Correction of a rare malformation of the penis in two boys with a single cavernous body and classical bladder extrophy

Abstract

Bladder extrophy - is a difficult malformation for the treatment. Aplasia of one of the cavernous bodies of the penis is an extremely rare pathology. In the available literature, we did not meet reports of correction of bladder extrophy in children with a single cavernous body of the penis.From 1990 to 2020, 545 children (364 boys) with extrophy have been repair. Two (0.5 %) boys only had a classic bladder extrophy combined with the aplasia of one cavernous body. The first patient with classical extrophy and multiple malformations (fingers of the right hand, fingers of the right foot, aplasia of the left kidney, left cavernous body, left testicle and hypoplasia of the left half of the scrotum) the penis with the single cavernous body on the right was somewhat thinner, but formed anatomically correctly with the presence of a urethra and closed prepuce. The primary closure of the bladder was performed with bilateral iliac osteotomy and the joint of pelvic bones at the age of 1 year. The patient had the anatomically properly formed bladder sphincter and urethra, penile correction was not required. After the operation, urinary continence had been achieved.The second boy was with classical extrophy and epispadia of the single cavernous body, he was given stage treatment. At the age of 5 days (2004) the primary closure of the bladder and the connection of the pelvic bones were performed. At the age of 4 years (2008) the formation of the urethra was carried out, the correction of the dorsal deformation of the single cavernous body by excision of the chord and the application of multiple superficial cross-cutting on the tunica albuginea. Bladder neck plastic by Kelly technique in combination with bilateral osteotomy of pelvic bones was performed at the age of 7 years in (2011). The boy of 15 years (2019) had a bladder volume of 350-400 ml. Dry 4-5 hours. Urinate freely with a wide stream. Then a two-stage plastic of the distal urethra was performed. We used the Bracka technique with augmentation of the glans penis and implantation a free graft of the mucous lip. We achieved a good result.Using modern penile correction technologies help to socially adapt patients with rare and difficult malformations.

Keywords

• bladder extrophy
• epispadia
• single cavernous body
• plastic of urethra
• rare malformation of the penis