Bracka verses Byar's two-stage repair in proximal hypospadias associated with severe chordee: a randomized comparative study.

INTRODUCTION: Proximal hypospadias associated with severe chordee represents a major surgical challenge and the debate over its optimal treatment is ongoing. The objective of this study is to compare the outcome of two-stage Bracka and Byar's repair in proximal hypospadias. MATERIALS AND METHODS: This study was conducted from January 2013 to February 2018 in a tertiary care centre. Patients of hypospadias with severe chordee who required urethral plate transection were included in the study. Patients were randomly divided into two groups by simple randomization method. Bracka staged repair was done in Group A and Byar's staged repair in Group B. Postoperatively complications including graft loss, flap necrosis, fistula formation, meatal stenosis, stricture, diverticula formation, residual chordee were noted in both the groups and compared. p value of < 0.05 was considered statistically significant. RESULTS: Over a period of 5 years, 74 patients in group A and 68 patients in group B were operated. Fistula occurred in 6.8% and 10.2% in group A and group B, respectively (p value 0.629). Meatal stenosis was seen in 4% in group A and 3% in group B (p value 0.731). Stricture was seen in 1% in each group (p value 0.339). Diverticula formation was seen in 2% in group B and none of the patient in group A (p value 0.960). None of the patient had recurrence of chordee in either group. CONCLUSION: Bracka and Byar's two-stage repair have similar postoperative outcome and the choice between the two depends up on the surgeon's choice and experience rather than scientific evidence.

Disorders of Sex Development: A 10 Years Experience with 73 Cases from the Kashmir Valley.

PURPOSE: To present the clinical data, investigative profile, and management of patients with disorders of sex development (DSD) from the endocrine unit of a tertiary care university hospital. MATERIALS AND METHODS: This retrospective study included 73 cases of DSD, evaluated and managed at Department of Endocrinology, Sher-i-Kashmir Institute of Medical Sciences, Srinagar, Kashmir, over a period of 10 years from September 2008 to August 2018. RESULTS: Twenty-nine patients (39.7%) had 46 XY DSD and twenty-nine patients (39.7%) had 46 XX. Sex chromosome DSD was diagnosed in 15 (20.5%) patients. Of 29 patients with 46 XY DSD, 17 (58.6%) had 5α-reductase type-2 deficiency (5α-RD) and 6 (20.7%) had complete androgen insensitivity syndrome. In our patients with 5α-RD, the history of consanguinity was documented in nine (52.9%) patients. Two patients had testosterone biosynthetic defect and one patient had partial androgen insensitivity syndrome. Of 29 patients with 46 XX DSD, 16 (55.1%) had congenital adrenal hyperplasia (CAH). Of 15 patients with sex chromosome DSD, 7 patients had Turner's syndrome, 7 had Klinefelter's syndrome, and 1 patient had mixed gonadal dysgenesis. CONCLUSION: In our study, equal number of patients had 46 XY DSD and 46 XX DSD. We are for the first time reporting from India that the most common cause of 46 XY DSD is 5α-RD, whereas CAH is the most common cause of 46 XX DSD as reported previously.

Posterior Reversible Encephalopathy Syndrome after Augmentation Cystoplasty in a Child with Neurogenic Bladder.

Posterior reversible encephalopathy syndrome (PRES) or leukoencephalopathy syndrome was introduced into clinical practice in 1996 by Hinchey et al., to describe unique syndrome, clinically expressed during hypertensive and uremic encephalopathy, eclampsia, and immunosuppressive therapy. Hyperperfusion with resultant disruption of the blood-brain barrier results in vasogenic edema, but not infarction, most commonly in the parieto-occipital regions. The severity of this clinical symptom varies. For example, the visual disturbance can manifest as blurred vision, homonymous hemianopsia, or even cortical blindness. Patients may be mildly confused or agitated but can become comatose. Other symptoms less commonly seen include nausea, vomiting, seizures, and brainstem deficits. Chronic kidney disease (CKD) and acute kidney injury are both commonly present in patients with PRES. We are presenting a rare case of neurogenic bladder who developed PRES after augmentation cystoplasty due to underlying CKD.

Buck's fascia repair with glanuloplasty in hypospadias surgery: A simple approach with excellent outcome.

BACKGROUND: Hypospadias is a common problem encountered in surgical practice and its repair is challenging because of various complications. Urethro-cutaneous fistula is the most common postoperative complication (incidence of 0-33%). Different types of intermediate protective layers have been used in attempts to decrease UCF formation; however, no single surgical technique is ideal. OBJECTIVE: The aim of this study was to compare use of dartos fascia and Buck's fascia as intermediate layers in prevention of the formation of UCF. STUDY DESIGN: This was a prospective, comparative study conducted over a period of 2 years from January 2014 to December 2015. Patients with primary hypospadias without or with mild ventral penile curvature were included in the study. Patients were categorised into two groups, A and B, with alternate patients assigned to each group. Patients in group A underwent Snodgrass repair with urethroplasty by two-layer subepithelial closure and dartos tissue as an intermediate layer. Patients in group B underwent a urethral repair followed by Buck's fascia repair as intermediate layer and glanuloplasty after excision of a triangular skin strip on either side of the urethral plate. Patients were followed at regular intervals for a minimum of 6 months and complications were noted. RESULTS: Over a period of 2 years, 160 patients were included in the study: 80 patients in group A and 80 patients in group B. The age of patients ranged from 1 year to 4.6 years, with a mean age of 1.8 years. Postoperative complications are listed in the summary table. DISCUSSION: A protective intermediate layer between the neourethra and the skin can be used to reduce fistula formation. We describe a technique of urethroplasty using Buck's fascia as intermediate layer and glanuloplasty, with excellent results. Buck's fascia over the corpora spongiosum which is deficient ventrally in hypospadias is not completely absent, and can be easily used to cover the neourethra, needs minimal dissection and hence vascularity of tissues is preserved (summary Fig.). We used this Buck's fascia as a second protective layer over the neourethra in an attempt to decrease UCF formation, and compared it with use of dartos fascia. In our study, UCF occurred in 2.5% of patients in the Buck's fascia group and 12.5% of patients in the dartos group, a statistically significant difference. We believe that the native Buck's fascia lateral to spongiosum is a more appropriate, natural, and strong layer to cover the neourethra. CONCLUSION: Use of Buck's fascia as an intermediate layer along with glanuloplasty is simple and very effective in preventing UCF formation and glanular dehiscence. We recommend the use of Buck's fascia as an intermediate layer to cover the neourethra to reduce incidence of postoperative complications and improve results.

Congenital duodenal diaphragm and enteroliths: A Unique complication.

We report an unusual case of duodenal diaphragm (DD) with "wind-sock" anomaly in a 6-year-old male. The child presented with an acute duodenal obstruction precipitated by multiple pigmented stones completely blocking the duodenum.

Congenital pouch colon syndrome: a report of 17 cases.

BACKGROUND: Congenital pouch syndrome (CPC) is a rare condition seen in association with anorectal malformation that occurs almost exclusively in northern India. We reviewed cases seen in our institution to study aspects of clinical presentation, diagnosis, embryogenesis and management and raise awareness of this relatively infrequent entity. PATIENTS AND METHODS: From March 2002 to September 2004, 17 neonates/infants (11 males and 6 females) treated for CPC associated with anorectal malformations included 13 with type IV and 4 with type I CPC. Diagnosis was made by a single large air-fluid level on the infantogram occupying more than 50% of the entire abdominal dimension. RESULTS: In all patients, the pouch had fistulous communication with the genitourinary system, and there were other associated anomalies as well. Of 13 patients with pouch colon type IV, 11 neonates underwent laparotomy, ligation of the fistula, excision of the colonic pouch and end colostomy as a stage 1 procedure. Subsequently, these patients underwent definitive surgery, i.e. abdominoperineal posterior sagittal anorectoplasty (AP-PSARP), with or without covering colostomy. Two of 4 patients with type 1 CPC underwent laparotomy, ligation of the fistula and colorrhaphy as a first-stage operation before AP-PSARP. In our series, 4 patients were diagnosed intraoperatively and were treated in accordance with their operative findings. Post-operatively, there were no major complications except wound infection in some patients. There was one death that was not related to surgery. CONCLUSION: There are variants of the anomaly, but the possibility of CPC needs to be kept in mind as a possible association with anorectal malformations.