Ovo Testicular Differences in Sexual Development

Ovo testicular type is amongst the least common of all Disorders of Sexual Differentiation. A child born with ambiguous genitalia due to Ovo testicular DSD has severe hypospadias with unilateral or bilateral undescended gonad, Mullerian structures, and 46XX chromosomes. With recent sharing of expertise, there has been improved timely diagnosis, proper assignment of sex, and judicious approach in the surgical management with a long-term follow-up including hormonal supplements for the development of secondary sexual characters. The phallus size is adequate and thus most of these children are reared as boys. Successful management is achieved by a team approach involving the pediatric geneticist, endocrinologist, Paediatric surgeon, radiologist, and a gynecologist, especially in advanced medical centers. Long-term follow-up is necessary to monitor the outcome of surgical procedures and the need for re-do if any, titrate the hormonal requirements, and address the issues related to psychology, marriage, infertility, and rehabilitation in the society.