A cognitive behavioural model and therapy for utero-vaginal agenesis (Mayer-Rokitansky-Küster-Hauser syndrome: MRKH)

Utero-vaginal agenesis, also called the Mayer-Rokitansky-Küster-Hauser Syndrome (MRKH), is a congenital abnormality of the female genital tract, characterized by the non-formation of the vagina and the uterus. It is a common cause of primary amenorrhoea. Little is known about the psychological impact and management of this condition.

We describe a specific model of the core negative psychological impact of diagnosis and medical treatment of MRKH and a cognitive-behavioural therapy of MRKH based on the model (CBT-MRKH). The Medical Research Council's (2002) framework for the development and evaluation of complex health interventions was used for intervention development and evaluation.

Evidence from a recent cross-sectional study and a small randomized controlled trial (RCT) provides preliminary support for the model and treatment (Heller-Boersma, Schmidt and Edmonds, in press; Heller-Boersma, Schmidt and Edmonds, 2007), and this is further validated by extensive qualitative material gathered over the course of the RCT from participants.

The model and treatment described may also be applicable to a number of other congenital or acquired gynaecological conditions such as premature ovarian failure, breast cancer, early onset endometrial cancer, female genital mutilation, Turner's Syndrome, ovarian dys/agenesis or, Complete Androgen Insensitivity Syndrome, all of which have a psychological impact not dissimilar to MRKH in terms of these women's sense of self and femininity.