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

M Ellaithi,T Nilsson,D Gisselsson,A Elagib,H Eltigani,I Fadl-Elmula

doi:10.1186/1472-6874-6-6

Abstract

BackgroundFemale genital mutilation (FGM) is commonly practiced mainly in a belt reaching from East to West Africa north of the equator. The practice is known across socio-economic classes and among different ethnic, religious, and cultural groups. Few studies have been appropriately designed to measure the health effects of FGM. However, the outcome of FGM on intersex individuals has never been discussed before.Case presentationThe 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.ConclusionFGM pose a threat to the diagnosis and management of children with abnormal genital development in the Sudan and similar societies.

Highlights

Female genital mutilation (FGM) is commonly practiced mainly in a belt reaching from East to West Africa north of the equator
FGM pose a threat to the diagnosis and management of children with abnormal genital development in the Sudan and similar societies
According to the National Demographic and Health Survey (1989/1990) infibulation is the predominant type of FGM practiced in Sudan throughout most of the northern, north-eastern and north-western regions, with a small overall decline in the 1980s, when a shift occurred from infibulation to clitoridectomy [6]

Summary

Background

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]. 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 circumcision or total infibulation, refers to the complete removal of the clitoris and labia minora, excision of the labia majora, and stitching of the whole raw area leaving a very small posterior opening for the passage of urine and menstrual blood [2]. According to the National Demographic and Health Survey (1989/1990) infibulation is the predominant type of FGM practiced in Sudan throughout most of the northern, north-eastern and north-western regions, with a small overall decline in the 1980s, when a shift occurred from infibulation to clitoridectomy [6]. Ultrasound examination confirmed the presence of a 15 × 15 mm diameter soft tissue mass in the position of the male prostate (Figure 4)

Conclusion

Mulholland L

11. Baasher T

Findings

14. Mitelman F: ISCN