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Persistent unexplained congenital clitoromegaly in females born extremely prematurely.
Back to Health A to Z. Differences in sex development DSD is a group of rare conditions involving genes, hormones and reproductive organs, including genitals. Some adults and young people with DSD prefer to use the term intersex. You or your child may have sex chromosomes bundles of genes usually associated with being female XX chromosomes or usually associated with being male XY chromosomes , but reproductive organs and genitals that may look different from usual. This happens because of a difference with your genes or how you respond to the sex hormones in your body, or both. It can be inherited, but there is often no clear reason why it happens.
Management of the enlarged clitoris, because of its import for sexual function, has been and remains one of the most controversial topics in pediatric urology. Early controversy surrounding clitoroplasty resulted from many factors including an incomplete understanding of clitoral anatomy and incorrect assumptions of the role of the clitoris in sexual function. With a better understanding of anatomy and function, procedures have evolved to preserve clitoral tissue, especially with respect to the neurovascular bundles. These changes have been made in an effort to preserve clitoral sensation and preserve orgasmic potential. It is the goal of this manuscript to describe the different procedures that have been developed for the surgical management of clitoromegally, with emphasis on the risks and benefits of each.
Clitoromegaly or macroclitoris [1] is an abnormal enlargement of the clitoris that is mostly congenital or acquired, though deliberately induced clitoris enlargement as a form of female genital body modification is achieved through various uses of anabolic steroids, including testosterone , and may also be referred to as clitoromegaly. The different grade of genital ambiguity is commonly measured by the Prader classification , [6] which ranges, in ascending order of masculinisation, from 1 : female external genitalia with clitoromegaly through 5 : pseudo-phallus looking like normal male external genitalia. Clitoromegaly is a rare condition and can be either present by birth or acquired later in life. If present at birth, congenital adrenal hyperplasia can be one of the causes, since in this condition the adrenal gland of the female fetus produces additional androgens and the newborn baby has ambiguous genitalia which are not clearly male or female. In pregnant women who received norethisterone during pregnancy, masculinization of the fetus occurs, resulting in hypertrophy of the clitoris; [8] however, this is rarely seen nowadays due to use of safer progestogens.