Misleading leads: Wilms tumor with a metachronous lesion appearing in the remaining kidney.

Medullary dysplasia may be present in the kidneys of children with the Beckwith-Wiedemann Syndrome (BWS). This is usually visible only at the microscopic level, but superimposed pyelonephritis in a child with the BWS led to gross changes that produced a filling defect on CT scanning of the remaining kidney. The finding could have been misinterpreted as a metachronous wilms tumor.

Transanal mucosectomy: a modification of the Soave procedure for Hirschsprung's disease.

In the Soave procedure for Hirschsprung's disease, the mucosectomy of the aganglionic segment of colon is performed transabdominally. The authors describe an innovative modification of the standard Soave procedure, in which a transanal mucosectomy is performed as the abdominal incision is made. The transanal mucosectomy technique was compared with the conventional approach. From 1974 to 1993, 51 patients underwent a Soave procedure for Hirschsprung's disease. Twenty-six (51%) had the standard Soave procedure (STD) (1974-1984), and 25 (49%) had the modified procedure (MOD) (1984-1994). In the latter, the entire mucosal dissection was performed through a transanal approach. Surgical data were accumulated by database analysis. Each case was entered into a database at the time of operation, and follow-up data were obtained through mail-in questionnaires or personal communication. The two groups were comparable with respect to number, gender distribution, and average age at the time of operation. There were two superficial wound infections in the STD group and two superficial wound infections and one death (secondary to cardiac arrest in a patient with trisomy 21 and a severe congenital cardiac anomaly) in the MOD group. The average postoperative stay for the STD group was 10.8 days; that for the MOD group was 6.8 days (t test: P < .0001). The operating time was shorter for the MOD procedure. Follow-up data were available for 25 (96.2%) patients in the STD group and 25 (100%) in the MOD group. The two groups were comparable in all respects except for the average length of follow-up, which was longer for the STD group. No patients had, urinary incontinence or sexual dysfunction (when applicable). Overall, patients/parents in both groups rated the outcome as "good." This modification of the Soave technique for Hirschsprung's disease is both safe and effective. Compared with the standard procedure, it is associated with shorter operating time (because the transanal mucosectomy is performed as the abdomen is opened) and a shorter stay postoperatively. The results have been excellent.

A complete ileo-tumor-colic fistula in a child with Burkitt's lymphoma.

A boy with abdominal Burkitt's lymphoma developed rectal bleeding, abdominal distension, pain, and fever three weeks after diagnosis. Imaging studies revealed a necrotic tumor mass allowing a fistulous pathway from the ileum to the proximal colon. A laparotomy was performed, with resection of the large necrotic tumor. The child recovered, and has had no further evidence of Burkitt's disease. The radiological evaluation performed in this case ensured proper medical management and surgical intervention during a life-threatening event. The possibility of bowel perforation as a complication of therapy for abdominal lymphoma must be recognized.

Paratesticular malignant mesothelioma associated with abdominoscrotal hydrocele.

This is the report of 14-year-old boy with a malignant mesothelioma of the tunica vaginalis contained in an abdominoscrotal hydrocele. A review of the literature shows that this aggressive tumor is very rare and has been reported only in adults.

Congenital central hypoventilation syndrome. A report of successful experience with bilateral diaphragmatic pacing.

Bilateral diaphragmatic pacing was successfully performed in an 18-month-old child with congenital central hypoventilation syndrome (Ondine's curse) as an alternative to long-term mechanical ventilation. Subsequent complications were related more to cor pulmonale and tracheostomy care than to the pacing itself.