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Congenital megalourethra: spectrum of presentation and insights in embryology and management

Prakash Advait, Bhavesh Doshi, Sangram Singh, Bhupendra Parekh.

Abstract
Background: Congenital megalourethra is a rare urogenital mesenchymal anomaly of the male anterior urethra and erectile tissue of penis. The disease is characterized by severe dilatation of the penile urethra due to congenital absence of the corpus spongiosum and corpus cavernosum. It presents as two types, a milder scaphoid type and severe fusiform type. The prognosis is related to the degree of associated renal impairment and the severity of accompanying congenital anomalies. Some isolated case reports are available but very few case series have been published till date. This is one such rare case series.
Methods: Since July 2010, we have encountered three cases of congenital megalourethra in our department. All these cases had different patient profile, clinical manifestations and types. Two patients had isolated megalourethra which is very rare entity while one patient had megalourethra with associated prune belly syndrome. All the patients were investigated thoroughly. Micturating Cystourethogram (MCU) is required to confirm the diagnosis.
Results: Reduction urethroplasty was performed in all the three patients successfully. All patients passed urine in single stream without any urethral dilatation. There were no complications.
Conclusions: Congenital megalourethra is a rare disease which warrants proper diagnosis and timely intervention for optimum outcome. Due to paucity of literature many cases are misdiagnosed and incorrectly treated the embryology, presentations and current management of this rare disorder are discussed herewith along with review of pertinent literature.

Key words: Congenital megalourethra, Scaphoid, Fusiform, Reduction urethroplasty



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