Draft:Unfused scrotum

An Unfused scrotum is a technical term to describe a labia like scrotum that has not fully fused, it is largely caused by androgen insensitivity syndrome (AIS), Persistent Müllerian duct syndrome (PMDS), or other intersex conditions such as issues in metabolic synthesization of cholesterol such as in leydig cell hypoplasia. During male differentiation the basal labia fuses into a scrotum, the vulva expands into a phallus, and the ovotestis are converted into testis and descend. It generally manifests itself with hypospadias, and cryptorchidism. It is related to permanent labial fusion caused by conditions such as congenital adrenal hyperplasia (CAH).

Presentation

Genitalia showing partially unfused scrotum, and partially complete phallus

Genitalia showing major hypospadias and unfused scrotum

During fetal development all external genitalia appears female, male genitalia is catalyzed via androgens primarily dihydrotestosterone via interaction with an androgen receptor (AR) via a process known as genital differentiation. In most XY chromosomal individuals the ovotestis are converted into testis via an SRY gene, which produces androgens which stimulates the development of male genitalia. The two separate folds which make up labia fuse and expand downwards, fusion can be delayed and the folds can remain partially separate via a insensitivity or lack of androgens during development or atypical utero development. Complete insensitivity results in external typical female genitalia, while partial results in ambiguity. While an unfused scrotum can be caused by abnormal sexual development in utero it can also be caused by overly elastic skin which results in scrotal expansion and dysgenesis.

Intersex microphallus genitalia with hypospadias, and cryptorchidism with female pattern fat and bone distribution

There are no physical complications from an unfused scrotum, other then cosmetic appearance. Generally an unfused scrotum presents itself with other genital differences including hypospadias, although it can appear isolated. Conditions such as partial androgen insensitivity syndrome, androgen deficiency, 5α-Reductase 2 deficiency, or otherwise often manifest with an unfused scrotum. Performing intersex genital surgery for cosmetic purposes is considered a human right violation.