RESUMO
STUDY OBJECTIVES: Mayer-Rokitansky-Küster-Hauser Syndrome (MRKH) is characterized by the congenital absence of the uterus and vagina, sometimes with associated extragenital anomalies. Currently there is limited literature on pelvic pain and co-morbid pain syndromes in people with MRKH. The aims of this scoping review were to summarize existing literature on pelvic and generalized persistent pain syndromes associated with MRKH and to identify knowledge gaps for further research into this field. METHODS: This scoping review followed the Joanna Briggs Institute framework. The population of interest was patients with a diagnosis of MRKH. MEDLINE, CINAHL, Scopus, Cochrane, Embase and Emcare databases were searched. Excluded were articles that did not meet the inclusion criteria or critical appraisal standards. The resultant articles were reviewed by 2 independent researchers while a third was used in cases of disagreement. A descriptive analytical method was used for data analysis. RESULTS: We screened 3348 articles for eligibility, 39 articles met criteria, which described 1353 cases of MRKH. 4 studies described baseline pelvic pain in MRKH, 19 described acute presentations and 13 described post-intervention pain levels. CONCLUSION: Despite the paucity of research, this review found that cyclical pelvic pain was mostly present in women with uterine remnants, whilst pelvic pain in those without remnants was poorly understood. There were no studies exploring generalized persistent pain syndromes in MRKH. Further cross-sectional studies are needed to elucidate prevalence and levels of pain syndromes in MRKH.