Twenty three patients with congenital choledocal dilatation were treated between 1977 and 1992 at our department. All patients underwent the excision of dilatated extrahepatic bile duct and hepaticoenterostomy uneventfully. Endoscopie retrograde cholangiopancreatography (ERCP) was performed on 9 patients who sometime presented abdominal pain after the operation. Five of 9 patients were found to have long remnant intrapancreatic bile duct from 10mm to 25mm in length. These five patients showed the same pancreaticobiliary maljunction (PBMJ), in which the pancreatic duct joins the common bile duct (P-C type). One patient had the remnant intrapancreatic bile duct (25 mm in length), in which there were 2 small caliculi. These stones were removed successfully with endoscopic sphinclcrotomy technique. Long-term follow up of patients with choledochal dilatation revealed some problems related to remnant intrapancreatic bile duct, such as pancreatitis, calculus and carcinoma. Therefore, we need to remove dilatated choledochus including intrapancreatic bile duct as completely as possible at the initial operation. ERCP is recommended to patients with abdominal pain, and with PBMJ of P-C type postoperatively

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In many hospitals lymph nodes are dissected by pick-up procedure but in our hospital systematically for the patients with neuroblastoma discovered by mass screening. To clarify the characteristics of the metastatic lymph nodes we analysed neuroblastoma patients treated in Hokkaido University Hospital. As a results, there were no correlations among primary sites, pathological diagnosis, urinary VMA-HVA concentration and the cases with positive lymph nodes. By systematic lymphadenectomy. positive lymph nodes were found in 6 out of 26 cases. Postoperative complications occured in five cases, consisting of four chylous ascites and one adhesion ileus. Relaparotomy was required in two cases. As the prognosis of these patients are generally very well, systemic lymph nodes dissection should not be required in early cases. But in cases with positive lymph nodes or advanced cases, lymph nodes around the aorta shuold be carefully looked for.

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The area of papilla of Vater including the pancreaticobiliary duct junction site was histologically investigated by serial sections in 15 autopsy cases of biliary atresia (BA). Fifteen human fetuses aged 12 to 28 gestational weeks and autopsy materials of 25 infants and 30 adults without pancreaticobiliary diseases were similarly investigated as controls. The length of the common pancreaticobiliary channel was 6.3 ± 1.5 mm in 7 BA cases, in which both the pancreatic and bite ducts were present in the papilla of Vater, and it was significantly (p<0.01) longer than that in infant and adult controls. In all 7 cases, the pancreaticobiliary junction site was located near the region where both the bile and pancreatic ducts penetrated the duodenal wall. In the other 8 BA cases, in which the bile ducts were absent in the papilla of Vater, it was histologically supposed that the bile ducts were secondarily disappeared near the outer duodenal wall. The length from the duodenal orifice in the major duodenal papilla to the outer duodenal wall, i.e., nearly the length of the papilla of Vater, was significantly longer (p<0.05) in the 15 BA cases than in normal infant controls. These results indicated that BA has a long pancreaticobiliary common channel and there is much possibility of the same etiology in either BA or CBD. It was thus suggested that pancreaticobiliary maljunction is etiologically responsible not only for CBD but also BA.

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In Japan, it has been considered that serum bilirubin (T.bil.) less than 2.0mg/dl after radical operation of biliary atresia (BA) means 'jaundice-free'. To assess the right jaundice-free level, 27 patients (operated from 1977 to 1993) whose T.bil. were less than 2.0mg/dl, were reviewed. They were divided into two groups according to bilirubin level, group A (T.bil.<1.0mg/dl, n=15) and group B (1.0≦T.bil.<2.0mg/dl, n=12). The occurrence of the cholangitis was not significantly different in two groups. The recurrent and prolonged jaundice was recognized in two patients in the group B, whereas none in the group A. The patients in the group B had significantly higher levels of serum transaminase (p<0.01). γ-GTP (p<0.05), and T. B. A. (p<0.05). The grade of esophageal varices were significantly higher in the group B (p<0.01). The association of the hypersplenism was not significantly different, but both two severe cases received PSE or splenectomy belonged to the group B. From these results it was considered that there would be significant differences in clinical outcome between the two groups. Therefore the patients who have higher bilirubin level than 1mg/dl should be observed more carefully, and it would be necessary to reconsider the serum bilirubin level for 'jaundice-free'.

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Anal endosonography was performed in 17 patients, aged 7 to 18, after surgical correction of anorectal malformations. Ten of the 17 had high type, 5 had intermediate type, and 2 had low type anomalies. They had the transverse images for the anal canal and the images were obtained at 5cm, 4cm, 3cm, 2cm. and 1cm from the anal verge, respectively. In the low type anomaly, external and internal anal sphincters were visualized as hyperehcoic band and hypoechoic band, respectively. However, 1 of the 2 showed a partial defect of the internai sphincter after anal transposition. In the intermediate type anomaly, the external anal sphincter was observed as a hyperechoic band in all of the 5 patients. However, the border or its echo level was unclear. And the internal sphincter could not be observed. In the high type anomaly, the hyperehoic band was observed in all of the 10 patients. However, its border or the echo level was not clear. In addition, the presence of external sphincter muscle in the high and the intermediate type anomalies was less remarkable than that in the low type anomaly. As for the presence of internal sphincter muscle, 5 of the 10 with high type anomaly showed a presence of the internal sphincter muscle and other 5 did not.

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Although the respiratory management with high frequency oscillatory ventilation (HFOV) has been generally performed for neonates with congenital diaphragmatic hernia (CDH) in Japan, the entry criteria for extracorporeal membrane oxygenation (ECMO) based on the data from the patients underwent HFOV has not been reported. To establish the entry criteria for ECMO in CDH patients treated with HFOV, the oxygenation index (OI) and the alveolar-arterial oxygen gradient (A-aDO_2) were retrospectively studied from 20 neonates with CDH treated with HFOV in our institutions from 1987 through 1993. OI and A-aDO_2 values were based on preductal and postductal arterial gas data drown from the admission to following 72 hours and detail ventilatory conditions. Patients were divided into two groups: candidates for ECMO. who underwent ECMO and died without ECMO. and non-candidates, who survived without ECMO. An preductal and postductal OI(mean value for 4 hours)>30, a postductal A-aDO_2≧620 torr for 4 hours and a postductal A-aD0_2≧580 torr for 8 hours showed a better sensitivity along with a specificity of 100% than the previous entry criteria that have been used in our institutions; a postductal OI>40, a postductal A-aDO_2≧610 torr for 8 hours and a postductal A-aDO_2≧600 torr for 12 hours. In addition, a combination of a preductal and postductal OI>30 indicated a sensitivity and a specificity of 100%.

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Lung thorax transverse area ratio (LT ratio) was proposed for ultrasonograpnic evaluation of lung hpoplasia in fetal diaphragmatic hernia. LT ratio was defined as a square area of bilateral lungs divided by that of thorax measured at the chest transverse section containing 4 chambers of the heart. The coefficient of variation of the measurements were 8.3% in inter-examination reproducibility and 6.4% in inter-examiner reproducibility. The LT ratio of 156 normal fetuses from 17 to 38 gestational weeks ranged from 0.43 to 0.64 (0.52 ± 0.04). There was no significant difference among 5 groups of 17-20, 21-25, 26-30. 31-35 and 36-38 gestational weeks. The LT ratios of 17 cases with diaphragmatic hernia ranged from 0.08 to 0.42 (the mean ± SD; 0.20±0.09), which was significantly lower than that of the normal controls. In the 13 cases without major cardiac anomalies, the LT ratio was significantly correlated with postnatal preductal arterial blood PO_2, PCO_2, PH and A-aDO_2. There was a significant difference in a requirement of HFO (high frequency oscillation) and ECMO (extracorporeal membrane oxygenation) and survival rate between two groups with the LT ratio greater than 0.20 and less than 0.20. From these data, measurement of the LT ratio using a fetal ultrasonography may be a reliable method for evaluating the degree of lung hypoplasia in diaphragmatic hernia.

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A normal-sized penis may appear to be very short in obese infants or boys because of fat deposits in the pubic region. In these cases, no surgical treatment is required and the condition is usually self-correcting. On the otherhand, true buried penis is a congenital abnormality which needs to be corrected by operation. Because the skin is unattached to the penile shaft, the penile shaft appears as if it is absent. However, when the skin is drawn back, there is a normally developed corpus. In the past 6 years. 45 cases of phimosis were operated and 10 of them were accompanied with buried penis. The operation is started by correcting the phimosis. Circumcision is contraindicated since it may cause the lack of the skin. Then U-shaped and Z-shaped skin incision lines are marked ventral and dorsal side of the penis respectively. After freeing the penile shaft from the tethering fibromuscular bands between the penile shaft and abdominal wall. U-shaped incision is approximated to Y-shaped line and Z-shaped incision are sutured by changing the position of the skin flaps. The results of the operation are successful in all 10 cases with normal appearance and function. We describe a simple technique of correcting the buried penis which provide excellent functional and cosmetic results.

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Supraumbilical skin incision was first utilized in 1986 by Tan and Bianchi. From June 1994 to August 1995. we tried pyloromyotomy with this procedure in 15 infants (23-98 days of age at operation, 12 males. 3 females). Wound infection was recognized in one child but healed soon with antibiotics. All incisional wounds were incorporated well into the umbilical creases. From the cosmetic standpoint, the supraumbilical incision is as satisfactory as that of laparoscopic approach, while supraumbilical approach is simpler.

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Informed consent is very important in pediatric surgery practices and rather unique, because the parent or the guardian of a patient must be informed and give the consent. Until now they seldom had a chance to observe the operation itself. From June 28 to December 31, 1994, the authors transmitted pictures of 114 pediatric operations in real time to the family at the different room in the hospital. This was a trial in the extended informed consent. After observing the operative pictures with real-time monitoring system. 109 out of 114 parents or guardians answered questions about this trial. Many thought that viewing the operation markedly improved understanding the disease and was worthwhile. Many commented that they felt a sense of, closeness to their child during the operation. Less than 10% of respondents give any negative opinions.

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A 14-year-old girl was admitted with right lower abdominal pain. At first, acute appendicitis was suspected, but abdominal ultrasonography revealed an unhomogenous iutraabdoniinal tumor and fluid collection. Based on the findings of ultrasonography, CT scan, MRI and the clinical history, we thought it to be ovarian hemorrhage. So, initially she received conservative treatment. On the 4th hospital day, because of ongoing anemia we did laparotomy. The right ovarian hematoma was ruptured and the intra abdominal blood volume was about 300ml. Wedge excision of the right ovarian hcmatoma was performed. Ovarian hemorrhage is thought not to be so rare in grown-up women, but in a review of the Japanese literature only 12 cases of adolescent girls under 15 years old including our case are reported.

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Congenital ureteral valves are so rare that up to the end of 1994, only 35 cases have been reported in Japan, including 14 pediatric cases. We encountered a 14-year-old male patient with an ureteral valve. He had left flank pain as his chief complaint. His preoperative diagnosis was left hydronephrosis resulting from stenosis of the ureleropelvic junction. During subsequent surgery, we confirmed a diaphragmatic ureteral valve having a pinpoint opening in the center of the lesion, and removed it. Most cases of this disease reported in the literature have been diagnosed during surgery or at postmortem examinations. However, there are recent reports suing that it was diagnosed preoperatively and that the treament was conducted non-invasively by using the endoscopic method. Keeping in mind that this disease can be a factor associated with disturbances of the upper urinary passage, it is important to attempt to diagnose this disease before surgery.

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Epignathus is a rare disease of newborns that often causes respiratory distress. We present a case of a large epignathus that was diagnosed antenatally. This lesion was detected at 28 weeks and 1 day of gestation as a fetal anomaly with polyhydramnios. Despite efforts to prolong gestation with amniocentesis, uterine contractions started. During Ihis period, a multi-disciplinary team approach was initiated, including specialists in pediatric surgery, neonatoloey, anesthesilology and oral and maxillo-facial surgery. At 32 weeks and 4 days of gestation, the infant was delivered by cesarean section after 7 minutes of general anesthesia. The umbilical cord was clamped and cut at 6 minutes after delivery, and orotracheal intubation was performed. Thereafter, a tracheotomy was performed because the tumor was so large that observation and manipulation were difficult. Since tha stalk of the tumor was visible, ligation using rubber tubing on the stalk was performed. The infant needed respiratory support for 6 days. The tumor shrank and became necrotic; it was resected on the 6th postoperative day. The tracheotomy tube was removed at 44 days of age and the infant was discharged from our hospital at 66 days of age.

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An 18 month-old boy presented with nonpalpable testes, Laparoscopic examination revealed that the spermatic cord entered through the internal inguinal ring on both sides. However, they were both found to end blindly in the inguinal canals on either side, without any associated gonad. Histologically. a rudimentary epididymis with a vas deferens was found on the left side, and a spermatic cord without a vas deferens on the right side. Testicular vanishing syndrome was suspected, but subsequently, a karyotype of XXY was detected. Klinefelter's Syndrome is usually associated with small, firm testes. but association of vanishing testes seems rare. The pathogenesis of vanishing testes in this case is not clear, but the XXY karyotype alone would seem insufficient to explain this rare association. The possibility of other factors after complete masculinization of the external genitalia should be sought.

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A case of congenital dilatation of the bile duct which was diagnosed at 25 weeks of gestation is herein reported. The baby girl was born at 38 weeks of gestation. She was asymptomatic until 6 months of age. at which time an abdominal mass was observed to be growing in the right upper quadrant region and the liver function test also demonstrated a slight deterioration. She underwent an operation consisting of a cyst excision and a Roux-en-Y hepaticojejunostomy. The postoperative course has been uneventful for 29 months. Antenatally diagnosed cases are expected to increase owing to the development and widespread use of ultrasound examinations during pregnancy. Early surgery is desirable because of the risk of either progressive liver dysfunction, hepatic fibrosis or a perforation of the cyst.

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We reported a female patient with tubular duplication of entire colon confirmed by consecutive two operations. Immediately after birth, her parents noticed fecal discharge from her vestibular region as well as her anus. At the age of 2, she was admitted to our clinic for surgical repair. On physical examination, rectal duplication with vestibular and anal opening was suspected. Removal of recto-vestibular fistula was carried out and the rectum was denuded of mucosa. At the age of 13. she was re-admitted to our clinic because of intestinal obstruction. At laparotomy. a duplicated colon, from the cecum to the sigmoid, closely adjacent to antimesenteric border of the colon proper was found. A hemicolectomy together with septum cxcision of the proximal end of the tranverse colon was carried out. Incklcental appendectomy alos revealed a double-barreled appendix. Postoperative barium enema and colonofiberscopy confirmed the entire colon duplication. After the second operation, ulcerative colitis supervened, but this 18 years old girl is now enjoying her schoolife. This is an extremely rare case of tubular duplication of the appendix, entire colon, and rectum.

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