Diagnostic and surgical strategies for intractable spontaneous intracranial hypotension. Case report

The authors present the case of a 55-year-old man suffering from intractable spontaneous intracranial hypotension, in whom conservative treatment with 19 weeks of bed rest was not effective. In this period the patient twice underwent surgery for bilateral chronic subdural hematoma, a complication of spontaneous intracranial hypotension. Conventional radionuclide cisternography, magnetic resonance imaging, and computerized tomography myelography did not demonstrate cerebrospinal fluid (CSF) leakage. Repeated radionuclide cisternography with the patient in an upright position revealed leakage of the tracer at upper cervical levels. Computerized tomography myelography with breath holding also showed CSF leakage of the contrast medium bilaterally at upper cervical levels. The patient underwent surgery, and bilateral C-2 and C-3 spinal nerve root pouches were sealed off from the subarachnoid space with oxidized cellulose cotton and fibrin glue. Epiarachnoid spaces around the root sleeves were also sealed to ensure complete resolution of the CSF leakage. After the surgery, the patient was completely free of the disease. In the case of intractable persistent spontaneous intracranial hypotension, surgical treatment is preferable to long-term conservative management. To identify CSF leakage, radionuclide cisternography with the patient in the upright position is useful. When obvious leakage is encountered, surgical sealing of the lesion should be performed via a subarachnoid approach.     

Intracranial hypotension.

The authors present the case of a 55-year-old man suffering from intractable spontaneous intracranial hypotension, in whom conservative treatment with 19 weeks of bed rest was not effective. In this period the patient twice underwent surgery for bilateral chronic subdural hematoma, a complication of spontaneous intracranial hypotension. Conventional radionuclide cisternography, magnetic resonance imaging, and computerized tomography myelography did not demonstrate cerebrospinal fluid (CSF) leakage. Repeated radionuclide cisternography with the patient in an upright position revealed leakage of the tracer at upper cervical levels. Computerized tomography myelography with breath holding also showed CSF leakage of the contrast medium bilaterally at upper cervical levels. The patient underwent surgery, and bilateral C-2 and C-3 spinal nerve root pouches were sealed off from the subarachnoid space with oxidized cellulose cotton and fibrin glue. Epiarachnoid spaces around the root sleeves were also sealed to ensure complete resolution of the CSF leakage. After the surgery, the patient was completely free of the disease. In the case of intractable persistent spontaneous intracranial hypotension, surgical treatment is preferable to long-term conservative management. To identify CSF leakage, radionuclide cisternography with the patient in the upright position is useful. When obvious leakage is encountered, surgical sealing of the lesion should be performed via a subarachnoid approach.     

Recurrent subdural hematoma caused by cerebrospinal fluid leakage. Case report

The authors report a case of a recurrent subdural hematoma (SDH) that was caused by a persistent cerebrospinal fluid (CSF) leak from an L1-2 fistula. A 34-year-old man experienced severe headaches due to SDH, and he underwent aspiration of subdural fluid four times due to recurrent collections. Further evaluation with computerized tomography (CT) myelography demonstrated extradural extravasation of contrast through an L1-2 fistula. The patient underwent an L1-2 laminectomy; a small dural defect with CSF leakage at the left nerve root sleeve was found and was repaired. Following the repair, the patient had no further recurrence of SDH. Recurrent SDH, caused by spontaneous CSF leakage through a lumbar CSF fistula, is extremely rare. In cases of recurrent SDH, radiographic workup with spinal CT myelography should be considered.     

Cerebrospinal fluid leak demonstrated by three-dimensional computed tomographic myelography in patients with spontaneous intracranial hypotension

BACKGROUND: Precise determination of the spinal level of a cerebrospinal fluid leak is important in the diagnosis and treatment of spontaneous intracranial hypotension. The authors report two cases of SIH in which a cerebrospinal fluid leak was demonstrated by three-dimensional computed tomographic (3D-CT) myelography. CASE DESCRIPTION: By overlaying 3D-CT images of contrast-enhanced CSF and the spine, the point of leakage was clearly depicted in the three-dimensional spinal structure, which assisted targeted epidural blood patch under the guidance of fluoroscopy. Although associated chronic subdural hematoma had to be treated by burr hole drainage, the patients' postural headaches subsided after treatment. CONCLUSION: Clear CSF images in relation to vertebral bones are obtainable with 3D-CT myelography, rendering this modality very useful for the diagnosis and treatment of spontaneous intracranial hypotension.     

Recurrent spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension: a prospective study

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.     

False localizing sign of C1-2 cerebrospinal fluid leak in spontaneous intracranial hypotension

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.     

Treatment of spontaneous intracranial hypotension secondary to C-2 meningeal cyst by surgical packing--case report

A 41-year-old man presented with progressive worsening of postural headache. Computed tomography (CT) showed bilateral subdural hematomas without prior history of trauma. The diagnosis was spontaneous intracranial hypotension (SIH). Conservative treatment with oral steroids failed to prevent gradual deterioration of the patient's consciousness. CT myelography revealed massive cerebrospinal fluid (CSF) leakage between the C-1 and C-2 levels. The leak was repaired surgically via a laminectomy. A cyst, thought to be a meningeal cyst, was discovered adjacent to the right C-2 nerve root, and CSF was seen seeping out from around the cyst after a Valsalva maneuver. The presumed dural defect of the cyst was sealed by packing with muscle fragments and fibrin glue. The symptoms disappeared soon after surgery. He was discharged 1 month after surgery without deficits. Most SIH cases are benign and can be managed conservatively, or by the epidural blood patch method. Surgery is more invasive than the epidural blood patch method, but should be performed in patients with a high cervical lesion and massive CSF leakage.     

Treatment of spontaneous intracranial hypotension with percutaneous placement of a fibrin sealant. Report of four cases

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.     

Intrathecal saline infusion in the treatment of obtundation associated with spontaneous intracranial hypotension: technical case report

OBJECTIVE AND IMPORTANCE: Spontaneous intracranial hypotension is an increasingly recognized cause of postural headache. However, appropriate management of obtundation caused by intracranial hypotension is not well defined. CLINICAL PRESENTATION: A 43-year-old man presented with postural headache followed by rapid decline in mental status. Imaging findings were consistent with the diagnosis of spontaneous intracranial hypotension, with bilateral subdural hematomas, pachymeningeal enhancement, and caudal displacement of posterior fossa structures and optic chiasm. INTERVENTION: Despite treatment with lumbar epidural blood patch, worsening stupor necessitated intubation and mechanical ventilation. Contrast-enhanced magnetic resonance imaging and computed tomographic myelography of the spine failed to demonstrate the site of cerebrospinal fluid fistula. The enlarging subdural fluid collections were drained, and a ventriculostomy was performed. Postoperatively, the patient remained semicomatose. To restore intraspinal and intracranial pressures, intrathecal infusion of saline was initiated. After several hours of lumbar saline infusion, lumbar and intracranial pressures normalized, and the patient's stupor resolved rapidly. Repeat computed tomographic myelography accomplished via C1-C2 puncture demonstrated a large ventrolateral T1-T3 leak, which was treated successfully with a thoracic epidural blood patch. Follow-up magnetic resonance imaging demonstrated resolution of intracranial hypotension, and the patient was discharged in excellent condition. CONCLUSION: Spontaneous intracranial hypotension may cause a decline of mental status and require lumbar intrathecal saline infusion to arrest or reverse impending central (transtentorial) herniation. This case demonstrates the use of simultaneous monitoring of lumbar and intracranial pressures to appropriately titrate the infusion and document resolution of intracranial hypotension. Maneuvers aimed at sealing the cerebrospinal fluid fistula then can be performed in a less emergent fashion after the patient's mental status has stabilized.     

Multi-level disruption of the spinal nerve root sleeves in spontaneous spinal cerebrospinal fluid leakage--two case reports

A 37-year-old male and an 18-year-old male presented with spontaneous spinal cerebrospinal fluid (CSF) leakage from multiple nerve root sleeves. Both patients suffered abrupt onset of intense headache followed by nausea, dizziness, and one patient with and one without positional headache. Radioisotope spinal cisternography of both patients revealed that the CSF leaks were not localized in a special zone but distributed to multiple spinal nerve root sleeves. Magnetic resonance (MR) myelography suggested that the spinal CSF column was fully expanded to the root sleeves. The extraspinal nerve bundles demonstrated numerous high intensity spots. Both patients were treated conservatively, and their symptoms resolved within one month. Repeat radioisotope cisternography and MR myelography confirmed the spine was normal after recovery. We suggest that spreading disruption of the arachnoid membrane occurs at the nerve root sleeves due to CSF overflow into the spinal canal.     

Bilateral multiple cervical root avulsions without skeletal or ligamentous damage resulting from blast injury: case report

OBJECTIVE AND IMPORTANCE: We describe a unique case of multiple bilateral cervical root injuries without ligamentous or bony injury secondary to a sandblast accident. CLINICAL PRESENTATION: A 19-year-old man sustained a sandblast injury to his face, neck, chest, and upper extremities, with immediate loss of motor and sensory function occurring in both of his upper extremities. Cervical spine x-rays, computed tomography, and magnetic resonance imaging demonstrated no fracture, soft tissue abnormality, or malalignment. The restriction of deficits to the patient's upper extremities suggested a central cervical spinal cord injury, bilateral brachial injuries, or a conversion disorder. INTERVENTION: Cervical computed tomographic myelography revealed multiple bilateral nerve root injuries. CONCLUSION: This case report is unique in the literature in that it describes a patient with multiple cervical nerve root injuries secondary to sandblast injury without ligamentous or bony injury. Although magnetic resonance imaging remains the diagnostic modality of choice in patients with acute spinal cord injury, it is deficient in demonstrating cervical root injury in the acute setting. In this setting, computed tomographic myelography is superior.     

Spectrum of subdural fluid collections in spontaneous intracranial hypotension

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.     

Minimally invasive repair of spontaneous intracranial hypotension

Spontaneous spinal CSF leakage with the development of intracranial hypotension is a well-described entity. Cerebrospinal fluid leaks, mostly from the thoracic spine, are the major cause of spontaneous intracranial hypotension (SIH). Conservative treatment options include hydration, oral caffeine, and epidural blood patching. Alternatively, open surgical correction of meningeal diverticula is a therapeutic option. The authors describe 4 cases of spontaneous spinal CSF leakage producing symptoms of intracranial hypotension. All patients had multiple spinal diverticula with an identified leaking level. The patients were treated using a minimally invasive approach via surgical correction of the meningeal diverticulum.     

Intracranial hypotension and recurrent pleural effusion after snow-boarding injury: a manifestation of cerebrospinal fluid-pleural fistula.

BACKGROUND DATA: Intracranial hypotension causing postural headaches has been described after occult and postsurgical cerebrospinal fluid (CSF) leaks and rarely isolated lumbar punctures. The occurrence of a CSF-pleural communication is much rarer, and a high level of suspicion aids in prompt recognition. PURPOSE: Early detection and anatomic delineation of the site of CSF-pleural fistula allows prompt intervention, results in resolution of symptoms and prevents the complication of meningitis. STUDY DESIGN: A case of intracranial hypotension with postural headaches is described after spinal surgery, with demonstration on computed tomography (CT) myelography of a rare CSF-pleural fistula. METHODS: The clinical presentation, postoperative intervention and imaging as well as laboratory data are presented. RESULTS: Chest X-ray showed recurrent pleural effusion after placement of chest tube, and serial head CT studies revealed decreasing ventricular size with development of severe headaches. Myelogram and CT postmyelogram demonstrate the CSF-pleural communication, allowing appropriate surgical repair. CONCLUSION: Severe headaches with a recurrent pleural effusion after thoracic spinal surgery may indicate presence of a CSF-pleural fistula, an unusual complication of thoracic spinal surgery.     

Epidural blood patch for the treatment of intracranial hypotension due to cerebrospinal fluid leakage after thoracic spine surgery

We report a case of a 44-year-old woman successfully treated by an epidural blood patch for intracranial hypotension due to cerebrospinal fluid (CSF) leakage into the thoracic cavity after thoracic spine surgery. The patient was admitted to our hospital with the complaint of postural headaches. She had received anterior thoracic instrumentation for thoracic disc herniation four months earlier. Lumbar puncture demonstrated low CSF pressure, and Gd-enhanced MR images displayed diffuse dural enhancement. Accordingly, she was diagnosed as having intracranial hypotension. 111In-DTPA cisternography revealed a CSF leakage into the left thoracic cavity, possibly caused by dural laceration during thoracic spine surgery. To avoid the risk of direct surgery, we performed epidural blood patch; 3 ml of autologous blood was injected into the epidural cavity. Postoperatively postural headaches immediately disappeared. MRI taken one year later revealed disappearance of diffuse dural enhancement, and 111In-DTPA cisternography revealed no CSF fluid leaks. Epidural blood patch seems to be a choice of treatment for CSF leak after spinal surgery.     

Spontaneous intracranial hypotension mimicking aneurysmal subarachnoid hemorrhage

OBJECTIVE: An excruciating headache of instantaneous onset is known as a thunderclap headache. A subarachnoid hemorrhage is the prototypical cause, but other serious disorders may also present with a thunderclap headache, including cerebral venous sinus thrombosis, carotid artery dissection, and pituitary apoplexy. We report a group of patients with thunderclap headaches as the initial manifestation of spontaneous intracranial hypotension caused by a spinal cerebrospinal fluid leak. METHODS: Among 28 patients with spontaneous intracranial hypotension due to a documented spinal cerebrospinal fluid leak, four (14%) initially experienced an excruciating headaches of instantaneous onset. RESULTS: The mean age of the four patients (two men and two women) was 35 years (range, 24-45 yr). Nuchal rigidity was present in the three patients who sought early medical attention, and they underwent emergency computed tomographic scanning, lumbar puncture, and cerebral angiography to rule out an aneurysmal subarachnoid hemorrhage. The delay between the onset of headache and diagnosis of intracranial hypotension ranged from 4 days to 5 weeks. A fourth patient did not seek medical attention until 1 month after the ictus. CONCLUSION: Spontaneous intracranial hypotension should be included in the differential diagnosis of thunderclap headache, even when meningismus is present.     

Connective tissue disorders with spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension: a prospective study

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.     

Evaluation of spontaneous intracranial hypotension: assessment on ICP monitoring and radiological imaging

We describe two recent cases of spontaneous intracranial hypotension. A 38-year-old woman developed a severe postural headache. Magnetic resonance imaging (MRI) showed marked dural enhancement. Histopathological findings of dural biopsy showed numerous dilated vessels in the dura, rather than hypertrophic change. Lumber CSF pressure was 5 cmH2O and RI cisternography suggested CSF leakage. A 58-year-old woman with postural headache and vertigo had bilateral subdural haematoma associated with diffuse dural enhancement on MRI. Lumber CSF monitoring confirmed persistent low pressure ranging from 0-5 cm H2O. MRI myelography revealed multiple CSF pouches along the whole spinal axis. CSF leakage was demonstrated on Radioisotope (RI) cisternography. Both cases described in this report were diagnosed as spontaneous intracranial hypotension caused by CSF leakage from spinal meningeal diverticula and were successfully treated by intravenous Factor XIII administration.     

Significance of empty sella in cerebrospinal fluid leaks.

OBJECTIVE: The role of elevated cerebrospinal fluid (CSF) pressures in the pathophysiology of various CSF leaks is not clear. Empty sella syndrome (ESS) is a radiographic finding that can be associated with elevated CSF pressures and may represent a radiographic indicator of intracranial hypertension. We present our experience with CSF leaks of various causes, the prevalence of ESS in the spontaneous and nonspontaneous categories, and the potential pathophysiology and unique management issues of the spontaneous CSF leak group. METHODS: We conducted a retrospective review of medical records, imaging studies, and surgical treatment of CSF leaks in patients treated by the senior author. RESULTS: Sixteen patients with spontaneous CSF leaks and 12 patients with nonspontaneous CSF leaks were surgically treated from 1996 through 2002. In the spontaneous group, 15 patients had complete imaging of the sella turcica. Ten had completely empty sellae and 5 had partially empty sellae, for a total of 100% (15 of 15). In the nonspontaneous group, 9 patients had complete imaging of the sella. Only 11% (1 of 9) had a partially empty sella and that was a congenital leak. Comparison of proportions between these 2 groups was significant (P = 0.01). The spontaneous group consisted primarily of obese, middle-aged females (13 of 16 patients). CONCLUSION: Empty sella probably represents a sign of elevated intracranial pressure that leads to idiopathic, spontaneous CSF leaks. Spontaneous CSF leaks are strongly associated with the radiographic finding of an empty sella and are more common in obese females, similar to benign intracranial hypertension. This unique population may require more aggressive surgical and medical treatment to prevent recurrent or multiple leaks.     

Transient oculomotor cranial nerves palsy in spontaneous intracranial hypotension

Transient sixth cranial nerves palsy may occur in rare cases after lumbar puncture, spinal anesthesia and myelography as well as in more rare cases of spontaneous intracranial hypotension. We report three cases of spontaneous intracranial hypotension with sixth cranial nerves palsy. One of these patients presented also third cranial nerve palsy, never reported in spontaneous intracranial hypotension.