Congenital scrotal anomalies are classified as penoscrotal transposition, bifid scrotum
, ectopic scrotum, and accessory scrotum.
Patients may present with almost complete female phenotype or ambiguous external genitalia, hypospadias, micropenis, bifid scrotum
, single urogenital opening, or a combination of these defects.
We reviewed the literature for patients diagnosed with Klinefelter syndrome before puberty with penoscrotal malformations; we searched PubMed using key words: Klinefelter syndrome, hypospadias, prepubertal age, chordee, bifid scrotum
, and scrotal transposition (Table 1).
He initially presented with a penoscrotal hypospadias and bifid scrotum