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

Abstract

The Mayer-Rokitansky-Küster-Hauser syndrome is the congenital absence or underdevelopment of the uterus and vagina even though the external genitalia, ovaries and ovarian function are normal. This condition is uncommon in Cameroon. A 23-year-old woman of the Fulbé tribe, a predominantly Islamic tribe of the northern part of Cameroon, complained of the absence of menstruation after age of puberty and lower abdominal pain occurring almost at the same period every month. She has been married polygamously for 10 years and has been having normal, satisfactory sexual intercourse. The sonographic and laparoscopic findings of this patient were consistent with Mayer-Rokitansky-Küster-Hauser syndrome. The patient was counseled for in vitro fertilization and surrogacy. Patients with Mayer-Rokitansky-Küster-Hauser syndrome typically present with primary amenorrhea during adolescence. With the existing medical technology in Cameroon, this condition is easily accessible in tertiary healthcare facilities. Patients with Mayer-Rokitansky-Küster-Hauser syndrome could become mothers through in vitro fertilization and surrogacy, but the cost is prohibitive in Cameroon.

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

“…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.…”

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

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

“…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.…”

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

“…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 ].…”