Purpose: To assess 5-year treatment and peritoneal fluid culture (PFC) outcomes of acute appendicitis at our institution.
Methods: We retrospectively examined 171 patients (115 men, 56 women) treated for acute appendicitis between June 2007 and May 2012 with the following particulars: age, sex, eligibility for emergent surgery, perforated appendicitis (PA), PFC results, and postoperative complications. The PFC results of patients with PA and those with non-perforated appendicitis (NPA) were compared. Furthermore, patients with PA were divided into two groups based on the necessity of a postoperative change in the antibiotic regimen. PFC results and postoperative complications were compared between the two PA groups.
Results: Of 171 patients, 153 (103 men, 50 women) were treated according to the therapeutic criteria for acute appendicitis at our institution. Thirty-five patients were treated conservatively, and 118 patients underwent emergency surgery. E. coli, Pseudomonas aeruginosa, Streptococcus species, and Bacteroides species were predominantly detected in the PFC results, which were positive in 12% of NPA and 74% of PA patients. Bacteria with resistance to antibiotics were detected in 37% of PA patients. Postoperative complications occurred in 1 NPA and 11 PA patients. Of 43 PA patients, 30 underwent a change in the postoperative antibiotic regimen, and had a higher postoperative complication rate than those who did not change the antibiotic regimen; the incidence of positive PFC results and drug resistance development were not significantly different between the two groups.
Conclusion: Of 153 patients with acute appendicitis, 93.5% were treated without complications. This verifies the validity of the therapeutic criteria adopted at our institution.

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Purpose: The prognosis of chronic constipation in children is generally good, and most children show improvement of their defecation following treatment by glycerin enema and laxative administration. Children resistant to long-term conservative therapy and having almost no natural bowel movement except by glycerin enema were evaluated by 3D-CT for vertebral arch dysraphism.
Methods: From 2003 to 2011, we performed barium enema examination and anorectal manometry on 157 children with severe constipation and suspected of having Hirschsprung disease. Six of these children were diagnosed as having Hirschsprung disease or pseudo-Hirschsprung disease. The remaining 151 were diagnosed as having chronic constipation and were treated conservatively, among which 20 (13.2%) were found to be resistant to conservative treatment. These children showed almost no natural bowel movement except when induced by glycerin enema, and they underwent lumbar 3D-CT. They were compared with 29 nonconstipation children who underwent abdominal CT to examine for appendicitis, intussusception, neuroblastoma, or other diseases.
Results: Dysraphism of the vertebral arch was found at L5 and S1 in 55.0% and 90.0% of the children in the severe constipation group compared with 3.4% and 34.5% of those in the nonconstipation group, respectively.
Conclusions: The percentages of children with L5 and S1 dysraphism of the vertebral arch were significantly higher in the severe chronic constipation group than in the nonconstipation control group.

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Purpose: Testicular microlithiasis (TM) is a relatively rare condition characterized by calcification within seminiferous tubules. Although TM is regarded as a potential risk of tumorigenesis and infertility in adults, little is known about it in the pediatric population. We conducted a retrospective investigation of the prevalence and clinical features of pediatric TM detected by ultrasound examination.
Methods: A total of 212 pediatric outpatients (mean age, 3.0 ± 2.9 years; range, 0–16 years) underwent testicular ultrasound examination for undescended testis, inguinal hernia, hydrocele testis, retractile testis, funicular hydrocele, testicular atrophy, epididymitis and testicular neoplasm at our clinic in 2012. TM was defined as the ultrasonographic confirmation of 5 or more calcific deposits in one visual field. The incidence of TM and its clinical features determined from clinical records were retrospectively examined.
Results: TM was found in 5 patients (2.4%), who were 3 of 91 patients with undescended testis (2 were treated and 1 was untreated), one of three patients with contralateral testis after orchiectomy for testicular atrophy, and one patient with epididymitis. Their mean age was 10.0 ± 3.4 (range, 8–16) years. Four of them had underlying conditions such as nephrotic syndrome, Down syndrome, acute leukemia, and severe motor and intellectual disabilities.
Conclusions: TM in pediatric patients might be a sign of impaired testicular function and indicative of a future risk of infertility or tumorigenesis. Regular and long-term careful monitoring is required for such patients.

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We present a successfully treated female infant suffering from congenital tracheal stenosis combined with hypoplasia of right lung, left pulmonary artery sling and bridging bronchus. A 9-month-old female was referred to our hospital with aggravation of stridor caused by upper respiratory infections. Chest-enhanced CT scan revealed congenital tracheal stenosis combined with right lung hypoplasia. Four days after admission, she developed sudden respiratory failure immediately after continuous weeping, subsequently requiring intubation and mechanical ventilation. As her respiratory condition did not improve, we performed slide tracheoplasty and tracheopexy at day 7 after admission. We hypothesized that the acute respiratory failure might be related to tracheal flexing caused by a mechanism similar to postpneumonectomy syndrome, where the trachea was translocated anterior to the aortic arch with slide tracheoplasty. Furthermore, tracheopexy with pericardium roll was added to alleviate postoperative tracheomalasia. The postoperative course was noneventful: she extubated at postoperative day 12, and discharged three weeks after operation.

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We report a rare case of dumb-bell shaped ganglioneuroma with paraplegia. A 2-year-old boy suffered from paraplegia with fever recovered naturaly after his symptoms diminished in a month. Eight months later, however, paraplegia recurred with fever. He was admitted to our hospital, and CT and MRI images showed a spinal tumor. The tumor was located below the right kidney and extended to the retroperitoneum, spinal canal, and vertebral body, as a dumb-bell shaped mass. We suspected the tumor was ganglioneuroma, and tumor biopsy was thus conducted. For exclusive diagnosis of ganglioneurobrastoma and for reduction of neurologic symptoms, we performed partial resection of the tumor in the retroperitneum and spinal canal. Histopathological diagnosis revealed the tumor as ganglioneuroma. In 1-year lapse, the remaining tumor was not enlarged, and his paraplegia was slightly improved. In conclusion, we considered the tumor was originated from a sympathetic ganglion of the retroperitoneum, which differentiated gradually with time, causing cord compression symptoms by the differentiated tumor enlargement.

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Here, we report a case of successful resolution of severe hepatopulmonary syndrome (HPS) in biliary atresia by living donor liver transplantation (LDLT). A 14-year-old girl diagnosed as having biliary atresia (BA) with HPS was referred to our hospital for LDLT. She underwent Kasai portoenterostomy 65 days after birth. Her jaundice and hypoxia worsened from 11 days after birth. On admission, her data on oxygenation were as follows: SpO₂ in room air was approximately 80%, arterial blood gas analyses showed PaO₂ of 39 mmHg, and perfusion scintigraphy showed a shunt ratio of 41%. From these findings, this patient was determined to have severe HPS. LDLT was performed using an extended left lobe graft from her father. She underwent relaparotomy and ileostomy twice owing to bowel perforation 6 and 9 days after LDLT. One month after LDLT, the shunt ratio increased to 59.9% transiently, but her respiratory condition improved gradually. Six months after LDLT, her data on oxygenation significantly improved as follows: SpO₂ in room air, 100%; PaO₂, 90.0 mmHg; and shunt ratio, 11.9%. We recommend that although the complication rate after LDLT for BA with severe HPS is relatively high, LDLT should be performed with utmost care. However, there is no significant evidence for the long-term prognosis after LDLT; therefore, it is necessary to closely monitor such a patient for a long period of time.

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Late-onset congenital diaphragmatic hernia (CDH) complicated by intestinal strangulation is rare, especially in central tendon defect subtype. As far as we have investigated, no cases of late-onset CDH complicated by postoperative mediastinal abscess have ever been reported until now. A one-year-old female, with a past surgical history of ventricular septal defect repair at the age of three months, was admitted to our hospital complaining of dyspnea. Emergency laparotomy diagnosed the patient as having the central tendon defect type of CDH with hernia sac, accompanied by incarceration of the transverse colon and left hepatic lobe. Partial colectomy and direct closure of the defect were performed. Postoperatively, she continued with a high fever, and she subsequently developed a mediastinal abscess of 5 cm in diameter. Emergency abscess drainage was then undertaken on postoperative day 12. Because an echo-guided percutaneous drainage or an open-drainage via median sternotomy was considered infeasible, we instead performed thoracoscopic drainage. The postoperative recovery was favorable and noneventful. A postoperative mediastinal abscess after central tendon defect repair is extremely rare, and thoracoscopic abscess drainage may be a feasible option for treatment.

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A 9-year-old boy, who had been suffering from headache, vomiting, and hypertension, was found to have bilateral adrenal tumors and was referred to our hospital for further exploration. Computed tomography and magnetic resonance imaging revealed bilateral adrenal tumors. Serum noradrenalin, urinary noradrenalin, normetanephrine, and VMA levels were high and ¹²³I-MIBG scintigraphy revealed selective uptake of the radiotracer in the bilateral adrenal glands. We finally made the diagnosis of bilateral adrenal pheochromocytoma and performed bilateral partial adrenalectomy to preserve his adrenal function. Later, he underwent a genetic test for von Hippel-Lindau disease because of his family history, and he was diagnosed as having the disease. The adrenocortical steroid administered for postoperative replacement was gradually tapered off over a period of four months. There have been few reports on the effectiveness and feasibility of partial adrenalectomy for bilateral adrenal pheochromocytoma, particularly in the pediatric population. The case of our patient presented here showed that this procedure might be a good therapeutic option to avoid a long-term administration of supplemental steroids by preserving adrenal function. Long-term and close monitoring for the recurrence of the disease in the residual glands is necessary.

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We report a 2-month-old male infant who underwent emergency surgery for presentation of acute peritoneal irritation after admission. At laparotomy, only chylous ascites was observed in the operative field, without any important underlining pathological condition. Only peritoneal toilet and drainage as well as postoperative low-fat diet were pursued during follow-up treatment. His clinical course was not eventful, and did not without evidence of associated disease or recurrence. The signs and symptoms of peritonitis due to extravasation of chyle into the peritoneal cavity is a rare condition. This rare event was reviewed.

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A four-year-old boy was suffering from upper abdominal pain and vomiting for two days before admission into our hospital. Findings of ultrasonography and CT examinations revealed enlargement of the gallbladder and thickening of the wall with an ischemic pattern due to torsion of the gallbladder on diagnosis. Because the enlarged gallbladder was also twisted, and necrotic on laparoscopic examination, cystectomy was performed. It is known that clinical manifestations of torsion of the gallbladder are not specific, and preoperative diagnosis is usually difficult. In this case, the abdominal ultrasonography and enhanced CT facilitated the diagnosis. Torsion of the gallbladder is a rare condition in infancy; however, it is important to perform differential diagnosis in cases with acute abdomen.

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Pediatric rectal impalement injury is relatively rare. We report a case of rectal impalement injury associated with urinary tract damage. An 11-year-old boy fell down from a chair, and a broken leg of the chair stuck into his anus. The piece of stuck leg was removed by his family. At the emergency room, penetrated rectal injury was diagnosed by proctoscopy. He was transferred to our hospital for further evaluation and treatment. CT scan revealed injuries of the rectum, seminal vesicles and bladder. An emergency operation was performed 8 hours after injury. Laparoscopic examination revealed intact intra-abdominal organs, except for the bladder perforation. Repair of the bladder was performed laparoscopically, followed by sigmoid colostomy. The postoperative course was uneventful. Based on our results, laparoscopic evaluation was useful in the treatment of pediatric rectal impalement.

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A 1,996 g girl was born at 32 weeks and 4 days of gestation. Antenatal scanning had demonstrated intestinal dilatation from 24 weeks of gestation. A scan on the day of delivery showed worsening dilatation and intraluminal echogenicity. Fetal volvulus and intestinal hemorrhage were suspected; therefore, an emergency cesarean section was performed. At laparotomy, the proximal jejunum was found to end as a huge blind pouch. A typical apple-peel tract was found. In addition, both the jejunum and ileum side were involved resulting in necrotic changes. Correction of the volvulus, resection of the necrotic intestine, ileostomy and jejunostomy were performed. At the end of the initial operation, the length of the residual jejunum was 6 cm and that of the ileum was 31 cm. Weight gain was obtained with a combination of parenteral nutrition (PN) and enteral nutrition (EN) into the ileum. Seventy-six days after the initial operation, jejunoileostomy was performed owing to worsening cholestasis and deteriorating liver function. Following the surgery, cholestasis continued to worsen and oral prednisolone was commenced resulting in the normalization of liver function 142 days after birth. PN was discontinued 210 days after birth, and she was discharged from hospital 217 days after birth. She was readmitted because of poor weight gain and metabolic acidosis due to short bowel syndrome, and intermittent PN was initiated 265 days after birth. She started solid food at 13 months without problem and stopped PN at 15 months. Particular care must be taken in managing sepsis and cholestasis in apple-peel atresia complicated with jejunal and ileal volvulus. A staged operation with a combination of PN and EN should be considered.

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We report a case of duodenal duplication cyst connected to the pancreaticobiliary duct. A ten-month-old girl was admitted to our hospital because of vomiting. Abdominal CT showed the cyst of pancreatic head. On the upper gastrointestinal series, the cyst had protruded into the duodenal lumen. These findings suggested the presence of duodenal duplication cyst, and an operation was performed accordingly. We performed operative cholangiography, and the cyst was confirmed to have connected to the pancreaticobiliary duct. Marsupialization was done to avoid pancreaticobiliary duct injury. Pathological findings further demonstrated the presence of duodenal duplication cyst. The postoperative course was uneventful, and she was discharged on postoperative day 8.

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Acinar cell carcinoma of the pancreas is an exocrine malignant neoplasm with features of acinar cell differentiation. It accounts for about 1% to 2% of all pancreatic neoplasms, and pediatric cases are extremely rare.
A 12-year-old girl presented with complaints of vomiting, weight loss, and general malaise was examined. Abdominal CT showed a mass measuring 47 mm from the pancreas head with multiple hepatic and lung metastases. Tumor biopsy revealed a pancreatic acinar cell carcinoma. She was treated with a combination chemotherapy consisting of intravenous infusion of gemcitabine (GEM) and oral administration of tegafur-gimestat-otastat potassium (TS-1). Although progression of the disease was slowed, she suffered from the side effects of GEM, such as fever and general discomfort. Therefore, GEM was replaced with TS-1 monotherapy. The patient’s lung metastases progressed, and she died 496 days after treatment. We reviewed 42 cases of pancreatic acinar cell carcinomas. Some patients who underwent surgical resection achieved long-term survival; however, chemotherapies employed were ineffective against acinar cell carcinomas, and the prognosis of patients with metastasis was very poor.

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High-output heart failure in a neonate due to pulmonary sequestration is rare. We report a case of neonatal heart failure relieved with thoracoscopic resection of an intralobar pulmonary sequestration. A male neonate of 39-week gestational age presented with respiratory distress, chest X-ray and echocardiography revealed high-output heart failure. A computed tomography showed a left lower pulmonary sequestration with venous drainage into the azygos system. Preoperative diagnosis is an extralobar pulmonary sequestration, and high-output heart failure is caused by arteriovenous shunt in the pulmonary sequestration. We intervened with thoracoscopic resection on day-of-life 21. Intraoperative diagnosis is an intralobar pulmonary sequestration. Postoperative course was uneventful, and the patient was discharged on postoperative day 20 without any sign of heart failure.

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