Duplicación rectal extrofiada asociada a malformación anorrectal y transposición pene-escrotal con hipospadias perineal: Reporte de un caso clínico / Exstrophy of rectal duplication associated with anorectal malformation and penoscrotal transposition with perineal hypospadias: A case report

Se presenta el caso de un paciente masculino quien requirió tratamiento por agenesia anorrectal con fístula rectouretral y transposición pene-escrotal con hipospadias perineal, acompañados de una masa perineal. La tumoración perineal se encontró íntimamente adherida y en continuidad al recto, lo que la hace compatible con una duplicación rectal extrofiada. La reconstrucción quirúrgica de la anomalía se realizó en etapas hasta lograr resultados funcionales y estéticos aceptables.

We present the case of a male patient who required treaatment due to anorectal agenesis with recto urethral fistula and penoscrotal transposition with perineal hypospadias, associated with a perineal tumor. The perineal tumor was found strongly adhered and contiguous to the rectum which makes it compatible with an exstrophy of rectal duplication. Surgical reconstruction of the birth defect was performed in stages until acceptable biological function and esthetic results were obtained.

Congenital Penile Urethrocutaneous Fistula.

Congenital Penile Urethrocutaneous Fistula (CPUF) is an extremely rare anomaly. Commonly congenital urethrocutaneous fistulae are associated with anorectal anomalies and urethral duplication. The posterior urethra is the part, which is mostly affected. To the best of our knowledge, only 11 such cases have been reported so far in the English literature. A case of CPUF with a short review of literature.

Congenital megalourethra complicated by circumcision

Congenital megalourethra [CMU] is a rare anomaly of the male urethra in which there is a non-obstructive dilatation of the penile urethra. Children usually present with an abnormal appearance of penis and ballooning of the penis during voiding. Diagnosis is easy by typical external appearance of the floppy penis. We report a case of CMU with an abnormal looking penile urethra resulted due to circumcision, carried out by the inexperienced traditional circumcision practitioner, [Nai]. A six years old child was referred with an abnormal appearance of the penis. At the age of two months, a traditional circumcision practitioner [Nai] carried out his circumcision, which led to an abnormal looking penile urethra. During this period, the child had two episodes of urinary tract infection. His urinary stream was good and he had no other urinary symptoms. Examination revealed a good sized penis with floppy large opening of the distal penile urethra. There was a wide urethral plate on the ventral surface of glans penis and the most distal part of corpora cavernosa were not palpable. Child, however, had normal erection. Diagnosis of congenital megalourethra complicated by circumcision was made because of the typical external appearance. Ultrasonography revealed bilateral hydroureter with hydronephrosis. Micturating cystourethrogram showed grade 4 vesicoureteric reflux on right side and grade 3 on left side. Isotope renal scans showed bilateral renal scarring, good renal function on both sides and differential functions of 35% and 65% on right and left side respectively. The child was operated and bilateral ureteric reimplantation performed. Six months follow-up visits showed stable renal functions, no evidence of vesicoureteric reflux. He is awaiting for repair of megalourethra, which shall be performed by Nesbit technique.2

Congenital urethral fistula.

A 3-year-old male child was presented with the history of passage of urine coming from the undersurface of penis and its tip since birth. An opening was present on the ventral aspect of midshaft of penis through which a catheter could be passed. It was diagnosed as urethral fistula which was managed surgically.

Bifidite' ure'thrale en Y. [a' propos de 2 cas]

Urethral duplicities are scarce malformations dominated by urethral bifidity in "y', sometimes associated to other congenital malformations. Discovery age is generally neonatal. Diagnosis is clinical, anatomic type is determinal by anterograde and retrograde opacifications. Treatment often poses restenosis problems. Two cases are treated at pediatric urology service, whose surgical intervention was a failure in one case; in the other case the reestablishment of urethral conduct is always under way, and we foresee terminal urethrosplasy in the future with a very good prognosis

Hipospadias / Hypospadias

Para avaliar o estado atual do tratamento das hipospadias, foi realizado um estudo sobre condutas e tecnicas utilizadas frente a esta ma formacao congenita. O local do estudo foi a Unidade de Urologia Pediatrica da Fundacao Puigvert - IUNA - Instituto de Urologia, Nefrologia e Andrologia - Universidade Autonoma de Barcelona (Hospital de La Santa Cruz y San Pablo). As codutas frente a uma hipospadia praticamente divergem se o meato uretral esta longe ou perto da glande e, nestas duas situacoes, surgem as varias taticas cirurgicas que podem ser agrupadas em tratamento das hipospadias distais e proximais. Como as implicacoes referentes a tecnica, resultados, complicacoes, etc. sao distintas a cada um destes grupos, toda esta revisao ficara assim dividida.