A case of Mayer Rokitansky Kuster Hauser syndrome

Abstract

Purpose & study object: We herewith present a case of unusual mullerian duct anomaly presenting with primary amenorrhea and some Past history suggestive of hymenotomy (details not available). Introduction: Mullerian duct anomaly is approximately 6.7% in general population. Incidence is higher in infertility 7.3% and recurrent miscarriages 16%. We present here a case of MRKH syndrome, which is a Abnormality of development of the female genital tract: partial or complete absence (agenesis) of the uterus, absent or hypoplastic vagina, normal fallopian tubes, ovaries, normal external genitalia. Material & Method: Basic investigation of USG was performed which revealed unicornuate uterus, elongated tapered structures suggestive of Rt. Hydrosalpinx and normal ovaries. To evaluate further communication between uterus and vagina, CT scan & MRI were performed. Graded C.T. cystogram also showed a false tract like structure going towards perineum. Since these findings were inadequate for further management and treatment – diagnostic laparoscopy and cystoscopy was performed. Conclusion: There was unicornuate uterus with rt. tube showing hydrosalpinx with blood collection, absent cervix and absent proximal vagina. These findings were confirmed with help of diagnostic laproscopy and cystoscopy.