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Pseudovaginal Perineoscrotal Hypospadias

Pseudovaginal Perineoscrotal Hypospadias Causes

The disorder can be an offset of congenital adrenal hyperplasia; it can also be due to certain intersex conditions that include early testosterone production such as: 5-alpha reductase deficiency, partial androgen insensitivity syndrome, congenital adrenal hyperplasia due to 17-alpha hydroxylase deficiency, and testiscular dysgenesis.

Pseudovaginal Perineoscrotal Hypospadias Definition

Pseudovaginal perineoscrotal hypospadias (PPSH) is a abnormal configuration of an infant's external genitalia. The genitalia then appears to be a midway between a male's scrotum and a female's vagina.

Pseudovaginal Perineoscrotal Hypospadias Diagnosis

PPSH is diagnosed when the infant is born. In some cases the condition is observed via ultrasound.

Pseudovaginal Perineoscrotal Hypospadias Symptoms and Signs

PPSH is usually represented by a phallus in between the clitoris and the penis, a urethral opening on the perineum, and a partially closed urogenital opening. In most cases internal female reproductive structures are not present.

Pseudovaginal Perineoscrotal Hypospadias Treatment

Management of PPSH often begins with sex assignment, which proves to be difficult since the genitalia do not resemble either sex. Infants in this condition are usually raised female. They are also offered surgical reconstruction, or testosterone treatment.

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