Plexiform Neurofibroma of Clitoris.

The most frequent genital presentation of neurofibromatosis in females is clitoromegaly. We report a case of a 5-year-old girl with neurofibromatosis type 1 with clitoral plexiform neurofibromatosis. Clitoroplasty was done, and the histopathology confirmed the diagnosis. Though rare, plexiform neurofibroma of clitoris should always be considered as a differential diagnosis in children with clitoromegaly before embarking on detailed investigations.

A safer way of suturing in Foker's technique.

BACKGROUND/PURPOSE: Foker's technique allows esophageal lengthening facilitating end to end anastomosis in long gap esophageal atresia. The problem faced with this technique is that the traction sutures cut through the tissues leading to re-operations. Our aim was to find a technique of suturing that will prevent the sutures from cutting through the esophagus. METHODS: After dissection of the upper and lower esophageal pouches, purse string sutures were placed, two each on both pouches. Clips were applied at the ends of both the pouches. Sutures were brought out on the posterior chest wall and traction applied. This was tried in a total of three cases. Case 1 was a newborn with pure esophageal atresia, Case 2 was an eighteen month old child with cervical esophagostomy and gastrostomy, and Case 3 had esophageal atresia with distal fistula. Two cases were done thoracoscopically and the third one by thoracotomy. RESULTS: In all three cases sutures held and lengthening could be obtained. In the first case it took twelve days, in the second case six days, and in third case eight days for the ends to come together. CONCLUSION: This modification of traction sutures is simple and reduces the risk of suture disruption.

Role of alpha adrenergic blocker in the management of posterior urethral valves.

PURPOSE: To evaluate the effect of Terazosin (alpha1 adrenergic blocker) on bladder emptying in children with posterior urethral valves. MATERIALS AND METHODS: Forty-two children with significant post void residual urine after valves ablation were placed on Terazosin ranging from 0.25 to 2 mg. Post void urine at the commencement and at follow up was monitored with abdominal ultrasound. RESULTS: Post void residual urine significantly reduced in 40 patients (95%) who were put on Terazosin. Mean pretreatment PVR was 15.7 ml and mean PVR at the last follow up was 2.4 ml (P = 0.000). This was a reduction of 85% in the pretreatment post void residual urine volume. All the patients had improvement in urinary stream. One patient reacted to Terazosin with hypotension necessitating its withdrawal. Mean follow up was 17 months. CONCLUSION: Terazosin has proved to be safe and results in significant improvement in bladder emptying in our patients with posterior urethral valves. Randomized controlled trial and long-term follow up are necessary to further define the role of alpha1 adrenergic blocker therapy in children with posterior urethral valves. This study will become the justification for such a study.

Posterior urethral valves: a single center experience over 7 years.

PURPOSE: To evaluate the outcome of management of 65 consecutive children with posterior urethral valves (PUV) using two techniques of valves ablation. METHODS: Retrospective review of 65 consecutive children with PUV managed over 7 years. There were two groups based on the methods of valves ablation: either Mohan's urethral valvotome for primary cases or electrocautery fulguration for patient with previous intervention. Serum electrolyte and serial post-void residue (PVR) were monitored in outpatient clinic. Alpha-blockers were started in all cases with high PVR. Management outcome of patient who had valves ablation with Mohan's valvotome were compared with those who had endoscopy fulguration. RESULTS: Sixty-one patients were regular on follow-up with a median age at presentation of 45 days. Nineteen (31%) patients were diagnosed antenatally. There were 32 refluxing ureters in 26 patients; 58 (95%) underwent primary valve ablation (37 with Mohan's urethral valvotome, 21 fulguration). Reflux disappeared in 10 (36%) refluxing ureters. There were five (8.2%) urethral strictures (all five in fulguration group), eight residual valves (four in each group), and three recurrent urinary tract infections. Three patients had nephrectomy and one patient had renal transplantation. There was no death. The mean serum creatinine for all the patients at presentation and at the last follow-up were 1.2 and 0.5 mg/dl, respectively (P = 0.031). At follow-up, mean PVR of valvotome group was 2.5 ml and fulguration group was 2 ml (P = 0.282). The median follow-up period was 24 months. CONCLUSIONS: Early ablation of PUV and detailed attention to bladder management gives a better outcome. With Mohan's valvotome incidence of stricture is less and incidence of residual valves is comparable to endoscopic ablation of PUV.