Am J Hum Genet. 1988 Nov;43(5):726-32.

Dural ectasia is a common feature of the Marfan syndrome.

Pyeritz RE, Fishman EK, Bernhardt BA, Siegelman SS.

Source

Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD 21205.

Abstract

Widening of the lumbosacral spinal canal was found in 63% of 57 patients with the Marfan syndrome and in none of 57 age- and sex-matched non-Marfan control patients, who underwent CT scanning for routine clinical indications. The bony abnormalities in mild cases consisted of thinning of the pedicles and taminae and erosion of the neural foraminae and were generally limited to L5 and S1. More severe changes were present in 13 patients, two of whom had associated neurologic signs, and included meningoceles or near total erosion of a pedicle. Presence and severity of vertebral abnormalities were associated with neither any other clinical feature nor overall phenotypic severity. Dural ectasia can be added to the list of pleiotropic manifestations of the Marfan syndrome.

PMID:: 3189335; [PubMed - indexed for MEDLINE]
PMCID:: PMC1715546

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Research Support, U.S. Gov't, P.H.S.

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RR 00722/RR/NCRR NIH HHS/United States

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