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Incomplete Uretheral Duplication Simulating Epispadias in a Newborn

Ahmed H. Al-Salem

Department of Pediatric Surgery, Maternity and Children Hospital

Dammam, Saudi Arabia

 

Correspondence:

Ahmed H. Al-Salem, FRCSI, FACS,FICS

P. O. BOX 61015, QATIF 31911; SAUDI ARABIA

FAX: 966 3 8630009; e-mail: This email address is being protected from spambots. You need JavaScript enabled to view it.

 

Abstract

Urethral duplication is a very rare congenital malformation that is divided into complete and incomplete forms. Most cases of the complete type are diagnosed in the neonatal period or early childhood with the presentation of double urinary streams or urinary dribbling. Incomplete urethral duplication on the other hand can be missed as they can be asymptomatic or present with intermittent discharge from the abnormal urethral opening. We report a case of incomplete urethral duplication simulating epispadias in a male newborn.

Key words: urethral duplication, incomplete urethral duplication, epispadias

 

Introduction

Urethral duplication is a rare and interesting congenital anomaly. It is divided into complete and incomplete forms and although there are several theories to explain its embryological origin, the exact mechanism of its etiology is not known and no single theory explains all the various types of urethral duplications [1, 2, 3]. The most common form of urethral duplication consists of a blind incomplete accessory urethra opening on the dorsum of penis [4]. This type is usually asymptomatic and many cases pass unnoticed till adulthood especially if the child is not going to be circumcised. We report a case of incomplete urethral duplication simulating epispadias discovered in a newborn at the time of circumcision.

Case report

A newborn male, a product of full term normal spontaneous vaginal delivery was referred for circumcision. Clinically, there were no abnormalities detected. There was complete foreskin (Fig. 1). At the time of circumcision, there was an abnormal opening on the dorsum of the penis extending from the tip of the glans to the distal part of the shaft of the penis resembling epispadias (Fig. 2). It was decided to postpone the circumcision till the time of epispadias repair. He was followed up in the clinic. At the age of 3 months, an abdominal and pelvic ultrasound revealed no abnormalities. He was admitted to the hospital at the age of 9 months for repair. Examination under anesthesia revealed a normally located external urethral meatus. Dorsally, there was an epispadiac grove extending from the glans of the penis to the distal part of the shaft of the penis. It was blind and not communicating with the normal urethra representing an incomplete urethral duplication. The normal urethra was catheterized with a size 8 French Foley’s catheter (Fig. 3). The lining of the grove was excised and the defect was closed (Fig. 4). Post-operatively, he did well and was discharged home on the 5th post-operative day.

Discussion

Urethral duplication is a rare congenital anomaly [5]. It is generally divided into two types, complete and incomplete forms. Complete urethral duplication, is defined as two urethras originating from the neck of the bladder that separate the external openings. Incomplete urethral duplication may or may not originate from the neck of the bladder, and may not have an external opening. Incomplete urethral duplications are more common than complete urethral duplications. Most urethral duplications occur in the same sagittal plane one on top of the other [6]. Less commonly, the openings of urethral duplications lie side by side in the frontal plane. According to Williams and Kenawi, urethral duplications have been divided into epispadiac, hypospadiac, spindle and collateral types [7]. There are several classifications for urethral duplication but the most accepted one is that described by Effman [7, 8, 9]. According to this, urethral duplication is divided into three types. Type I is a blind incomplete urethral duplication. It is subdivided further into two types. In type IA, the duplicated urethra opens on the distal surface of the penis but does not communicate with the urethra. This is the most common type. In type IB, the duplicated urethra opens like an extension from the urethra and ends blindly in the periurethral tissue. This is very rare. In type II, there is complete urethral duplication. It is also divided further into two types.

simulating epispadias 1 simulating epispadias 2
Figure 1. Clinical photograph showing a normally looking penis with a normal foreskin Figure 2. Clinical photograph showing incomplete urethral duplication simulating epispadias
simulating epispadias 3 simulating epispadias 4
Figure 3. Clinical photograph showing a catheterized normal urethra with a dorsal incomplete urethral duplication   Figure 4. Clinical photograph showing a normally looking urethra and glans penis after repair.

In type IIA - 1 there are two noncommunicating urethras arising independently from the bladder or coursing independently to open as two different meati. In type IIA-2, the duplicate urethra arises from the first urethra and courses independently to open as a second meatus. In type IIB, the two urethras arise from the bladder or posterior urethra and then unite to open as a single external urethral meatus. A subtype of urethral duplication is the Y- type where there is a single urethral opening and the duplicated urethra opens in the rectum or perineum [10, 11]. This type of urethral duplication is often associated with other severe associated malformations such as anorectal agenesis, Prune Belly syndrome and cloacal extrophy [12]. In the type III urethral duplication is a component of partial or complete caudal duplication. According to the classification of Effman et al, our case was type IA, the duplicated urethra opens on the distal surface of the penis but does not communicate with the urethra or bladder. This is considered the most common type of all urethral duplications. This type is usually asymptomatic may not be discovered till adult-hood. In our patient, this was not going to be discovered if wasn’t planned for circumcision. Our patient had complete foreskin and the duplicated urethra was hidden underneath it. On the other hand, patients with complete urethral duplication can be asymptomatic or more commonly present with a double stream, incontinence, dribbling of urine, outflow obstruction or recurrent urinary tract infection.

The treatment of urethral duplication depends on several factors such as the clinical manifestation and type of anomaly. Some patients with mild or no symptoms do not require surgery. On the other hand, surgery should be considered for patients who have symptoms such as incontinence or double stream. In our patient surgery was done for cosmetic reason.

 

 

 

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