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Ambiguous Genitalia and Differences of Sexual Development (DSD)
Published in S Paige Hertweck, Maggie L Dwiggins, Clinical Protocols in Pediatric and Adolescent Gynecology, 2022
If isolated clitoromegaly is presentIf suspect mass effect from tumor: Excisional biopsy indicatedIf clitoral enlargement with normal laboratory findings: Consult with pediatric gynecologist/surgeon/urologist, likely can be monitored without any treatment, but definitely until patient old enough to consent for any surgical procedure
Variation of sex differentiation
Published in Joseph S. Sanfilippo, Eduardo Lara-Torre, Veronica Gomez-Lobo, Sanfilippo's Textbook of Pediatric and Adolescent GynecologySecond Edition, 2019
Anne-Marie Amies Oelschlager, Margarett Shnorhavorian
Assessment of the clitorophallus: It is important to note that palpation of the corporal tissue is the most accurate method for assessing width and length of the corporal tissue. In the newborn, swelling of the labia and the clitoral hood may be misleading; therefore, care must be taken to retract the clitoral hood if the tissue is edematous to assess for true clitoromegaly. For the most accurate measurement, the clitorophallus should be stretched and measured from the pubic ramus to the tip of the glans (stretched length). The width should be measured in the midshaft. The tethering of the clitorophallus on the ventral side by residual urethral tissue is called a chordee, and this may prevent stretching to assess the length. If stretching the tissue is not possible, palpating the corporal tissue is an option. For a term male infant, a typical stretched phallic length is >2.5 cm and width is >0.9 mm. Stretched length less than 2 cm is considered a microphallus. For a term female infant, clitoromegaly is defined as width greater than 6 mm and length greater than 9 mm. It is important to note that this is impacted by gestational age, gestational size (large or small for gestational age), and twinning.42
Normal and abnormal development of the genitalia
Published in David M. Luesley, Mark D. Kilby, Obstetrics & Gynaecology, 2016
Rebecca Deans, Sarah M Creighton
Clinical presentation of both these conditions is usually mild ambiguity of the genitalia (clitoromegaly) at birth or in early childhood in an XY female. However, the presentation can be variable, and a number of these patients will present to a gynaecologist with virilisation at puberty. Müllerian structures are absent and Wolffian structures are present. The testes are intra-abdominal in childhood, and often descend to the inguinal canal or labioscrotal folds after puberty. Without childhood intervention, secondary sexual development is usually masculine, with poor breast development and normal pubic and axillary hair. The incidence of these conditions is unknown, but with the new scientific knowledge of these enzymes over the past decade, 17-β-hydroxysteroid dehydrogenase type 3 deficiency is now being diagnosed in some cases previously labelled as CAIS. In cases diagnosed in childhood, the management and assignment of gender are difficult. There have been insufficient cohorts raised as either males or females from childhood to evaluate the outcomes of adult gender identity, sexual function, psychological outcomes and quality of life. Fertility may be possible as a male, although infertility is common. Diagnosis is by DNA serum samples, and urinary steroid profile (either 24 hour or spot sample).
Clitoroplasty: A variant of the technique by Acimi
Published in Arab Journal of Urology, 2018
Smail Acimi, Mohammed Ali Acimi, Lamia Debbous, Mimouna Bessahraoui, Ibtisseme Bouanani
Recently, two variants of the clitoroplasty technique, by complete separation of the glans with its neurovascular bundle, have been described for surgical correction of an enlarged clitoris. The technique is described by Pippi Salle et al. [11], which places the corpora cavernosa around the vaginal orifice and the glans repositioned at the bifurcation of the corpora cavernosa. The other technique is characterised by excision of the distal and internal part of the corpora cavernosa, as well as a reduction in the volume of the glans [6]. This technique gives a significant and symmetrical reduction in the length and diameter of the corpus cavernosum. Reducing the diameter of corpus cavernosum is an important step in the surgical correction of clitoromegaly because this part of the bodies is very thin in a woman. This can only be achieved after complete release of the glans with its neurovascular bundle. In addition, when the glans is large, the reduction in its volume is needed and every effort should be made to protect the glans mucosa in this reduction of the volume.
Pseudoclitoromegaly from acute T-cell lymphoblastic leukemia
Published in Baylor University Medical Center Proceedings, 2023
Krista Birkemeier, Hale E. Wills, Tesneem Issa, Amanda Farris, Joanna Stacey, Pamela Greene, Wajahat Dawood, Kurren Desai, Carrie Laborde, Bradley Trotter
Clitoromegaly, abnormal enlargement of the clitoris, may be congenital or acquired and is usually the result of elevated androgens. Congenital clitoromegaly from hormonal disturbances is often noticeable at birth. Acquired clitoromegaly develops later in life. Clitoromegaly can be divided into two causal categories, hormonal (endocrinopathies, masculinizing tumors, exposure to androgens, and virilization syndromes) and nonhormonal (neurofibromatosis, epidermoid cysts, infection, and tumors).1 While acute lymphoblastic leukemia is the most prevalent childhood cancer, accounting for 20% of all cancers diagnosed in the pediatric population,2 a presenting complaint of clitoromegaly is unique.
Severe hyperandrogenemia in postmenopausal woman as a presentation of ovarian hyperthecosis. Case report and mini review of the literature
Published in Gynecological Endocrinology, 2017
Adam Czyzyk, Justyna Latacz, Dorota Filipowicz, Agnieszka Podfigurna, Rafal Moszynski, Piotr Jasinski, Stefan Sajdak, Michal Gaca, Andrea R Genazzani, Blazej Meczekalski
Gynecological examination revealed clitoromegaly. Her body mass index (BMI) was 31.0 kg/m2, indicating obesity. In the past the patient was diagnosed with hypertension and hypothyroidism, and for that reason she was during losartan therapy (50 mg daily) and l-thyroxine supplementation (75 μg daily). Her self-reported menarche occurred at the age of 12. The patient was pregnant three times and delivered spontaneously in term three times. Her last menstruation was in four years prior to admission. For most of her life she reported regular cycles. Unscheduled bleedings started one year before menopause.