ABSTRACT
Gynaecological developmental abnormalities can be broadly classified into two groups: Müllerian duct anomalies and differences in sex development (DSD). Mullerian duct anomalies occur in approximately 5% of females. Initial management of obstructed Mullerian duct anomalies should be with menstrual suppression to allow time for preoperative planning. Magnetic resonance imaging is the imaging modality of choice, and these patients should be managed in a specialist tertiary centre. DSD encompasses a group of conditions where the development of chromosomal, gonadal or anatomical sex is atypical. These cases should be managed by a multidisciplinary team in a specialist tertiary referral centre. Feminising genital surgery is best delayed until adolescence or adulthood when the girl/woman can be fully informed and involved in the decision-making process. Full disclosure of the diagnosis is imperative, and psychological support should be available for the girl and her family from the time of diagnosis and throughout adult life.
