[Acute abdomen in a patient with Mayer-Rokitansky-Kuster-Hauser syndrome].
BACKGROUND
Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a malformation of female genital tract (incidence 1 in 4000 female newborn children). It appears as a result of a disorder in the development of Millerian cannals. Etiology is unknown. Syndrome MRKH is the most frequent cause of primary amenorrhoea (90%). Patients with MRKH have a normal female phenotype, with normal pubic hairness and thelarche, and female karyotype (46XX) followed by primary amenorrhoea. Hormonal status corresponds to healthy women, where the appearance of ovarian tumors and tumors on rudiment parts of uterus is possible.


METHODS
We presented a case of acute abdomen in a patient with previously not diagnosed MRKH. The diagnosis was done during the operation. Small pelvis and an abdominal part were filled with torquated tumor lump, where ovaries, oviducts, uterus or something resembling rudiment of uterus were not recognized through careful examination. Furthemore, the patient had a short, dead-end vagina. Tumorectomy was done and hystopathological finding showed the presence of vascular leiomyoma.


CONCLUSIONS
The diagnosis of complex syndromes, such as MRKH, can, despite modern diagnostics, be absent for non-medical and psycho-social reasons. We can expect ovarian and uterine pathology on hypoplastic structures in these patients, as well as in healthy women. Vascular leiomyoma in the patients with MRKH was not found in the available literature.
Petrić
Aleksandra
A
Klinicki centar, Ginekolosko-akuserska klinika, Nis, Srbija. sidunja@bankerinet.net
Stefanović
Milan
M
Vukomanović
Predrag
P
Zivadinović
Radomir
R
Tubić
Aleksandra
A
Janjić
Zoran
Z
D000006:Abdomen, Acute / Q000209:etiology*; D000008:Abdominal Neoplasms / Q000150:complications* / Q000473:pathology / Q000601:surgery; D018229:Angiomyoma / Q000150:complications* / Q000473:pathology / Q000601:surgery; D005260:Female; D005836:Genitalia, Female / Q000002:abnormalities*; D006801:Humans; D008875:Middle Aged; D013577:Syndrome