Proximal hypospadias with skin-covered shaft of penis and absent urethral plate: An unknown variant and its management.

We report a case of proximal hypospadias with an absent urethral plate having intact ventral shaft skin with hooded dorsal prepuce and a good glans groove. Such a variant of hypospadias has not been reported in the literature.

INTRODUCTION

Deficient ventral skin between the hypospadiac meatus and the glans is the characteristic feature of all the types of hypospadias except some milder glanular variants and the distal variant called “Megameatus with Intact Prepuce”.[12] Complete absence of urethral plate is an unknown feature in hypospadias. A case of proximal hypospadias is reported where normal skin was present on the ventral surface of the penile shaft between the hypospadiac meatus and a grooved glans with no urethral plate. To the best of our knowledge, this is the first report of a hypospadias variant unknown in the English literature. The embryology and management is discussed.

CASE REPORT

A 16-month-old boy presented with absent urethral opening at the tip of the penis and voiding through an opening on the undersurface near the base of the penis. On examination, there was a wide oval urethral opening at the penoscrotal junction lined with healthy pink mucosa. The glans groove was good and ventral prepuce was deficient. The ventral shaft was completely covered with normal skin, with an oblique midline raphe between the meatus and the glans groove [Figure 1]. Bracka's staged repair was done using inner preputial free graft and tubularizing the same after 6 months [Figure 2]. The result of the repair was satisfactory [Figure 3].

Figure 1

Wide hypospadiac meatus, glans groove, hooded prepuce and normal skin with median raphe covering the ventral penile surface with no visible urethral plate

Figure 2

Graft after 6 months

Figure 3

Result 3 months after the second stage

DISCUSSION

Abnormally located ventral opening of the urethral meatus anywhere from the ventral aspect of the glans to the perineum is a constant feature of hypospadias.[1] Abnormal ventral curvature (chordee) and deficient foreskin ventrally with dorsal hood may not be present in all cases.[12] In the megameatus variant and in some glanular forms of hypospadias, ventral glans may partially or completely conceal the glans.[2] In all types of hypospadias, the urethral plate, the remnant of unformed urethra, extends distally from the hypospadiac meatus as a strip of epithelium overlying the connective tissues.[1] In our case, proximally located hypospadiac meatus, glans groove lined with epithelium, deficient foreskin along the ventrum of glans and dorsal preputial hood are characteristic features of a hypospadias. The unique feature in this case is the ventral penile shaft between the hypospadiac meatus and the glans being covered with normal skin, with a well-formed median raphe instead of a urethral plate. Embryologically, the hypothesis of “arrest of development” provides the most plausible explanation for the concomitant occurrence of three typical features of hypospadias, including penile curvature, hypospadiac meatus and deficient foreskin.[34] However, the absence of a urethral plate and completely formed shaft skin, as in our case, finds no answer in any of the hypotheses.

We chose to stage the repair by Bracka's method[5] for the following reasons: (1) the skin was not well supported by dartos on the ventral aspect to provide enough vascularity to the neourethra, (2) incising a skin strip for tubularization from the ventrum would have needed dorsal skin for ventral cover and, hence, was not as good a cosmesis as in Bracka's repair, which preserves the whole shaft skin for covering the neourethra, (3) the well-formed median raphe, otherwise, had to become part of the urethral tube and (4) tubularized grafted skin is less prone to form diverticulum.