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True hermaphroditism: a report of two cases.
D Maji1, B Mukhopadhyay, U R Chaudhuri
1Department of Endocrinology, IPGME & R, Calcutta.
The Journal of the Association of Physicians of India
|November 1, 1990
Summary
This study details two cases of true hermaphroditism, a rare condition. Both 46XX individuals with ovotestis underwent gonadectomy, with one also receiving mastectomy and hysterectomy.
Area of Science:
- Reproductive Endocrinology
- Human Genetics
- Pediatric Endocrinology
Background:
- True hermaphroditism, characterized by the presence of both ovarian and testicular tissue, is a rare disorder of sexual development.
- Genetic and hormonal factors contribute to the complex presentation of intersex conditions.
Observation:
- Two cases are presented: a 2-year-old child with ambiguous genitalia and bilateral ovotestis, and a 20-year-old male with gynecomastia, bleeding, and seminal discharge, exhibiting a right ovotestis, uterus, ovary, and hypoplastic fallopian tubes.
- Both individuals were cytogenetically 46XX and assigned male gender.
Findings:
- Surgical intervention included gonadectomy in both cases.
- The second case also underwent bilateral mastectomy and hysterectomy due to the presence of female internal organs.
Implications:
- These cases highlight the phenotypic variability of true hermaphroditism in 46XX individuals.
- Management requires a multidisciplinary approach, considering genetic, hormonal, and surgical aspects for optimal patient outcomes.