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Management of male pseudohermaphroditism: a case report spanning twenty-one years
T Quattrin1, S Aronica, T Mazur
1State University of New York, Buffalo School of Medicine.
Insights
This case study highlights the importance of interdisciplinary care for infants with ambiguous genitalia. Comprehensive management, including psychological support, enabled a patient with male pseudohermaphroditism to effectively navigate her condition.
Area of Science:
- Pediatric Endocrinology
- Medical Psychology
- Surgical Management
Background:
- Ambiguous genitalia management necessitates a multidisciplinary approach.
- This case details the long-term care of a patient initially assigned male but raised female.
Observation:
- The patient was diagnosed with male pseudohermaphroditism (46 XY karyotype with testes).
- She received comprehensive clinical management over 21 years, including psychological support.
Findings:
- Despite unsuccessful reconstructive surgery, the patient developed full awareness and acceptance of her endocrine condition.
- Patient's personal testimony validates the effectiveness of the clinical management strategy.
Implications:
- Effective management of disorders of sex development (DSD) requires integrated medical and psychological care.
- Long-term patient-centered support is crucial for individuals with DSD to achieve positive outcomes.
Abstract:
Management of infants born with ambiguous sex organs requires the close collaboration of a team of professionals: pediatric endocrinologist, pediatric psychologist, gynecologist, and surgeon. The interdisciplinary teamwork of a group of professionals is described in the case of a patient announced at birth as a male but within days reannounced and reared as a female. Over the next 21 years, she became completely aware of her endocrine condition, including its medical classification (male pseudohermaphroditism), her chromosomal (46 XY) and gonadal (testes) status, and her early history of male sex assignment. The clinical management described herein helped this person deal effectively with her condition despite unsuccessful reconstructive vagal surgery. Substantiation of this is provided by the patient's personal comments.