Embryology
Anthony R. Mundy, John M. Fitzpatrick, David E. Neal, Nicholas J. R. George in The Scientific Basis of Urology, 2010
Nondisjunction and translocation anomalies are not confined to gametogenesis, but can also occur during the early mitotic cell divisions in the process of cleavage. In the resulting state, termed mosaicism, the embryonic tissues contain a varying ratio of cell lines with differing karyotypes depending on the phase of cleavage at which nondisjunction occurred, such as two-cell, four-cell, and eight-cell embryo. Abnormalities of the sex chromosomes are often found in mosaic form. Ovotesticular disorders of sex development (previously termed true hermaphroditism) (2) can be explained on this basis. Affected individuals possess both ovarian (XX) and testicular (XY) tissues that coexist in streak-like gonads termed ovotestes. Gonadal mesenchyme carrying a Y chromosome differentiates as testicular tissue, whereas tissue derived from the original population of non–Y embryonic cells differentiates passively down the female (ovarian) pathway. Karyotypes show a varied pattern, including 45 X/46 XY and 46 XX/47 XXY. Turner’s syndrome and Klinefelter’s syndrome are often associated with mosaic karyotypes.
Surgical treatment of disorders of sexual development
Mark Davenport, James D. Geiger, Nigel J. Hall, Steven S. Rothenberg in Operative Pediatric Surgery, 2020
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.
Gender-Specific Disease
Mary Ann G. Cutter in The Ethics of Gender-Specific Disease, 2012
Part of the difficulty in establishing a naturalist view of gender is rooted in the difficulty of establishing a naturalist view of sex. In the last century, biologists and clinicians have recognized that human beings display a range of sexual diversity (Reis 2007). Despite controversies surrounding the nomenclature, standard clinical texts still report that there are those who fall in so-called exclusive genetic classes (e.g., a normal homologous XX [female] or non-homologous XY [male]). There are those who do not fall within either class (e.g., those with Turner's syndrome involving XO or a single X chromosome, and those with Klinefelter's syndrome involving the presence of one or two additional X chromosomes in a male). There are those who fall in both: a so-called “true hermaphrodite” possesses at least one ovary and at least one testis, or at least one ovotestis, a “male pseudohermaphrodite” has testes (and not ovaries or ovotestes) and an XY chromosomal complement, and a “female pseudohermaphrodite” has ovaries (and not testes or ovotestes) and an XX chromosomal complement (Dreger et al. 2005, 729).
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.
Related Knowledge Centers
- Gonad
- Gonadal Dysgenesis
- Hermaphrodite
- Intersex
- Ovary
- Clitoris
- Testicle
- Sox9
- Sex-Determining Region Y Protein
- Reproductive System of Gastropods