Spontaneous intracranial hypotension syndrome treated with a double epidural blood patch

Acta Anaesthesiol Scand 2012; ••: ••–•• Printed in Singapore. All rights reserved © 2012 The Authors Acta Anaesthesiologica Scandinavica © 2012 The Acta Anaesthesiologica Scandinavica Foundation ACTA ANAESTHESIOLOGICA SCANDINAVICA doi: 10.1111/j.1399-6576.2012.02742.x Case Report Spontaneous intracranial hypotension syndrome treated with a double epidural blood patch J. M. Beleña1, M. Nuñez2, J. Yuste1, J. F. Plaza-Nieto3, F. J. Jiménez-Jiménez3 and S. Serrano4 1 Department of Anesthesiology and Critical Care, Hospital Universitario del Sureste, Madrid, Spain, 2Department of Anesthesiology and Critical Care, Hospital Universitario Ramón y Cajal, Madrid, Spain, 3Section of Neurology, Hospital Universitario del Sureste, Madrid, Spain and 4Department of Radiology, Hospital La Moraleja, Madrid, Spain Spontaneous intracranial hypotension (SIH) is considered to be a very rare disease. It is characterised by an orthostatic headache in the absence of a past history of a trauma or a dural puncture. SIH is caused by a spontaneous spinal cerebrospinal fluid (CSF) leakage demonstrated by neuroradiological studies in most of the patients. Conservative treatment usually includes bed rest, hydration and administration of caffeine or steroids. However, when the patient is refractory to the conservative treatment, an epidural blood patch (EBP) is performed. We report a 34-yearold woman with SIH and no neuroradiologically demonstrable clear point of CSF leakage, who was treated with a double EBP at two different levels (lumbar and thoracic) in the same procedure. The patient was successfully managed, and she was still S pontaneous intracranial hypotension (SIH) is a postural headache syndrome of acute or subacute onset, unrelated to dural puncture, surgery or trauma, although sometimes it is associated with underlying connective tissue disorders.1,2 Besides headache, other clinical features may include nausea, vomiting, dizziness, balance problems, vertigo, tinnitus, marked exacerbation by Valsalva manoeuvre,3 and a wide variety of ocular manifestations (unilateral or bilateral abducens nerve palsy, or less frequently, other oculomotor palsies, visual field defects of ophthalmoplegia).4 In fact, this syndrome can occur in the context of several diseases, such as subdural hematoma or brainstem compression, and it can also occur with loss of cerebrospinal fluid (CSF) through the cribriform plate or otorrhea. SIH has an incidence estimated at 5/100.000,3 and is more common in women than men.5 SIH is due to a leak of CSF from a tear of the dura, which occurs most often at the exit zones where the spinal roots leave the subarachnoid space.3 The diagnosis of SIH is made on the basis of clinical symp- asymptomatic at the 18 months follow-up. After review of literature, we observed that execution of a double EBP at the same time is not a common procedure for treatment of SIH. We consider that simultaneous use of two EBP could be useful as a novel treatment in those cases of SIH without demonstration of CSF leakage. Accepted for publication 11 June 2012 © 2012 The Authors Acta Anaesthesiologica Scandinavica © 2012 The Acta Anaesthesiologica Scandinavica Foundation toms, lumbar puncture (showing low CSF opening pressure), radiological studies [brain magnetic resonance imaging (MRI) can show meningeal enhancement, spine MRI, computed tomography myelography], and radionuclide cisternography showing thoracolumbar dural leaks (less often at cervical level) in some patients.5–14 Conservative therapy includes bed rest, hydration and administration of caffeine or steroids.3,7,15 When conservative measures fail, the use of autologous epidural blood patches (EBP) is recommended.5–7,15–20 Because of the use of a single EBP does not relieve the symptoms in a variable percentage of patients, some of them require 2 or 3 ones.6,15,17 We report one patient with SIH, and no points of CSF leakage, who directly received a double EBP at two different levels (lumbar and thoracic) with a good outcome. Case report A 34-year-old woman, with a previous history of two epidural anaesthesia procedures 3 and 4 years 1 bs_bs_banner J. M. Beleña et al. ago (without complications), was evaluated because of a 3-month history of severe fronto-occipital headache of subacute onset and a gradual progression. That was accompanied by neck pain, nausea and vomiting, and was exacerbated by the upright position, physical activity and Valsalva manoeuvre, whereas it gradually resolved on lying down. Neuroimaging studies, including brain MRI, and cervical, thoracic and lumbar spine MRI, showed no abnormalities. A lumbar puncture showed an opening pressure of CSF of 5 cmH2O, and radionuclide cisternography did not detect any point of CSF leakage. Routine CSF analysis showed 6 cells/mm3, and protein 65 mg/dl abnormality. Headache was not relieved with conservative measures, such as bed rest, hydration, common analgesics and caffeine. The patient presented an affected functional status; she usually experienced a severe headache of 7/10 on the visual analog scale and has limitation when standing. A double EBP at two different levels (lumbar and thoracic) was then chosen as treatment once the written informed consent for the technique was obtained. Both procedures were carried out by median puncture in sitting position. The patient received a dose of 2 g of cefazolin iv as standard antibiotic prophylaxis. After skin preparation (strict asepsis), one anaesthesiologist began the lumbar epidural puncture, and an 18-G Tuohy needle was inserted at L1-L2 level. The epidural space was identified by the air loss of resistance technique. At the same time, another anaesthesiologist, using a rigidly aseptic technique, performed a venous puncture at the antecubital area of the patient’s right arm, withdrew 17 ml of blood, removed the needle from the syringe and handed the syringe to the first anaesthesiologist (without breaching the integrity of the sterile fields) who injected 15 ml of blood epidurally. Next, we repeated the same procedure at T10-T11 level for the second EBP without incidences. The patient remained in the supine position for 1 h following the procedure, symptomatic relief was achieved, and 1 h later she was discharged after advice to report fever, back or radicular pain, or other untoward symptoms immediately. Two days later, the patient was referred back to the clinic reporting fever (37.8°C) with no other symptoms. A lumbar-thoracic spine MRI showed hyperintensity in T2-weighted images and hypointensity in T1-weighted images in the anterior epidural space, with homogenous contrast enhancing from T10 to S1 levels (Fig. 1). T2-weighted images showed six small laminar collections (1 mm) in the 2 Fig. 1. Magnetic resonance imaging T1 sagittal. Shows a hypointense image in the anterior epidural space, with homogeneal contrast enhancing from T10 to S1 levels (between arrows), consistent with the blood patch. posterior epidural space between these two levels as well (Fig. 2). These images were consistent with the spreading of blood patch in the epidural space. The fever disappeared spontaneously in 24 h, and the patient remains asymptomatic with no headache or other related symptoms reported after 18 months of follow-up. Discussion The reported patient presented with a typical SIH in the absence of any point of CSF leakage by using the radionuclide cisternography. The patient was refractory to the conservative treatment, so we decided to perform an EBP. Berroir et al.6 used early EBP empirically in patients with clinical suspicion of SIH without previously performing lumbar puncture or identifying a CSF leak, with or without MRI changes. This group obtained a complete cure in 57% of patients after one EBP, and in other 20% after a second EBP, and they looked for CSF leak by MRI, CT myelography and/or radionuclide cisternography after the failure of two to four EBPs. More recently, Ferrante et al.15 achieved excellent results with a single EBP in 90% of patients after pre-medication with acetazolamide and maintaining the patients in a 30-degree Trendelenburg SIH syndrome treated with double EBP Fig. 2. Magnetic resonance imaging T2 sagittal. Shows a hyperintense posterior epidural blood patch (between arrows). position an hour before, during, and 24 h after the procedure. The other patients required two (5%) or three (5%) EBPs. Based on the results of EBP for treatment of SIH, we decided (after performing lumbar puncture, neuroimaging and radionuclide methods) to try two blind EBPs. The majority of the spontaneous leaks occur at the level of the spine, particularly at the thoracic level.21 For this reason, and in the absence of a definite CSF leak, we decided to perform one of the EBP at this level. Lately, Franzini et al.22 proposed a novel physiopathological hypothesis of SIH based on considerations about the spinal venous drainage system. The authors think that the dural tear (even when clearly identified) is not the cause of the disease but the effect of the epidural hypotension maintained by the inferior cava vein outflow to the heart. The upper thoracic plexiform venous network drains into the superior vena cava system, and the lumbar epidural venous network drains into the inferior vena cava system. These two systems communicate at the thoracolumbar junction. The inferior vena cava system is affected (much more than the upper one) by dynamic modifications due to the strong muscles pumping blood from the inferior limbs during standing and walking. This causes a negative pressure in the inferior cava vein that results in overdrainage of venous blood from the epidural spinal vein network via lumbar collectors through antireflux venous valves. This decrease in spinal epidural pressure and in the volume of the epidural veins results in modification of the gradient between two pressures: epidural space (negative) and CSF (positive in orthostatic conduction). This modification results in aspiration of CSF into the epidural space and veins, with a considerable outflow of CSF from the subarachnoid compartment to the radicular veins. This hypothesis may explain the developing of SIH without neuroradiological evidence of CSF leaks. They used EBP at the lumbar level using autologous blood and fibrin glue. The goal of this procedure was not to seal CSF leaks, but instead help in reversing the CSF-blood gradient within the epidural space along the entire cord, minimising the outflow of CSF along the spinal cavity. Of the 28 patients studied, 27 were available to undergo follow-up at 3 months (70.4% did not show any clinical symptoms), 22 patients at 1-year follow-up visit (81.8% were completely asymptomatic) and 11 patients at 3-year follow-up visit (83.3% were completely free from clinical symptoms). Based on this theory, we performed a second EBP at the lumbar level in the same procedure. We tried to combine the effectiveness of the puncture at two different levels (lumbar and thoracic) at the same time to improve the results of the technique. Double EBP offered a higher probability of success because it covered a more extensive area, besides minimising the risk of spinal compression (with consequent back pain or subdural hematoma) because we needed a smaller amount of blood in each of both punctures. On the other hand, with this technique, we avoid not only to bring the patient under Trendelenburg position for a long time, with the discomfort that it carries, but also the inconvenience of two separated punctures for the patient. Our patient was successfully managed, and this resulted in a complete cure of her orthostatic headache. The double EBP did not show more adverse events than the ones attributed to the single technique. The execution of a double EBP at the same time is not a common procedure for treatment of SIH. We only found a case report where it was performed, but in this case, they identified CSF leakage (by radionuclide cisternography) at the upper cervical vertebral level and at the middle thoracic level, and the patient was successfully managed by injecting an EBP at each level of leakage.20 It is possible that just with one EBP, the patient had been cured, as Ferrante et al. demonstrate in 3 J. M. Beleña et al. most of their cases,15 but reported experience of other authors6,17,20 lead us to believe that the double EBP would ensure the success of the treatment, avoiding that symptoms persist for a longer time. We consider that simultaneous use of two EBP in those cases of SIH without demonstration of CSF leakage could be useful. Conflicts of interest and source of funding: None. 12. 13. 14. 15. References 1. Schievink WI, Gordon OK, Tourje J. Connective tissue disorders with spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension: a prospective study. 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Spontaneous intracranial hypotension syndrome: a novel speculative physiopathological hypothesis and a novel patch method in a series of 28 consecutive patients. J Neurosurg 2010; 112: 300–6. Address: José María Beleña C/ Ronda del Sur 10. 28500 Arganda del Rey, Madrid Spain e-mail: jmaria.belenab@salud.madrid.org