Mayer-Rokitansky-Küster-Hauser症候群に対する造腟術後に両側付属器腫瘍を認め腹腔鏡下手術を施行した１例

抄録

　Mayer-Rokitansky-Küster-Hauser syndrome (MRKH syndrome) is a congenital disorder characterized by uterine and vaginal hypoplasia or defects due to Müllerian duct dysplasia. We report a case of MRKH syndrome in which a patient underwent laparoscopic surgery for bilateral adnexal tumors 20 years after laparoscopic-assisted vaginoplasty, and it was a rare case in that we could observe the intraperitoneal after the vaginoplasty. The patient was diagnosed with MRKH syndrome type I at the age of 20 and underwent a modified laparoscopic Davydov procedure at our hospital. At the age of 40, she had right lower abdominal pain, and a CT scan revealed suspected ovarian tumor. Multiple 10 cm-sized cysts were found in the pelvis. Intra-abdominal findings showed that the left ovary was normal with a 7-cm large monocystic paraovarian cyst, and the right ovary had multiple 7-cm large cysts. A white scar was seen at the peritoneal suture site at the time of vaginoplasty, and there was a mild adhesion to the rectum. A right salpingo-oophorectomy and a left salpingectomy were performed. The operative time was 122 minutes, and blood loss was 20 mL. Histopathological diagnoses were left paraovarian cyst and right serous cystadenoma. The degree of structural abnormality in MRKH syndrome is variable. We approached surgery with the possibility that the anatomy of the adnexa might be different from the usual structure because of the effects of vaginoplasty, but in this case the anatomy of the adnexa was preserved, and the surgery could be performed as usual.