Purpose: The long-term treatment results and problems of extremely low birth weight infants (ELBWIs) were examined.
Method: We had 15 ELBWIs who underwent peritoneal drainage or open abdominal surgery from 1981 to 2012. These 15 ELBWIs were divided into two groups according to birth weight, i.e., less than 500 g and from 500 to 1,000 g. The survival rate of each group was calculated and compared with that of very low birth weight infants (VLBWIs). The problems presented by ELBWIs who survived were revealed.
Results: Seven of the 15 ELBWIs survived, with a survival rate of 46.7%. Three of the 15 ELBWIs were less than 500 g, and 2 of the 3 infants, who were encountered after 2003, survived. Among the surviving 5 ELBWIs weighing between 500 and 1,000 g, two patients showed MR, which was caused by Down syndrome and PVL. The 2 ELBWIs weighing less than 500 g who survived also showed MR, which was caused by PVL and hydrocephalus.
Conclusions: In recent years, lifesaving possibilities have become high even in ELBWIs of less than 500 g. However, MR is observed at high rates (4/7, 57.1%) caused by central nervous system damage such as PVL. These are the problems presented by ELBWIs during the postnatal period.

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Purpose: Gastric volvulus is defined as a rotation of all or part of the stomach by more than 180 degrees along its axis. Surgical repair is required in acute or recurring cases. We analyze the efficacy and safety of our laparoscopic three-point anterior gastropexy (L3pAG).
Methods: A systematic retrospective review of 6 patients (4 boys and 2 girls) who were surgically treated by L3pAG between 2000 and 2013 in our institutes was performed.
Result: The age at surgery ranged from 3 years and 5 months to 11 years and 5 months with an average of 91.8 ± 35.8 months. All the cases were idiopathic volvulus, and they involved developmental disorder in 5, mental retardation in 4, Hirschsprung’s disease in 1, and bronchopulmonary foregut malformation in 1. All of the 5 patients with acute onset successfully received emergent decompression with a nasogastric tube and were followed by elective surgery. The mean operation time for L3pAG was 96.7 minutes. The operative blood loss was minimum, and no severe postoperative complication developed except for mild hyponatremia and fever in 1 patient. The enteral nutrition could be restarted on postoperative day 5.3 on average. No recurrence was observed during an average follow-up period of 46.8 ± 18.8 months (2 years to 6 years and 2 months).
Conclusion: Elective surgery could be performed in patients with acute gastric volvulus after successful treatment with a nasogastric tube. L3pAG might be an effective surgical option in cases of primary volvulus.

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Purpose: On the basis of the results of a questionnaire survey of the participants of the Kyushu Pediatric Surgical Congress, we evaluated the usefulness of interval appendectomy for an appendix mass.
Methods: Comparative analyses were performed for the following procedures for patients aged 15 years and younger undergoing appendectomy: initial open appendectomy vs initial laparoscopic appendectomy, initial appendectomy with appendix mass vs initial appendectomy without appendix mass, and initial appendectomy with appendix mass vs interval appendectomy with appendix mass.
Results: Initial laparoscopic appendectomy, compared with initial open appendectomy, had a significantly longer operation time (94.0 ± 42.1 min vs 68.0 ± 31.8 min) and significantly reduced wound infection rates (0.8% vs 4.7%). Initial appendectomy with appendix mass, compared with initial appendectomy without appendix mass, had significantly longer operation time (122.6 ± 65.3 min vs 83.5 ± 38.8 min) and postoperative hospitalization (10.3 ± 7.2 days vs 5.7 ± 3.1 days), and more overall adverse postoperative events (21.4% vs 5.4%). Interval appendectomy with appendix mass, compared with initial appendectomy with appendix mass, had significantly fewer overall adverse postoperative events (0% vs 21.4%) and longer postoperative hospitalization (4.3 ± 1.9 days vs 10.3 ± 7.2 days). However, interval appendectomy with appendix mass, compared with initial appendectomy with appendix mass, had a significantly longer total hospitalization (19.0 ± 6.5 days vs 11.9 ± 8.4 days). No significant differences were observed in operation time and intraoperative complication between initial appendectomy with appendix mass and interval appendectomy with appendix mass.
Conclusion: The current results reaffirmed the difficulty of treatment of an appendix mass. Interval appendectomy had significant therapeutic usability for an appendix mass from the standpoint of adverse postoperative events and postoperative hospitalization. However, interval appendectomy was disadvantageous with regards to total hospitalization.

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Purpose: The differential diagnosis of cystic lesions at the hepatic hilum detected prenatally remains difficult. The aim of this study was to determine the factors that are helpful for differentiating between biliary atresia (BA) and congenital biliary dilatation (CBD) shortly after birth.
Methods: We reviewed the medical records of patients with prenatally detected cysts at the hepatic hilum in four institutions between April 2000 and October 2013. We divided the patients into BA and CBD groups, on the basis of operative findings and cholangiography. The preoperative findings of ultrasound and laboratory tests were compared in these groups.
Results: Ten patients were enrolled (BA, n = 6 and CBD, n = 4). In the BA group, two were diagnosed as having Type I cysts, with the others having Type IIId. The mean direct bilirubin (D-Bil.) levels shortly after birth (at 1 to 7 days of life) were significantly higher in the BA group than in the CBD group (2.7 ± 0.5 vs 0.5 ± 0.1 mg/dl, p = 0.0001). The mean AST and ALT levels shortly after birth were not significantly different between these groups, whereas in the BA group, they increased markedly after 20 days of life.
Conclusions: The high D-Bil. levels shortly after birth in the patients with the prenatally detected cysts at the hepatic hilum can indicate that the condition is BA rather than CBD. They should be monitored closely and undergo early radical operation before cholestatic liver injury begins.

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In the present report, we describe a case of Gross type D esophageal atresia (EA) that was diagnosed preoperatively. The patient was a male infant delivered at 41 weeks and 5 days, with a birth weight of 3,836 g. He was suspected of having EA on the basis of findings of drooling, cyanosis, and meconium staining. In addition, the medical staff encountered difficulty in inserting a nasogastric tube. However, chest radiography did not indicate coiling of the nasogastric tube, and thus esophagography was performed. Esophagography indicated the upper esophageal pouch, trachea, and lower esophagus. On the basis of these findings, he was diagnosed as having Gross type D EA. He was then transferred to our hospital and underwent primary anastomosis and fistula ligation on the same day. After the operation, a proximal tracheo-esophageal fistula (TEF) scar resulted in the development of tracheomalacia and tracheal collapse. Therefore, we performed tracheostomy 28 days after the surgery for fistula ligation. The infant’s respiratory condition became stable after the tracheostomy. He was weaned from mechanical ventilation and transferred to the pediatrics unit 30 and 42 days after the fistula ligation, respectively. Although the preoperative diagnosis of Gross type D EA is difficult, severe respiratory complications may develop if proximal TEF is overlooked. Hence, proximal TEF should be considered in all cases of EA. We consider that preoperative bronchoscopy is the most appropriate method for diagnosing proximal TEF.

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A male infant was born at 36 weeks of gestation with a birth weight of 3,095 g. Soon after birth, he was referred to a local hospital for mild respiratory disorder. On the first day of life, he was able to start oral feeding with normal infant formula; then he excreted bloody stool on postnatal day 3, which suggested that he had cow’s milk allergy. Intake of extensively hydrolyzed formula could not stop the bloody stool. Therefore, pediatric elemental formula (Elental-P^®; ED-P) was started on postnatal day 12. However, he was unable to tolerate ED-P because the amounts of gastric residues of ED-P gradually increased. Then, he was transferred to our hospital for evaluation of intolerance to both milk and elemental formula and for starting central venous nutrition. Although oral intake of sugar and electrolyte solution resulted in no gastroenteric symptoms, oral intake of both ED-P and fat emulsion induced poor gastric output and the disappearance of intestinal tract gas, as shown by abdominal X-ray. Oral intake of both own mother’s milk and another elemental formula without soybean ingredients induced no gastroenteric symptoms, and he was well when fed orally without parenteral nutrition. IgE-RAST for cow’s milk and soybean showed negative findings and the drug-induced lymphocyte stimulation test (DLST) for cow’s milk showed positive findings (1,474%). However, DLST for ED-P showed negative findings (167%; cut-off value, 180%). His clinical course suggested that he had food allergy to both milk and soybean ingredients.

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We have selected extravesical ureteral reimplantation for vesicoureteral reflux (VUR) since 2009 because hematuria and spasms are minimal. To clarify its efficacy and safety, we retrospectively evaluated the surgical outcomes of our extravesical ureteral reimplantation for VUR associated with paraureteral diverticula in children. Five patients (8 ureters) underwent extravesical ureteral reimplantation between 2009 and 2014. Two of them had unilateral VUR with ipsilateral diverticulum and 3 had bilateral VURs with bilateral diverticula. The median age of the 5 patients, 4 of whom were male, at surgery was 5.7 years old (range, 1.8–14.9). Mean follow-up duration was 49.4 months (range, 32–67). VUR was Grade I in no ureter, Grade II in 2 ureters, Grade III in 2 ureters, Grade IV in 3 ureters, and Grade V in 1 ureter. Patients with unilateral diverticulum could leave the hospital 1 day postoperatively, and patients with bilateral diverticula could leave the hospital 3 days postoperatively. No patient had febrile urinary tract infection after surgery. One patient had hydronephrosis at 3 weeks postoperatively, but none had hydronephrosis at 3 and 12 months postoperatively. Extravesical ureteral reimplantation is safe and effective for VUR associated with paraureteral diverticula in children because there are few complications and minimal perioperative morbidity.

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A 9-year-old boy was referred to our hospital for severe lower abdominal pain with inflammatory signs. Abdominal CT scans showed a 5-cm-size solid mass located near the sigmoid colon. Antibiotics administration failed to improve the inflammatory signs. On the basis of abdominal MRI, it was suspected that the tumor included hematoma, and he underwent laparoscopic single-site surgery. The solid tumor of 5.5×5.0×3.5 cm³ size located in the pelvic cavity was completely removed. Histopathological and genetic evaluation of the tumor confirmed epithelioid inflammatory myofibroblastic sarcoma (EIMS). Surgical resection is the most important treatment for EIMS because adjuvant therapy is less effective. The patient did not show any sign of recurrence within 2 years after surgery; however, he needs to be closely observed for a long time.

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A male neonate, weighting 2,755 g and without anus formation, was born by cesarean section. Since abdominal X-ray imaging showed giant colonic gas, he was suspected of having a congenital pouch colon (CPC). On day 1, emergency laparotomy was performed. The pouch-shaped colon with a colovesical fistula was identified. After the fistula was transected, a colostoma was formed on the pouchshaped colon. Stasis of feces in the pouch-shaped colon was evident, requiring manual irrigation several times a day. Following his transfer to our hospital, contrast enema revealed the existence of transverse and ascending colon, suggesting CPC type 3 according to the literature. During sacroabdominoperineal anorectoplasty, pull through was carried out using the rest of the colon with the pouch-shaped colon excised. On postoperative year 3, the patient still had frequent bowel movements, requiring medication or enema. Here, we present a rare case of CPC focusing on postoperative function of defecation.

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Spigelian hernia in the pediatric population is rare. We report on a case of Spigelian hernia in an 8-month-old female infant. She was transferred to our hospital immediately after birth because of a bulging mass in the left abdomen. Her left upper abdominal wall showed a 5-cm-diameter protrusion. The diagnosis of Spigelian hernia was made on the basis of ultrasonography performed 1 week after birth, which revealed a defect of the muscle layer and herniated bowel loops under the skin. Since the probability of incarceration seemed low, elective operation was scheduled at 8 months of age. At the time of operation, a large hernia sac (5.0 × 3.5 cm in size) containing transverse colon was protruded through the Spigelian aponeurosis defect, which was located lateral to the margin of the rectus abdominis muscle. The hernia sac was excised and the Spigelian aponeurosis defect was closed by direct suturing of the external margin of the rectus abdominis muscle to the transversus abdominis muscle. She was discharged from the hospital one day after the operation. The patient is free from recurrence 1 year postoperatively.

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A case of mesenchymal hamartoma of the chest wall in a 0-day-old female neonate is reported. The baby was vaginally delivered following an unremarkable prenatal course. Computed tomography revealed that the tumor originated from the posterior part of the left 6th rib and with a bony outer shell. This tumor was suspected as osteochondroma, and was progressively growing and should then be considered malignant. Total resection of the tumor with the posterior portion of the 6th rib was performed. A prosthetic material was not used to close the chest wall defect. The histopathological diagnosis was mesenchymal hamartoma of the chest wall from the rib. Neither recurrence nor scoliosis was observed one year after surgery. This tumor is extremely rare; however, its Xp, CT and MRI findings are specific, and it is necessary to consider it when treating chest wall mass lesions in neonates. According to the literature, this tumor should be surgically resected; however, several recent reports revealed that the tumor tends to regress or vanish spontaneously. We discuss the therapeutic strategy and its prognosis in this report.

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We report a case of primary infant iliopsoas abscess. A 3-month-old male infant was brought to our hospital because of groin mass with redness. Blood analysis results indicated inflammation, and abdominal-pelvic computed tomography showed an abscess in the right iliopsoas muscle. After admission, intravenous infusion of antibiotics was started, but antibiotic therapy alone was insufficient. We performed emergency percutaneous abscess drainage with a small incision. Fifteen days after the operation, the patient was discharged from our hospital. Culture of abscess fluid was positive for methicillin-sensitive Staphylococcus aureus. Blood culture on admission was negative, and pelvic magnetic resonance imaging (MRI) on the second day after admission did not show coxitis, spondylitis or iliosacral arthritis. The etiology of iliopsoas abscess in this patient was unknown. The major presenting symptom of infant iliopsoas abscess is groin swelling. Computed tomography (CT) and MRI are necessary for the differential diagnosis of infant groin mass with redness.

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The spontaneous perforation of a choledochal cyst is rare, and the treatment, including both single-stage and two-stage operations, is still controversial. We report the case of choledocal cyst perforation in a 1-year-old girl who was initially diagnosed as having intussusception. Repeated CT revealed a choledochal cyst and increased volume of ascites. Definite diagnosis was performed by laparoscopy; then, laparoscopic external cholecystostomy was performed for biliary drainage. As definitive surgery, excision of the choledochal cyst by Roux-en-Y hepaticojejunostomy was successfully performed 40 days after the drainage operation. We propose that two-stage operation including laparoscopic cholecystostomy is safe and reliable in the treatment of perforated choledochal cyst.

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