The birth of an intersex child prompts a long term management strategy that involves a myriad of professionals working with the family. It is estimated that genital anomalies occur in 1 in 4500 births. There has been progress in diagnosis, surgical techniques, understanding psychosocial issues, and recognising and accepting the place of patient advocacy. The Lawson Wilkins Pediatric Endocrine Society (LWPES) and the European Society for Paediatric Endocrinology (ESPE) considered it timely to review the management of intersex disorders from a broad perspective, to review data on longer term outcome, and to formulate proposals for future studies. The methodology comprised establishing several working groups whose membership was drawn from 50 international experts in the field. The groups prepared prior written responses to a defined set of questions resulting from an evidence based review of published reports. At a subsequent gathering of participants, a framework for a consensus document was agreed. This paper constitutes its final form.

Advances in identification of molecular genetic causes of abnormal sex with heightened awareness of ethical issues and patient advocacy concerns necessitate a re-examination of nomenclature.¹ Terms such as intersex, pseudohermaphroditism, hermaphroditism, sex reversal, and gender based diagnostic labels are particularly controversial. These terms are perceived as potentially pejorative by patients,² and can be confusing to practitioners and parents alike. The term “disorders of sex development” (DSD) is proposed, as defined by congenital conditions in which development of chromosomal, gonadal, or anatomical sex is atypical.

The proposed changes in terminology are summarised in table 1⇓. A modern lexicon is needed to integrate progress in molecular genetic aspects of sex development. As outcome data in individuals with DSD are limited, it is essential to employ precision when applying definitions and diagnostic labels.^3,4 It is also appropriate …