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.
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- Chu N. S. Marfan's syndrome and epilepsy: report of two cases and review of the literature. Epilepsia. 1983 Feb;24(1):49–55. doi: 10.1111/j.1528-1157.1983.tb04865.x. [DOI] [PubMed] [Google Scholar]
- Cilluffo J. M., Gomez M. R., Reese D. F., Onofrio B. M., Miller R. H. Idiopathic ("congenital") spinal arachnoid diverticula. Clinical diagnosis and surgical results. Mayo Clin Proc. 1981 Feb;56(2):93–101. [PubMed] [Google Scholar]
- Fishman E. K., Zinreich S. J., Kumar A. J., Rosenbaum A. E., Siegelman S. S. Sacral abnormalities in Marfan syndrome. J Comput Assist Tomogr. 1983 Oct;7(5):851–856. doi: 10.1097/00004728-198310000-00019. [DOI] [PubMed] [Google Scholar]
- Gott V. L., Pyeritz R. E., Magovern G. J., Jr, Cameron D. E., McKusick V. A. Surgical treatment of aneurysms of the ascending aorta in the Marfan syndrome. Results of composite-graft repair in 50 patients. N Engl J Med. 1986 Apr 24;314(17):1070–1074. doi: 10.1056/NEJM198604243141702. [DOI] [PubMed] [Google Scholar]
- Hall J. R., Pyeritz R. E., Dudgeon D. L., Haller J. A., Jr Pneumothorax in the Marfan syndrome: prevalence and therapy. Ann Thorac Surg. 1984 Jun;37(6):500–504. doi: 10.1016/s0003-4975(10)61142-3. [DOI] [PubMed] [Google Scholar]
- Mitchell G. E., Lourie H., Berne A. S. The various causes of scalloped vertebrae with notes on their pathogenesis. Radiology. 1967 Jul;89(1):67–74. doi: 10.1148/89.1.67. [DOI] [PubMed] [Google Scholar]
- Murdoch J. L., Walker B. A., Halpern B. L., Kuzma J. W., McKusick V. A. Life expectancy and causes of death in the Marfan syndrome. N Engl J Med. 1972 Apr 13;286(15):804–808. doi: 10.1056/NEJM197204132861502. [DOI] [PubMed] [Google Scholar]
- NELSON J. D. The Marfan syndrome, with special reference to congenital enlargement of the spinal canal. Br J Radiol. 1958 Oct;31(370):561–564. doi: 10.1259/0007-1285-31-370-561. [DOI] [PubMed] [Google Scholar]
- Newman P. K., Tilley P. J. Myelopathy in Marfan's syndrome. J Neurol Neurosurg Psychiatry. 1979 Feb;42(2):176–178. doi: 10.1136/jnnp.42.2.176. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Pagès M., Echenne B., Pagès A. M., Dimeglio A., Sires A. Multicore disease and Marfan's syndrome: a case report. Eur Neurol. 1985;24(3):170–175. doi: 10.1159/000115842. [DOI] [PubMed] [Google Scholar]
- Pyeritz R. E., McKusick V. A. The Marfan syndrome: diagnosis and management. N Engl J Med. 1979 Apr 5;300(14):772–777. doi: 10.1056/NEJM197904053001406. [DOI] [PubMed] [Google Scholar]
- Pyeritz R. E. The Marfan syndrome. Am Fam Physician. 1986 Dec;34(6):83–94. [PubMed] [Google Scholar]
- Strand R. D., Eisenberg H. M. Anterior sacral meningocele in association with Marfan's syndrome. Radiology. 1971 Jun;99(3):653–654. doi: 10.1148/99.3.653. [DOI] [PubMed] [Google Scholar]
- Streeten E. A., Murphy E. A., Pyeritz R. E. Pulmonary function in the Marfan syndrome. Chest. 1987 Mar;91(3):408–412. doi: 10.1378/chest.91.3.408. [DOI] [PubMed] [Google Scholar]
- Thierry A., Archimbaud J. P., Fischer G., Freidel M., Mansuy L. La méningocèle sacrée antérieure. Revue de la littérature et présentation d'un cas. Neurochirurgie. 1969 Sep-Oct;15(5):389–412. [PubMed] [Google Scholar]
- WILNER H. I., FINBY N. SKELETAL MANIFESTATIONS IN THE MARFAN SYNDROME. JAMA. 1964 Feb 15;187:490–495. doi: 10.1001/jama.1964.03060200022003. [DOI] [PubMed] [Google Scholar]
- Weir B. Leptomeningeal cysts in congenital ectopia lentis. Case report. J Neurosurg. 1973 May;38(5):650–654. doi: 10.3171/jns.1973.38.5.0650. [DOI] [PubMed] [Google Scholar]
- le Mercier Y., Decazes J. M., Mechali D., Redondo A., Aboulker J., Coulaud J. P. Méningite purulente par inoculation chirurgicale d'une méningocèle sacrée antérieure. Une complication rare de la maladie de Marfan. Ann Med Interne (Paris) 1980;131(5):289–290. [PubMed] [Google Scholar]