EDS & spontaneous spinal CSF leaks

[Guest guest]

http://www.neurosurgery-online.com/abstracts/5401/NURO54010065_abs.html

Neurosurgery. January 2004, Volume 54, Number 1 pp 65-71

Connective Tissue Disorders with Spontaneous Spinal Cerebrospinal Fluid Leaks

and Intracranial Hypotension: A Prospective Study

Wouter I. Schievink, M.D.; Ora Karp Gordon, M.D.; Tourje, M.D.

Maxine Dunitz Neurosurgical Institute, Cedars-Sinai Medical Center, Los

Angeles, California GenRisk Adult Genetics Program, Cedars-Sinai Medical

Center, Los Angeles, California Imaging Medical Group, Cedars-Sinai Medical

Center, Los Angeles, California

OBJECTIVE: Intracranial hypotension attributable to a spontaneous spinal

cerebrospinal fluid (CSF) leak is an increasingly recognized cause of postural

headaches. The cause of these leaks is poorly understood, but it is likely

multifactorial and may involve a primary connective tissue disorder. We

undertook a study to estimate the contribution of systemic connective tissue

disorders to the development of spontaneous spinal CSF leaks.

METHODS: We examined a group of 18 consecutive patients with spontaneous spinal

CSF leaks for features of a connective tissue disorder.

RESULTS: The mean age of the 15 female patients and 3 male patients was 38 years

(range, 2255 yr). Seven patients (38%) demonstrated stigmata of a systemic

connective tissue disorder, and three distinct types of disorders could be

identified, as follows. 1) The association of spontaneous spinal CSF leaks and

minor skeletal features of Marfan syndrome was noted for three patients. 2)

Ehlers-Danlos syndrome Type II was noted for two patients. 3) Joint

hypermobility associated with marked attenuation of the dorsal muscular fascia,

precluding proper wound closure, was noted for two patients. In addition,

isolated small-joint hypermobility was observed for five patients (28%).

Slit-lamp ocular examinations, echocardiographic evaluations, histopathological

examinations of skin biopsy specimens, and renal scanning did not reveal any

other features of a systemic connective tissue disorder.

CONCLUSION: Findings suggesting connective tissue disorders are common among

patients with spontaneous spinal CSF leaks, and manifestations may be subtle. A

variety of disorders can be identified, probably reflecting genetic

heterogeneity. Problems with wound healing may occur as a result of the systemic

nature of the underlying connective tissue disorder.

Key words: Cerebrospinal fluid leak; Ehlers-Danlos syndrome; Headache;

Intracranial hypotension; Marfan syndrome