Complete duplication of the bladder, urethra and external genitalia in a male neonate with an imperforate anus

Abstract

A rare case of a male neonate with complete duplication of the bladder, urethra and external genitalia with associated anomalies is reported. In addition to the above, the patient had a hypoplastic left kidney, bilateral bifid scrotums (four hemi-scrotums), an imperforate anus and a mass on the perineum. Key wordsbladder, duplication, external genitalia, male neonate, urethra. Case reportA one-day-old neonate with multiple congenital abnormalities was referred to our hospital. Review of the history disclosed a normal vaginal delivery by a 21-year-old primigravida. The pregnancy was uneventful and the mother had an insignificant medical history. On examination the patient had complete duplication of the external genitalia ( Fig. 1) with a double penis and bilateral bifid scrotums (four hemi-scrotums) with bilateral undescended testes. The penis on the right side was smaller and clinically normal compared to the left sided larger abnormal penis. Meatuses could be seen on both glandes. The anus was imperforate with a mass attached to the perineum with a clinically abnormally-developed foot hanging from the mass.Abdominal sonar was done which showed a normal right kidney, but the left kidney could not be visualized. A computed tomography (CT) scan of the abdomen and pelvis could not differentiate the bladders but showed a functioning normal right kidney and a hypoplastic left kidney (Fig. 2). A magnetic resonance (MR) scan was also done which showed the rectum to be tapered and 1 cm away from the perineal skin. On the MR scan the perineal mass appeared to contain fat, bone and some muscle tissue (Fig. 3). A cystogram was done with retrograde filling through the right urethra at first, followed by the left side. Two bladders without reflux were demonstrated with the left bladder smaller and no connection between the two (Fig. 4).In order to show the ureters, an intravenous pyelogram was done with no visual contrast handling on the left side, and a nephrogram and pyelogram on the right side with drainage into the larger right-sided bladder. Little residual contrast was left in the bladder on the post void film. A dimercapto-succinic acid (DMSA) renogram was done which showed non-function of the left kidney.The first procedure done on the patient was a loop colostomy to decompress the bowel. After a few weeks the mass on the perineum was removed with the finding of an articulation of the bone in the mass with the pelvis. Histology showed a mature teratoma. Six weeks later reconstruction of the urogenital system and rectum was performed. Preoperative cystoscopy was done to evaluate the internal anatomy of the urethras and bladders. A lower abdominal laparotomy was done with separation of the two bladders and subsequent left cystectomy. A vesicorectal fistula was found to this side. The vas deferens of both sides could be seen entering the internal inguinal rings. Subsequently a simple amputation of the left penis was done. A rectal pull through procedure was performed after closure of the laparotomy wound. Histo...

Complete Urogenital and Anorectal Duplication in a Dog

“…Interestingly, duplication of the urethra was found to have a male preponderance in individuals ranging from 4 months to 10 years of age on presentation with anal urine voiding and incontinence as presenting symptoms [19]. Reports in another study describe duplication of the external genitalia or of the lower intestinal tract, comparable to the female dog described in our case, as the most common [7].…”

Webbed Penis Associated With Urethral Duplication: A Case Report

“…The anatomical variations of a duplicated urethra have been classified and modified by various authors (3,4). Effermann et al 4 and rectum, as well as anal atresia, anterior ectopic anus, mental retardation, spinal anomalies, diplopodia of the foot, intestinal malrotation, and umbilical hernia have been reported in the literature (5)(6)(7)(8). We could not find any case of webbed penis associated with urethral duplication.…”

Incomplete bladder duplication with multiple congenital anomalies: A rare presentation

“…Associated duplication anomalies of the lower gastrointestinal tract have been reported in up to 42% of cases and spina bifida, meningocele, or myelomengocele has been reported in 15% of cases. [ 5 ] Berrocal et al . also showed associated duplication anomalies of the external genitalia, gastrointestinal malformations, duplications of the spine, spina bifida conditions, and various fistula formations between the urogenital and gastrointestinal tract.…”