Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension

Abstract

Spinal cerebrospinal fluid (CSF) leaks are often implicated as the cause of the syndrome of spontaneous intracranial hypotension, but they have rarely been demonstrated radiographically or surgically. The authors reviewed their experience with documented cases of spinal CSF leaks of spontaneous onset in 11 patients including their surgical observations in four of the patients. The mean age of the six women and five men included in the study was 38 years (range 22-51 years). All patients presented with a postural headache; however, most had additional symptoms, including nausea, emesis, sixth cranial-nerve paresis, or local back pain at the level of the CSF leak. All patients underwent indium-111 radionucleotide cisternography or computerized tomographic (CT) myelography. The location of the spontaneous CSF leak was in the cervical spine in two patients, the cervicothoracic junction in three patients, the thoracic spine in five patients, and the lumbar spine in one patient. The false negative rate for radionucleotide cisternography was high (30%). Subdural fluid collections, meningeal enhancement, and downward displacement of the cerebellum, resembling a Chiari I malformation, were commonly found on cranial imaging studies. In most patients, the symptoms resolved in response to supportive measures or an epidural blood patch. Leaking meningeal diverticula were found to be the cause of the CSF leak in four patients who underwent surgery. In three patients these diverticula could be ligated with good result but in one patient an extensive complex of meningeal diverticula was found to be inoperable. Two patients had an unusual body habitus and joint hypermobility, and two other patients had suffered a spontaneous retinal detachment at a young age. In conclusion, spontaneous spinal CSF leaks are uncommon, but they are increasingly recognized as a cause of spontaneous intracranial hypotension. Most spinal CSF leaks are located at the cervicothoracic junction or in the thoracic spine, and they may be associated with meningeal diverticula. The radiographic study of choice is CT myelography. The disease is usually self-limiting, but in selected cases our experience with surgical ligation of leaking meningeal diverticula has been satisfactory. An underlying connective tissue disorder may be present in some patients with a spontaneous spinal CSF leak.

Frequency of spontaneous intracranial hypotension in the emergency department

“…Spontaneous intracranial hypotension is an important cause of new daily persistent headaches, particularly among young and middle-aged adults [1]. Spontaneous intracranial hypotension is caused by a spontaneous spinal cerebrospinal fluid (CSF) leak, often in the setting of an underlying generalised connective tissue disorder [1].…”

“…Spontaneous intracranial hypotension is caused by a spontaneous spinal cerebrospinal fluid (CSF) leak, often in the setting of an underlying generalised connective tissue disorder [1]. The clinical hallmark of spontaneous intracranial hypotension is the presence of a headache that is exacerbated by the upright position and relieved by ***recumbancy.…”

“…The headache of spontaneous intracranial hypotension is not always orthostatic and various other headache patterns have been described. The onset of headache may be acute, mimicking a subarachnoid haemorrhage (SAH) [1]. In addition to headache, a wide variety of associated symptoms has been reported, such as neck pain, photo-or phonophobia, diplopia, parkinsonism and even coma.…”

“…Typical imaging features on computed tomography (CT) or magnetic resonance imaging (MRI) also may contribute to a diagnostic challenge with sagging of the brain mimicking a Chiari malformation, enhancement of the pachymeninges mimicking infectious or neoplastic disease, subdural fluid collections mimicking a primary subdural haematoma, and pituitary hyperaemia mimicking a pituitary tumour [1]. Because opening pressure generally is low in these patients, a traumatic tap is common, further causing confusion with SAH [1]. Treatment for the underlying spontaneous spinal CSF leak includes bed rest and hydration, epidural blood patching, percutaneous injection of fibrin glue and surgical repair [1].…”

“…Because opening pressure generally is low in these patients, a traumatic tap is common, further causing confusion with SAH [1]. Treatment for the underlying spontaneous spinal CSF leak includes bed rest and hydration, epidural blood patching, percutaneous injection of fibrin glue and surgical repair [1].…”