K. E. Mammen

Results of Wilms’ tumour management in two tertiary-care hospitals in Asia

In the period 1985–1995, 87 children underwent surgery for Wilms’ tumour; 16 were lost to follow-up. Of the remaining children, 27 presented with stage I disease, 11 with stage II, 12 with stage III, 14 with stage IV, and 6 with stage V. One child was not staged. The histology was favourable Wilms’ tumour in 44, anaplastic in 12, unclassified in 8, clear-cell sarcoma in 4, and rhabdoid tumour in 3. Although a total nephrectomy was generally performed, partial renal surgery was performed for 6 bilateral and 4 unilateral tumours, the latter including 2 fused kidneys. Preoperative chemotherapy was employed with benefit in massive tumours, tumour in fused kidneys, bilateral tumours, and preoperatively diagnosed inferior vena caval tumour thrombi. Postoperative chemotherapy, employed in all cases, consisted of actinomycin D and vincristine with the addition of adriamycin in anaplastic and advanced-stage tumours. Ten children underwent second-line chemotherapy for disease unresponsive to the above management, but only 1 of these is currently free of disease. Postoperative tumour-bed radiotherapy, used in selected cases, prevented local recurrence in stage I and II disease. However, 20% of stage I and II patients not receiving radiotherapy developed tumour-bed recurrence. Twenty-three children have died and 5 with advanced disease and incomplete follow-up are presumed to be dead. Nine children are currently on treatment; 34 have successfully completed treatment, the disease-free survival in stages I–V being 81%, 75%, 42%, 14%, and 50%, respectively. Overall disease-free survival was 69% for Wilms’ tumour of favourable histology and 50% for anaplastic tumours. The 3 patients with rhabdoid tumours and 3 of 4 with clear-cell sarcomas have died. Wilms’ tumour management in the developing world is compromised by cases lost to follow-up and late presentation with massive tumours and advanced stage. Preoperative chemotherapy is advantageous in a number of cases, and postoperative radiotherapy should be deployed more frequently.

Gender assignment in male pseudohermaphroditism: an Indian perspective

Parents of 21 out of 30 children with male pseudohermaphroditism (MPH) opted for a male upbringing for their child and appropriate management was instituted. The phallic size in these 21 children varied from adequate to microphallus, although at least one externally visible testis was present in all cases. Nine children who were assigned a female gender were being reared as girls at home prior to medical consultation and 7 of them had a female-type vulvar outlet. This predominance of male gender assignment in MPH is in striking contrast to the Western experience. The reasons for this difference in India are discussed in the light of the Indian social and economic background.

Bilateral high loop ureterostomy in the primary management of posterior urethral valves in a developing country

Abstract Of 200 consecutive cases of posterior urethral valves (PUV) managed in a single institution, 26 underwent bilateral high loop ureterostomy (BU) as their first operative intervention. Indications for BU were persistently raised serum creatinine levels after bladder drainage, severe urosepsis, and urinary ascites, especially in a neonate or infant. After a median period of 1 year of diversion, the serum creatinine fell from a median value of 2.5 to 0.6 mg%. Twelve cases have been fully undiverted without deleterious effects (median serum creatinine level 0.5 mg%). Three patients died. Of the 14 renal units with documented persistent vesicoureteric reflux at the time of undiversion, only 1 has been reimplanted. One child has undergone bladder augmentation.

Pneumonostomy in the surgical management of bilateral hydatid cysts of the lung.

Abstract During the period 1994–1998, three patients with bilateral hydatid cysts of the lung (HCL) underwent operative removal of the cysts. In three of the six lungs operated upon the conventional technique was used: after removal of the cyst and suture closure of bronchial leaks, the chest was closed with an intercostal drainage tube. Two of these patients developed bronchopleural fistulae requiring rethoracotomy and prolonged hospital stays. The other three lungs were operated upon using the pneumonostomy technique: after excision of the cyst a separate catheter is fixed within the residual lung cavity and brought out through the adjacent chest wall, effectively marsupialising the residual cavity to the atmosphere. All these patients had an uneventful postoperative recovery. We conclude that the pneumonostomy technique is a very useful method of treating HCL surgically, especially when the cysts are bilateral and complicated.

Isolated splenic hemangioma presenting with bleeding into serous body cavities

We present two children with massive bleeding into the serous body cavities accompanied by intractable consumption coagulopathy. One had a large spleen palpable at admission, the other developed progressive splenomegaly while in hospital. Neither child had any external evidence of angiomatous lesions. A splenic hemangioma was suspected clinically and on abdominal ultrasound; the diagnosis was confirmed at laparotomy. Splenectomy resulted in a prompt cure in both cases.

Giant inflammatory pseudotumor of the lung masquerading as pleural effusion

We report a child with a mass lesion filling the entire left chest cavity and presenting with clinical features of a left-sided pleural effusion. The mass was excised with dramatic relief of symptoms. Histopathologic examination revealed an inflammatory pseudotumour.

Ectopic vasal insertion into the posterior urethra: Report of two cases

Two cases of ectopic vas deferens insertion into the posterior urethra are reported. A search of the English literature revealed only two such cases previously reported. We advocate routine cystourethroscopu when investigating childhood epididymitis.

Neonatal sacrococcygeal teratoma communicating with the rectum

A unique case of benign sacrococcygeal teratoma communicating with the rectal lumen at birth is reported. A large air-fluid level in the teratoma was seen on the plain X-ray film.

Aplasia cutis congenita: new evidence supporting the amniotic band theory of origin

We present two cases of aplasia cutis congenita (ACC). The scalp alone was involved in the first baby, who died from massive sagittal sinus hemorrhage. The second neonate presented with circumferential constrictive trunk ulcers and healed scars on the thighs, axillae, and temples in addition to the scalp lesion. This baby was successfully treated with local flap cover of the scalp and trunk ulcers. A biopsy from the edge of the scalp ulcer revealed a dermoid cyst. This provides new evidence in support of the amniotic band theory of origin of ACC.

Single-system ureteral ectopia

We present our experience of six children with bilateral single-system ureteral ectopia and two children with unilateral single ectopic ureters and contralateral renal agenesis. The wide spectrum of malformations indicates an embryological insult in addition to anomalous ureteric buds. There appears to be an element of antenatal ureteral obstruction affecting some renal units and sparing ectopic contralateral units. Ureteric reimplantation into the embryologically diverted bladder, without additional bladder neck surgery or augmentation, resulted in a satisfactory outcome in the majority of cases. Reimplantation techniques may require modification when the bladder is very small, and a simple ureteric nipple has proven effective in preventing reflux.