Female genital mutilation of a karyotypic male presenting as a female with delayed puberty

The patient first presented as a female with delayed puberty. Hormonal analysis revealed a normal serum prolactin level of 215 Mu/L, a low FSH of 0.5 Mu/L, and a low LH of 1.1 Mu/L. Type IV FGM (Pharaonic circumcision) had been performed during childhood. Chromosomal analysis showed a 46, XY karyotype and ultrasonography verified a soft tissue structure in the position of the prostate.FGM pose a threat to the diagnosis and management of children with abnormal genital development in the Sudan and similar societies.Female circumcision, or female genital mutilation (FGM), is defined by the World Health Organization as "all procedures involving partial or total removal of the external female genitalia or other injury to the female genital organs, whether for cultural or other non-therapeutic reasons" [1]. Girls are commonly circumcised between the ages of 4 and 10 years, but in some communities the procedure may be performed on infants, or it may be postponed until just before marriage or even after the birth of the first child [2]. Female circumcision is practiced in 28 countries in the sub-Saharan and northeastern regions of Africa [3]. It affects over 132 million girls and women worldwide, with an additional two million girls at risk of some form of the practice each year [4]. The practice is known across socioeconomic classes and among different ethnic and cultural groups, including Christians, Muslims, Jews, and followers of indigenous African religions [5].Based on the amount of tissue removed, Toubia has classified FGM into four main types: Type I, which is known in Sudan as "sunna", represents the excision of a part or the entire clitoris. Type II involves clitoridectomy and excision of parts of the labia minora. Types III and IV are called infibulations. Type III represents clitoridectomy, removal of the labia minora, excision of the labia majora, and stitching of the anterior two thirds of the labia majora leaving a small posterior opening. Type IV, Pharaonic