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Cormac O. Maher, Fredric B. Meyer, and Bahram Mokri

Spontaneous spinal cerebrospinal fluid (CSF) leaks are an increasingly recognized cause of intracranial hypotension. In this report the authors review the indications for surgery, surgical techniques, and surgery-related outcomes for these lesions. The major presenting symptoms include postural headaches, nausea, vomiting, and diplopia. Often, there is no history of traumatic injury. The most common cranial magnetic resonance (MR) imaging features include pachymeningeal gadolinium enhancement and sagging of the brain. On spinal MR images, diverticula are frequently noted. In cases in which symptoms are severe and refractory to less invasive measures, surgical intervention is indicated. Tears in the dura or leaking diverticula that are identified as the sources of the CSF leak often can be ligated or repaired. When a source of CSF egress is not found intraoperatively, packing the epidural space with blood-soaked Gelfoam or muscle at the appropriate level can lead to relief of symptoms. Occasionally the dural defect is large, irregular, or has attenuated borders that may not be possible to repair with sutures. These may be repaired by packing the defect with muscle or blood-soaked Gelfoam. Indications for and outcomes of surgery in patients with this condition will become more defined as surgeons gain experience with these procedures.

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Wouter I. Schievink, Fredric B. Meyer, John L. D. Atkinson, and Bahram Mokri

✓ 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.