Normogonadotropik primer amenore nedeni; fibrodisplazi ossifikans progresiva Normogonadotropik primary amenorrhea; fibrodysplasia ossificans progressiva

Objectives: Fibrodysplasia ossificans progressiva (FOP) is a severely disabling heritable disorder of connective tissue characterized by congenital malformations of the great toes and progressive heterotopic ossification that forms qualitatively normal bone in characteristic extraskeletal sites. In the literature, two women with FOP had live birth an one woman had voluntery abortion. In our case, we report an 18 years old girl with FOP who suffered from primer amenorrhea. The affect of FOP to the women reproductive system has not been understood yet by time maybe FOP may take place between the reasons of primer amenorrhea. Case: An 18 years old, virgin patient who had the diagnosis of FOP applied to our hospital suffering from amenorrhea. Although the normal development of sekonder sexuel development she had no menstruel bleeding. With the 10 days of intervals, totally 6 hormonal measurements were performed and the results were in normal range. Neither pelvic examination nor pelvic ultrasonography determined pelvic-vaginal abnormality such as vaginal obstruction or hematocolpos. Theoretically uterus should not be affected by FOP. In our case, even the consecutive cyclic treatment for 6month the menstrual bleeding could not be started. This situation may be explained by the unknown mechanisms of FOP which can influence the menstrual cycle. Amac: Fibrodisplazi ossifikans progresiva (FOP) ilerleyici heterotropik ossifikasyonla birlikte konjenital