ABSTRACT: A case of sirenomelic baby of 34 weeks gestation is reported. The baby was stillborn and was having fused lower extremities. There were associated multiple congenital anomolies. Autopsy revealed multiple bowel atresias. Review of literature is discussed.

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ABSTRACT: The posterior urethral valve is the commonest cause of bladder outlet obstruction in children. With the improvement of primary health care system and due to the widespread use of ultrasonography during antenatal checkup the incidence of early diagnosis of posterior urethral valve has increased. The ultrasonography, estimation of blood biochemistry and micturating cystourethrography are the primary modes of investigation in the postnatal period. After an initial period of catheterization and vasodilatation by administration of fluid and electrolytes, endoscopic rsection of valve is the first choice of treatment. The technique of endoscopic valve resection has been standardized. The neonatal resectoscope (9F) cannot be used in many premature or small for date babies. Although major improvement has been made, further miniaturization of the neonatal resectoscope is the need of the day. Vesicostomy is the preferred form of temporary urinary diversion, without any long term morbidity. Supravesical diversion has limited but definite role. There are very few long term follow up studies of posterior urethral valve managed on a uniform protocol. Our knowledge about the pathophysiology of the patient with posterior urethral valves, ability to perform video urodynamics and apply it to plan the treatment has improved. The ongoing prospective studies will define the exact role of invasive urodynamics and the scope of secondary surgical intervention.

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ABSTRACT: Children with posterior urethral valve (PUV) obstruction constitute a significant number of hospital admissions in our Pediatric Surgery Unit. We present a single institution experience in the management of children with PUV. Over the past three years (2000-2002) 90 patients with PUV were treated in our hospital. Majority of them presented in the first year of life. Seventy one percent (64/90) had renal failure at presentation. Voiding cystourethrography (VCUG) was the mainstay of the diagnosis. Associated vesicoureteric reflux (VUR was present in (51/90) 56 percent of cases, and was mostly unilateral (90percent). After initial per urethral catheter drainage, 75 percent showed an improvement in the renal function. Primary urethral fulguration of valve was done mostly in older children whereas neonates and non-responders to catheter drainage underwent vesicostomy. The high incidence of diversion in our series was due to non-availability of adequate size resectoscope. Short-term follow up showed adequate growth and nutrition in children on vesicostomy with recovery of renal function. However, a significant number of renal units (90 percent unilateral and 10 percent bilateral) showed poor function with functional obstruction in DTPA scan. This report highlights the treatment protocol and problems faced in the management of children with PUV in a Paediatric Surgical Unit of a medical college hospital set-up.

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ABSTRACT: Fournier's gangrene, an anerobic necrotizing fasciitis, is a serious pathologic entity primarily affecting adults. Herein we report three cases of Fournier's gangrene, all under the age of one month and presenting almost simultaneously. These were treated by broad spectrum antibiotics and repeated surgical debridement. All survived with no complications. Polymicrobial infection was held responsible for the gangrene. Stress was given on vigorous treatment which included hemodynamic stabilization, para\enteral broad spectrum antibiotics and surgical debridement. Though measures like institution of hyperbaric oxygen and application of various topical agents have been described in the literature, the cases in the present series responded well to combination of antibiotics and surgical debridement.

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ABSTRACT: Antenatal diagnosis and assessment has improved the understanding of the natural history of posterior urethral valves from inception to post natal life. More babies receive medical and surgical care in early neonatal life than ever before. However, it is not yet clear whether it reduces the incidence of end stage renal failure secondary to posterior urethral valves. The exact form and role of fetal intervention remains to be defined. It is certain that open fetal surgery has no role today. Vesico amniotic shunt may well be replaced by fetal endoscopy, although the indications, timing, and technique need to be further standardised. Once again whether it will indeed improve the outcome is terms of postnatal bladder dynamics and end stage renal failure remains to be seen. In the meanwhile, the diagnosis, assessment and fetal intervention must strictly remain limited to dedicated teams with experience and commitment to improving the outcome of such babies.

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ABSTRACT: Purpose: To observe the clinical, biochemical and imaging patterns of Posterior Urethral Valve (PUV) patients and how they change after initial treatment and during follow-up. Methods: 79 patients of posterior urethral valves were studied between January, 2000 and June, 2002. Their age ranged from 1 day to 11 years. Presenting features of these patients were studied clinically. Biochemical studies as blood urea, serum creatinine, electrolytes and imaging studies as Micturating Cystourethrogram (MCU), Ultrasonography (USG) and renal isotope scan were performed. Result: 55 percent of the patients presented under one year of age. The most common presentation was poor urinary stream followed by dribbling of urine. 37 percent of the patients presented with palpable kidneys, 62 percent with recurrent Urinary tract infection (UTI). Blood urea and Serum creatinine were raised in 91 percent of patients. Electrolyte abnormalities were present in 22 cases. USG showed hydronephrosis in 87 percent of cases. Micturating Cystourethrogram (MCU) showed reflux in 45 percent of cases. Renal scan showed bilateral hydronephrosis, reduced Glomerular Filtration Rate (GFR) and delayed excretory pattern.

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ABSTRACT: We have reviewed 130 patients of posterior urethral valves in a single center in Kolkata over the last 4 years. Primary endoscopic valve ablation was performed in 104 patients(80 percent). Diversion was required in 19 (14.6 percent) patients who were not fit for primary valve ablation. Four did not improve after valve ablation and required diversion. Three more cases were referred to us after vesicostomy done elsewhere. One neonate expired due to septicemia. In comparison to our earlier report, the rate of diversion in our center is steadily declining and more and more neonates are being treated by primary valve ablation.

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ABSTRACT: The mortality of posterior urethral valve (PUV) has dropped in the recent past because of its early diagnosis and better medical and surgical care. The incidence of PUV in our study was 3.5 per 1000 Pediatric Surgical OPD cases. Nearly half (47.68 percent) presented in the first three months of life and 75 percent presented with urinary symptoms. Inspite of various non invasive procedures, the voiding cystourethrogram (VCU) still stands out as the most important investigation for deciding the course of action. Primary valve ablation should be tried in al cases before embarking upon diversion. In our series, loop cutaneous ureterostomy was found to have better results than cutaneous vesicostomy. For long-term follow up, serial estimation of serum creatinine and examination of urine for pus cells are the two most simple, yet effective tests for assessing the prognosis. DMSA/DTPA renal scan and urodynamic studies are required in problematic cases only. Out of 516 cases assessed for long term follow up (5-20 years), only 312 (60.5 percent) are coning for regular follow up and 52 percent are leading normal life with normal renal parameters. Out the these 312 cases, only 48 percent have attained 80th percentile of height and 70th percentile of weight and the rest are below 50th percentile in term of height and weight as compared to normal age matched control group.

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ABSTRACT: The management of patients with posterior urethral valves from four Pediatric Surgery Centres in India has been reviewed. The statistics from these centers give us an idea of the presenting symptoms, the treatment modalities used and most importantly the subsequent follow up of these children, under our difficult circumstances. Endoscopic fulguration of the valves has been the treatment of choice excepting in one center where the surgeon has used hook for valvotomy.

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