any symptoms of GER. During these periods the patient recieved 15 to 20ml/kg of 5% glucose solution twice every 3 hours apart per os. After each meal was fed the patient was layed supine or prone for 3 hours, respectively. The following conditions were measured and compared between supine and prone positioning; the number of refluxes, number of refluxes of more than 5 minutes' duration, the duration of longest single reflux and percentage of time that the esophageal pH was below 4. Although remarkable GER was noted during supine positioning, prone positioning significantly diminished the extent of reflux during first 2 hours after feeding.

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It is well-known that congenital hemihypertrophy has a tendency to be associated with other abnormalities and/or tumors. Here we reported such cases. Case 1 : A 5-month-old boy with hemihypertrophy of left side extremities, first noted at the age of two months, was transferred to the authors' hospital. He had abnormal findings on the right lobe of liver which was the contralateral side of hemihypertrophy. Hepatic hemangioma was diagnosed, but a malignant tumor was undeniable because he had sufferred from congenital segmental hemihypertrophy. After the liver was partially resected, capillary hemangioma was histologically evidenced. Case 2 : A one-month-old girl was transferred to the authors' hospital because of right hemihypertrophy noted from birth. She had the cutaneous hemangiomas and varicosities. Her diagnosis was Klippel-Weber syndrome and she had been followed. At the age of 2 years and 6 months, a huge tumor in right lower abdominal cavity was palpated under thick subcutaneus tissue. Right ovarian cyst was diagnosed. Six months later, right oohorectomy was done. Serouscystadenoma of ovary with borderline malignancy was histologically diagnosed. One month after the oohorectomy, she suddenly died from asphyxiation at her home. Autopsy was not done. She had multiple abnormalities : hemihypertrophy, macrodactyly, subcutaneous mass, exostosis, epidermal nevus, varicosities, scoliosis, macrocephaly, mental retardation, asymmetric eye size, high myopia, epibulbal tumor, optic disc pallor, strabismus, cataract, nystagmus, chorioretinal degeneration. Her condition was thought to be Proteus syndrome, originally named by Wiedemann et al. in 1983.

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Iatrogenic esophageal perforation in a newborn may happen during intensive care of prematures. Its clinical and roentogenographic findings resemble those of esophageal atresia or double esophagus. One case of traumatic pharyngoesophageal pseudodiverticulum in a low-birth-weight newborn was presented in this paper. A Japanese female infant was born weighing 1600g at 38-week gestation. Soon after delivery, she was transferred to our hospital because of dyspnea and cyanosis. We unsuccessfully attempted to pass a catheter into the stomach. In contrast X-ray examinations carried out with isotonic water soluble contrast material through a catheter was demonstrated a pseudodiverticulum. The lesion extended to the level of the diaphragm in the posterior mediastinum. A diagnosis of accidental esophageal perforation was made. It was caused by oropharyngeal suction at birth. She was treated successfully with antibiotics and gastrostomy. Since esophageal perforation in a newborn was first reported by Eklof in 1968, 90 cases have been reported in literatures. Therapy should be selected and based upon not only the type and extent of injury, but the underlying condition of an infant

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Ovarian torsion with inguinal hernia in 3- and 4-month-old girls were reported. Taxis was attempted but failed in both patients. On admission, 5X4cm, and 4X3cm, oval-shaped tender masses, which were heterogenic echogenic on US were found at the left inguinal region with redness and swelling of the left labia majora pudendi. At exploration, clockwise torsion (360 degrees) of the left ovary and tip of Fallopian tube was found in both patients. Histologic examination showed massive hemorrhage and complete necrosis of the ovaries.

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A case of neurocristopathy is reported in a 3-day-old boy admitted to our hospital for cyanosis and respiratory distress. His respiratory condition required for respiratory support. He had an extensive aganglionosis, and underwent an jejunostomy operation on the 23rd day of his life. His associated anomalies are congenital central hypoventilation syndrome, Mobius syndrome, sensory deafness and autonomic imbalance. All of these disorders are consist with a clinical entity of'the neurocristopathy'reported by Bolande in 1974. This is the first report on neurocristopathic syndrome associated with the five different diseases. Pathogenesis of neurocristopathy is discussed.

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Congenital stricture of the mid-ureter or lower one third of the ureter is extremely rare. Five children with this type of ureteral stricture were treated during the past 17 years. In these five children, two presented with abdominal tumor, one with hydronephrosis on prenatal ultrasonography, and one with growth retardation due to renalfailure. In one boy, the disorder was detected through the preoperative X-ray examination for hypospadias. In addition to routine IVP and voiding cystourethrography, all children had retrograde pyelography prior to or at the time of their operation. In two cases, the stricture was found at the middle ureter, and in three cases at the lower one third of the ureter. Three children had contralateral abnomalities; two with multicystic kidney and one with PUJ stenosis. In the selection of operative procedure it depends on whether or not the lower ureter distal to the stricture has normal ureteral movement. In two of cases, normal peristalsis of the distal ureter was demonstrated on IVP, and in one on antegrade pyelography under fluoroscopy. In two cases, intraoperative pressure-flow-study revealed normal intraluminal pressure of the distal ureter. End-to-end ureteral anastomosis with resection of the stricture lesion was made in all children. On Postoperative IVP their hydronephrosis and hydroureter improved in all cases.

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Our recent experiences of two cases with pseudotumor of the lung were peresented. Case 1 was a 7 year-old female who presented with a two-week history of fever. An X-ray film of the chest showed tumor in the right lower lung field. Tumor markers and PPD were negative. The tumor which was located in the segment 7-10 was extirpated without excising the adjacent lung tissue. Case 2 was a 7 year-old female who was referred to us due to a tumor shadow in the left lower lung field on the chest X-ray film which was taken because of the positive PPD. She underwent extirpation of the tumor which was located in the segment 8. Histological diagnosis of both tumors was inflammatory pseudotumor of the lung. Surgical excision is the choice of treatment in order to rule out the malignant variety. However, it is important to limit the extent of excision after making an immediate histological diagnosis from frozen section because of the benign feature of this disease.

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Congenital epulis is a rare tumor of the gingiva and only 60 cases have been reported in Japan. A female newborn was noted to have a mass, mesuring 18X15X14mm, arising from the right upper gingiva into the oral cavity. The tumor was excised at the 30th days of age. Postoperative course was uneventful and no recurrence has been recognized for 1 year until the time of this report. Histological examination of the biopsy specimen revealed that the tumor was granulomatous epulis, an extremely rare entity in newborn. Although congenital epulis is generally characterized by the presence of granular cells, they were not observed in this case. The pathology, clinical features and treatment were described with reference to previous reports.

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A total of 34 prenatally diagnosed cases of meconium peritonitis, including one personal and 33 reported cases, are reviewed retrospectively. Of these 34, meconium peritonitis was diagnosed by workup for obstetric fetomaternal problems in 23, by regular ultrasound examinations in 10, and by routine checkup preceding pregnancy termination. In addition to intraabdominal calcification and intestinal dilatation, fetal ascites, abdominal cyst and abdominal mass were sonographically revealed in 17, 5, and 5 cases, respectively. Four of these antenatally diagnosed fetuses underwent abdominal paracentesis, and another 3 were delivered by cesarean section. Previous reports show that none of those cases detected before the 26th week of gestation could survive after birth. Based on these results, a systematic approach to obstetrical management of fetuses with meconium peritonitis is outlined as follows. First, fetal paracentesis should be conducted in an attempt to ensure an adequate pulmonary growth when a developement of fetal ascites results in a rapid increase of the abdominal circumference before the 28th week of gestation. Secondary, elective preterm cesarean section could be justified to improve the operative outcome after 28 weeks of gestaion or once fetal lung maturity is obstetrically considered adequate.

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We reported a case of immature teratoma of the ovary with gliomatosis peritonei. A ten-year-old girl was admitted to our hospital for evaluation of an abdominal mass. On physical examination a huge mass was palpable in the lower abdomen. Serum alpha-fetoprotein (17.8ng/ml) and CA125 (300U/ml) levels were elevated. At laparotomy, a large left ovarian tumor with adhesion to the omentum was found, and multiple white nodules were present on the surface of omentum and peritoneum. The primary tumor weighed l,535g and measured 18X15X9 cm. Histological diagnosis of ovarian solid tumor was grade 1 immature teratoma. The omental and peritoneal implants were composed exclusively of mature glial tissue (grade 0). After operation, she was treated with combination of cisplatin, vinblastine, and peplomycin (PVP) for four courses. She has been doing well for 15 months postoperatively. Gliomatosis peritonei is a rare complication of ovarian teratomas. Twenty-one cases of gliomatosis peritonei in children, including the present case, were reported in Japan. Fifteen cases were immature ovarian teratoma, and 6 mature teratoma. All of 16 cases described in prognosis were alive and well from 7 months to 21 years after the operation. It is suggested that gliomatosis peritonei associated with ovarian teratoma has a favorable prognosis.

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