A case of Mayer-Rokitansky-Küster-Hauser syndrome in a low-resource tertiary hospital in Douala, Cameroon

Egbe, Thomas Obinchemti; Kobenge, Fidelia Mbi; Arlette, Metogo Mbengono Junette; Nyemb, Jacque-Ernest; Mbu, Robinson Enow

doi:10.1177/2050313x19830817

Cited by 4 publications

(4 citation statements)

References 16 publications

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“…A link between a loss-offunction mutation in the WNT4 gene and MRKH has been described in the medical literature. 11,12 We present a case of Mullerian agenesis in a patient with a biopsy-confirmed large fibroid tumor, from the vestigial Mullerian remnant that grew large enough to obstruct her only right kidney resulting in dialysis dependency. Our patient who had worsening renal function, solitary kidney, amenorrhea, and a pelvic mass was incidentally discovered to have MRKH with leiomyoma at index presentation with no documented history of MRKH.…”

Section: Discussionmentioning

confidence: 99%

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Incidental Finding of Leiomyoma in Mayer-Rokitansky-Kuster-Hauser Syndrome

Ibidapo-Obe

Okudo

Filani

2021

Journal of Investigative Medicine High Impact Case Reports

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Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a sexual developmental disorder. In this disorder, there is a congenital absence of the uterus and vagina with normal external genitalia. The etiology is not well understood. Variations of this condition exist that may include congenital abnormalities and psychological problems. In this article, we discuss the case of a 47-year-old African American female who presented with acute renal failure, solitary right kidney, and a pelvic mass extending from the pelvis to the right hypochondrium determined to be a fibroid. The patient was managed by a multidisciplinary team, dialyzed, and planned for removal of the mass. While understanding the low probability of having fibroids without a uterus, fibroids should not be excluded from such patients. It is also important to consider the emotional and psychological well-being of such patients.

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Section: Discussionmentioning

confidence: 99%

“…A link between a loss-of-function mutation in the WNT4 gene and MRKH has been described in the medical literature. 11 , 12 …”

Section: Discussionmentioning

confidence: 99%

Incidental Finding of Leiomyoma in Mayer-Rokitansky-Kuster-Hauser Syndrome

Ibidapo-Obe

Okudo

Filani

2021

Journal of Investigative Medicine High Impact Case Reports

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“…In addition, the patient's care remains holistic, taking into account the psychosocial aspects, vaginoplasty to improve sexual practice and the possibility of medical assistance, the cost of which is prohibitive in our country. 4,16,17 CONCLUSION MRKHS syndrome is a rare condition. The association with a voluminous ovarian tumor is even rarer, if not exceptional.…”

Section: Treatmentmentioning

confidence: 99%

Mayer-Rokitansky-Kuster-Hauser syndrome and ovarian benign teratoma: a case report

Adjoby¹,

Koffi²,

Effoh³

et al. 2020

Int J Reprod Contracept Obstet Gynecol

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Mayer-Rokitansky-Kuster-Hauser syndrome (MRKH) is an unknown congenital etiology disorder characterized by agenesia or hypoplasia of the Müller ductal system, including the upper vagina, uterus and fallopian tubes. The occurrence of an associated ovarian tumor is rare, with fewer than 20 cases reported to date according to the literature. We report the case of a 14-year-old girl, virgin, who had not yet seen her menarche, complaining of an abdomino-pelvic mass associated with pain. The ultrasound performed revealed a large left ovarian tumor and an absence of uterus. The indication of a laparotomy confirmed the ovarian mass and a complete absence of uterus associated with vaginal hypoplasia. The contralateral ovary was present, and of normal appearance. The pathological examination was in favor of a mature benign multi-tissular teratoma. This is the first case described in our service. The mode of transmission of this entity appears to be autosomal dominant with low penetrance and variable expressivity, suggesting that the incidence of this syndrome is likely underestimated. With the development of techniques of medical assistance to procreation, maternity remains possible, particularly through gestational surrogacy.

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“…The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is not very much documented in most African countries. In Cameroon, a few studies have focused on creating awareness on the existence of this syndrome and giving orientations on how such a case can be handled [ 1 ], others have depicted the experience of such women in suburban areas while showing the psychological distress these women go through [ 2 ]. Also known as Mullerian agenesis, this syndrome is the situation where the Mullerian canals responsible for the formation of the uterus and the vagina fail to develop at the 6 th month of embryogenesis, thereby causing a girl to be born with either one of these organs or none at all [ 3 ].…”

Section: Introductionmentioning

confidence: 99%

How does the experience of the medical encounter of women with the Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome impacts on them in Africa?

Ngoumou¹

2022

Pan Afr Med J

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Introduction the MRKH syndrome is a rare congenital malformation in women. As most rare conditions, this syndrome usually causes confusion in patients and even in physicians. The medical encounter of young girls with the syndrome is intertwined in a complex matrix of social, economic and cultural factors that only contribute in creating more confusion in the young girl. Methods the study is qualitative and designed to describe the first encounter of women with the MRKH syndrome at the physician. To that effect, an interview guide was designed for in-depth interviews with 05 MRKH women from varied African countries (Cameroon, Côte-d’Ivoire, and Senegal) selected using the snowball technique. Results findings from this study revealed that most women go to the hospital and return more confused as no clear information is given to them on their condition. Also, cultural, social and religious beliefs on the one hand seems to blur the physician’s judgment to provide appropriate remedies like instrumental dilatation for rudimentary vagina, surrogacy for uterine infertility factor, adoption, etc. and on the other hand, these beliefs also act as a barrier for these young girls who are not strangers to the culture. Conclusion the study suggests that, physicians give to the patients all necessary information for them to take elective decisions on their health. It also suggests that awareness should be raised on this condition.

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A case of Mayer-Rokitansky-Küster-Hauser syndrome in a low-resource tertiary hospital in Douala, Cameroon

Cited by 4 publications

References 16 publications

Incidental Finding of Leiomyoma in Mayer-Rokitansky-Kuster-Hauser Syndrome

Incidental Finding of Leiomyoma in Mayer-Rokitansky-Kuster-Hauser Syndrome

Mayer-Rokitansky-Kuster-Hauser syndrome and ovarian benign teratoma: a case report

How does the experience of the medical encounter of women with the Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome impacts on them in Africa?

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