| Spinal abnormalities and the atypical form of the Mayer-Rokitansky-Küster-Hauser syndrome. | |
| | |
MedLine Citation:
|
PMID: 1294137 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
|
In 96 patients with congenital absence of the uterus and upper vagina, the Mayer-Rokitansky-Küster-Hauser (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. |
| | |
Authors:
|
E H Strübbe; J A Lemmens; C J Thijn; W N Willemsen; B S van Toor |
Related Documents
:
|
8577497 - Unusual radiological manifestations of lemierre's syndrome: a case report. 17159757 - Anesthetic management of klippel-trénaunay syndrome and attendant gastrointestinal hem... 1123177 - Liver ultrastructure in gilbert's syndrome. 11579407 - Prenatal sonographic diagnosis of klippel-trénaunay-weber syndrome with cardiac failure. 19804087 - Short qt syndrome. 2341447 - Congenital snapping knee. habitual anterior subluxation of the tibia in extension. |
Publication Detail:
|
Type: Journal Article |
Journal Detail:
|
Title: Skeletal radiology Volume: 21 ISSN: 0364-2348 ISO Abbreviation: Skeletal Radiol. Publication Date: 1992 |
Date Detail:
|
Created Date: 1992-12-04 Completed Date: 1992-12-04 Revised Date: 2004-11-17 |
Medline Journal Info:
|
Nlm Unique ID: 7701953 Medline TA: Skeletal Radiol Country: GERMANY |
Other Details:
|
Languages: eng Pagination: 459-62 Citation Subset: IM |
Affiliation:
|
Department of Radiology, Rijnstate Hospital, Arnhem, The Netherlands. |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
Adolescent Adult Classification Female Humans Kidney / abnormalities Klippel-Feil Syndrome / classification, pathology Middle Aged Mullerian Ducts / abnormalities, pathology Scoliosis / pathology Spina Bifida Occulta / pathology Spine / abnormalities* Syndrome Uterus / abnormalities*, pathology Vagina / abnormalities*, pathology |
| Comments/Corrections | |
Erratum In:
|
Skeletal Radiol 1993;22(2):120 |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Isolation, mapping, and characterization of two cDNA clones expressed in the cerebellum.
Next Document: Docking of dogs: practical and ethical aspects.