Clitoromegaly in Childhood and Adolescence: Behind One Clinical Sign, a Clinical Sea

Abstract

The clitoris is a highly complex organ whose structure has only been clarified in recent years through the use of modern imaging techniques. Clitoromegaly is an abnormal enlargement of this organ. It may be congenital or acquired and is usually due to an excess of androgens in fetal life, infancy, or adolescence. Obvious clitoromegaly in individuals with ambiguous genitalia is easily identifiable, whereas borderline conditions can pass unnoticed. Case reports of clitoromegaly with or without clinical or biochemical hyperandrogenism are quite numerous. In these subjects, a comprehensive physical examination and an accurate personal and family history are needed to investigate the enlargement. We reviewed the literature on the conditions that may be involved in the development of clitoromegaly in childhood and adolescence.

Extensive Pelvic Plexiform Neurofibroma Presenting As Clitoromegaly in a 3-Year-Old Female: Presentation and Management with MEK Inhibitor

“…These include neurofibromatosis, epidermoid cysts, intradermal nevus, other tumors such as hemangioma or leiomyoma, malignant tumors such as rhabdomyosarcoma, and various syndromes which may be associated with clitoromegaly. 1 Clinical features on examination that suggest a nonhormonal etiology include an irregular or asymmetric clitoris. 1 Further investigations include diagnostic imaging such as ultrasound or MRI.…”

“…1 Clinical features on examination that suggest a nonhormonal etiology include an irregular or asymmetric clitoris. 1 Further investigations include diagnostic imaging such as ultrasound or MRI. Genetic evaluation is warranted if an underlying syndrome is suspected.…”

“…5 Contiguous extension to the urinary tract, particularly the bladder, or to the retroperitoneum is possible with PN and subsequently, diagnostic imaging is important to evaluate the extent of the lesion. 1,3 Management of clitoral neurofibromas depends on the extent of involvement. Clitoroplasty with preservation of the neurovascular bundle and glandular tissue is the suggested treatment when possible.…”

“…Clitoral enlargement in infancy or childhood requires consideration of both hormonal or nonhormonal causes. 1 Plexiform neurofibromas (PN) involving the external genitalia are a rare cause of nonhormonal clitoromegaly and occur in the context of neurofibromatosis type 1 (NF1). NF1 is an autosomal dominant neurocutaneous disorder with an incidence of 1 in 3,000.…”

Clitoral sizes and anogenital distances in term newborns in Nigeria

“…Using the definition of mean + 2 SD, we define clitoromegaly as a clitoris greater than 9.9 mm in length. In many neonates, no cause is found (idiopathic clitoromegaly) [15] but, it can also reflect exposure of the fetus to fetal or maternal androgens. Congenital adrenal hyperplasia is the commonest cause of pathological clitoromegaly in the newborn [6].…”