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Mayer-Rokitansky-Küester-Hauser (MRKH) syndrome with rectovestibular fistula and imperforate anus.
MedLine Citation:
PMID:  20656398     Owner:  NLM     Status:  In-Process    
Abstract/OtherAbstract:
OBJECTIVES: The Mayer-Rokitansky-Küester-Hauser (MRKH) syndrome can be either isolated uterovaginal agenesis or associated with other organ anomalies. However, MRKH co-existing with rectovestibular fistula and imperforate anus is uncommonly seen. We present the specific clinical manifestation and discuss the diagnosis and treatment for this unique subtype of MRKH.
STUDY DESIGN: We reviewed 133 cases with MRKH admitted in our hospital within a 10-year span. Among them, three cases of uterovaginal agenesis concomitant with rectovestibular fistula and imperforate anus were reported in detail.
RESULTS: This scenario of MRKH with rectovestibular fistula was characterized by the common features of two openings in the perineum along with an imperforate anus. Our patients were older girls whose main complaints were of primary anomerrhea and leakage of loose stool. A one-stage repair of anorectovaginoplasty was used in two patients, and with laparoscopic assistance in one case. Thirty-two similar cases in the previous relevant literature were also systematically reviewed.
CONCLUSION: MRKH with rectovestibular fistula and imperforate anus is extremely rare. A thorough understanding of this unique entity helps establish the correct and timely diagnosis, and also avoids inappropriate operative treatment.
Authors:
Shu Wang; Jing He Lang; Lan Zhu
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Publication Detail:
Type:  Journal Article     Date:  2010-07-24
Journal Detail:
Title:  European journal of obstetrics, gynecology, and reproductive biology     Volume:  153     ISSN:  1872-7654     ISO Abbreviation:  Eur. J. Obstet. Gynecol. Reprod. Biol.     Publication Date:  2010 Nov 
Date Detail:
Created Date:  2010-10-22     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0375672     Medline TA:  Eur J Obstet Gynecol Reprod Biol     Country:  Ireland    
Other Details:
Languages:  eng     Pagination:  77-80     Citation Subset:  IM    
Copyright Information:
Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.
Affiliation:
Department of Obstetrics and Gynecology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Science, Beijing 100730, PR China.
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