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Separation of 3-year-old brachio-thoraco-omphalo-ishiopagus bipus conjoined twin girls is reported. Detailed preoperative assessment and a multidisciplinary approach were essential prerequistes to surgery. The difficulties of managing the extensive body wall defect resulting from separation are discussed. Despite the use of subcutaneous and intraperitoneal tissue expanders, a large area of prosthetic material was required to reconstruct the abdominal wall. Skin cover was achieved using a combination of meshed allogeneic skin, homograft split skin, and autologous cultured keratinocytes. One twin died in the early postoperative period, but the other has recovered well and is awaiting further rehabilitative treatment.

Purpose Maintaining adequate renal function without compromising cure in children with bilateral Wilms' tumor is surgically demanding and challenging. The aim of this study was to assess renal function and outcome in children treated in one institution for bilateral Wilms' disease by renal salvage procedures. Methods This study is a retrospective review of all patients with histologically proven bilateral nephroblastoma who underwent a renal salvage procedure between November 1973 and June 2002. The median follow-up time was 52 months (range, 8 to 326 months). Results Twenty-three patients were included in the study. The median age at diagnosis was 19 months (range, 5 to 65 months). Patients who presented before 1982 (n = 5) were treated surgically first followed by chemotherapy. The remainder (n = 18) received initial chemotherapy before the operation. Of the 46 kidneys, 18 had a nephrectomy. The remaining 28 underwent a renal salvage procedure. At follow-up, 19 patients had good renal function, 2 had satisfactory function, and 2 had renal failure. Seventeen of the 23 children are alive and tumor free at follow-up (74%). Four children died of distant metastases and 2 of renal failure. Local recurrence in the salvaged kidney was detected in 1 patient 12 years after surgery after the commencement of immunosuppression for renal transplantation. Conclusions These results confirm that renal salvage procedures, in combination with chemotherapy, are a safe and effective way of treating children with bilateral Wilms' disease. In addition, renal salvage surgery can maintain satisfactory renal function in the majority of these patients without an increased risk of local recurrence. When transplantation is required, the remaining native kidney should be removed to prevent tumor recurrence.

Background/purpose The aim of this study was to review the management of the gastrointestinal tract in cloacal exstrophy and to assess the effect of spinal dysraphism on nutritional outcome. Methods Twenty-two patients with cloacal exstrophy were reviewed retrospectively. Gastrointestinal and spinal anomalies were documented. The need for nutritional supplementation was recorded. Weights at 1 and 5 years of age were used to assess growth in childhood. Results Ten patients were treated initially with ileostomy, 7 with colostomy, and 3 without stoma. Two patients died before surgical reconstruction. Total parenteral nutrition (TPN) was utilized in 8 infants for a median of 10 days (range, 5 to 200). Three patients had complications with colostomies requiring conversion to ileostomy. Patients with an ileostomy required more nutritional supplementation compared with patients with a colostomy (50% v 28%). High-output stoma losses were more common in patients with an ileostomy (40% v 14%). Fourteen patients (67%) with spinal dysraphism had a higher incidence of failure to thrive in the first year of life (69% v 26%) and multiple episodes of enteritis (40% v 25%). Two neonates with duodenal atresia and small bowel deletion died within the first month of life. One patient with short bowel syndrome died of TPN-associated liver disease at 6 months of age. There were no other deaths. Conclusions The gastrointestinal tract contributes significantly to the morbidity and mortality in cloacal exstrophy. Nutritional supplementation is more frequently required in patients with an ileostomy. Stoma complications were higher in those with a colostomy. Morbidity is high in patients with spinal dysraphism.

Purpose: Demographic data, clinical presentation, associated abnormalities, and radiologic findings were evaluated to outline diagnostic criteria that may lead to the diagnosis of xanthogranulomatous pyelonephritis in children. Methods: Eleven boys and 8 girls with a mean age of 3.4 ± 1.7 years were classified into obstructive xanthogranulomatous pyelonephritis (n = 13), which was associated with nephrolithiasis and nonobstructive (n = 6), which mimicked Wilms' tumor. Results: Twelve children with obstructive diffuse involvement of the renal parenchyma, 1 with left-sided obstructive focal involvement in a horseshoe kidney (group 1), and 6 with nonobstructive diffuse xanthogranulomatous pyelonephritis (group 2) showed a male to female ratio of 1.2:1 and 2:1, respectively. Mean age was 4.1 ± 1.2 years in group 1 versus 1.8 ± 1.5 years in group 2; P =.001. The common features were renal mass, hematuria, and anemia (100%; P =.07) and leucocytosis (77% v 83%; P =.097). Main differences between the 2 groups were acute inflammatory syndrome (0 v 33%; P =.01), recurrent urinary infection (54% v 17%; P =.05), isolation of Proteus mirabilis as a pathogen (69% v 0; P =.001), and renal stones (100% v 0; P =.001). Preoperative diagnosis was accurate in all 13 (100%) children with obstructive xanthogranulomatous pyelonephritis. Radiologic features that were not consistent with Wilms' tumor in group 2 were absence of sharp definition and encapsulation of the mass, ill-defined margins with inflammatory infiltration of the perinephric fat and focal inflammatory tissue destruction. Nephrectomy was technically difficult because of extensive adhesions to the retroperitoneum, psoas muscle, and surrounding structures in both groups. Conclusions: Xanthogranulomatous pyelonephritis must be considered in the differential diagnosis of a child presenting with a renal mass, anemia, and elevated inflammatory markers. Treatment by nephrectomy is curative. J Pediatr Surg 36:598-601. Copyright © 2001 by W.B. Saunders Company.

Continent, catheterizable enterostomies (Malone stomas) have been formed in 25 patients. A reversed appendicocecostomy was used for the majority, although experience has led to modifications of this procedure. Antegrade enemas are regularly administered via these stomas in the treatment of fecal incontinence and intractable constipation. There have been only two important surgical complications, and the technique has proved to be highly successful. Follow-up of the patients has shown that individual requirements can be met with variations in the administration of the enemas.

Retroiliac ureters have been found in association with a variety of urogenital abnormalities but the iliac artery usually retains a retroperitoneal position. We report 7 children with various urogenital abnormalities (cloacal malformation 4, bladder agenesis 1, urogenital sinus 1, neuropathic bladder 1) in whom the lower aorta and iliac arteries were so distorted as to pose a significant operative hazard. In all cases the distal aorta and/or iliac arteries lay within the peritoneal cavity, often anterior to the bladder. In 3 cases the aorta was fixed by a short ligament to the umbilicus, and in 3 patients one of the iliac arteries ran across the pelvis behind the pubic bones before entering the leg. In one patient an anomalous iliac artery was inadvertently divided during reconstruction of a cloaca; when recognized, the vessel was rejoined. Recognition of an association between urogenital or cloacal malformations and a major vascular anomaly is of great importance to reconstructive surgeons who, if not forewarned, could unintentionally compromise the arterial supply to one or both lower limbs.

Between 1982 and 1988, 36 children with advanced Wilms' tumour underwent percutaneous trucut needle biopsy followed by chemotherapy before definitive surgery. Nephrectomy was performed after a median of 14 weeks of chemotherapy. Substantial reduction in tumour bulk was achieved in 94% of patients. Biopsy morbidity was low and complete concordance between the histological assessment of the tumour in the biopsy specimen and at subsequent nephrectomy was confirmed in 26 of 28 (93%) patients. The overall clinical value of trucut biopsy was 83% (30/36 patients). Survival rates in this high-risk group were comparable to those of children with less advanced disease. Chemotherapy may be the primary treatment of choice for patients with Wilms' tumour. Percutaneous biopsy allows definition of histology in most patients without increasing morbidity.

Twenty-seven patients with sacrococcygeal teratoma were followed for a mean period of 5 years (range, 2 to 12 years). Eleven (41%) had some form of functional impairment. Fecal and/or urinary incontinence was present in nine children and two had weakness of the lower limbs secondary to sciatic nerve palsy. Tumors with large intrapelvic extensions requiring an abdominoperineal approach for resection were associated with a higher incidence of functional sequelae (67%). It is recommended that patients with sacrococcygeal teratoma (particularly those with a presacral extension) be closely monitored for functional deficits so that early treatment can be instituted.

Agenesis of the penis is an extremely rare anomaly, occurring only once in 30 million births. This low incidence is responsible for the limited experience with this anomaly. There are only about 70 published cases, most reports being of one or two patients. This complex malformation requires urgent assessment at birth for several reasons: Female sex assignment is required early. Gonadectomy should be performed in the first few days of life to prevent male gender sex marking from the testosterone surge occurring between the tenth and the 60th day of life. Early gonadectomy and genital reconstruction helps the family to accept the child's altered gender and to reduce psychological problems.

The possibilities for treating children with renal stone disease by extracorporeal shock wave lithotripsy (ESWL) and percutaneous nephrolithotomy (PCNL) have not been widely explored. We report ten children, aged between 5 and 16 years, treated by PCNL, and six children, aged between 6 and 15 years, treated by ESWL alone. A 16-year-old spina bifida child with a staghorn calculus was treated electively by a combination of the two methods. Hospital stay was 3 to 8 days for treatment by PCNL or ESWL, and was only 11 days for the combination treatment. Complete stone clearance was achieved in 12 children followed for 3 months. No significant complications occurred. ESWL and PCNL are suitable methods for the management of renal calculi in children.

Our experience with single stage primary hypospadias repair in 96 patients over a 12-month period is reviewed. The ages ranged from 7 months to 14 years with an average of 3.5 years. Seventeen patients were 18 months or younger at the time of operation. Meatal advancement and glanuloplasty (MAGPI) was performed in 46 patients mainly with coronal and glanular hypospadias. There was a fistula rate of 4%. The modified Mathieu and Mustarde operations were used for subcoronal and distal hypospadias. The transverse preputial island flap technique was found to be very versatile and was employed in 33 patients. The fistula rate was 18% and proximal urethral stricture rate 9%. These complication rates are considered quite acceptable in busy centers where surgeons in training are involved in these operations. It is concluded that by mastering a relatively limited number of techniques, single stage hypospadias reconstruction can be undertaken in almost all cases at the optimal time.

Between 1964 and 1987, 12 patients with mesoblastic nephroma were treated. The BP was measured preoperatively in five patients, four of whom were hypertensive. Following nephrectomy, the BP returned to normal. Plasma renin levels were measured in one patient; they were markedly elevated pre-operatively, but returned to normal following tumour excision. Immunoreactive renin staining, using a polyclonal antibody to human renin, was performed in the 12 patients. Staining was positive in ten patients. The most intense staining was noted in the areas of cortex entrapped within the tumour, and in perivascular spaces not associated with entrapped cortex. These findings suggest that hypertension secondary to increased tumour associated renin production is a feature of congenital mesoblastic nephroma.