caa a22 4500 469094672 CHVBK 20180323133027.0 cr unu---uuuuu 170328e19921001xx s 000 0 eng 10.1007/BF00190992 doi (NATIONALLICENCE)springer-10.1007/BF00190992 Spinal abnormalities and the atypical form of the Mayer-Rokitansky-Küster-Hauser syndrome [Elektronische Daten] [Ernst Strübbe, J. Lemmens, Cornelis Thijn, Wim Willemsen, Bert van Toor] In 96 patients with congenital absence of the uterus and upper vagina, the Mayer-Rokitansky-KüsterHauser (MRKH) syndrome, it proved possible to distinguish between the typical and the atypical form using laparoscopy. The typical form was characterized by symmetrical nonfunctioning muscular buds (the Müllerian duct remnants) and normal fallopian tubes, and the atypical form by aplasia of one or both buds, one bud smaller than the contralateral one, with or without dysplasia of one or both fallopian tubes. The atypical form was found in 52 patients (54.2%). Radiographs of the spine showed that congenital spinal abnormalities, especially the Klippel-Feil (KF) syndrome, were seen in 14 of the 52 patients with the atypical form only. Renal agenesis or ectopia together with the MRKH and KF syndromes, known as the MURCS association (MU: Müllerian duct aplasia; R: renal agenesis/ectopia; CS: cervical somite dysplasia), was diagnosed in 10/52 patients in the atypical group. From our results we conclude that additional cervical spine films in patients with the MRKH syndrome are indicated only in the atypical form the syndrome. In those cases where the MRKH syndrome is associated with the KF syndrome, the MURCS association should be considered. International Skeletal Society, 1992 Congenital abnormalities, genitourinary system nationallicence Congenital abnormalities, cervical spine nationallicence Laparoscopy nationallicence Radiography nationallicence Strübbe Ernst Deparment of Radiology, Rijnstate Hospital, Arnhem, The Netherlands aut Lemmens J. Department of Radiology, University Hospital Nijmegen, The Netherlands aut Thijn Cornelis Department of Radiology, University Hospital Groningen, The Netherlands aut Willemsen Wim Department of Obstetrics and Gynecology, University Hospital Nijmegen, The Netherlands aut van Toor Bert Department of Surgery, Academical Medical Center, University of Amsterdam, The Netherlands aut Skeletal Radiology Springer-Verlag 21/7(1992-10-01), 459-462 0364-2348 21:7<459 1992 21 256 https://doi.org/10.1007/BF00190992 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/BF00190992 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Strübbe Ernst Deparment of Radiology, Rijnstate Hospital, Arnhem, The Netherlands aut NATIONALLICENCE 700 1- Lemmens J. Department of Radiology, University Hospital Nijmegen, The Netherlands aut NATIONALLICENCE 700 1- Thijn Cornelis Department of Radiology, University Hospital Groningen, The Netherlands aut NATIONALLICENCE 700 1- Willemsen Wim Department of Obstetrics and Gynecology, University Hospital Nijmegen, The Netherlands aut NATIONALLICENCE 700 1- van Toor Bert Department of Surgery, Academical Medical Center, University of Amsterdam, The Netherlands aut NATIONALLICENCE 773 0- Skeletal Radiology Springer-Verlag 21/7(1992-10-01), 459-462 0364-2348 21:7<459 1992 21 256 Metadata rights reserved Springer special CC-BY-NC licence nationallicence SWISSBIB 469094672 BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer