Congenital urinary incontinence in a 4-year old girl due to isolated epispadias: A case report and literature review

Abstract

Epispadias is a congenital anomaly in which the urethral meatus opens dorsally on top of the penis, medial to the glands, or with absent or bifid clitoris in females. It often occurs as part of a complex anomaly called the bladder extrophy epispadias complex (BEEC) along with exposed bladder and bifid pelvic brim. The diagnosis of bladder extrophy epispadias complex is often clinical with identifications of the various component of the anomalies. Although this condition is rare it present with wide spectrum of deformities which ranged from mild to more complex anomalies, its management especially the complex variety has been a major challenge to pediatric surgeons worldwide. With a male to female ratio of 2-3:1, the occurrence of isolated epispadias in female patient is even rarer. Our patient is a 4-year old girl who presented with urinary incontinence since birth. She has never achieved urinary continence up to the age of 4-years, there is no history of any prior urethral procedures, no history of any trauma to the perineum, no passage of stone or blood in the urine. Child is the second of her mother's three children and her younger ones has achieved urinary continence. Pregnancy, delivery and puerperal history were essentially uneventful. On examination, we found a young girl, calm, but apprehensive otherwise stable, abdomen was full, moves with respiration, no area of tenderness, no palpable organomegaly, no intra-abdominal masses felt, there was distorted female genitalia with bifid clitoris and patulous urethral meatus. There was a normal vaginal orifice with intact hymen with poorly developed mons pubis and labia. A clinical diagnosis of isolated epispadias with urinary incontinence to rule out ectopic ureter in a 4-year old girl was made. She was worked up and prepared for surgical repair, and had a single stage procedure done. Post-operative cosmetic appearance of the genitalia was acceptable. Urethral catheter was removed on the postoperative day 10 and patient had achieved some continent of urine. She generally did well and has been on outpatient follow up visits at the pediatrics surgical outpatient clinic.