Purpose : This study was to evaluate the adequate starting time of pediatric surgical training in infantile hypertrophic pyloric stenosis (IHPS) at Showa University Hospital. Materials and Methods : The records of 94 patients who underwent pyloromyotomy from 1989 to 2003 were reviewed retrospectively. Results : Sixty-one of the operations were performed by surgeons with less than 5 years of post-graduate training, while 23 operations were performed by surgeons with 2 years of post-graduate training. Twentyfive were neonatal HPS. of which 15 were carried out by surgeons with less than 5 years of post-graduate training. including 9 by 3rd and 1th year surgeons. Consultant pediatric surgeons supervised 68 of the procedures. The mean operating time gradually decreased as post-graduate training progressed, from was a high of 60 minutes for surgeons with less than 2 years of post-graduate training to a low of 38 minutes for those with more than 8 years of training. No surgical complications were observed. Conclusions : The conventional pyloromyotomy for IHPS can be safely performed by a surgeon with less than 5 years of post-graduate trainees in the presence of appropriate support from staff pediatric surgeons.

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Purpose : The aim of our study was to assess, based on fetal MRI, the degree of lung hypoplasia with congenital diaphragmatic hernia, and to plan a therapy strategy based on the results. Methods : Between December 1993 and March 2004 we examined the fetal MRIs of 24 cases diagnosed by prenatal US. The lung volume was evaluated based on the detected number of slices obtained in coronal images from the level of the vertebrae toward the abdomen. We calculated the HL (hypoplastic lung) index by dividing the number of slices by the anterior to posterior length of the thorax. We assessed the utility of the HL index by studying its correlation with the postnatal course. Results : The overall survival rate was 63%. The HL index was statistically lower in the patients who needed ECMO (p<0.001). Moreover, the HL index was statistically lower in nonsurvivors (p<0.01). The patients were categorized into 3 groups : Group 1. HL index<0.06 ; Group 2, 0.06≦HL index<0.13 ; Group 3. 0.13≦HL index. Group 1 : all 5 cases needed ECMO : the survival rate was 20%. Group 2 : there were 7 cases : one needed ECMO and the other 6 were stabilized relatively early : the survival rate was 57%. Group 3 : there were 7 cases : all were saved without complications. Conclusion : The HL index is useful in assessing the degree of lung hypoplasia. ECMO was needed and we should consider fetal therapy for Group 1 in the future. It is possible to save the patients in Group 2 by applying a strict prenatal therapy strategy. We must also consider reducing the stabilization for the patients in Group 3 who showed ill-sided lung.

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Purpose and Methods : The adhesive-type, central venous (CV) catheter anchoring device is useful for improving post-treatment QOL, because it secures the CV catheter without sutures and does not leave an ugly scar. We evaluated the usefulness of the device in 20 patients between July 2002 and December 2003. Results : The anchoring device could be used in patients aged from 27 days to 28 years. The duration of CV catheter placement ranged from 7 to 122 days (mean, 29.5 days). The catheter was removed for the following reasons : Capable of oral intake : 14 patients : fever : 4 patients : bilateral shoulder pain : 1 patient : cutaneous lesion at the adhesion site : 1 patient. Although all catheter tip cultures were negative in patients with fever, the culture with cutaneous lesion was positive. In the case of cutaneous lesion, impetigo at the adhesion site was treated conservatively and healed without scarring. As regards anchoring technique, disinfection, and device replacement, caution is required to avoid accidental removal, but the technique itself is easy, and there were no problems with respect to strength and durability in use for 2 weeks. Conclusions : The adhesive-type anchoring device is more expensive than sutures, but is useful in terms of strength and durability with the major advantage that it leaves no ugly scar. In children with a lengthy life after treatment, the pediatric surgeon should choose a treatment modality and device that avoids any reduction in QOL, such as limitations on the clothes that can be worn because of a noticeable scar.

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Purpose : The aim of this study was to assess the usefulness of fetal ultrasonography in retrospective differential diagnosis of cases in which biliary dilatation is revealed during the prenatal period, and to assess the usefulness of hepatobiliary scintigraphy in cases of biliary dilatation revealed by ultrasonography during the prenatal or neonatal period. Methods : We reviewed 5 cases of biliary dilatation, as revealed by prenatal ultrasonography, and 3 cases which had jaundice and white-colored stool. Results : In all cases, fetal ultrasonography revealed stagnant, immobile cysts at the porta hepatis. On the other hand, hepatobiliary scintigraphy revealed 3 patterns : 1) the radionuclide reached the duodenum, 2) the radionuclide did not reach the duodenum, but reached the dilated biliary duct, or 3) the radionuclide did not reach the dilated biliary duct. 3 cases had pattern 1, 2 cases had pattern 2, 1 case had pattern 3 with CBD, and 2 cases had pattern 3 with biliary atresia (I-cyst). Conclusion : Intraoperative cholangiography is necessary for differential diagnosis of cases with biliary dilatation revealed during the prenatal or neonatal period.

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Purpose : Intussusception of the school age is comparatively rare. The feature of school age intussusception was examined. Methods : We studied eight children in 143 cases with intussusception which we have treated over an 11-year period (1993-2003). Results : The patients consisted of 5 boys and three girls, a ratio of 1.7 : 1. Their ages ranged from 8 to 15 years. Main symptoms were abdominal pain (100%), vomiting (25%) and bloody stool (25%). 4 patients (50%) were cured successfully without surgical intervention. The other patients underwent laparotomy and were cured by Hutchinson's maneuver. The ype of intussusception were 5 ileo-colic type, two ileo-ileo-colic type and one ileo-ileo type. In two cases. Meckel's diverticulum was found. One patient had pathologic leading points such as Meckel's diverticulum. the other had no correlation. Seven cases were idiopathic intussusception. The recurrence was two (25%) of the non-surgical cases, but those cases were successfully treated by enema reduction. Conclusions : In school age intussusception, bloody stool and vomiting are comparatively in frequent symptoms. It presents abdominal pain in many cases, and requires the differential diagnosis with appendicitis. Moreover, an idiopathic intussusception is not rare in school age.

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Purpose : Recently, prenatal diagnosis of GCMP has become more common, and early surgery is necessary for neonates with GCMP. However, there is no consensus regarding the surgical treatment for GCMP. Radical surgery in the early neonatal period is difficult to perform because of severe adhesion, and so many patients usually undergo cyst drainage alone or enterostomy. Here, we report four patients with GCMP, who underwent primary radical surgery early after birth, and then we discussed the surgical strategy for GCMP based on our cases. Materials and Methods : From 1984 to 2004, 16 neonates with GCMP underwent surgery at Shizuoka Children's Hospital. GCMP was caused by perforation due to intestinal volvulus in all cases. One patient, who had hemorrhagic shock due to perinatal hepatic injury before surgery, was excluded from this review. The patients were divided into two groups according to the type of surgery : group I (underwent drainage alone or enterostomy first, and elective surgery later, from 1984 to 2001 : n=11) and group II (underwent primary anastomosis of intestines with minimal dissection of adhesion and the cyst wall, from 2002 to 2004 : n=4). Operating time, amount of blood loss, postoperative complications. CRP levels, and length of hospital stay (LOS) were compared between the two groups. Results : Comparison of operating time, and amount of blood loss showed no significance between the two groups. Postoperatively, group I had sepsis (n=4), intestinal obstruction (n=2), wound infection (n=2), intraabdominal bleeding (n=1), and anastomotic leak (n=1), Only one patient died because of sepsis. Group II had mild wound infection (n=1), and intestinal perforation (n=1). The LOS in the group II was significantly shorter than that in group I (130±48 days vs, 34±14 days, p<0.005). The levels of CRP in group II decreased earlier after operation than in group I. Conclusions : In our recent cases, primary anastomosis of intestines with minimal dissection of adhesion and the cyst wall was performed on 4 patients. All of them recovered earlier and were hospitalized for a short period. In patients having no bleeding tendency, we should consider primary radical surgery in the early neonatal period.

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Infantile myofibromatosis is a rare mesenchymal tumor, which originates from fibroblast. We report two cases with infantile myofibroma, diagnosed pre and postnatally respectively. The tumors developed pediculately on the skin in both cases. Pathological finding in one case showed a lower grade malignancy, including component of myofibrosarcoma. In general this tumor is usually present at birth and early infancy and has a good prognosis. However, in the case with multivisceral involvement or a low grade malignancy, the prognosis is often poor. Therefore, even if complete surgical resection is performed in this tumor, a careful follow-up is needed.

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Alimentary tract duplications are rare congenital malformations. They can occur throughout the alimentary tract, but only 5% in the rectum. In Japan, only 11 cases including this one have been reported as anal canal duplications. Ten of them are female, and 1 is male. We present a 12-year-old female with anal canal duplication. On the first examination, she was noted to have a small pit just behind the normal anus. Fistulography revealed the tubular structure about 2 cm in length along the normal anal canal. The fistula was excised, and pathologically proved to be anal canal duplication. Histological examination showed transitional epithelium and squamous lining with a smooth muscle component around the fistula, but no ganglion cells found out. The etiology of the anal canal duplication is thought to be a duplication of the dorsal cloaca. Owing to formal semblance, we guess this lesion might be misdiagnosed and be treated as anal fistula.

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A case of duodenal duplication presenting with acute abdomen in a 3-month-old girl is reported. In this case, a large abdominal cyst was detected by prenatal ultrasonography at 26 weeks of gestation. After the delivery, she was carefully followed by echo. Nevertheless at 3 months old, she was emergently admitted to our hospital complaining of sudden onset of abdominal pain and revealed to be in pre-shock status. So, emergency laparotomy was carried out. At operation, the duplication cyst was found to arise from the medial side of the second portion of the duodenum. The cyst did not communicate with the pancreatic duct, duodenum lumen or bile duct. After the cyst was diagnosed as a duodenal duplication, partial excision of the cyst and cautery of the mucosa of the common wall was performed. Her post-operative course was uneventful. In summary, duplication cyst can cause acute abdomen. Laparoscopic examination or operation should have been chosen before crisis when duodenal duplication was suspected.

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We report a rare case of antenatally detected hemangiopericytoma of spleen. The abdominal mass was detected by fetal echogram at 28 weeks' gestation in this patient. The patient was delivered by Caesarean section at 29 weeks' gestation because of fetal distress. The patient was intubated and he received mechanical ventilation just after birth because he had a respiratory disorder due to the huge abdominal mass. Blood examinations revealed anemia and thrombocytopenia. Ultrasonography and CT scan revealed a giant mass in the left upper abdomen. Emergent tumor resection was performed 8 hours after birth. The tumor arising from the spleen was completely resected. There was no surgical complication. Histological diagnosis was congenital hemangiopericytoma. The patient was extubated 38 days after operation, and discharged 96 days after operation. No recurrence was found for 7 months after the operation.

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A case of long tubular colonic duplication with imperforate anus is presented. A baby girl was found to have ano-vestibular fistula and ano-cutaneous fistula with hemivertebrae and rib anomaly. She had no genitourinary anomalies. Ba-enema showed a long tubular colonic duplication beginning from hepatic flexure opened to the anus. Posterior sagittal anorectoplasty (PSARP) was performed. The septum of duplicated rectum was divided using GIA and the ano-vestibular fistula was resected. And anorectoplasty was done using ano-cutaneous fistula. No farther procedure was performed to the duplicated colon. One year after the operation, she remains without any complications. Tubular colonic duplication is a rare congenital anomaly in early childhood. Many operative techniques have been reported such as complete resection of duplicated colon with or without neighboring colon, complete or partial division of the septum. However, we believe that a simple technique such as PSARP is enough for the long colonic duplication without obstruction.

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We describe our experience with 6 children with testicular yolk sac tumor (YST) treated in the past 12 years. The age of patients ranged from 4 months to 27 months with an average of 15 months. Hydrocele testis occurred in 2 cases. In 4 cases the tumor was located in the right testis, and in the other 2 cases in the left. All patients underwent high orchiectomy as the initial treatment. Four patients presented with Stage I disease and the remainder presented with Stage III 0 or Stage IIIB disease. All patients were free of evidence of disease after a mean follow-up period of 4.8 years. Three patients received platinum-based combination chemotherapy. In conclusion, high orchiectomy alone seems adequate for Stage I testicular YST. Post-operative chemoprevention is likely to be required, if tumor cell invasion into vessels is histologically revealed in the resected specimen. Serial monitoring of the AFP degradation curve plays an important role in the management of post-operative testicular YST. Patients with metastatic disease or recurrent disease can be treated successfully with platinumbased combination chemotherapy.

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Immature teratoma of the stomach is extremely rare. We are reporting a case with a giant immature teratoma of the stomach which was detected with prenatal ultrasonography. An accurate pre-operative diagnosis was obtained prior to surgery based on imaging studies and laboratory data, and the tumor was completely excised during the neonatal period. No chemotherapy was required post-operatively, and the patient has been event free over 2 years after resection. Extra-gonadal immature teratoma of infant has an excellent prognosis compared to adult case, and no adjuvant therapy is necessary if complete resection of the tumor is achieved. Our patient developed anastomotic stenosis at the gastric fundus and microgastria. It was hard to avoid, because the stomach had already been thinned with compression of the tumor. Considering the excellent prognosis of this tumor, we should always consider preservation of the stomach as far as possible to maintain satisfactory post-operative gastric function.

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Esophagogastric dissociation was performed in two patients with gastroesophageal reflux disease (GERD) who were severely neurologically impaired. One patient underwent the procedure to control recurrent GERD after Nissen fundoplication and it was performed as the primary operation in the other. Although minor leakage and stricture at esophagojejunal anastomosis was observed in one patient, a postoperative improvement in respiration, quality of life and weight gain was apparent in both patients. Based on the experience of the procedure in these patients, it is concluded that esophagogastric dissociation, which completely blocks gastroesophageal reflux and resolves symptoms of GERD, can be a surgical option for either primary or recurrent GERD in patients with severe neurological impairment.

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A 1 year 8 months girl was presented to our hospital for vomiting, abdominal distension, and agonizing pain. She had been previously treated as rotavirusassociated diarrhea 3 days before the presentation. Initial evaluation from the abdominal X-ray showed free air and revase a shaped nasogastric tube. She was suspected as acute gastric volvulus with perforation and emergency laparotomy was performed. The stomach showed mesenteroaxial volvulus and was detorsed 180 degrees in a counter-clockwise fashion. Gastric perforation of the major flexure was also detected. Resection and suture of a perforative area, and gastropexy were performed. She recovered without incident and was discharged 14 postoperative days later. Acute gastric volvulus in childhood is rare, but an emergency condition. Although the use of nasogastric tube decompression and keeping a prone position was initial treatment of gastric volvulus, earlier surgical correction should be required for acute gastric volvulus in childhood because it leads to fatal complications such as perforation and shock condition.

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A 15-year-old child was diagnosed as alveolar echinococcosis of the liver, and posterior & lateral segment resection was carried out. Before the operation, multiple nodular lesions were found in the bilateral lung by chest X-P and CT, and was diagnosed as pulmonary lesions. After the operation, we treated the patient with albendazole. No adverse reaction was found and the ELISA level decreased significantly. Multiple lesions in the lung reduced in size significantly. Albendazole was a safer and more effective drug. Though reports concerning echinococcosis in children are quite rare in Japan, we should be aware that the area contaminated by echinococcus is enlarged from the Hokkaido Islands to mainland Japan, Honshu, nowadays. Thus the possibility of encountering the disease is increased.

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