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Bone mineral density (BMD) was measured to establish the optimal timing for pectus bar (PB) removal after the Nuss procedure (NP). Twenty-three patients who had PB removal after NP were assessed according to: age at PB insertion/removal, duration of insertion, clinical outcome and BMD. BMD was measured just prior to insertion (in-BMD) and just prior to removal (out-BMD) and %BMD was determined by dividing subject BMD by BMD for age-matched controls. Age at insertion ranged from 4.3–2.7 years and age at removal ranged from 6.3–14.1 years. Duration of insertion ranged from 1.4–3.9 years. There were two cases of recurrence after NP. In these cases, PB insertion occurred at 5 and 4 years, and removal was at 6 and 8 years respectively and both BMD and %BMD were below normal. In the nine cases with no recurrence, PB removal occurred between 6–8 years old; BMD was normal, and %BMD was higher than in the two cases with recurrence. BMD and %BMD would appear to be valuable markers for the timing of PB removal.

We report our experience of shunt infections (SIs) following intestinal cystoplasty (CP) in patients with neurogenic bladder (NB) and hydrocephalus. METHODS: We reviewed 34 patients with NB who underwent intestinal CP between 1984 and 2005. All had ventriculoperitoneal (VP) shunts converted to ventriculopleural (VL) shunts prior to intestinal CP to prevent SIs. SI secondary to intestinal CP was defined as infection within 30 days of intestinal CP. RESULTS: No SIs were reported. Mean age at shunt conversion was 9.5 years (range, 2–25 years), mean length of follow-up after conversion was 12.8 years (range, 2–18 years), and mean period between conversion and CP was 12 days (range, 0–30 days). Pleural effusion (PE) occurred post-conversion in 13/34 subjects (41%). Ten were treated with diuretics for a mean of 12 days, two were treated conservatively, and one required revision to a ventriculoatrial shunt. Delayed PE occurred in two patients at 7 and 18 months, secondary to respiratory infections. In the literature, there are six reports of SIs after intestinal CP in 216 NB patients. Overall, seven of 216 patients (3.2%) had SI. CONCLUSION: VP shunts should be converted to VL shunts prior to intestinal CP because this appears to prevent SIs.

Alpha bungarotoxin (α-BTX) is a neurotoxin isolated from the venom of Bungarus multicinctus that binds specifically to the β-subunits of nicotinic acetylcholine receptors (nAChR) on myotube membranes. The purpose of the present study was to investigate the distribution of α-BTX-sensitive nAChR in Hirschsprung's disease (HD) to understand the histopathological features of HD, especially the increase in acetylcholine esterase (AChE) positive nerve fibres. METHODS: Confocal microscopy was used to study the expression of FITC (fluorescein isothiocyanate)-α- BTX, anti-synaptophysin (A-SY) antibody, and anti-neurofilament (A-NF) antibody to determine the distribution of nAChR and ganglion cell and nerve fibres in colon specimens from five cases of HD and three normal controls. RESULTS: Quantitative assessment of the immunoreactivity of colonic muscle and colonic mucosal epithelium from an aganglionic segment of HD bowel demonstrated markedly increased nAChR compared with colonic muscle and colonic mucosal epithelium from a ganglionic segment of HD bowel and normal bowel (p < 0.0001, respectively), both of which have only a few positive nAChR. In colonic muscle from aganglionic and transitional segments of HD, there were many nAChR around hypertrophic nerve trunks identified by A-NF and A-SY staining. CONCLUSION: We suggest that abnormal expression of nAChR in HD might be implicated in causing gastrointestinal dysmotility because of their localization around hypertrophic nerve trunks.

Teratomas arising from the oral cavity are relatively rare and can cause life-threatening airway obstruction immediately after birth. We report a case of a huge teratoma arising from the soft palate detected prenatally. To save the patient, a caesarean section and ex utero intrapartum treatment (EXIT) were planned at 29 weeks of gestation. However, during EXIT, the patient's cardiopulmonary status deteriorated due to unstable cord blood flow secondary to uterine contractions. EXIT was abandoned, the patient was delivered and an emergency tracheotomy performed. The tumour was successfully excised 4 hours after tracheotomy. The tumour weighed 1,591 g and was 20 × 22 × 12 cm. The patient, a female, weighed 715 g. Histopathology showed Grade II teratoma. The postoperative course was uneventful and she is now 5 years old with no neurological sequelae. EXIT is indicated in patients who have a high risk for airway obstruction immediately after birth. However, if EXIT must be abandoned, as in this case, urgent tracheotomy is mandatory. Planned prevention of airway obstruction at delivery is indispensable for successful outcome and requires excellent coordination among obstetricians, neonatologists, anaesthesiologists and paediatric surgeons.

Magnetic resonance imaging (MRI) is generally an efficient tool for establishing the differential diagnosis of soft tissue tumours. We simultaneously encountered two patients with adipose tumours in the neck. Both had similar clinical features and MRI findings: lobular high intensity tumours on both T1- and T2-weighted images equivalent to subcutaneous fat with septal formation. The provisional diagnosis in both cases was lipoblastoma and both had complete excisions. Histopathological diagnoses, however, were different (lipoblastoma and fibrolipoma), indicating that while MRI is excellent for demonstrating the anatomical detail of soft tissue masses, it is not specific enough to be used alone for differentiating between adipose tumours. In this report, we assess the value of MRI and recommend a more appropriate and patient-friendly protocol for the evaluation for adipose tumours in children.

Percutaneous endoscopic gastrostomy (PEG) placement is associated with considerable complications. We added laparoscopic monitoring to improve outcome. METHODS: Thirty-four patients who had laparoscopy-assisted PEG (LAP-PEG) were reviewed. A 5 mm supraumbilical trocar and two 5 mm working ports were required for LAP-PEG. A needle was placed percutaneously into the stomach under laparoscopic and gastroscopic control. A wire was placed through the needle, encircled with a snare, and the PEG completed. The anterior wall of the stomach was then anchored to the abdominal wall. RESULTS: Thirty-one subjects had cerebral palsy. Age at LAP-PEG ranged from 5 months to 25 years (mean, 8.1 years). Weight ranged from 4.7 kg to 25.9 kg (mean, 12.2 kg). In 23 patients, LAP-PEG was performed with laparoscopic Nissen fundoplication. In 11 patients, it was performed for reasons such as gastric volvulus and nutritional supplementation. Mean operating time was 67 minutes, and all procedures were performed safely without intra- or postoperative complications. CONCLUSION: LAP-PEG is our method of choice for gastrostomy because it allows the first and last parts of a conventional PEG procedure to be well controlled and safe instead of being blind.

Objective: To examine whether Lapprotector™, a protective film and ring device for protectingwounds, can prevent wound infection after open appendectomy. Methods: We performed open appendectomy on 64 patients between 2004 and 2006. In September 2005, we started using Lapprotector™ to protect the site of incision (McBurney's point). Patients were dividedinto two groups as follows: Lapp(−), n =32; Lapp(+), n =32. Patient demographics were not statisticallydifferent and antibiotic protocols were identical. Results: In the Lapp(−) group, the appendix was perforated in seven patients (21.9%) and not perfo-rated in 25 patients (78.1%). In the Lapp(+) group, the appendix was perforated in nine patients (28.1%) and not perforated in 23 patients (71.9%). For perforated cases, incisional wound infection was seen in three out of seven patients (42.9%) in the Lapp(−) group and in no patient (0%) in the Lapp(+) group, a significant difference (p

This is the first report of a primary mucinous cystadenoma (MCA) arising from behind the posterior peritoneum of the descending colon in a paediatric patient. A large intra-abdominal cystic lesion was found incidentally during renal ultrasonography in a 14-year-old girl. Imaging studies showed a 13 × 9 × 15 cm homogeneous cystic lesion with mild contrast enhancement of the wall. The cyst appeared to originate from the retroperitoneum, but was separated from the left kidney, ovary, and pancreas. At laparotomy, there was a cyst behind the posterior peritoneum of the descending colon. The cyst was successfully excised, and histopathology showed MCA. Although primary MCA in the retroperitoneum is extremely rare in children, it should be considered in the differential diagnosis of an intra-abdominal cyst, since it needs to be excised to eliminate the risk of infection, recurrence, and malignancy.

Lymphangioma of the tongue is relatively rare and may cause facial structural deformity. Using a combination of a V-shaped and central resection, we successfully treated a 6-year-old girl who had massive lymphangioma of the tongue. Postoperatively, her tongue was located completely within her mouth with good cosmetic results. Sensory and motor nerves to the tongue appeared to be intact. Her speech was also improved.