Managing disorder of sexual development surgically: A single center experience

Abstract

Introduction: Ambiguous genitalia are a major cause of parental anxiety and create psychological and social problems to patient, if not managed properly. Here we present our experience in managing patients with ambiguous genitalia. Material and Methods: We retrospectively reviewed clinical records of all patients with ambiguous genitalia managed surgically at out institute between December 1989 and January 2011. Relevant history, clinical examination , investigations and surgical procedures performed were analyzed and results were evaluated in terms of anatomical, functional and psychosexual outcomes. Results: Female pseudohermaphroditism was the most common cause of genital ambiguity in our patients. Male and female genitoplasty was done according to gender of rearing, genital anatomy and parental choice. Twenty six patients (86.6%) reported satisfactory cosmetic outcome and 22 (73.3%) satisfactory functional outcome on long term follow-up. Among the 24 patients diagnosed as male pseudohermaphroditism 14 (82.3%) patient have reported satisfactory cosmetic outcome and 13 (76.4%) have reported satisfactory functional outcome. In patients with mixed gonadal dysgenesis and true hermaphroditism satisfactory cosmetic and functional outcome was seen in 70% patient. Conclusion: Managing patients of genital ambiguity according to gender of rearing, genital anatomy and parental choice carries good prognosis in terms of anatomical, functional and psychosexual outcome.

Keywords

• Disorder of sexual differentiation
• genital ambiguity
• intersex
• sexual ambiguity