Urethral Anomalies

Abstract

During the development of the phallic part of a urogenital sinus, the urethral plate tubularizes by midline meeting of its elongated folds. Simultaneously, the outer ectodermal genital folds fuse at the midline, which is demarcated by the perineal and penile raphe.

Hypospadias as a common urogenital anomaly will not be discussed herein, but two variants, chordee without hypospadias (CWH) and megameatus intact prepuce hypospadias variants, will be discussed with some details.

Isolated epispadias without exstrophy is a rare anomaly; the incidence being 1 in 120,000 in males, the male-to-female ratio is 2.3:11. The first recorded case is attributed to the Byzantine Emperor Heraclius for his unknown disease. Isolated epispadias remained unknown and untreated until it was described by Morgagni in 1761; it is classified into glanular, penile, pubic, or penopubic epispadias.

Congenital megalourethra is a rare form of functional obstructive uropathy caused by dysgenesis of the penile corpora cavernosa and spongiosa, which results in extensive dilatation of the penile urethra; it has been postulated that it may be caused by abnormal development or hypoplasia of the penile erectile tissue secondary to distal urethral obstruction.

Urethral duplication is a rare congenital anomaly affecting mainly boys, with a variable clinical presentation because of the different anatomical patterns. This pathological condition may be easily underdiagnosed especially in patients with other associated anomalies such as hypospadias or bladder exstrophy.

Congenital urethroanal fistulas are very rare in the absence of associated anorectal malformation; in the male child, it represents less than 1 % cases of all anorectal malformations, but urethral fistula associated with anorectal malformations is a common finding among children who had anorectal malformations.

Urethral agenesis with complete absence of the urinary meatus is a very rare anomaly, which comes in two forms: either with normal phallus or in combination with penile agenesis. It is a very rare reported anomaly as most cases die intrauterine; combined bladder and urethral agenesis is also an extremely rare anomaly; only 22 live births have been reported of the 60 known cases.

Congenital urethral narrowing is known as Cobb’s collar and Moormann’s ring. It can appear either as a ridge of tissue (Cobb’s collar I) or a definite stricture at the junction between prostatic and membranous urethra (Cobb’s collar п) or even as a very tight pinhole opening (Cobb’s collar Ш).