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Mayer-Rokitansky-Kuster-Hauser syndrome: complications, diagnosis and possible treatment options: a review.

Abstract Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a disorder in women that presents as Mullerian agenesis. These patients present internal genitalia abnormalities that include the absence of a uterus and the upper two-thirds of the vagina. In this review, current diagnostic methods, accompanying complications (congenital and psychological) and non-surgical and surgical treatments are summarized. Ultrasound and MRI have been the most documented options in MRKH syndrome diagnosis. Many women with MRKH syndrome have renal, skeletal, hearing or cardiac congenital anomalies and increased levels of psychological distress. Non-surgical interventions can be used to create a sexually functional neovagina through vaginal dilation, and surgical interventions provide alternate methods of creating a neovagina. Additionally, vaginal tissue engineering and gene therapy might provide more effective approaches in solving MRKH syndrome.
PMID
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Authors

Mayor MeshTerms
Keywords

Creatsas method

Frank’s method

Mayer–Rokitansky–Kuster–Hauser syndrome

Mullerian agenesis

Vecchietti procedure

neovagina

tissue engineering

vaginal dilation

Journal Title gynecological endocrinology : the official journal of the international society of gynecological endocrinology
Publication Year Start




PMID- 24948340
OWN - NLM
STAT- MEDLINE
DCOM- 20150514
LR  - 20140821
IS  - 1473-0766 (Electronic)
IS  - 0951-3590 (Linking)
VI  - 30
IP  - 9
DP  - 2014 Sep
TI  - Mayer-Rokitansky-Kuster-Hauser syndrome: complications, diagnosis and possible
      treatment options: a review.
PG  - 618-23
LID - 10.3109/09513590.2014.927855 [doi]
AB  - Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a disorder in women that
      presents as Mullerian agenesis. These patients present internal genitalia
      abnormalities that include the absence of a uterus and the upper two-thirds of
      the vagina. In this review, current diagnostic methods, accompanying
      complications (congenital and psychological) and non-surgical and surgical
      treatments are summarized. Ultrasound and MRI have been the most documented
      options in MRKH syndrome diagnosis. Many women with MRKH syndrome have renal,
      skeletal, hearing or cardiac congenital anomalies and increased levels of
      psychological distress. Non-surgical interventions can be used to create a
      sexually functional neovagina through vaginal dilation, and surgical
      interventions provide alternate methods of creating a neovagina. Additionally,
      vaginal tissue engineering and gene therapy might provide more effective
      approaches in solving MRKH syndrome.
FAU - Bombard, David S 2nd
AU  - Bombard DS 2nd
AD  - Pharmaceutical Research Institute, Albany College of Pharmacy and Health Sciences
      , Rensselaer, NY , USA.
FAU - Mousa, Shaker A
AU  - Mousa SA
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20140620
PL  - England
TA  - Gynecol Endocrinol
JT  - Gynecological endocrinology : the official journal of the International Society
      of Gynecological Endocrinology
JID - 8807913
RN  - Mullerian aplasia
SB  - IM
MH  - 46, XX Disorders of Sex Development/*complications/diagnosis/genetics/surgery
MH  - Congenital Abnormalities/diagnosis/genetics/surgery
MH  - Female
MH  - Humans
MH  - Mullerian Ducts/*abnormalities/surgery
MH  - Surgically-Created Structures
OTO - NOTNLM
OT  - Creatsas method
OT  - Frank's method
OT  - Mayer-Rokitansky-Kuster-Hauser syndrome
OT  - Mullerian agenesis
OT  - Vecchietti procedure
OT  - neovagina
OT  - tissue engineering
OT  - vaginal dilation
EDAT- 2014/06/21 06:00
MHDA- 2015/05/15 06:00
CRDT- 2014/06/21 06:00
PHST- 2014/06/21 06:00 [entrez]
PHST- 2014/06/21 06:00 [pubmed]
PHST- 2015/05/15 06:00 [medline]
AID - 10.3109/09513590.2014.927855 [doi]
PST - ppublish
SO  - Gynecol Endocrinol. 2014 Sep;30(9):618-23. doi: 10.3109/09513590.2014.927855.
      Epub 2014 Jun 20.