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Surgical treatment of disorders of sexual development
Published in Mark Davenport, James D. Geiger, Nigel J. Hall, Steven S. Rothenberg, Operative Pediatric Surgery, 2020
Rafael V. Pieretti, Patricia K. Donahoe
True hermaphrodites have well-developed, non-dysgenetic male and female gonadal tissue in many combinations, i.e. a testis on one side and an ovary on the other, two ovotestes, or a normal gonad on one side and an ovotestis on the other. Although over 80% of these patients have a 46,XX karyotype, testicular tissue is present. The patient with a small phallus should be raised as a female. However, the final decision should be made following adequate discussion with the parents or guardians about various options and choices of how their child can be reared. The patient with a large phallus already committed as a male should be raised as a male. Gonads should be bivalved and biopsied longitudinally. The gonadal tissue commensurate with the sex of rearing (ovary tissue is peripheral, testicular is central) should be salvaged. Perineal Müllerian reconstruction should be accompanied by removal of Wolffian structures if the female sex is chosen. If the phallus is adequate for male gender assignment, ovarian and Müllerian structures should be removed, followed by hypospadias repair. Testicular prostheses can be inserted later, should the testicular tissue be inadequate.
Sex Chromosome Anomalies
Published in Merlin G. Butler, F. John Meaney, Genetics of Developmental Disabilities, 2019
L. Hamerton John, A. Evans Jane
De la Chapelle (27) suggested that 1 in 25,000 newborn males has a 46,XX karyotype and that, in series of patients with Klinefelter syndrome, the incidence is 1 in 25. The clinical phenotype is similar to that of Klinefelter syndrome, except that XX males are shorter than XXY males and perhaps less prone to mental impairment (Fig. 6). Otherwise, the features are similar, with normal external genitalia and small testes with the same histological changes. Secondary sexual characteristics, hormonal status, and rates of gynecomastia and sterility are similar to 47,XXY subjects. Some 46,XX individuals are true hermaphrodites with both infertile testes and ovaries or ovotestis. In some males, there may be some ambiguity in the external genitalia.
Disorders of sexual development
Published in Prem Puri, Newborn Surgery, 2017
Gonadal biopsies must be taken along the longitudinal axis of the gonad as both ovarian and testicular tissue may be found at the polar ends of the gonad (Figure 109.5). Patients with TH may have an ovary (O) and testicle (T); bilateral ovotestes (OT); or an O and OT.
Mixed gonadal dysgenesis with an ovotestis on imaging mimicking ovotesticular disorder of sexual differentiation
Published in Baylor University Medical Center Proceedings, 2021
Samantha Fine, Kenneth Ford, Bradley Trotter, Hoang-Kim Le, Matthew Crisp, Jose Santiago, Krista Birkemeier
This case is unique because the contralateral gonad had the imaging appearance of an ovotestis, and histology was necessary to differentiate MGD from ovotesticular DSD. While both may contain a mixture of male and female sexual features, the gonad does not contain primordial follicles in MGD but they are numerous in ovotesticular DSD.5 This distinction is important since MGD has a much higher gonadal malignancy risk.6 Also, it is important to understand that a typical imaging appearance of ovary, testis, or ovotestis may still represent a dysgenetic streak gonad, so biopsy is necessary.7
Ovotestis at 18 years: an accidental discovery in an internally displaced persons’ camp in North-Eastern Nigeria
Published in Journal of Obstetrics and Gynaecology, 2019
Hadiza Abdullahi Usman, Bala Mohammed Audu, Mohammed Bukar, Ahmed A. Mayun
She had psychosocial counselling and support from her managing team, a psychologist, her parents and religious clerics on the findings and the management options. A right gonadectomy and a Modified Randolph-Hung reduction clitoroplasty (Fonkalsrud et al. 1977) was carried out. The histology of the excised gonad confirmed ovotestis in the ratio of 65%:45% of ovarian to testicular tissue arranged cranio-caudally (Figure 1(C)). She was relocated to a host community and has been psychologically stable.