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Wouter I. Schievink, Reid C. Thompson, Christopher T. Loh and M. Marcel Maya

✓ An excruciating headache of instantaneous onset, or thunderclap headache, may be caused by a variety of serious disorders, including aneurysmal subarachnoid hemorrhage, cerebral venous sinus thrombosis, pituitary apoplexy, and carotid artery or vertebral artery dissection. The authors describe a patient with this type of headache who was found to have a spontaneous retroclival hematoma.

A 49-year-old woman experienced an instantaneous excruciating headache. Results of computerized tomography (CT) scans of the head were normal, but on examination of the cerebrospinal fluid xanthochromia was found. Magnetic resonance (MR) imaging of the cervical spine revealed a retroclival hematoma. Three cerebral angiographic studies did not reveal the source of the hemorrhage and a repeated MR image demonstrated resolution of the hematoma. The patient made an uneventful recovery.

Spontaneous retroclival hematoma is an exceedingly rare type of intracranial hemorrhage and may be associated with normal findings on CT scans. Spontaneous retroclival hematoma should be included in the differential diagnosis of thunderclap headache.

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Wouter I. Schievink, M. Marcel Maya and Mary Riedinger

Object. Intracranial hypotension due to a spontaneous spinal cerebrospinal fluid (CSF) leak is an increasingly recognized cause of postural headaches, but reliable follow-up data are lacking. The authors undertook a study to determine the risk of a recurrent spontaneous spinal CSF leak.

Methods. The patient population consisted of a consecutive group of 18 patients who had been evaluated for consideration of surgical repair of a spontaneous spinal CSF leak. The mean age of the 15 women and three men was 38 years (range 22–55 years). The mean duration of follow up was 36 months (range 6–132 months). The total follow-up time was 654 months. A recurrent spinal CSF leak was defined on the basis of computerized tomography myelography evidence of a CSF leak in a previously visualized but unaffected spinal location. Five patients (28%) developed a recurrent spinal CSF leak; the mean age of these four women and one man was 36 years. A recurrent CSF leak developed in five (38%) of 13 patients who had undergone surgical CSF leak repair, compared with none (0%) of five patients who had been treated non-surgically (p = 0.249). The recurrent leak occurred between 10 and 77 months after the initial CSF leak, but within 2 or 3 months of successful surgical repair of the leak in all patients.

Conclusions. Recurrent spontaneous spinal CSF leaks are not rare, and the recent successful repair of such a leak at another site may be an important risk factor.

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Wouter I. Schievink, M. Marcel Maya and James Tourje

Object. Spontaneous intracranial hypotension due to a spinal cerebrospinal fluid (CSF) leak is an important cause of new daily persistent headaches. Spinal neuroimaging is important in the treatment of these patients, particularly when direct repair of the CSF leak is contemplated. Retrospinal C1–2 fluid collections may be noted on spinal imaging and these are generally believed to correspond to the site of the CSF leak. The authors undertook a study to determine the significance of these C1–2 fluid collections.

Methods. The patient population consisted of a consecutive group of 25 patients (18 female and seven male) who were evaluated for surgical repair of a spontaneous spinal CSF leak. The mean age of the 18 patients was 38 years (range 13–72 years). All patients underwent computerized tomography myelography. Three patients (12%) had extensive retrospinal C1–2 fluid collections; the mean age of this woman and these two men was 41 years (range 39–43 years). The actual site of the CSF leak was located at the lower cervical spine in these patients and did not correspond to the site of the retrospinal C1–2 fluid collection.

Conclusions. A retrospinal fluid collection at the C1–2 level does not necessarily indicate the site of the CSF leak in patients with spontaneous intracranial hypotension. This is an important consideration in the treatment of these patients because therapy may be inadvertently directed at this site.

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Wouter I. Schievink, M. Marcel Maya and Franklin M. Moser

✓ Spontaneous intracranial hypotension due to a cerebrospinal fluid (CSF) leak in the spine is an important cause of new, daily persistent headaches. Most patients respond well to conservative treatments including epidural blood patching. Limited options for effective treatment are available for patients in whom these treatments fail. The authors treated four patients (mean age 38 years; range 26–43 years) with percutaneous placement of a fibrin sealant. All these patients presented with intractable positional headaches. The CSF leak was located in the lower cervical spine in three patients and in the lower thoracic spine in one patient. Four to 20 milliliters of fibrin sealant was injected at the site of the CSF leak. Two of the four patients became asymptomatic within days of the procedure and thus avoided surgery. There were no complications of this procedure. Percutaneous placement of a fibrin sealant is a safe, minimally invasive treatment for spontaneous spinal CSF leaks and should be considered in patients in whom conservative treatment has failed.

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Wouter I. Schievink, M. Marcel Maya, Franklin G. Moser and James Tourje

Object. Spontaneous intracranial hypotension is a noteworthy but commonly misdiagnosed cause of new daily persistent headaches. Subdural fluid collections are frequent radiographic findings, but they can be interpreted as primary rather than secondary pathological entities, and uncertainties exist regarding their optimal management. The authors therefore reviewed their experience with subdural fluid collections in 40 consecutive patients with spontaneous spinal cerebrospinal fluid (CSF) leaks and intracranial hypotension.

Methods. The mean age of the 26 female and 14 male patients was 43 years (range 13–72 years). Subdural fluid collections were present in 20 patients (50%); 12 of these patients (60%) had subdural hygromas alone, and eight (40%) had subacute to chronic subdural hematomas (SDHs) associated with significant mass effect. The subdural hygromas resolved within several days to weeks following treatment of the underlying CSF leak. Three patients with SDHs underwent evacuation of the hematoma prior to the establishment of the diagnosis of spontaneous intracranial hypotension, but the SDHs did not resolve until the underlying spinal CSF leak was treated. In the remaining five patients, the CSF leak was treated primarily and the SDHs resolved over a 1- to 3-month period without the need for evacuation.

Conclusions. Subdural fluid collections are common in spontaneous intracranial hypotension, varying in appearance from thin subdural hygromas to large SDHs associated with significant mass effect. These collections can be safely managed by directing treatment at the underlying CSF leak without the need for hematoma evacuation.

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Daniel J. Hoh, Marcel Maya, Alexander Jung, Skorn Ponrartana and Carl L. Lauryssen

Object

Various C1–2 instrumentation techniques have been developed to treat atlantoaxial instability. Screw fixation of C1–2 poses a risk of injury to the vertebral artery and internal carotid artery (ICA). Injury to the ICA caused by C-1 screws is extremely rare, but has been described. To characterize this risk, the authors studied the anatomical relationship of the ICA to the lateral mass of C-1.

Methods

The authors studied 100 patients who had undergone computed tomography scanning and magnetic resonance imaging of the neck to assess the position of the ICA in association with the C-1 lateral mass. Each ICA was classified into 1 of the following 4 zones: Zone 1 (medial to lateral mass), Zone 2 (medial half of lateral mass), Zone 3 (lateral half of lateral mass), and Zone 4 (lateral to lateral mass). For patients with an ICA ventral to the lateral mass, the shortest distance between the ICA and lateral mass was measured to determine the margin of error with an overpenetrated bicortical screw.

Results

Of the 100 patients, 58% had a left ICA in Zones 2 and 3 with a mean distance from the anterior cortex of 3.5 ± 1.5 mm (± standard deviation), and 74% had a right ICA in Zones 2 and 3 with a mean distance from the anterior cortex of 3.9 ± 1.6 mm. Both ICAs anterior to the lateral mass were noted in 47% of patients, and 84% had ≥ 1 ICA anterior to the lateral mass. When the ICA was anterior to the lateral mass, it was more commonly in the lateral half (left ICA in 91% and right ICA in 92%). The left ICA was in Zone 1 in 1% and Zone 4 in 41%. The right ICA was in Zone 1 in 1% and Zone 4 in 25%.

Conclusions

A high percentage of patients demonstrate an ICA directly ventral to the C-1 lateral mass, which poses a risk of ICA injury caused by an overpenetrated bicortical screw.

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Wouter I. Schievink, David Palestrant, M. Marcel Maya and George Rappard

Spontaneous spinal CSF leaks are best known as a cause of orthostatic headache, but may also be the cause of coma. The authors encountered a unique case of a spontaneous spinal CSF leak causing coma 2 days after craniotomy for clipping of an unruptured aneurysm. This 44-year-old woman with autosomal dominant polycystic kidney disease underwent an uneventful craniotomy for an incidental anterior choroidal artery aneurysm. No intraoperative spinal CSF drainage was used. Two days after surgery the patient became comatose with a left oculomotor nerve palsy. Computed tomography scanning revealed a right extraceberal hematoma and loss of gray–white matter differentiation. The hematoma was evacuated and a diagnosis of hemodialysis disequilibrium syndrome was made. Continuous hemodialysis and hyperosmolar therapy were instituted without any improvement. The CT scans were then reinterpreted as showing sagging of the brain, and the patient was placed in the Trendelenburg position which resulted in prompt improvement in her level of consciousness. A CT myelogram demonstrated an upper thoracic CSF leak that eventually required surgical correction. The patient made a complete neurological recovery. Neurological deterioration after craniotomy may be caused by brain sagging caused by a spontaneous spinal CSF leak, similar to intracranial hypotension due to intraoperative lumbar CSF drainage.

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Parham Moftakhar, Moise Danielpour, Marcel Maya and Michael J. Alexander

Vein of Galen malformations are rare congenital intracranial vascular malformations. Based on reports in the literature, spontaneous thrombosis or regression of these lesions is rare. Patients have variable outcomes from an asymptomatic course to death. The reasons behind spontaneous thrombosis are not entirely understood. Here the authors present a case of an infant diagnosed with a vein of Galen malformation in utero that subsequently went on to thrombose or regress. A review of the published cases on this phenomenon and the potential causality are discussed.

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Wouter I. Schievink, M. Marcel Maya, Brian K. Pikul and Charles Louy

Subdural hematoma is a relatively common complication of long-term anticoagulation, particularly in the elderly. The combination of anticoagulation and cerebral cortical atrophy is believed to be sufficient to explain the subdural bleeding. The authors report a series of elderly patients who were on a regimen of anticoagulation and developed chronic subdural hematomas (SDHs) due to a spontaneous spinal CSF leak. They reviewed the medical records and imaging studies of a consecutive group of patients with spontaneous intracranial hypotension who were evaluated at Cedars-Sinai Medical Center. Among 141 patients with spontaneous spinal CSF leaks and spontaneous intracranial hypotension, 3 (2%) were taking anticoagulants at the time of onset of symptoms. The mean age of the 3 patients (1 woman and 2 men) was 74 years (range 68–86 years). All 3 patients had chronic SDHs measuring between 12 and 23 mm in maximal diameter. The SDHs resolved after treatment of the underlying spontaneous spinal CSF leak, and there was no need for hematoma evacuation. Epidural blood patches were used in 2 patients, and percutaneous placement of a fibrin sealant was used in 1 patient. The presence of an underlying spontaneous spinal CSF leak should be considered in patients with chronic SDHs, even among the elderly taking anticoagulants.