Hypothesis / aims of study
Introduction:
True hermaphroditism, or ovotesticular disorder, is a rare intersex condition where both ovarian and testicular tissue are present in an individual. This condition results from abnormal gonadal development and presents diverse phenotypic features depending on the distribution of the gonadal tissues. True hermaphroditism poses significant clinical and psychosocial challenges and requires a multidisciplinary management approach. This case report discusses the care of a 26-year-old female with true hermaphroditism, focusing on sexual health challenges like dyspareunia and the importance of integrated medical, surgical, and psychological interventions.
Study design, materials and methods
Objective:
The purpose of this report is to emphasize the importance of a multidisciplinary approach in managing true hermaphroditism, particularly in addressing sexual health issues such as dyspareunia. The report highlights the need for personalized care plans, including hormonal therapy, surgical correction, and psychological support, to enhance sexual function and overall quality of life. The goal is to ensure that patients with this condition receive comprehensive, patient-centered care that addresses both the physical and emotional aspects of their health.
Results
Case Report:
A 26-year-old female with a 46 XX karyotype and a history of true hermaphroditism was referred to our urogynecology clinic for dyspareunia. She had undergone multiple surgeries in childhood, including bilateral gonadectomy, reconstructive surgery for ambiguous genitalia, and repeat gonadectomy for residual testicular tissue. Gonadal tissue was preserved for potential fertility, and she had been on hormonal therapy with regular menstruation while using combined oral contraceptives.
Married for 8 months, the patient reported difficulty with intercourse due to vaginal narrowing. Physical examination revealed Tanner stage 4 development, indicating normal secondary sexual characteristics. Vaginoscopy revealed clitoromegaly, vulval fat loss, vaginal introitus atresia, and a small anteverted uterus. Short vaginal length and stenosis at the introitus led to the decision for surgical intervention.
Surgical treatment involved vaginal dilation using Hegar dilators (size 7-26), but bleeding from the posterior forchette required a Fenton’s perineorrhaphy to widen the vaginal opening. The procedure was successful, and the patient was discharged the same day. Postoperative care included vaginal dilators, vaginal estrogen, and hormonal therapy (Prugyluton).
At the two-week follow-up, the patient’s healing was progressing well. By six months, she reported no pain during intercourse and improved sexual function. She continued to use vaginal dilators, with gradual increases in size, and her condition remained stable at the one-year follow-up. The patient also expressed interest in future fertility, and surrogacy was discussed as a potential option for biological parenthood.
Interpretation of results
Discussion:
True hermaphroditism is rare, with about 90% of cases presenting with a 46 XX karyotype. The clinical presentation can vary depending on the presence and functionality of gonadal tissues. Early diagnosis and intervention are crucial, requiring a multidisciplinary approach to address medical, surgical, and psychosocial aspects. Individuals with true hermaphroditism often experience sexual health challenges, including dyspareunia and vaginal stenosis, resulting from abnormal genital development or post-surgical changes.
In this case, vaginal stenosis was addressed through surgical intervention, including vaginal dilation and Fenton’s perineorrhaphy. Hormonal therapy and the use of vaginal dilators were effective in maintaining vaginal health and improving sexual function. Fertility is often compromised in true hermaphroditism, but assisted reproductive technologies, such as surrogacy, offer potential solutions for biological parenthood.
Concluding message
Conclusions:
True hermaphroditism is a rare condition requiring a comprehensive, multidisciplinary management approach. Early diagnosis and tailored interventions, including surgery, hormonal therapy, and psychological support, can significantly enhance the patient’s quality of life. This case emphasizes the importance of personalized care and highlights the need for further research to refine management strategies and reduce stigma surrounding intersex conditions. With continued support, individuals with true hermaphroditism can lead fulfilling lives and have opportunities for fertility and parenthood.