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SummaryColonic volvulus is a rare disease in children. Delayed diagnosis of the condition can often be fatal, especially in pediatric patients with mental retardation. We herein present the case of a female pediatric patient with colonic volvulus, prune belly syndrome, and mental retardation. Preoperative CT scans showed the characteristic signs of this disease. The volvulus occurred in the proximal colon of the colostomy. The release of the colonic volvulus and reconstruction of the colostomy were performed without the resection of the ischemic colon. The postoperative clinical course was uneventful.

This report presents the case of a 6-day-old male with cystic biliary atresia (CBA), and the cyst was detected antenatally. Antenatal ultrasonography suggested the possibility of CBA or a choledochal cyst at 16 weeks' gestation. However, the cyst disappeared during the later gestational period. The cyst was detected again by preoperative imaging. Surgical cholangiography at 30 days of age confirmed CBA, but the common hepatic duct (CHD) was extremely narrow. The histopathological findings revealed the partial obstruction of CHD. These findings suggest that correctable CBA (I cyst) may change into uncorrectable CBA (IIId).

This report describes the case of an ileal perforation secondary to clamping of the exomphalos minor in 3-day-old girl. Eighteen accidental clamping or cutting cases were found in the published work. It is thought that this defect resulted from either the clamping, ligation or cutting of the bowel in a small unrecognized omphalocele. These complications are extremely rare, but should be held in mind when performing an umbilical ligation.

We herein report a case of type I congenital cystic adenomatoid malformation of the lung (CCAML) with non-immune hydrops fetalis (NIHF), a mediastinal shift and polyhydramnios diagnosed at 24 weeks' gestation by ultrasonography. The fetus was treated with a cyst-amniotic shunt at 29 weeks' gestation. Following a postnatal whole resection of the right lung, postpneumonectomy syndrome appeared and, as a result, the infant died 13 months after delivery due to respiratory failure. Only 19 cases demonstrating CCAML associated with NIHF have been reported previously in Japan. Four cases showed a spontaneous resolution of NIHF, while 5 cases with type I CCAML, which all underwent fetal intervention, demonstrated an excellent outcome.

Purpose: The aim of this study was to determine if there is latent manganese toxicity in patients with biliary atresia. Methods: Fifteen children with biliary atresia were examined postoperatively with regard to whole-blood manganese levels using brain magnetic resonance imaging (MRI) and I-123 iodoamphetamine (IMP) per rectal portal scintigraphy. Results: Nine (60%) of the 15 had high whole-blood manganese levels (mean, 4.1 μg/dL; range, 1.2 to 9.6; normal, 0.5 to 2.5), and these 9 had hyperintense globus pallidus on T1-weighted images, with no corresponding signal change in T2 sequences. I-123 IMP per rectal portal scintigraphy was done for 13 patients to evaluate portosystemic shunt flow. 12 (92%) of these patients had an increased flow. Mean shunt ratio was estimated to be 41% (range, 0.6 to 98; normal, Conclusions: Some patients with biliary atresia in the postoperative period have manganese deposits in globus pallidus on T1-weighted images and high whole-blood manganese levels, possibly caused by increased portsystemic shunt, and a latent or subclinical encephalopathy is also present. J Pediatr Surg 35:450-453. Copyright © 2000 by W.B. Saunders Company.

An 8-year-old girl was introduced to our department due to the presence of a left painless submandibular mass. The mass had been initially noticed at 7 years of age. Preoperative imaging showed the mass to have originated from the left submandibular gland. The mass was removed with a part of submandibular gland attached to it. The pathologic findings showed the mass to be pleomorphic adenoma without any malignant components. The postoperative clinical course was uneventful. During the 1-year follow-up period, no recurrence was noticed. In addition to the clinical report of our case, we reviewed the pertinent Japanese literature to clarify the clinical features of this disease in children.

Objective Wound dehiscence caused by surgical site infection (SSI) presents a complicated problem. Negative pressure wound therapy (NPWT) was developed to treat wound dehiscence. Nutritional treatment using arginine has also been recently shown to be effective for the treatment of pressure ulcers. Therefore, wound complications due to SSI were treated using NPWT combined with nutritional therapy with an arginine-rich supplement (ARS). Methods Six pediatric patients with wound dehiscence due to SSI received this combined therapy. Results The average age of the patients was 12.2 mo. The operations that these patients underwent included laryngotracheal separation, radical operation for spinal bifida, gastrostomy, colostomy, anorectoplasty, and tumor extirpation. A local wound infection induced wound dehiscence in all patients. Therefore, NPWT was introduced with an enteral administration of ARS. All wounds completely healed within 1 mo after the introduction of this combined therapy without any other complications from the NPWT or ARS. A follow-up study at 6 mo after this therapy was completed showed no complications associated with the wounds. Conclusion This combination therapy using NPWT and ARS administration was effective in inducing early healing of infected wound complications after surgery.

Infants, especially extremely low birth weight infants (ELBWIs, birth weight1,000 g) continue to have a high mortality after gastrointestinal (GI) perforation. In Japan, the overall mortality rate for neonates under 30 days having GI perforation was 31.6% in 2003. From 1974 to 2003, 34 cases of GI perforation in neonates were treated surgically in Fukuoka University Hospital. The overall mortality rate was 50% (17 of 34). Etiologies included necrotizing enterocolitis (NEC) (35.3%), meconium peritonitis (25%), idiopathic (25%), and gastric perforation (11.8%). The present series was divided into four groups: survival and non-survival neonates of the early (1974 to 1997) and recent (1998 to 2004) periods. Several prognostic factors of neonatal GI perforation were compared between several groups. The gestational week (GW) at birth, birth weight (BW) and weight at operation were significantly lower for non-surviving neonates in the recent period compared with the other three groups. Although a real improvement in surgical outcome was noted with improved neonatal intensive care management, the mortality rate was still high, especially in extremely premature cases under both 1,000 g and 29 GWs. The vast majority of these extremely premature babies thus comprised the NEC patients. It is therefore necessary to substantially improve the medical treatment level for such premature babies.

We herein present a megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) case followed by ultrasound (US) examinations before birth. During the prenatal US examination of a 34-year-old woman, an enlarged bladder with bilateral hydronephrosis and hydroureter of the fetus were detected. The amniotic fluid was normal in the second trimester but polyhydramnios was observed in the third trimester. A female baby was born by cesarian section weighing 2632 g. Imaging studies detected an enlarged bladder with bilateral hydronephrosis, hydroureter and microcolon. Laparotomy on the 9th day confirmed a short small bowel with caliber change and the existence of ganglion cells and plexus of the nerve in the intestine. These findings correlated with the previously reported characteristics of MMIHS. An enlarged bladder in the second trimester and polyhydramnios in the third trimester have been reported in many cases of MMIHS. These findings may thus help to accurately diagnose MMIHS before birth.

This report documents a new endoscopic management modality for congenital membranous stenosis in the third portion of the duodenum. Standard approaches to duodenal stenosis in newborns include a laparotomy with an enteroenterostomy, bypassing the obstruction, or a duodenoduodenostomy with excision. We successfully developed a modification of the endoscopic treatment modality for congenital duodenal diaphragm.

A 12-year-old girl had the hair on the right side of her head pulled during a quarrel, after which a subgaleal hematoma (SGH) developed over her right cranium. The subcutaneous swelling progressed to the forehead, and a marked exophthalmos then developed on the left side. The bilateral, liquefied SGH was removed surgically, and two drainage catheters connected to a vacuum-drain pump were introduced. After the surgery, the SGH disappeared. The liquefied hematoma was aspirated by puncturing the superolateral portion of the left orbit. Thereafter, the left exophthalmos rapidly disappeared. A chemical analysis of the SGH revealed that it contained extremely low levels of fibrinogen and platelets, and high levels of fibrinogen and fibrin degradation products, suggesting that secondary fibrinolysis had occurred in the subgaleal space. Subgaleal hematomas are usually treated conservatively. However, closed observation is necessary, and if increased expansion is seen, aspiration with a closed drainage system should be considered.

We report herein the case of a 2-year-old girl with short bowel syndrome who developed chest pain 2 weeks after a totally implantable central venous access catheter had been surgically placed to facilitate total parenteral nutrition. A lateral chest X-ray and contrast flow study subsequently revealed that the catheter tip had been inserted into the azygous vein. Although this complication is very rare, it may be encountered more commonly with the increasing use of this type of venous access. Thus, we believe that a lateral chest X-ray should be routinely performed on all patients following catheter insertion to check for possible malposition of the tip in the azygous vein.