Management of metastatic Wilms tumor: an eleven year experience.

During the last 11 years, 101 patients of Wilms tumor were seen. Of these, 13 patients had metastatic disease at onset while 24 patients presented with relapse at a latter date. Seven patients have been cured, all had favourable histology. The risk factors associated with relapse were found to be unfavourable histology, lymph node involvement, age more than 6 years, diffuse spill, capsular and vascular invasion, and aneuploidy. Of the 18 patients who had relapsed, 14 were found to be aneuploid. Judicious use of various therapeutic options like radiotherapy to metastatic sites, second look surgery, resection of pulmonary metastasis and use of cis-platinum, VP-16 and Ifosfamide as "alternative chemotherapy protocols" were used to achieve cure in these advance cases.

Congenital adrenal hyperplasia: experience at intersex clinic, AIIMS.

During 1981-88, 63 cases of female pseudohermaphroditism (FPH) were seen at the Intersex clinic at AIIMS, of whom 34 (54%) were diagnosed as due to congenital adrenal hyperplasia (CAH). Though ambiguity was present at birth in most cases, only one child was brought immediately after birth, while 14 presented after one year. Family history of affected siblings and fetal wastage was present in 10. Salt wasting symptoms were present in 13 (38.2%), evidence of early virilization in 10 (29.4%) and generalised hyperpigmentation in 7 (20.6%). Clitoromegaly was present in 30 children with labial fusion in 10 and scrotalisation of labia in 6. The urogenital opening was single in 25 (73.5%). Buccal smear was positive for sex chromatin in 19. Chromosomal pattern showed 46 XX in 33. Dyselectrolytemia was present in 16 children. Bone age was advanced in all. Adrenal hyperplasia could be documented in 3 on CT scan. All the girls were put on hydrocortisone or prednisolone, and fluodrocortisone was given only to children with salt wasting CAH. Children with CAH are being brought to medical attention much too late and investigative and therapeutic facilities are grossly inadequate. There is a need to educate primary care physicians for early case detection and provide minimum diagnostic and therapeutic facilities in regional centres.

Mixed gonadal dysgenesis and dysgenetic male pseudohermaphroditism--a critical analysis.

A 10 year prospective study of 14 patients with mixed gonadal dysgenesis (MGD) and six patients with dysgenetic male pseudohermaphroditism (DMP) is reported. All of them had internal mullerian structures, along with unilateral or bilateral dysgenetic testes, ambiguous external genitalia. Twelve had been brought up as male, nine of whom had a unilateral descended testis. Eight had been reared as females, as they had bilaterally undescended gonads, and ambiguous genitalia. Clinical examination, retrograde genito-urethrography and cytogenetic studies suggested the diagnosis in 16 patients, while four were diagnosed on inguino-abdominal exploration for undescended testis. This report delineates more clearly the clinical profile of these orders. All the patients reared as male were assigned the male gender following abdominal gonadectomy, retention of scrotal testis and male genitoplasty. The eight patients who were reared as females underwent bilateral salpingo-gonadectomy and female genitoplasty. This management differs from the usual recommendation that all such children should be reared as females. Ten patients (50%) had maternal history of previous abortion/stillbirth, or drug intake in the first trimester of pregnancy suggesting a role of these factors in the etiology. All cases of DMP had a 46,XY karyotype, while eight of 14 cases of MGD had mosaicism with 45X/46,XY cell lines in blood or gonadal cultures. The clinicopathological features of patients of MGD and DMP were similar. It is suggested that these two disorders represent different spectra of the same disorder. A unifying concept of etiopathogenesis is proposed.

Role of ultrasound guided percutaneous antegrade pyelography (USPCAP) in the diagnosis of obstructive uropathy.

Ultrasound guided percutaneous antegrade pyelography (USPCAP) was performed in 6 cases between 1 month and 8 years age, presenting with obstructive uropathy. The procedure in this age group was characterized by (i) ease of performance without sedation in the neonates and with sedation in older children, and (ii) use of smaller needles and catheters. The procedure enabled delineation of the intimate anatomy of complicated urological abnormalities such as obstructed duplex system, primary megaureters, posterior urethral valves, prune belly syndrome, obstructive hydronephrosis and vesicoureteric reflux. Percutaneous renal puncture may be established rapidly using ultrasonic guidance in severely ill pediatric patients with obstructive uropathy. B-mode ultrasonography has proved to be rapid, accurate and free of radiation hazards, making it applicable to children with little or no sedation. Guided by ultrasound, percutaneous antegrade pyelography should be considered in the few, selected children with obstructive uropathy when the diagnosis is critical for management and difficult with the usual imaging procedures.

Hormonal therapy in undescended testes.

A clinical study trial of HCG therapy in 78 selected prepubertal cryptorchids (40 unilateral and 38 bilateral) having a total of 116 undescended gonads revealed that complete descent occurred in 12.5% gonads (13% in unilateral and 12% in bilateral group). Good response was observed in only those gonads which were located in the inguinal canal and at the external ring, irrespective of it being right or left sided gonad. In patients with impalpable testes, though there was no complete descent, yet partial descent was noted in 18% (21/116 gonads). The response was better in the older children.

Spina bifida occulta: radiographic and operative correlation.

The clinical and myelographic evaluation in 24 children with spina bifida occulta has been correlated with the operative findings. Myelograms were done using Myodil in 11 patients and Metrizamide in 13 patients. The diagnostic quality of myelograms done with Metrizamide was comparatively better. Myodil myelography failed to demonstrate diastematomyelia in one case and a dural sac in 3 cases of lipomeningomyelocele, whereas, Metrizamide failed to demonstrate a sac in one case of lipomeningomyelocele only. Worsening of the neurological status was observed in only 3 cases in whom myelography was done with Myodil. The radiographic and operative correlation has been discussed.

Use of appendix in biliary atresia.

Antiperistaltic appendiceal conduit based on its vascular pedicle was used as hepaticoporto-appendico-jejunostomy (HAJ) in 9 infants with advanced stage of biliary atresia. Operative procedure was simple and less time consuming. The postoperative cholangitis was conspicuously absent, possibly due to the role played by the presence of lymphoid follicles in the wall of the appendix. Our early experience with this technique shows that antiperistaltic appendiceal conduit works quite satisfactorily. The procedure has been successfully utilised to reduce the problems contributing post-operative morbidity and mortality in cases with the advanced disease. The HAJ procedure provides a conduit for bilioenteric drainage, that closely resembles to normal anatomy.