Purpose: To investigate whether age at primary surgery and ductal remnants affect long-term outcome in biliary atresia. Method: Cases (n=93) were categorized by age at primary surgery (A,<0 days; B,&ge;70 days), and the rates of disappearance of jaundice and long-term outcomes were investigated. Cases of late surgery (&ge;90 days) were also analyzed. Fifty-five cases in which jaundice disappeared were categorized by age at primary surgery (C,<70 days; D,&ge;70 days), and their long-term outcomes were compared. In 86 cases in which pathological examination was performed, the relationship between the presence of ductal remnants and the diameter of them with the disappearance of jaundice and long-term outcome was analyzed. The relationship between the ductal remnants and long-term outcome was also analyzed in cases in which jaundice disappeared. Results: There was no difference between groups A and B in the rate of disappearance of jaundice (A, 54.2%; B, 65.3%) and long-term outcome. The rate of late surgery group was also 64.3%. There was no difference between groups C and D in the long-term outcome. Ductal remnants were present in 67 of 86 cases. The rate of disappearance of jaundice (68.7%) in them was significantly higher than that in the 19 cases without ductal remnants (26.3%). Neither age at primary surgery nor diameter of ductal remnants affected outcome in cases in which jaundice disappeared. Conclusion: Age at primary surgery did not affect the outcome of biliary atresia. The presence of ductal remnants positively affected the long-term outcome.

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Purpose: We reviewed our experience of acute abdomen in severe motor and intellectual disabilities at our facility. Methods: We have experienced 15 cases of acute abdomen during January 1994 through December 2010. Variables including physiological examination, vital signs, laboratory data, diagnostic rate, and complications were reviewed. Results: The primary symptom for acute abdomen was abdominal distension in 73.3%, followed by high fever (26.6%), and vomiting (20%). The mean time from appearance of the first symptom to consultation with our facility was 19.9 hours. Among 8 patients with peritonitis diagnosed at the operation, four patients manifested with muscle rigidity preoperatively (p=0.029). Four patients had only abdominal distension (50%). As for the preoperative vital signs, those with tachycardia and low blood pressure tended to have peritonitis and postoperative complications. Correct preoperative diagnosis was achieved in 80% in our department while other reference facilities had 50%. All patients displayed elevation in the percentage of neutrophils preoperatively, compared with those in normal condition. The preoperative CRP was positive in 86.6%. Conclusion: A correct preoperative diagnosis of acute abdomen in cases with severe motor and intellectual disabilities is difficult because these patients show little sign of muscle rigidity despite their peritonitis. Preoperative vital signs and physical findings and elevation of the neutrophil percentage would be the clue for a correct preoperative diagnosis.

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We present a 4-month-old boy with transanal resection of a juvenile sigmoid polyp which led to colocolic intussusception. During conservative treatment for hematochezia, which was present since the infant was 2 months old, this patient developed bowel prolapse. At that time, the bowel prolapse was reduced manually but recurred thereafter. Radiographic contrast enema suggested colocolic intussusception involving the sigmoid colon, and the patient was diagnosed with prolapse of the intussuscepted bowel, and the prolapse was again reduced manually. However, the intussuscepted bowel prolapsed again, and radiographic contrast enema revealed a sigmoid polyp. Subsequently, colonoscopy revealed polyps at 3 sites in the sigmoid colon; and the polyps could not be resected endoscopically. The sigmoid prolapse recurred for a third time, and we performed polypectomy via the anal approach; the postoperative course was stable. Pathological findings of the resected polyps showed that the infant had juvenile polyposis. Minimally invasive anal polypectomy can be safely performed in this pediatric case where endoscopic polypectomy was difficult.

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We report a rare case of male infant presenting with serial two time intrauterine intestinal volvulus. Fetal sonography showed an arched dilated bowel loop at 29 weeks, the primary volvulus, which later resulted in ileal atresia. Preterm labor occurred at 35 weeks, which may have been induced by fetal stress resulting from the occurrence of the secondary volvulus. This is the first report showing serial two time intrauterine intestinal volvulus with ileal atresia.

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We report a case of Spigelian hernia associated with an undescended testis. The patient was a 10-month-old infant with right lower abdominal bulging and a right impalpable testis. MRI at 5 months. CT and US at 10 months revealed a right ventral hernia at the lateral border of the right rectus muscle and a small solid mass in contact with the ventral hernia, considered to be the right testis. Operation at 10 months showed the hernia sac underlying the thin external oblique muscle and fascia, and the right testis, having normal size and appearance but no gubernaculums, attached to the hernia sac, and the spermatic cord and vessels with enough length to be pulled down into the scrotum. Our case is the first reported one of this entity in Japan, and a review of the English literature revealed only 19 cases of Spigelian hernia with cryptorchidism in children younger than 15 years of age. Most of the reported cases showed similar anomalous anatomical features, suggesting that these are included in a common clinical entity as congenital anomalies.

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A 10-year-old girl complaining of lower abdominal pain and urinary retention was admitted to the pediatric emergency department. From the abdominal X-ray image, we suspected a lower abdominal tumor. However, abdominal ultrasonography revealed a distended bladder. On urethral catheterization, we detected a bulging imperforate hymen. The patient underwent surgery, which involved a circular incision of the hymen, and drainage of approximately 350ml coagulated, old blood. Thereafter, the entire circumference of the incisional wound was sutured. The postoperative course was good without re-adhesion of the hymen, and the onset of regular menstruation was observed after the surgery. Imperforate hymen is often observed in the neonatal period and adolescence. Adolescents tend to be hesitant to undergo inspection of the vulva; this may result in delayed diagnosis of such a condition. It is important to include the inspection of the vulva in the clinical examination of adolescents without menarche and complaining of lower abdominal pain without menarche. In addition, circular incision and suture of the incisional wound in the hymen is recommended in the treatment of this condition, in order to prevent hymen re-adhesion.

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A 10-year-old girl underwent Kasai operation for biliary atresia at 5 months of age, and living donor liver transplantation with left lateral segment graft at 9 months of age. She was referred to our hospital because of a sudden onset of right side abdominal pain and vomiting. CT scan showed the whirl sign, and she was diagnosed with having small bowel volvulus. At the emergent laparotomy, the twisted small intestine, with 270° counter-clockwise rotation, was reduced and no ischemic bowel was found. It was revealed that the elevated ileo-cecal region and upper jejunum adhered to the point of the Roux-en-Y loop passing through the transverse mesocolon. This adhesion caused the narrowing of the mesentery, and the volvulus. Adhesiolysis around the Roux-en-Y loop was performed. Eight days after the operation, she underwent re-laparotomy because of the recurrence of the volvulus. After the reduction of the volvulus, terminal ileum and upper jejunum were fixed to the retroperitoneum. The patient recovered well after this procedure. It was thought that the cause of the elevation of the ileo-cecal region was unfixation of the ileo-cecal region in infancy. It is suggested that this type of volvulus could occur as a possible complication in the patient with Roux-en-Y hepaticojejunostomy at liver transplantation in infancy.

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This is a case report of an 11-year-old male with torsion of wandering spleen in situs inversus totalis. At birth, he was diagnosed with situs inversus totalis, double outlet right ventricle, and common ventricle. This time, he suffered from the right upper quadrant pain with localized tenderness. Plain CT revealed a small round calcification like a fecalith in the right upper abdomen, and emergency surgery was performed on the preoperative diagnosis of acute appendicitis with intestinal malrotation. At laparotomy, though the appendix was normal, torsion of inverted spleen without suspensory ligaments resulting in infarction was detected. Blood supply was not restored by detorsion and splenectomy was performed. Wandering spleen is a rare form of developmental anomaly arising from either a congenital or acquired laxity of the suspensory ligaments of the spleen and can be a cause of torsion. In this case, coexisting situs inversus totalis and malrotation made it difficult to diagnose accurately. In situs inversus totalis, complicating splenic torsion is extremely rare, but it is necessary to consider the possibility of this entity.

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The multistaged extrathoracic esophageal elongation procedure is useful for preserving the native esophagus in long-gap esophageal atresia. However, this technique requires repeated meticulous dissection of the proximal esophagus to prevent injury to the esophageal wall and cervical nerves and vessels. Herein we report a case of type A esophageal atresia using Seprafilm adhesion barrier in a multistaged extrathoracic elongation procedure. A female baby was born at 36 weeks' gestation weighing 2,612g. A gastrostomy was performed at the age of 2 days on the diagnosis of esophageal atresia without tracheoesophageal fistula. At the age of 2 months, the cervical esophagostomy was fashioned while placing Seprafilm around the cervical esophagus as well as subsequent elongation procedures, starting "sham-feeding". Initial elongation was performed at the age of 5 months with 2.5cm elongation. Further elongation was performed at the age of 9 months and 14 months with 1.5cm and 1.8cm elongation respectively. The proximal esophagus was covered with a Penrose drain to pass through the subcutaneous tunnel to avoid infection. The adhesion around the cervical esophagus was sparse; thus it was not necessary to incise the previous scar to dissect the proximal esophagus extended to the level of the cricoid cartilage in both elongation procedures and the definitive operation, which was performed at the age of 18 months with tension-free end-to-end anastomosis.

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The treatment strategy for pediatric renal malignancy with cavoatrial extension is still controversial. We have treated four cases in the last twenty years. Case 1 was stage III nephroblastoma with intracardiac tumor extension and the tumor was resected under cardiopulmonary bypass with hypothermic circulatory arrest. Case 2 was stage IV nephroblastoma with inferior vena caval extension and the tumor was resected by side clamping of the inferior vena cava after neoadjuvant chemotherapy. Case 3 was stage IV MRTK with intracardiac tumor extension and the tumor was resected under cardiopulmonary bypass after neoadjuvant chemotherapy. Case 4 was stage IV nephroblastoma with intracaval extension and the tumor was resected with partial combined resection of the inferior vena cava after neoadjuvant chemotherapy and radiotherapy. The three cases other than case 3 are still alive and disease free. It seems important to investigate the extent of tumor thrombus adequately initially and an appropriate procedure should be chosen depending on the extent of tumor thrombus after neoadjuvant chemotherapy.

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Wandering spleen is an uncommon condition in children. We report a case of a 3-old-year boy with wandering spleen and gastric volvulus who was successfully treated by laparoscopic splenopexy and gastropexy. His initial symptom was acute abdominal pain and vomiting. Radiological study revealed gastric volvulus and malpositioned spleen which was located behind the stomach in the sub-hepatic area. Insertion of a nasogastric tube quickly improved the patient's condition. After gastric drainage, ultrasound sonography revealed that the spleen was repositioned to the upper left quadrant, and the flow of the splenic artery was normal. Elective laparoscopic splenopexy and gastropexy were planned. We performed a laparoscopic retroperitoneal pouch procedure without a balloon dilator or absorbable mesh. A vertical incision was made in the posterolateral peritoneum and a large retroperitoneal pocket was created by a blunt dissection. The spleen was placed in the pocket and the peritoneal closure was secured, leaving space for the splenic pedicle. The gastric fundus was fixed to the diaphragm by a single suture, and the greater curvature was fixed to the anterior peritoneum. Six months after surgery, the patient was doing well and remained symptom-free. While several laparoscopic techniques of splenopexy using retroperitoneal balloon dilator and absorbable mesh have been reported, our procedure is simple and safe because it excludes a large device and foreign material for small children.

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We present a case of a 10-month-old boy with a right axillary mass. It was 3cm in diameter, and felt spherical and elastically hard. The surface of the mass was smooth and its movability was relatively poor. Ultrasonography showed a solid mass with low and high echogenicities. CT and MRI revealed that it was composed of both fat and non-fat components. With a preoperative diagnosis of hamartoma of the axilla, surgical resection was performed. As there was no capsule and the boundary of the mass was unclear, it was resected en bloc with surrounding fat tissue. Histopathologically, there was an organoid structure consisting of mature adipose tissue, immature mesenchymal cell nests and intersecting fibrous trabeculae, and it was diagnosed as a fibrous hamartoma of infancy (FHI). Based on the experience of the presented case, we conclude that FHI should be included in a differential diagnosis of axillary masses in infants.

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We report a case of laparoscopic repair of epigastric hernia using Lapa-Her-Closure^<TM>. The patient was a 4-year-old girl with an epigastric hernia. The orifice of the hernia was located at a midpoint between the xiphoid process and umbilicus, the size of the hernia was 1×1cm, and its content was preperitoneal fat. We completed direct suture of the fascial defect laparoscopically. Two 5-mm ports were used via the umbilicus for the laparoscope and the left lateral abdomen for the working port. The orifice of the hernia covered by the falciform ligament was identified laparoscopically. A Lapa-Her-Closure needle with a 2-0 Ethibond was passed through the right rectus muscle, the suture was placed in the abdominal cavity, only the needle was pulled back at the level of subcutaneous fat, then the needle was passed through left rectus muscle to pick up the above 2-0 Ethibond; thus the defect was sutured and the knot was made subcutaneously. A total of 7 sutures were put over the hernia defect at a distance of 2cm cranially and caudally. The post-operative course was uneventful. The procedure was safe, quick, simple, and cosmetically acceptable.

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A 2-year-old girl was referred due to sudden onset vomiting. Abdominal ultrasonography and upper gastrointestinal series suggested that duodenal obstruction due to foreign body impaction accounted for the symptom. Subsequent gastrointestinal endoscopy revealed that it was congenital duodenal stenosis with a plugged mushroom. Diamond anastomosis was done, and Ladd's operation was added for the accompanying malrotation. Congenital duodenal stenosis should be considered in cases of upper GI tract obstruction even after the newborn period.

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