Purpose: Little is known about maternal factors behind developing NEC and histopathologic examination of placentas in infants who develop necrotizing enterocolitis has not been reported. So we investigated maternal factors and histopathological placental findings in infants with necrotizing enterocolitis. Among 111 extremely low birth weight infants born at our hospital from 2000 to 2008, six infants developed necrotizing enterocolitis. We compared the maternal factors and histological placental findings to the control group (n=105). Results: In the necrotizing enterocolitis group, the presence of placental chorioamnionitis was significantly higher than that in the control group (83.4% vs. 38.1% p=0.039). They were significantly more likely to have received antibiotics on the day before delivery (100% vs. 53.3% p=0.03) and the serum CRP level of the necrotizing enterocolitis group was significantly higher than that of the control group (3.24±2.32 vs. 1.26±1.67mg/dl p=0.0067). Conclusion: Necrotizing enterocolitis in the extremely low birth weight infant could be a neonatal disease associated with placental inflammation. So careful observation is necessary for extremely low birth weight infants with severe placental inflammation.

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Purpose: Recently antireflux surgery has been frequently performed in neurologically impaired (NI) children with gastroesophageal reflux disease (GERD) and they often develop postoperative complications. In this study we describe our experience of 59 consecutive fundoplication in NI. Methods: From 1991 to 2007, we performed Nissen fundoplication in 59 NI children (33 boys and 26 girls. mean age 10.5 years old) with GERD. Results: An open procedure was performed in 26 cases (44.1%) and a laparoscopic procedure was performed in 33 cases (55.9%). Postoperative recurrence of reflux was revealed in 11 cases (18.6%), and 2 patients with severe symptoms of it required reoperation. Bowel obstruction occurred in 7 patients subjected to open and 2 to laparoscopic procedure (p=0.0351). We performed gastrostomy in 58 patients. Complication occurred in 35 cases and 3 patients with severe symptoms of them required revision. Laryngotracheal separation (LTS) was performed pre/post operation in 10 cases with severe respiratory aspiration. Postoperative death occurred in 8 cases but none in children with LTS. Conclusions: When fundoplication is performed in NI children, prevention against recurrence of reflux and gastrostomy with easy management are important and LTS is effective in cases with severe respiratory aspiration.

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Purpose: Vesicoureteral reflux (VUR) is a common urinary tract anomaly which occurs in approximately 0.4-1.0% of infants and is associated with recurrent UTIs that may result in renal scarring. The endoscopic treatments become an established alternative to long term antibiotic prophylaxis and open surgery. The aim of this study is to examine the use of an endoscopic injection technique, specifically the Hydrodistension implant technique (HIT procedure) using Nonanimal stabilized Hyaluronic acid/Dextranomer Gel (Deflux^[○!R]). Subject and Methods: A total 16 infants and children (25 ureters) with a median age of 2 years and 2 months (range 6 months to 5 years and 8 months) underwent the HIT procedure. The reflux was grade II in 4 ureters, Grade III in 9 ureters and grade IV in 12 ureters. DMSA scan demonstrated renal scarring in 13 (52%). Results: Vesicoureteral reflux completely resolved in 21 (84.0%) ureters after a single injection of Deflux. Four ureters (16.0%) required a 2nd injection and resolved. However, two patients were found to have recurrent reflux one year after injection. The final resolution rate at one year after injection was 88.8%. All patients underwent endoscopic procedure on an out-patient basis. There were no complications. Conclusion: Endoscopic treatment using Deflux is a safe and effective method for eradicating VUR, but long term follow up is required to discover late relapsing cases.

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Purpose: This study aimed to analyze the outcomes and to evaluate the medical and ethical relevance of parental treatment decisions for fetuses following a prenatal diagnosis of abdominal wall defects. Materials and Methods: The medical records of 53 fetuses that had undergone a prenatal evaluation of abdominal wall defects from 1994 to 2008 were studied. The parental treatment decision was then divided into positive strategy and negative strategy groups. The gestational age at the time of diagnosis, the severity of the disease, major associated anomalies and the final outcomes were analyzed retrospectively with respect to each treatment decision. Results: The parental treatment decisions were positive in 29 cases and negative in 23 cases. One case died in utero before a decision was made. Twenty-seven neonates out of 29 positive treatment cases were born alive and underwent surgery. Four infants with a giant omphalocele died after the operation. Eighteen fetuses out of 23 negative treatment cases were terminated and one fetus died in utero. Four neonates were live-born and resulted in death following either limited or palliative therapy. Nineteen cases selected as negative treatment strategy were considered nonviable, retrospectively. Four fetuses with a giant omphalocele associated with total live herniation, were able to survive, however, with a poor quality of life. Conclusions: Although a small number of the cases with a negative treatment may have the possibility of surviving, the parental treatment decisions for the fetuses of prenatally diagnosed abdominal wall defects were generally considered to be medically and ethically relevant.

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We present a case of paraesophageal hernia in a 5-year-old boy with chronic vomiting since infancy. The patient vomited with excessive straining after mealtime. The initial examination in the emergency room noted an aberrant density on the chest X-ray. The patient was admitted with the suspected diagnosis of paraesophageal hernia to our hospital. An upper GI series examination confirmed the diagnosis of paraesophageal hernia. We performed laparoscopic esophageal hiatus repair, and fundoplication by the Dor-Nissen method. Recovery was satisfactory, and the patient was discharged on the 9th day after his operation. The patient has since been asymptomatic. At this time, laparoscopic surgery is prevalent and successfully applied to a multitude of disorders. This operative method is less invasive and safe for this disorder in a child.

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Mesenchymal hamartomas of the chest wall are rare benign tumors. It has been known that the tumor temporarily grows after birth and the growth subsequently stops. We experienced two neonatal cases of mesenchymal hamartomas of the chest wall. Both cases were conservatively treated because radical resection would cause a large defect of the chest wall. The tumor enlarged until 6 months after birth, but the size has been decreasing one year after birth in both cases. In one case, the tumor was exposed from the incision site for biopsy due to rapid growth that required partial resection including MCT (microwave coagulation therapy) several times. At 6 months after birth, the tumor growth stopped and we were able to close the skin at 11 months after birth. In the other case, although the tumor temporarily enlarged, it did not cause any symptoms. The tumor size has been decreasing for three years. The clinical course of these cases suggests that asymptomatic mesenchymal hamartomas of the chest wall in infants should be conservatively treated, because they are benign and have the feature that the tumor spontaneously starts regressing within a year after birth.

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An 8-year-old girl was admitted because of abdominal pain. A mass was palpated in the left upper abdomen and an x-ray and computed tomography of the abdomen showed a stuffed and dilated stomach. Bowel obstruction of the duodenum was initially suspected; however, a foreign body in the stomach was diagnosed preoperatively. The upper gastrointestinal endoscopy revealed a large trichobezoar in the stomach which could not be removed endoscopically. Laparotomy was performed using a wound retractor inserted through the skin not only into the peritoneal cavity but into the gastric wall in an attempt to prevent spillage of the gastric contents. The trichobezoar had to be dissected into pieces to remove it because it was stuck in the stomach. Postoperative course was uneventful and the patient was discharged from the hospital 5 days after the operation without any complications. A wound retractor hooked into the stomach wall is useful in terms of preventing pieces of trichobezoar from spilling.

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Gastroschisis is a rare congenital anomaly that results in an abdominal wall defect. Closing gastroschisis is a pathological condition in which the abdominal wall has closed, and the intestinal tubes become strangulated at the umbilicus. All known cases of closing gastroschisis have been reported from the United States and Europe. As of 2008, only 50 cases of closing gastroschisis have been reported in the English literature. Here, we report the first case from Asia. A newborn boy (2,664g) was delivered at the gestational age of 38 weeks and 2 days. A stick-like solid mass was found on his abdomen at birth, and we diagnosed the condition as congenital atresia of the jejunum and colon with short bowel syndrome. The patient was 17 months of age at the time of this report and continues to require parenteral nutritional support. Surgery involving an intestinal lengthening procedure is considered to be required in the future.

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A 4.5-year-old boy underwent liver transplantation. The spleen was saved. Immunosuppression was started with tacrolimus and methylprednisolone. Disorders in a liver function test appeared on post-operative day (POD) 7. We made a diagnosis of acute cellular rejection. Nevertheless the result of a liver function test (LFT) showed no reaction in spite of repeated steroid therapy. Jaundice was prolonged and massive ascites remained. Pathological findings of liver needle biopsy on POD 20 and 26 persisted in severe graft damage, so-called early chronic rejection. From the view point of responsiveness to therapy, coagulopathy and protein-losing ascites, 3mg/body of OKT3 was administrated from POD 24 and the dose of OKT3 was escalated to 5mg/body from POD 29. Temporal rebound in the liver function test appeared after the withdrawal of muromonab-CD3; thereafter disorders of LFT were normalized. Ascites and jaundice also disappeared, and clinical findings clearly improved. Pathological findings of liver needle biopsy on POD 47 and 61 certified drastic recovery of severe graft damages. We here presented a rare case of early chronic rejection after pediatric liver transplantation, successfully treated by OKT3.

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A 6-month-old boy, a 9-month-old girl, and a 10-month-old boy were admitted to our hospital for a painless and hard mass located in the axilla or upper arm; the tumors were 1.5, 5, and 3cm in size, respectively. In 2 of these cases, the tumors gradually enlarged during the follow-up periods. Tumor resection was performed in all cases, and the histological diagnosis was fibrous hamartoma of infancy (FHI). FHI is a rare tumor arising from the subcutaneous tissue during infancy. Microscopic examination revealed that the tumor consisted of fibrous trabeculae, mature adipose tissue, and immature mesenchymal cells. Preoperative diagnosis was difficult, but magnetic resonance imaging (MRI), especially fat-suppression MRI, proved beneficial in attaining the diagnoses of FHI. Although recurrences of FHI after surgery have been reported, repeated tumor resection is associated with good prognosis.

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The ceco-appendix region is rare as an omphalomesenteric duct remnant. A male infant was born at 35 weeks' gestation weighing 1,580g. Since he presented with hernia into the umbilical cord with bowel patency, an emergent operation was done. The bowel, inside the hernia sac, was strangulated with a band that was connected to a diverticulum-like structure. Operative diagnosis was Meckel's diverticulum, and we resected it. However, pathological findings showed that the resected bowel was not the diverticulum but the appendix and the cecum. The abnormal artery arose from the root of the mesentery, which we thought to be the omphalomesenteric artery, and ended in the wall of the appendix. This artery didn't branch and maintained the same diameter through all its length and abruptly ended. Furthermore, the appendicecal artery existed separately, suggesting that this artery may have been the omphalomesenteric artery as we thought, wherever it ended. In addition, the umbilical artery adhered to the patent cecum wall. From all these anatomical anomalies, we thought that the omphalomesenteric duct remnant was located at the cecal region. These structural anomalies may prevent bowels from normal reduction into the abdominal cavity, and contribute to the pathogenesis of hernia into the umbilical cord.

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A 12-day-old girl was referred to our hospital because of her poor feedings and abdominal distention. The body temperature was 37.9℃ and her abdomen was markedly distended. The white blood count was 12,310/mm^3, and C-reactive protein was 2.6mg/dl. Abdominal X-ray examinations revealed massive intestinal gases with pneumoperitoneum. At the emergent operation, a large volume of both intestinal gas and fluid was pouring out from the tip of the appendix. Therefore, panperitonitis due to perforated appendicitis was diagnosed. Then an appendectomy with peritoneal drainage was performed. The patient was discharged from our hospital on post-operative day 11 without event. Neonatal appendicitis is extremely rare. We reviewed reports of 45 Japanese cases, and discussed the clinical features of neonatal appendicitis.

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A prenatally diagnosed sacrococcygeal teratoma was excised during the neonatal period followed by partial excision of a lumbosacral teratoma with occult bifid spine at 3 months. The residual tumor was extended posteriorly to the spinal canal and caused right lower hemiplegia and dysuria. Imaging studies revealed that the tumor in the spinal canal was extended up to the lower thoracic spine level, severely compressing the spinal cord and further extended to the retroperitoneum through bilateral intervertebral foramina. The teratoma in the spinal canal was a subdural extramedullary tumor and was totally excised through laminotomy at 9 months of age. The paravertebral retroperitoneal part of the tumor was excised through a lower midline excision and an extraperitoneal approach. The tumor capsule was the dura mater, which was so extended and thin that the repair of the dura was technically very difficult. This approach provided a satisfactory operative view and a working space and was considered suitable and safe for bilateral retroperitoneal tumor excision. The postoperative course was uneventful except for transient liquorrhea without tumor regrowth. Fracture of the right femur and paralytic dislocation of the right hip joint necessitated surgical repair.

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Complete androgen insensitivity syndrome (CAIS) is characterized by normal female appearance and external genitalia in the presence of a 46XY karyotype. Although testosterone is produced at the normal testis, androgen receptor defects permit the development of female external anatomy. Because of the normal female appearance, these patients are raised as girls, and the diagnosis of CAIS is usually not made until amenorrhea is investigated. Herein we report 2 cases of CAIS diagnosed at laparoscopic inguinal hernia repair. In both cases, the laparoscope revealed the presence of the bilateral intra abdominal testis and the lack of female internal genitalia. The postoperative karyotyping (46XY) confirmed the diagnosis of CAIS. In our institute, the incidence of CAIS in girls with inguinal hernia was 0.4% (2/504), suggesting that any girls with inguinal hernia have a high likelihood of CAIS. Checking of internal genitalia at laparoscopic inguinal hernia repair is thought to be an easy and useful CAIS screening procedure.

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