Harzif Achmad Kemal, Ambalagen Sonia, Charilda Fistyanisa Elya, Mutia Heidi Dewi
Division of Reproductive Immuno-Endocrinology, Department of Obstetrics and Gynecology, Faculty of Medicine Universitas Indonesia, Dr. Cipto Mangunkusumo Hospital Jakarta, Indonesia.
Department of Obstetrics and Gynecology, Dr. Cipto Mangunkusumo Hospital Jakarta, Faculty of Medicine Universitas Indonesia, Pangeran Diponegoro Street No. 71, Kenari, Jakarta, Indonesia.
Int J Surg Case Rep. 2021 Apr;81:105711. doi: 10.1016/j.ijscr.2021.105711. Epub 2021 Mar 4.
Mayer Rokitansky Kuster Hauser (MRKH) syndrome is a congenital disorder involving reproductive, genitourinary, bone, and cardiac malformation. The incidence is 1 in 4000-5000 females livebirths. The phenotype is female 46 XX karyotype, normal secondary sexual characteristics, and normal functional ovaries. The occurrence of leiomyoma in uterine remnant in MRKH syndrome is a very rare case, even though several cases have been reported. The diagnosis and management approach, in this case, is quite challenging. Here, we report a 38 years old female who represents multiple leiomyomas on the rudimentary uterus, then we did laparoscopic removal of the fibroids and adjacent rudimentary uterus.
迈耶-罗基坦斯基-库斯特-豪泽（MRKH）综合征是一种先天性疾病，涉及生殖、泌尿生殖、骨骼和心脏畸形。发病率为每4000-5000例女性活产中有1例。其表型为女性46 XX核型、正常的第二性征和正常功能的卵巢。尽管已有数例报道，但MRKH综合征子宫残迹中发生平滑肌瘤是非常罕见的病例。在这种情况下，诊断和管理方法颇具挑战性。在此，我们报告一名38岁女性，其残角子宫上有多个平滑肌瘤，随后我们通过腹腔镜切除了肌瘤及相邻的残角子宫。
