Purpose: A transanal endorectal pull-through (TAEPT) procedure, which does not require any abdominal operation or laparoscopic assistance, has been advocated recently for Hirschsprung's disease. However, sufficient evaluation has not been made of the clinical defecation function. The aim of this study was to evaluate the clinical defecation function of short-segment aganglionosis, where TAEPT was performed following the Soave method, in comparison with conventional open Z-shaped anastomosis. Materials and Methods: The subjects are 17 cases that underwent a TAEPT procedure for short-segment rectal or rectosigmoid aganglionosis from 1998 to 2003 (TAEPT group) and 10 cases that underwent the open Z-shaped anastomosis for short-segment rectal or rectosigmoid aganglionosis from 1990 to 1998 (open group). Each case was evaluated for the use of enemas and suppositories, the number of stools per day, perianal skin rash, occurrence of postoperative enterocolitis requiring hospital stay, and RI-defecogram and functional continence score. Results: Enemas were administered in two cases (11.8%) out of the 17 in the TAEPT group. In one of them, enemas were used only for a postoperative period of six months. In the other case, enemas have been used for a year biginning three years after the operation. Enemas were administered in six cases (60.0%) out of the 10 in the open group. In the TAEPT group, the frequency of stools per day declined gradually. Initially, It it was four to five times per day at the early postoperative stage, and became two to three times per day with the elapse of a postoperative year. In the open group, it was two to three times per day in the first postoperative year, and became less than once per day with the elapse of three to four postoperative years. A perianal skin rash developed in three cases out of the 17 in the TAEPT group, but it disappeared with the elapse of two years. In the open group, no patients had a perianal skin rash. Enterocolitis requiring hospital stay occurred in one TAEPT case and in two open group cases. An RI-defecogram was measured for five in the TAEPT group and five in the open group. The results of the RI-defecogram showed that the maximum capacity of the rectum in the TAEPT group was significantly smaller than that in the open group, and the required time for excretion in the TAEPT group tended to be shorter than that in the open group. Both three TAEPT group members and two open group members, similar to the excretion patterns of the corresponding groups, showed good functional continence scores. Three open group members that presented an excretion delay in the RI-defecogram did not have good functional continence scores. Conclusions: Although the TAEPT group recorded a larger number of stools per day at the early postoperative stage, judging from the use of enemas and suppositories, the subsequent frequency of stools per day, and the result of the RI-defecogram, the defecation function of TAEPT group was considered to be better than that of open group. In order to carry out sufficient postoperative defecation functional evaluation, further long-term observation is required.

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Purpose: We report the analyses of gastroesophageal reflux (GER) with congenital diaphragmatic hernia (CDH). The incidence, clinical features, suspected mechanism of GER and specifically nutritional management were reviewed from our experience and collected literature. Methods: Nineteen neonates who had their CDH repaired at our institution from March 2002 to August 2004 were reviewed. We evaluated the predictive factor for GER related to the following features: prenatal diagnosis, gestational age, birth weight, side of hernia, utilization of nitric oxide (NO)・extracorporeal membrane oxygenation, direct or patch closure and liver herniation. Results: Five of 16 neonates (31%) had GER. Predictive factors for GER are NO inhalation and patch closure of the defect. Other features showed no significant correlation. Conclusion: Tube feeding is not feasible without an enteral feeding tube in these cases with GER, which may lead to various complications such as obstruct jaundice, bacterial translocation or sepsis. We suggest that an enteral feeding tube should be positioned during CDH repair, at least for those cases who require patch closure.

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Purpose: Constipation is one of the major side effects of the potent analgesic opioids. It is known that the opioid activates the central adrenergic neuron system and plays an important role in opioid-induced analgesia. But the role of the adrenergic system in the morphine-induced depression of colonic motility has not been clarified. Thus we examined the effect of β-adrenergic antagonists on the morphine-induced inhibition of colonic motility in rats. Methods: Male Wistar rats (250-350g) were anesthetized by urethane and α-chloralose (ip). The left femoral vein was catheterized for intravenous injections. The colonic motility was monitored through a balloon catheter connected to a transducer, which was inserted into the rat's colon 4cm from the anus. Each β-adrenergic antagonist was administered (iv) 5 min before morphine injection. Results: Morphine (100μg/kg) depressed colonic motility for 33 min. The administration of the nonselective β-adrenoreceptor antagonist propranolol (20μg/kg) and the β_1-adrenoreceptor antagonist atenolol (20μg/kg) significantly reduced the depression of the colonic motility, while β_2-adrenoreceptor antagonist ICI 118,551 hydrochloride (20μg/kg) produced little effect. Conclusions: The results suggested that morphine inhibits colonic motility by the activation of the adrenergic system, especially through the β_1-adrenergic system.

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Purpose: Although TNP-470 is a promising angiogenesis inhibitor, it is known to be very unstable in vitro or in vitro. To overcome the disadvantage of TNP-470, we adopted a drug delivery system (DDS) for the administration of TNP-470. The aims of this study are to determine whether TNP-470 using DDS would be effective and reduce the side effects in the treatment of a murine hemangioendothelioma model. Methods: The 5×10^6 hemangioendothelioma cells were implanted in the peritoneum of 4-week-old male nude mice. On the second day after the implantation, TNP-DDS was administered intraperitoneally. The 60 mice were divided into 4 groups by the concentration of TNP-470, namely Group-I: 70mg/kg body weight; Group-II: 100mg/kg; Group-III: 150mg/kg; Group-IV: 0mg/kg (saline only). We added untreated Group-V (n=10) to determine the natural course of the model. The body weight and tumor size were measured every two days and the hemoglobin concentration, platelet count, and blood pressure were measured every week. The Group I to IV mice were sacrificed on the 21^<st> day after the tumor implantation and blood was drawn to determine the serum concentration of VEGF and TNP-470. Results: The tumor growth was statistically suppressed in Group I, II and III, compared to Group IV. The plate count and hemoglobin concentration were maintained normally in Group I, II and III, though they decreased in Group IV at three weeks. The serum concentration of VEGF showed statistically lower levels in TNP-470-administered groups compared to Group IV. The body weight decreased transiently in TNP-470-administered groups, but recovered more quickly in Group I than Group II and III. TNP-470 concentration was maintained in Group I, II and III at the end of the study. Conclusions: TNP-470, which was administered by using DDS, suppressed the tumor growth and reduced the adverse effects in the murine hemangioendothelioma model.

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Purpose: Recently, increasing medical expenses and the declining birthrate have become important problems in our country. A retrospective study was conducted to determine the factors behind the increase of medical expenses in pediatric inguinal herniorrhaphy. Methods: A comparison examination of the medical expenses in pediatric hemi lateral inguinal herniorrhaphy was carried out among 3 hospitals in different areas in Japan during the past 2 years. All patients were less than 15 years of age. We reviewed the records of 292 patients, which were composed of the 126 patients admitted in the hospital in Tokyo, 119 patients in Kyoto, and 47 patients in Hokkaido. The costs, subdivided according to medical examination and treatment, were analyzed among the hospitals. Results: The hospital in Tokyo revealed the highest medical expenses, with a significant difference of 27,001 points, caused by the longest treatment periods, the most preoperative examinations and the largest amounts postoperative medication. These costs were 24,051 points in Kyoto, and 24,924 points in Hokkaido. The factors behind increasing medical expenses depended on each clinical pathway. Conclusion: On the basis of the results, unnecessary preoperative examination and postoperative medication should be eliminated to reduce the medical expenses. The methods of ultrasonography and laparoscopic examination are cost effective in the diagnosis of the contra lateral inguinal hernia. In the medical cost system of diagnosis procedure combination (DPC), we should demand the appropriate additional cost that corresponds to management for the children to provide safe surgery and nursing.

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Purpose: To assess the current clinical features, modality of treatment and outcome of pediatric lymphangioma. Patients and Methods: Inquiries about pediatric lymphangioma treated in the last 5 years were sent to pediatric surgical centers that are members of the Kyushu Pediatric Surgical Congress in the Kyushu and Okinawa Area. The replies were analyzed in terms of site, type, modality of treatment and outcome, among others. Results: Information from 217 cases originating from 23 institutions was collected. The locations of the main lesion were the neck in 87, torso in 50, axilla in 34, extremities in 27, face in 13, mediastinum in 10, mesentery in 8, and retroperitoneum in 7 cases. Sclerotherapy alone was performed in 102 cases and sclerotherapy with additional excision was used in 40 cases. Excision of the lesion alone was done in 35 cases. In 39 cases, the lesions were observed without any specific treatment. The sclerotherapeutic agent was OK-432 in 94% of the cases. Of all the 217 cases, 88 (40.6%) cases were completely cured without any residual tumor or recurrence. In 29.5% of all cases, the tumor mass greatly decreased. However, in 24% of the patients, treatment was not effective and the mass was stable or increased. There was no significant difference in the therapeutic effect between the modalities of the treatment. Conclusion: Sclerotherapy using the OK-432 is currently the mainstay of pediatric lymphangioma treatment. Though the results of treatment are generally satisfactory, there are still some cases where obtaining significant reduction of the tumor is a difficult task.

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Hepatoblastoma is the most common liver tumor among children. It usually presents with abdominal swelling and abdominal mass. We report an unusual neonate who presented with an acute abdominal crisis due to rupture of the liver tumor. A male neonate after vaginal delivery was admitted to our NICU because of abdominal distention. His gestational age was 37 weeks and 5 days and birth weight was 2,250g. Physical examination at the time of admission confirmed the presence of an abdominal mass extending to the level of the pubic bone. His blood pressure was 59/30 mmHg and his pulse was 150/min, and the hemoglobin level was 9.9g/dl. Ultrasonography of the abdomen detected an abdominal tumor with intra-tumor hemorrhage and a small amount of ascites. The serum alfa-fetoprotein level was markedly elevated to 1,147,000ng/ml. Computed tomography (CT) scan showed a huge tumor arising from the left lobe of liver and expanding extra-hepatically. Early left lateral segmentectomy was planned. However 3 days before the scheduled segmentectomy, hypovolemic shock developed. Emergent laparotomy was applied to left lateral segmentectomy. Four hundred milliliters of blood was aspirated from abdominal cavity. The rupture site of the hepatoblastoma was found to be on the posterior side of the tumor. Histological diagnosis was hepatoblastoma of the embryonal type. The postoperative clinical course was favorable. Multidrug chemotherapy using cis-platin was initiated on the 28^<th> postoperative day. After six cycles of treatment, the patient is now 1 year and 7 months old and is healthy and free from disease.

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A case of a 0-day-old girl. Bile was vomited immediately after birth. Small intestinal atresia was suspected after X-ray examination. It was confirmed by ultrasonography after hospitalization. The girl was diagnosed to have small intestinal atresia by intrauterine intussusception. Since we understood the situation of intestinal atresia before the operation, the operation was completed by upper abdomen midline incision. Ultrasonography is useful for intestinal atresia. There is no report of diagnosed intestinal atresia by intrauterine intussusception in reference to a newborn.

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We reported a case of Meckel's diverticulum found with a very rare complication. A 15-day-old baby had been treated for umbilical bleeding by an obstetrician. When the obstetrician tried to ligate the umbilical granulation tissue, the small intestine was delivered through the umbilicus. The infant was admitted to Fukuoka Children's Hospital, and underwent urgent laparotomy to reduce the strangulated intestine. The Meckel's diverticulm was found attached to the umbilical granulation tissue, and cause the incarceration of the intestine. The incarcerated ileum was necrotic, and partial resection of the ileum was performed. The postoperative clinical course was uneventful, and the patient is well now, one and a half year after surgery. It is necessary to carefully treat an umbilical lesion, such as bleeding, inflammation, granulation, or polyp, in cases of the omphalomesenteric duct or the urachal duct remnant.

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Progressive familial intrahepatic cholestasis (PFIC) is an inherited disorder of childhood in which cholestasis of hepatocellular origin often presents in the neonatal period. Our patient is a nine-year-old male child with PFIC who was admitted to our hospital to be evaluated for living-related liver transplantation. Our examination of the patient included a liver biopsy that revealed chronic cholestasis with a decreased γ-GTP level, confirming the diagnosis of PFIC (also known as Byler disease). Because we were unable to find a suitable donor for living-related liver transplantation, we opted for treatment with a high-dose choleretic agent, but liver fibrosis continued to worsen. In order to prevent liver cirrhosis, we performed a partial external biliary diversion (PEBD), which alleviated pruritus and improved liver function tests (eg, total bilirubin and total bile acid). One year after surgery, we performed an open liver biopsy that showed the fibrosis had not worsened. On the basis of our findings, we recommend that PEBD be performed in patients with PFIC refractory to medical management, before liver fibrosis progresses to an irreversible phase of cirrhosis.

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Thoracoscopic debridement for acute empyema has already become a standard operation in adulthood, as this procedure is minimally invasive. However, case reports in childhood are few, because of intercostal narrowness and the difficulty of differential lung ventilation. We report a case of pediatric acute empyema successfully treated with a video-assisted thoracoscopic operation. The patient is a nine-month-old boy. As his empyema did not resolve with thoracic drainage in another institution, he was transferred to our hospital. On the next day, thoracoscopic debridement in the fibrinopurulent loculated space was performed under differential lung ventilation. In this case, we could perform this procedure with instruments of 5.5mm diameter, under differential lung ventilation with a fogartycatheter. He had a favorable outcome. In a computed tomography scan on the 39th postoperative day, lung expansion was good and no intrathoracic fibrinopurulent tissue was found. At the moment this case is the youngest of all cases successfully treated with thoracoscopy in Japan. This result suggested that thoracoscopic debridement is a promising procedure in the management of pediatric acute empyema, especially in a patient who shows poor response to thoracic drainage.

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Intrathoracic kidney is an unusual developmental anomaly and its association with Bochdalek hernia is exceedingly rare. We present a case of a 26-month-old boy with diaphragmatic hernia and intrathoracic kidney. His chest radiograph was normal at the age of 1 month, but chest computerized tomography (CT) scan examining the cause of repeated bronchitis at the age of 8 months revealed the herniation of colon, liver, and right kidney in the right thoracic cavity. He had no sign of respiratory distress and had been followed under the diagnosis of right Bochdalek hernia to the age of 26 months, when a primary closure of a diaphragmatic defect was performed. The postoperative course was uneventful. Chest CT scan was useful in the evaluation of intrathoracic kidney and recommended as a diagnostic modality in a case with diaphragmatic hernia if the respiratory condition is stable.

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As the diagnostic methods for a fungus infection are various, the serum (1→3)-β-D-glucan level has been used most frequently among them. But there are some reports of various factors which raise the serum (1→3)-β-D-glucan level, such as human serum albumin. We experienced a case of high serum (1→3)-β-D-glucan level which we assumed to be induced by administration of human serum albumin, and we had to think a little about when the medication of the anti-fungus was stopped. An 8-month-old boy was admitted to our hospital because he had recognized diarrhea and vomiting frequently after a Ladd operation for malrotation. We administered anti-fungus to him because of fungal sepsis while total parenteral nutrition was treated. After removal of the catheter, the high serum (1→3)-β-D-glucan level continued though clinical symptoms were improved. It was suggested that this value had been raised more by administration of the human serum albumin. We stopped the anti-fungus medicine in consideration of clinical symptoms with high serum (1→3)-β-D-glucan level, and the revival of the infection sign did not reappear after that.

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We encountered a 2-year-old boy with a giant cell fibroblastoma (GCF). A small mass, sized 5mm in diameter was noticed at the right chest wall by the parents at 1 year of age. He had been observed for 1 year at a nearby hospital with questionable diagnosis of lymphangioma. The mass gradually enlarged and the patient was referred to us. On admission, the mass was 5.5cm in diameter, fixed tightly to the skin. It was covered by normal skin and no fluctuation or tenderness was seen. Ultrasonography showed a mixed pattern; however, CAT scan showed homogenous, low-density mass with a weak enhanced effect. No calcification was seen. T1 weighted MRI demonstrated homogenous, low-intensity signal and the T2 weighted image showed a variable high-intensity signal. Heterogenous enhancement was observed. These imaging studies suggested a neurogenic tumor with a mucinous component or myxoid liposarcoma. The en-bloc resection was perfomed with a part of the skin and fascia. Pathological examination revealed spindle-shaped cells, multinucleated giant cells, a sinusoid-like space with a mucinous component, and a positive resulf for CD34, so GCF was concluded. GCF is a rare mesenchymal neoplasm, mostly developed in boys in the first decade of life. It often presents as a painless, slow-growing subcutaneous mass, especially in the trunk and the upper limbs. The local recurrence rate is up to 50%; however no metastasis was previously reported.

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Mucosal prolapse syndrome of the rectum causes rectal bleeding in patients and it is relatively rare in children. MPS is characterized by mucosal lesions of the distal rectum. It is caused by a long period of stimulation at the rectal wall during squeezing. In the treatment of MPS, fecal control is necessary to avoid recurrence. We have treated two children with MPS. The first case was a twelve-year-old male. He complained of rectal bleeding. The double contrast barium enema and colonoscopy found circumferential polypoid lesions at the lower rectum. Defecography showed hypo-motility of the muscles of the levator ani. Endoscopic ultrasonography revealed the thickness of the first to third layer of the rectum and no abnormality at the fourth layer. Therefore, the diagnosis of MPS was made. He underwent trans-anal mucosectomy and had no relapse after the surgery. The other case was a seven-year-old male. He complained of severe constipation with fecal soiling. At the posterior wand of the rectum, flat mucosal thickness was found and the biopsy of the lesion provided the diagnosis of MPS. He had no symptom of rectal bleeding and we performed a conservative treatment for the incontinence of stool without surgery.

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A 10-month-old girl was admitted to Omihachiman City Hospital with complaints of ill humor and appetite loss. Abdominal ultrasonography showed a dilated stomach. Intrathoracic intestinal gas shadows were not seen in the plain X-ray film. Thereafter, computed tomography and barium enema study showed a herniation of the intestine into the left thoracic cavity. She was diagnosed with a late-presenting left congenital diaphragmatic hernia. Gastric dilatation was thought to be caused by the herniation of transverse colon, which compressed the duodenum and blocked gastric emptying. Elective surgical repair was performed, and the postoperative course was uneventful. She was discharged on the 7th day after the operation.

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We report a case of polyorchidism with inguinal hernia. A 6-year-old boy was admitted to our hospital because of left side inguinal hernia. A testis 9×8mm in size was recognized in the inguinal canal during the operation. There was a normal-sized testis 15mm in maximum length in the left scrotum. The spermatic duct from the internal inguinal canal connected these testes with the epididymis. The testis in the inguinal canal was resected because of the short vessel. Hypoplastic testicular tissue was revealed by histological examination. This case was classified as type 5 according to Toyoda's classification. Twenty-three cases were reviewed in the literature from Japan. According to this literature, 5 cases with surplus testes in the inguinal canal were resected within 6 cases, 3 surplus testes in the scrotum were reserved within 13 cases. It is not necessary to reserve a surplus testis affirmatively, because polyorchidism is regarded as the cause of sterility and a high carcinogenicity rate.

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We herein report a case of a bronchogenic cyst arising in a 7-month-old girl. A tiny hole in the anterior chest wall was noted shortly after birth. At the age of seven months the region was infected. After relieving the inflammation, a surgical resection was performed. The histological diagnosis was a bronchogenic cyst based on the existence of a ciliated stratified columnar epithelium and smooth muscles. The origin of the bronchogenic cyst may be explained by the migration of lung buds during the gestational period. However, a bronchogenic cyst arising in the subcutaneous region is rare. Only 16 cases of those have so far been reported in Japan to our knowledge. In particular, only 7 pediatric cases arising in the subcutaneous region of the anterior chest wall have been previously reported. When cystic lesions are detected in this area during the neo-infantile period, bronchogenic cyst should thus be included in the differential diagnosis.

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In 2 children (Patient 1: a 22-month-old girl with hypoganglionosis involving the entire intestinal tract, and Patient 2: a 15-month-old girl with megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS)), we performed extended resection of the small intestine involving the ileocecal region (the residual jejunum: 50 cm from Treitz's arch), right semicolectomy, and functional enterostomy to reduce the duration of retention of the intestinal contents and promote water absorption in the large intestine. In these children, total parenteral nutrition (TPN) was switched to oral ingestion, and the results were satisfactory.

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