Total Vaginectomy in a True Hermaphrodite: A Case Report

Area of Science:

• Endocrinology
• Genetics
• Reproductive Medicine

Background:

• True hermaphroditism (TH) is a rare disorder of sex development characterized by the presence of both ovarian and testicular tissue.
• Individuals with TH often present with ambiguous genitalia and may have a 46 XX or 46 XY karyotype, or mosaicism.

Observation:

• A 46 XX individual with TH presented with an ovotestis, uterus, vagina, and underdeveloped phallus, raised as male.
• The patient experienced breast enlargement at 14 and menarche at 17, prompting further investigation.
• Imaging and karyotyping confirmed Mullerian structures and a 46 XX karyotype.

Findings:

• Surgical intervention included mastectomy, hysterectomy, bilateral gonadectomy, and vaginectomy.
• Genital reconstruction and male hormone replacement therapy were administered.
• The patient was assigned male gender based on patient and parental preference and psychological disposition.

Implications:

• This case highlights the complex management of true hermaphroditism, emphasizing a multidisciplinary approach.
• It underscores the importance of patient-centered care and psychological support in gender assignment decisions.
• Successful gender affirmation in TH cases can lead to improved quality of life and psychosocial well-being.