Jiledar Rawat

Vaginal agenesis: Experience with sigmoid colon neovaginoplasty.

Aim: Objective of this study is to report our experience with sigmoid vaginoplasty in adolescents. Materials and Methods: A retrospective study of children with vaginal atresia and Mayer–Rokitansky–Kuster–Hauser syndrome. The sigmoid segment was used for vaginoplasty in all the cases. Results: Eight children were studied over a period of 7 years. The postoperative complications were ileus in 2, mucosal prolapse of the neovagina in 1, and minor wound infection in 1 patient. Seven patients are on regular follow-up. All the neovaginas were patent and functional. One patient had unacceptable perineal appearance, that is, badly scarred perineum as a late complication. None of the patients had vaginal stenosis or excessive mucus discharge, during follow-up visits. Out of the 7 patients, 2 patients are sexually active and satisfied. Conclusions: Sigmoid vaginoplasty is a safe and acceptable procedure for vaginal agenesis with good cosmetic results and acceptable complications rate. Sigmoid colon vaginoplasty is the treatment of choice because of its large lumen, thick walls resistant to trauma, adequate secretion allowing lubrication, not necessitating prolonged dilatation, and short recovery time.

Delayed presentation of anorectal malformations

Aims and Objectives: Delayed management of anorectal malformation (ARM) increases the surgical and functional complications for the patient. We defined “delayed presentation of ARM” and reviewed our patients with ARM to find out the incidence and causes of delayed presentation. Materials and Methods: Patients satisfying the criteria of “delayed presentation of ARM” were involved. Detailed information of each patient including the mode of presentation, associated anomalies, plan of management and follow-up was obtained from the hospital records. Results: Between 2003 and 2006, 43 patients satisfied our criteria of “delayed presentation of ARM”. There were 21 males and 22 females. Seventeen of these males presented with low-type ARM. Eleven of them were managed by a single-stage procedure. These “delayed presenters” had to live with constipation, inadequate weight gain and parental anxiety for a greater time. Analysis of the outcomes showed more functional complications in patients who had undergone failed perineal surgery previously. In females with low ARM, the procedure of choice was anterior sagittal anorectoplasty (ASARP). Single stage surgery provides good outcomes for most of low type of ARMs. High-type ARMs in males and females were managed by a staged procedure. Conclusion: “Delayed presentation of ARM” is a major group of ARM in our setup. The management and results of their treatment are not different from those of the early presenters. The most common cause of delayed ARM is wrong advice given by the health care providers followed by inadequate treatment elsewhere. Corrective surgeries taking second attempt in perineum always produces poor outcomes.

Congenital anterior urethral valves and diverticula: Diagnosis and management in six cases

BACKGROUND Anterior urethral valves (AUVs) are rare congenital anomalies causing lower urinary tract obstruction in children. Although they are referred to as valves, these obstructive structures often occur in the form of a diverticulum. The urethra in these cases shows saccular or bulbar dilatation known as anterior urethral diverticulum (AUD). They typically occur where there is a defect in the corpus spongiosum, leaving a thin-walled urethra. This segment of the urethra balloons out during voiding, simulating a mass that is sometimes visible along the ventral wall of the penis. The swelling is fluctuant and urine dribbles from the meatus on compression. The present study highlights the clinical approach in identifying the condition and its treatment options, especially for those, presenting with urethral diverticula. MATERIALS AND METHODS We have studied children with congenital anterior urethral valves and diverticula. Six patients of AUVs with diverticula were admitted during the period of 2000-2007 and were prospectively evaluated. The mean age of presentation was 16 months (15 days to 4 years). Straining at micturition and a palpable penile swelling were the most common presenting features. The diagnosis was established by voiding cystourethrogram (VCUG) and supported by ultrasonography (USG). All patients were treated with single-stage open surgical excision except one who died preoperatively due to urosepsis. Initial lay opening of the penoscrotal urethra and delayed repair were done in one patient. RESULTS The surgical outcome was successful in all but one patient, who died of delayed presentation with severe back pressure changes, urinary ascitis and urosepsis. On long-term follow-up, all patients demonstrated good stream of urine. The renal functions were normal and the patients had no evidence of urinary infections. CONCLUSION We propose that, the patients of AUVs, if not excessively delayed for treatment are otherwise well in terms of general condition as opposed to patients of posterior urethral valves. The diagnosis is easily established by VCUG and the severity is revealed by a sonogram. Open surgical excision is the method of choice for patients with a urethral diverticulum; however, cystoscopic fulguration is also feasible in selected patients. The outcome is excellent with minimal morbidity and mortality.

Unusual Ventriculoperitoneal Shunt Extrusion: Experience with 5 Cases and Review of the Literature

Distal migration of ventriculoperitoneal shunt is rare. We present this unusual complication in 5 patients. The lower end of the shunt was extruded from right lumbar region, cervical area, umbilicus and rectum. The cause of such extrusion is not known. The patients were managed by shunt removal followed by shunt replacement on the opposite side.

Diagnosis and management of congenital bladder diverticulum in infancy and childhood: experience with nine cases at a tertiary health center in a developing country

ObjectivesThe purpose of the study is to present the authors’ clinical and surgical experience with congenital bladder diverticula in nine pediatric patients at a developing world tertiary care center.Patients and methodsRecords of nine patients diagnosed and treated as congenital bladder diverticula from 2000 to 2007 were retrospectively reviewed for age, sex, chief complaints, associated anomalies, investigative work-up, operative notes, and postoperative follow-up.ResultsAll were males. Age at presentation ranged from six months to eight years (mean three years). All were diagnosed postnatally by ultrasound and/or voiding cystourethrography (VCUG) and confirmed on urethrocystoscopy. Open surgical excision of diverticulum was done in all the patients. Ureteral reimplantation was simultaneously done only in three patients with VCUG-documented high-grade vesicoureteral reflux (VUR). With an average follow-up of four years, gradual resolution of symptoms was seen in seven of nine patients whose postoperative follow up records were available. There was no diverticulum recurrence at the defined mean follow-up.ConclusionPediatric patients with recurrent urinary tract infections and voiding dysfunction should always be evaluated for congenital bladder diverticulum. Although investigations such as intravenous urography (IVU), urodynamic studies, nuclear renal scanning, and, sometimes, CT scan and MRI, form an important part of preoperative diagnostic work-up and postoperative follow up, USG and VCUG may be enough when availability and cost are the constraints. Diverticulectomy, with ureteral reimplantation for high-grade reflux, provides good results without recurrence.

Abdominal wall ectopic testis mimicking spigelian hernia

We encountered a patient with a swelling in the left lateral infraumbilical region and a left undescended testis. The appearance of the swelling was suggestive of a spigelian hernia. At operation, the swelling was actually an abdominal ectopic testis with associated inguinal hernia. This unusual finding led us to report this occurrence and present a brief review of the relevant literature.

Prolapse of bowel via patent vitello intestinal duct—a rare occurrence

This report describes the presentation of prolapse of small bowel through the patent omphalomesenteric or vitello intestinal duct in a child. In spite of diagnosing the anomaly earlier, there was delay in surgical intervention that led to prolapse of the small bowel through patent remnants, which was life threatening. The patient presented to us with questionable viability of prolapsed bowel. Early surgery is recommended for this entity. As this is a very rare occurrence, it is being reported with a brief review of the relevant literature.

Congenital lumbar hernia: 20 years' single centre experience.

Aim:  Congenital lumbar hernia is an uncommon anomaly with only few cases reported in the English literature. This study was done to study the incidence, associated conditions, management and complications of congenital lumbar hernia repair in paediatric patients.

Undescended testicle: An update on fertility in cryptorchid men.

Cryptorchidism or undescended testis is one of the most common anomalies encountered in paediatric urology and is estimated to affect 1 to 4 per cent of full term and upto 30 per cent of preterm male neonates. The associated problems of sub-fertility or infertility and malignant transformation have been recognized for long. Fertility is impaired after both unilateral and bilateral cryptorchidism. The reported paternity rates in adults are about two-third for unilateral undescended testis and less than one-third for bilateral disease. Over the last five decades, the concepts related to cryptorchidism have changed dramatically as knowledge about its effects has accrued from research conducted worldwide. The recommended age of orchidopexy has fallen progressively from adolescence to less than one year. The realization that the infantile testes are not in a state of ‘suspended animation’ and the recognition of the defect in the androgen dependent transformation of gonocytes into adult dark spermatogonia in cryptorchidism have been recognized as the primary cause of sub-fertility in these patients. This has paved the way for hormone therapy in an attempt to simulate the ‘post-natal gonadotropin surge’ or ‘mini-puberty’. This review summarizes the current knowledge about the various factors affecting the fertility status in cryptorchidism with a particular focus on the derangements in the development and maturation of the germ cells and the role of surgery, hormone therapy and antioxidants in reversing these changes.

Y-type urethral duplication in children: Management strategy at our center.

Aims: Report of seven children with Y-type urethral duplication (YUD). Materials and Methods: (A) Four staged operations were performed in patients having extensive perineal dissection (for rectourinary fistula separation and anterior mobilization of ventral urethra (VU)), tension rectocutaneous anastomosis, and children who were not toilet trained). These stages are (1) diversion sigmoid colostomy with anterior mobilization of VU as perineal urethrostomy via anterior sagittal approach; (2) Orthotopic urethral (OU) reconstruction; (3) anastomosis of OU and perineal urethra; (4) colostomy closure with management of complications. (B) The patients having VU onto the perineum underwent single stage urethral reconstruction. Results: The VU was urethrorectal/urethroanal in five and urethroperineal in two. Low anorectal malformation and upper urinary tract anomalies were present in 57.1% (4/7) and 14.7% (1/7) patients, respectively. Buccal mucosa free graft, transverse inner preputial flap, and perineal skin were tubularized for OU reconstruction. Mean age at 1st , 2nd , 3rd , and 4th surgery was 5 ± 0.78, 28 ± 0.78, 36 ± 0.78, 49 ± 0.78 months respectively. Three patients needed surgery for complications (urethrocutaneous fistula in two and urethral diverticulum in one) in a mean 3.12 ± 0.34 years of follow-up. Final uroflowmetry and fecal continence were good in all patients. Conclusions: The YUD is a difficult entity to manage. Although, staged procedure appears to be time consuming, but good and promising results can be achieved by staging the procedure.