• Childhood Kidney Diseases
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• 제10권2호
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• pp.233-237
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• 2006

MRKH 증후군은 비뇨생식기계 및 골격계에 기형을 동반하지만 쇄항에 대한 동반 기형유무 검사상에서 MRKH 증후군으로 진단된 경우는 거의 보고되고 있지 않다. 이에 저자들은 쇄항을 주소로 내원한 34주 미숙아에서 MRKH 증후군으로 진단된 본 증례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• Clinical and Experimental Reproductive Medicine
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• 제30권2호
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• pp.179-184
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• 2003

The Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome refers to a condition which presents as normal female secondary sex characteristics, normal external genitalia, congenital absence of the internal vagina, usually a rudimentary uterus in the form of bilateral noncanaliculated muscular buds, and normal tubes and ovaries with normal cytogenetic and endocrine evaluation, frequent association of renal, skeletal and other congenital anomalies. However, rarely, whole uterus or a segment of uterus may be present, but lacking a conduit to the introitus. If a partial endometial cavity is present in a segment of uterus, cyclic abdominal pain may be a complaint and furthermore endometiosis can be developed. Recently, we experienced a case of MRKH syndrome with the segments of uterus accompanying endometriosis in young woman. We present this case with a brief review of literatures.

• Archives of Plastic Surgery
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• 제38권3호
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• pp.333-337
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• 2011

Purpose: Various operations have been proposed to compensate for congenital absence of the vagina using ileal or colonic interposition. These methods involve laparotomy, which shows postoperative complications such as long scar and delayed recovery. One case of neovagina reconstruction with laparoscopic rectosigmoid colpopoiesis in Mayer-Rokitansky-Kuster-Hauser syndrome is presented to avoid laparotomic complications. Methods: Laparoscopic surgery was performed in a 27-year-old MRKH syndrome patient. After a cruciate incision, blunt dissection through two-finger wide space was created between the bladder and the rectum. A 14-cm rectosigmoid segment vascularized by a branch of sigmoid artery was isolated by laparoscopy. The distal end was sutured with vaginal vestibule mucosa. A continuity of intestine was restored by circular end-to-end proximate curved intraluminal stapler CDH29$^{(R)}$ through perineal opening. Results: Total operation time was 4 hr 15 min. Normal walking and ingestion were possible within 3 days and 4 days after surgery. The hospital stay was 7 days and the patient was followed up for 6 months. The neovaginal introitus was wide enough for inserting two fingers, and there has been no narrowing of the neovagina on palpation as confirmed by vaginogram. The patient had functional self-lubricating neovagina without excessive mucous production or the need for routine dilation or unnoticeable scar. Conclusion: The successful result of this laparoscopic vaginal reconstruction technique with rectosigmoid segment suggests that this technique can be considered for the option of vaginal reconstruction in girls with the MRKH syndrome.

• Archives of Plastic Surgery
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• 제48권2호
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• pp.185-188
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• 2021

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome can be treated through numerous surgical and nonsurgical methods. We present a surgical technique in which a neovagina was reconstructed and shaped by a vaginal expander with acellular porcine dermal matrix (XCM Biologic Tissue Matrix) and mucosal interposition using microfragments harvested from the hymen. In our case, we found this procedure to be safe and effective, resulting in satisfactory sexual function and good cosmetic results, without donor site morbidity. To our best knowledge, this tissue-engineered biomaterial has never been used for vaginal reconstruction before.