46,XX ovotesticular disorder of sex development
MONDO:0016281
46,XX ovotesticular disorder of sex development (46,XX ovotesticular DSD) is characterized by histologically confirmed testicular and ovarian tissue in an individual with a 46,XX karyotype.
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View 46,XX ovotesticular disorder of sex development on the outbreak map →Ovotesticular syndrome
Ovotesticular syndrome (also known as ovotesticular disorder or OT-DSD) is a rare congenital condition of atypical sexual differentiation where an individual is born with both ovarian and testicular tissue. It is one of the rarest disorders of sex development, with an incidence rate of approximately one in 100,000 live births. Commonly, one or both gonads is an ovotestis containing both types of tissue. Although it is similar in some ways to mixed gonadal dysgenesis, the conditions can be distinguished histologically. People with ovotesticular syndrome may be considered intersex.
Background only. Canonical identity remains MONDO; clinical-trial relationships come from ClinicalTrials.gov.Drugs being studied
Disease identity and terminology: MONDO. Trial relationships: ClinicalTrials.gov.