Abstract
Congenital absence of uterus and vagina, the Mayer-Rokitansky-Küster-Hauser Syndrome
(MRKHS), results from defective müllerian duct development during female embryogenesis;
it is the second most common cause of primary amenorrhea. Atypical forms of MRKHS
(type B) represent a heterogeneous group of disorders with associated anomalies of
other organ systems which frequently includes the renal and skeletal systems and several
individually occurring malformations.
We report two cases with MRKHS in which we diagnosed situs inversus totalis incidentally
during radiologic examinations. Abdominal situs inversus describes the mirror-image arrangement of the intra-abdominal organs in the abdominal
cavity and it is characterized by the presence of multiple congenital anomalies.
In this report we attempt to question whether the association between MRKHS and situs
inversus is a rare feature of the müllerian dysgenetic spectrum or whether it is the
result of random association.
Key Words
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Article info
Publication history
Published online: April 09, 2009
Identification
Copyright
© 2009 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.
