P0279 - What factors influence gender assignment and surgical reconstruction for an index case of ovotesticular disorder of sexual development in the Dominican Republic?

Introduction:
Gender assignment in children with disorders of sexual development (DSD) remains controversial. Low-and-middle income countries (LMIC) have fewer resources to complete a DSD workup compared with the United States (US), however many US surgeons visit LMIC to offer/teach surgical techniques. During a surgical mission in the Dominican Republic (DR), we evaluated a 20-month-old male with ambiguous genitalia, undescended left testicle, symptomatic right inguinal hernia, and umbilical hernia. Surgical exploration revealed ovotestis DSD (SRY negative, microarray negative, 46XX karyotype). Sociocultural preferences for gender assignment were unclear and further information was needed for shared decision making.

Methods:
A Google survey link surrounding this index case of ovotesticular DSD was distributed to pediatric physicians in the DR. Statistical analysis was performed to compare the respondent’s gender, occupation, years in practice, practice setting (public vs private), and prior experience managing DSD. After survey data was obtained and both pathology and cytogenetic evaluations were performed at the University of Pittsburgh Medical Center, a videoconference was held with the family to discuss gender assignment and future surgical reconstruction.

Results:
199 surveys were distributed; 53 were completed (26%). Nearly all (96%) of respondents believed a psychologist was critical to gender assignment. The only significant variable predicting delay in gender assignment and surgery was practice setting. Those involved in private (or combined) practice considered patient autonomy as the priority hence were more likely to wait on gender assignment and surgery. Conversely, those who practiced in a purely public setting considered patient anatomy and genotype the highest priority, also valuing physician expertise over family autonomy. These practitioners were more likely to believe the parents’ greatest concern was patient fertility.
With respect to our index case, the child’s parents indicated family autonomy, gender identity and fertility preservation influenced gender assignment. Consistent with private physician preferences, parents, local surgeons and an international surgeon were comfortable delaying surgical reconstruction, though preferred gender assignment before the child entered primary school. Fertility was not a central determinant in gender assignment once explained that natural conception was highly unlikely for either gender, based upon the child’s anatomy.

Conclusions:
Practice patterns in DSD management may be influenced by practice type among providers, and patient/parent preference appears to be the greatest predictor in private practice settings. This has important implications for timing of reconstruction and gender assignment in the DR and is important for international surgeons to understand. In an attempt to better comprehend local preferences for gender assignment, a physician survey was created which assisted both international and local surgeons in shared decision making for gender assignment of a child with ovotestis DSD. Such a model for humanitarian mission work can be applied in other countries to ensure sociocultural preferences are respected.