Connective tissue disorders with spontaneous spinal cerebrospinal fluid leaks an

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Neurosurgery. 2004 Jan;54(1):65-70; discussion 70-1. Related

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Connective tissue disorders with spontaneous spinal cerebrospinal

fluid leaks and intracranial hypotension: a prospective study.

Schievink WI, Gordon OK, Tourje J.

Maxine Dunitz Neurosurgical Institute, Cedars-Sinai Medical Center,

Los Angeles, California 90048, USA. schievinkw@...

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, 22-

55 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.

PMID: 14683542 [PubMed - indexed for MEDLINE]