Practical and functional classification of the double urethra: A variable, complex and fascinating malformation observed in 20 patients: Beyond the Abstract

Urethral duplication is a rare anomaly (estimated frequency is 1/5.5 millions of new-borns) with a huge spectrum of anatomical variants and difficulties in obtaining an adequate and complete classification system. These elements may interfere with the prompt identification of patients and the setting of a proper treatment.

The first description of urethral duplication dates back to Aristotle. In 1872 Schatz reported the first case of a foetus with urethral and bladder duplication, double vagina, uterus and anus. In the fifties, Gross and colleagues reported the first case series of patients with urethral duplication. 

The presence of asymptomatic patients, mis-diagnosis and lack of uniformity in reports make, with good chance, the number of described cases (nearly 400) the tip of the iceberg, 

The abovementioned peculiar features have led to problems in classifying the double urethra. The most used classifications refer only to males. Williams and Kenawi introduced the description of the duplication plane (collateral and sagittal) as related to the embryological origin. Effman, also uses their definition of complete and incomplete, taken from Gross, with a slightly different meaning. More recently Abouzeid has proposed an interesting algorithm for patients with urethral duplication excluding the “lambda type”. Considering our own personal experience and starting from the observations reported in Literature, we propose a new classification aiming at addressing the issues of the previous ones. 

The proposed classification is complete and helps the surgeon in diagnosing the malformation.  The correct diagnosis allows the setting of the most appropriate management and the formulation of a prognosis. However each case should be discussed individually to propose "ad hoc treatments". The fundamental step in patient assessment is the identification of the functional urethra and associated anomalies. Surgery can be successful in most cases but there are patients with complex associated anomalies influencing long-term outcomes. Therefore parents should be informed of the possibility of performing more than one procedure to achieve satisfactory aesthetic and functional results

Written by: Mario Lima and Francesca Destro

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