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Case Report
Mayer Rokitansky Küster Hauser Syndrome and Endometriosis (MRKH): A case report
1 Residency Program, Department of Gynecology and Obstetrics, Federal University of Amazon – School of Medicine, UFAM – Manaus, Amazonas, Brazil
2 Intern at School of Medicine, Federal University of Amazon, UFAM – Manaus, Amazonas, Brazil
3 Faculty member, Department of Gynecology and Obstetrics, Federal University of Amazon – School of Medicine, UFAM – Manaus, Amazonas, Brazil
Address correspondence to:
Isabela Goncalves Feitosa
Intern at School of Medicine, Federal University of Amazon, UFAM, Manaus, Amazon, Brazil – Manaus, Amazonas,
Brazil
Message to Corresponding Author
Article ID: 100056Z06FS2019
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How to cite this article
da Silva FGN, Bulcão FEA, Feitosa IG, dos Santos ES, da Silva MRV, da Silva PRL. Mayer Rokitansky Küster Hauser Syndrome and Endometriosis (MRKH): A case report. Case Rep Int 2019;8:100056Z06FS2019.ABSTRACT
Introduction: MRKH is a rare congenital malformation that affects about 1:4500 female newborns. Its cause is unknown, but the reproductive abnormalities are due to lack of development of the Müllerian ducts between the fifth and the sixth weeks of gestation. This syndrome is characterized by complete or partial vaginal agenesis, tubal, and uterine-cervical abnormalities. In clinical presentation, the disease typically presents itself as primary amenorrhea in an adolescent who has secondary sexual characteristics compatible with their age and may be accompanied by cyclic dysmenorrhea when a rudimentary uterus with functional endometrium is present.
Case Report: This case report describes follow-up appointments and outcomes of a young female patient who looked for gynecological care at The Getúlio Vargas University Hospital. She presented primary amenorrhea with normal development of secondary sexual characteristics. Within the diagnostic investigation it was demonstrated that there was MRKH and endometriosis occurrence. This specific patient had progressive cyclic pelvic pain that didn’t respond to conservator treatment. Therefore, laparoscopic hysterectomy was performed.
Conclusion: Diagnosis is most often based on a clinical suspicion associated with a detailed physical and gynecological examination. The anatomical treatment of the syndrome is surgical. The frequent association of this disease with somatic and psychosocial disorders requires a multidisciplinary therapeutic.
Keywords: Abnormalities; Amenorrhea, Endometriosis, Paramesonephric (Müllerian) ducts
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Author Contributions
Fernanda Goulart Nogueira da - Substantial contributions to conception and design, Acquisition of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Felipe Everton Araújo Bulcão - Substantial contributions to conception and design, Acquisition of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Isabela Goncalves Feitosa - Substantial contributions to conception and design, Acquisition of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Evelise Staevie dos Santos - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Maria Riselda Vinhote da S - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Paula Faculty member - Acquisition of data, Final approval of the version to be published
Guaranter of SubmissionThe corresponding author is the guarantor of submission.
Source of SupportNone
Consent StatementWritten informed consent was obtained from the patient for publication of this case report.
Data AvailabilityAll relevant data are within the paper and its Supporting Information files.
Conflict of InterestAuthors declare no conflict of interest.
Copyright© 2019 Fernanda Goulart Nogueira da Silva et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.
