Intracranial hypotension

Spontaneous Intracranial Hypotension in Childhood and Adolescence

Authors: Schievink WI, Maya MM, Louy C, Moser FG, Sloninsky L.

OBJECTIVES: To describe the clinical and radiographic manifestations of spontaneous intracranial hypotension, a rarely diagnosed cause of headache in children.
STUDY DESIGN: This study included patients 19 years of age or younger evaluated between January 1, 2001, and June 30, 2012, for spontaneous intracranial hypotension.
RESULTS: We evaluated 24 children (18 girls and 6 boys) with spontaneous intracranial hypotension (age at onset of symptoms: 2-19 years, mean 14.3 years). Twenty-three patients presented with orthostatic headaches and 1 presented with a nonpositional headache. A generalized connective tissue disorder was diagnosed in 54% of patients. Magnetic resonance imaging showed the typical changes of spontaneous intracranial hypotension in most patients (79%). Spinal imaging demonstrated a cerebrospinal fluid (CSF) leak with or without an associated meningeal diverticulum in 12 patients (50%) and with dural ectasia or meningeal diverticula in 10 patients (42%), and it was normal in 2 patients (8%). Twenty-three patients initially underwent epidural blood patching, but 8 patients also were treated with percutaneous injections of fibrin glue and 11 patients eventually required surgical correction of the underlying CSF leak. There was no morbidity or mortality associated with any of the treatments, but 5 patients required acetazolamide for rebound high intracranial pressure headache. Overall, outcome was good in 22 patients (92%) and poor in 2 patients (8%).
CONCLUSIONS: Spontaneous intracranial hypotension in childhood is rare. Most patients can be treated effectively using a combination of epidural blood patching and percutaneous injections of fibrin glue or surgical CSF leak repair in refractory cases.

Bladder and bowel dysfunction following small-volume epidural blood patch for spontaneous intracranial hypotension

Authors: Han IB, Ropper AE, Teng YD, Ryoo YH, Kim O.

Epidural blood patch (EBP) is an effective procedure for the treatment of spontaneous intracranial hypotension (SIH). Neurological compromise following EBP, although rare, is recognized as a serious potential complication. We describe a 33-year-old female patient in whom long-term bladder and bowel dysfunction developed following a small volume (10mL) EBP to treat SIH. We also discuss the possible pathophysiological mechanisms related to this complication in the postprocedure setting.

Concentric visual field defect related to spontaneous intracranial hypotension

Authors: Pilo-de-la-Fuente B, Gonzalez Martin-Moro J, Navacerrada F, Plaza-Nieto FJ, Jimenez-Jimenez FJ.

Spontaneous intracranial hypotension (SIH) is a rare syndrome characterized by postural headache associated with a low cerebrospinal fluid pressure in the absence of dural puncture or penetrating trauma. Cranial magnetic resonance imaging (MRI) typically shows diffuse pachymeningeal gadolinium enhancement, subdural fluid collections, prominence of cerebral venous sinuses and brain descent. Visual signs and symptoms have been described infrequently in patients with SIH. These include third or sixth nerve palsy, superior nasal quadrantanopia and temporal hemianopia. We report a 34 year-old woman who presented with a two-year history of orthostatic headache, dizziness and transient visual obscurations. Campimetry showed a bilateral concentric visual field defect. She also described that intermittently a transparent fluid leaked out of her nose. She had no past history of trauma, sinus surgery or intracranial surgery. Cranial MRI was normal. Neuro-ophthalmological examination ruled out any other causes of concentric visual field defects. Lumbar puncture showed a cerebrospinal fluid (CSF) opening pressure of 9 cm H(2)O. Radioisotope cisternography suggested a dural leak at cribiform plate. The cribiform plate region was repaired endoscopically with improvement of all symptoms. One year later she remains asymptomatic and the visual field defects have improved.

Stroke and Death Due to Spontaneous Intracranial Hypotension

Authors: Schievink WI.

BACKGROUND: Spontaneous intracranial hypotension has become a well-recognized cause of headaches and a wide variety of other manifestations have been reported. Recently, several patients with asymptomatic spontaneous intracranial hypotension were reported. I now report two patients with spontaneous intracranial hypotension who developed multiple arterial strokes associated with death in one patient, illustrating the spectrum of disease severity in spontaneous intracranial hypotension.

Spontaneous intracranial hypotension secondary to anterior thoracic osteophyte: Resolution after primary dural repair via posterior approach

Authors: Veeravagu A, Gupta G, Jiang B, Berta SC, Mindea SA, Chang SD.

INTRODUCTION: Spontaneous intracranial hypotension (SIH) is an uncommon syndrome widely attributed to CSF hypovolemia, typically secondary to spontaneous CSF leak. Although commonly associated with postural headache and variable neurological symptoms, one of the most severe consequences of SIH is bilateral subdural hematomas with resultant neurological deterioration.
PRESENTATION OF CASE: We present the case of a patient diagnosed with SIH secondary to an anteriorly positioned thoracic osteophyte with resultant dural disruption, who after multiple attempts at nonsurgical management developed bilateral subdural hematomas necessitating emergent surgical intervention. The patient underwent a unilateral posterior repair of his osteophyte with successful anterior decompression. At 36months follow up, the patient reported completely resolved headaches with no focal neurological deficits.
DISCUSSION: We outline our posterior approach to repair of the dural defect and review the management algorithm for the treatment of patients with SIH. We also examine the current hypotheses as to the origin, pathophysiology, diagnosis and treatment of this syndrome.
CONCLUSION: A posterior approach was utilized to repair the dural defect caused by an anterior thoracic osteophyte in a patient with severe SIH complicated by bilateral subdural hematomas. This approach minimizes morbidity compared to an anterior approach and allowed for removal of the osteophyte and repair of the dural defect.
Copyright © 2012. Published by Elsevier Ltd.

Spinal manifestations of spontaneous intracranial hypotension

Authors: Schievink WI, Chu RM, Maya MM, Johnson JP, Cohen HC.

Object The goal of the study was to elucidate the spinal manifestations of spontaneous intracranial hypotension. Methods The authors reviewed the medical records and imaging studies of 338 consecutive patients with spontaneous intracranial hypotension who were evaluated at their institution between 2001 and 2010. Results Twenty patients (6%; mean age 35.8 ; 5 males and 15 females) had convincing signs or symptoms referable to the spinal cord or spinal nerve roots. The spinal manifestations consisted of radiculopathy in 11 patients (unilateral in 8 and bilateral in 3), myelopathy in 8 patients, and bibrachial amyotrophy in 1 patient. The cervical spine was involved in 12 patients, the thoracic spine in 5, and the lumbosacral spine in 3. The spinal symptoms were positional in only 3 patients. The spinal manifestations occurred around the time of the headache onset in 16 patients, and months to years after the positional headache had resolved in 4 patients. A large extrathecal CSF collection causing compression of the spinal cord or nerve root was responsible for the spinal manifestations in the majority of patients. Treatment of the spinal CSF leak resulted in resolution of the spinal manifestations along with the headache, except for those in the patient with bibrachial amyotrophy. Conclusions Spinal manifestations are uncommon in cases of spontaneous intracranial hypotension, occurring in about 6% of patients, but myelopathy and radiculopathy involving all spinal segments do occur. Unlike the headache, the spinal manifestations usually are not positional and are caused by mass effect from an extradural CSF collection.

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