Experiences of Pelvic and Generalized Persistent Pain Syndromes in MRKH: A Scoping Review

R U Gaikaiwari, Reineth Prinsloo, C Prinsloo, S R Grover, Sonia Grover, I Wright, Ian Wright, Natalie Drever, N Drever
Journal of Pediatric and Adolescent Gynecology · 2024 · vol. 37(5) , pp. 477–494 · doi:10.1016/j.jpag.2024.07.002 · PMID:39038531 · W4400849740
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This scoping review of 39 articles found that cyclic pelvic pain in MRKH is linked to uterine remnants, while pain without remnants and generalized persistent pain syndromes remain poorly understood and require further research.

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Abstract

STUDY OBJECTIVE: Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome 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 comorbid 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. Articles that did not meet the inclusion criteria or critical appraisal standards were excluded. The resultant articles were reviewed by 2 independent researchers, and a third was used in cases of disagreement. A descriptive analytical method was used for data analysis. RESULTS: We screened 3348 articles for eligibility. Of these, 39 articles, which described 1353 cases of MRKH, met the criteria. Four studies described baseline pelvic pain in MRKH, 19 described acute presentations, and 13 described postintervention pain levels. CONCLUSION: Despite the paucity of research, this review found that cyclic pelvic pain was mostly present in women with uterine remnants, whereas 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 the prevalence and levels of pain syndromes in MRKH.

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MeSH descriptors

46, XX Disorders of Sex Development 46, XX Disorders of Sex Development 46, XX Disorders of Sex Development Congenital Abnormalities Congenital Abnormalities Mullerian Ducts Mullerian Ducts Pelvic Pain Pelvic Pain Pelvic Pain Chronic Pain Chronic Pain Chronic Pain Female Humans

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