Penoscrotal transposition: review of 53 patients.

Abstract

Penoscrotal transposition is a rare congenital abnormality of the external genitalia. We determine whether there is a genetic basis for this disorder, define the incidence of coexisting organ system anomalies, and compare the results of surgical techniques to correct transposition and hypospadias. We report the largest, single institution series of 53 patients 1 day to 30 years old with penoscrotal transposition. Of the patients 13% had a family history of penoscrotal transposition. Interestingly, we identified 1 family in which inheritance occurred in an X-linked recessive manner. There were 17 (32%) patients who had abnormalities in other organ systems, with the genitourinary system in 9 affected most. A total of 79% of patients had hypospadias and 81% chordee. These anomalies were corrected with a single stage Thiersch-Duplay urethroplasty in 6 patients and complex repair with bladder or buccal mucosa, or a staged procedure in 34. Complication rates for urethroplasty were similar. Correction of the transposition included a Glenn-Anderson technique in 37 patients, Singapore rotational flaps in 7 and V-Y procedure in 6. The Glenn-Anderson repair produced the best cosmetic results and was associated with a significantly lower incidence of complications (p = 0.001). We identified a subgroup of patients with a family history of penoscrotal transposition. Treatment requires an awareness of the association with other organ system anomalies. The Glenn-Anderson technique was the most successful method to correct transposition. Most patients required release of chordee and complex urethroplasty for hypospadias.