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Spontaneous Cervical (C1–C2) Cerebrospinal Fluid Leakage Repaired with Computed Tomography-Guided Cervical Epidural Blood Patch

Open AccessPublished:July 30, 2010DOI:https://doi.org/10.1016/j.jpainsymman.2010.04.010
      To the Editor:
      Intracranial hypotension syndrome (IHS) is often caused by persistent cerebrospinal fluid (CSF) leakage. IHS usually presents with orthostatic headache, exacerbated by an increase in intracranial pressure, but other symptoms can be present such as nausea, vomiting, dizziness, neck pain, and paresis of the VIth cranial nerve.
      • Mokri B.
      • Posner J.B.
      Spontaneous intracranial hypotension: the broadening clinical and imaging spectrum of CSF leaks.
      It can be spontaneous or related to a trauma, such as dural puncture or surgery, or medical causes such as dehydration. Thanks to improvements in neuroradiological imaging quality, magnetic resonance imaging (MRI) diagnosis of IHS is now more common and easier.
      • Bakir B.
      • Aydin K.
      • Talu G.
      • Ozyalcin S.
      Cranial magnetic resonance imaging in spontaneous intracranial hypotension after epidural blood patch.
      IHS is a benign condition,
      • Couch J.R.
      Spontaneous intracranial hypotension: the syndrome and its complications.
      often treated conservatively. If it does not resolve by itself, it usually can be treated with epidural blood patch (EBP), even if a Cochrane meta-analysis showed a lack of randomized controlled trials that confirm the efficacy of this approach.
      • Sudlow C.
      • Warlow C.
      Epidural blood patching for preventing and treating post-dural puncture headache.
      Rarely, IHS is caused by a high cervical problem and, to our knowledge, in the literature there are only three cases of C2 leakage treated with EBP
      • Rai A.
      • Rosen C.
      • Carpenter J.
      • Miele V.
      Epidural blood patch at C2: diagnosis and treatment of spontaneous intracranial hypotension.
      • Buvanendran A.
      • Byrne R.W.
      • Kari M.
      • Kroin J.S.
      Occult cervical (C1-2) dural tear causing bilateral recurrent subdural hematomas and repaired with cervical epidural blood patch.
      • Inamasu J.
      • Nakatsukasa M.
      Blood patch for spontaneous intracranial hypotension caused by cerebrospinal fluid leak at C1-2.
      with a large volume of blood (the authors repeated the procedure twice, with 10 mL each time).

      Case

      A 32-year-old previously healthy woman presented to our hospital with a monthly history of progressively worsening frontal headache, nausea, dizziness, and photophobia. Symptoms partially regressed when lying down and worsened when sitting up or standing. She did not present with any fever.
      The patient's symptoms had started subacutely after heavy physical activity. She also reported a nonconcussional craniofacial trauma some days before the start of the headache. She was brought to the neurological department of her local hospital. Computed tomography (CT) scan did not show any evident abnormality, but an MRI showed typical signs of IHS (pachymeningeal enhancement, subdural fluid collection at multilevels of the central nervous system, and venous engorgement); no clear image of the level of fistula was found. She was treated for a month with nonsteroidal anti-inflammatory drugs, intravenous caffeine, fluids and rest, without symptom relief. The patient was referred to our hospital, where we repeated an MRI that revealed collection of fluid in the suboccipital space (Fig. 1a ) at the upper cervical muscle level, along with subdural fluid collections (Fig. 1b) and enhancement of the pachymeninges (Fig. 1c). The CSF accumulation at the posterior atlanto-epistropheus level suggested the level of the fistula (C1–C2).
      Figure thumbnail gr1
      Fig. 1Different MRI scans showing IHS signs: a) collection of fluid at the level of the upper cervical muscles in the suboccipital space, b) subdural fluid collections, and c) enhancement of the pachymeninges.
      With the patient in a prone position and under CT guidance, an 18G needle was inserted in the epidural space at the C5–C6 level, using the air loss-of-resistance technique. Then, a radiopaque 18G epidural catheter was advanced to the C3 level, and we confirmed the level of the tip of the catheter through dye injection. As 1 mL was spread to C2, we decided to inject slowly, into the epidural space, only 2.5 mL of sterile autologous peripheral unclotted venous blood. As CT confirmed spreading of blood to C1 (anterior and posterior), we stopped the injection. At the end of the procedure, the catheter was removed. The patient was awake during the entire procedure and cooperated with a complete neurological examination. She did not complain of any neurological symptoms except neck pain for few hours. All her symptoms regressed within 24 hours after the procedure, and she was discharged after three days.
      MRI after 12 weeks did not show any signs of CSF at the atlanto-epistropheus level, other than improvement of the typical IHS signs, with the exception of stable subdural fluid accumulation at the cervicothoracic level (Fig. 2a and b ). The patient was completely symptom free, and she started working again one month after the procedure. At one-year follow-up, the patient did not complain of any other symptoms.
      Figure thumbnail gr2
      Fig. 2a) No sign of CSF accumulation in the suboccipital space and b) no sign of CSF behind C1–C2 and stable subdural fluid accumulation at the cervicothoracic level (arrow).

      Comment

      Recent evidence suggests that IHS is half as common as spontaneous subarachnoid hemorrhage, with an estimated annual incidence of 5 per 100,000 population.
      • Mea E.
      • Chiapparini L.
      • Savoiardo M.
      Application of IHS criteria to headache attributed to spontaneous intracranial hypotension in a large population.
      This syndrome has to be considered an important cause of persistent headaches, particularly among young and middle-aged individuals.
      MRI has improved the understanding of IHS and has greatly facilitated diagnosis without need of invasive procedures.
      • Schievink W.I.
      Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension.
      Because of the wide variety of headache patterns, MRI should be considered for all patients with unexplained headache to evaluate for spontaneous intracranial hypotension.
      • Schievink W.I.
      Spontaneous spinal cerebrospinal fluid leaks.
      Typical MRI findings include enhancement of the pachymeninges (the most well-known imaging abnormality, even if it could be absent in up to 20% of patients), subdural fluid collections, mostly located over the cerebral convexities, engorgement of venous structures, pituitary hyperemia, and sagging of the brain (a very specific sign of IHS).
      The objective of therapy is to stop the CSF leakage, restore CSF volume, and restore the buoyancy of the brain. The simplest therapy is bed rest, with the objective of diminishing hydrostatic pressure against the defect in the dural membrane and allowing the defect to heal. Adding caffeine to the regimen is often mentioned, but scientific proof of its efficacy is lacking.
      • Couch J.R.
      Spontaneous intracranial hypotension: the syndrome and its complications.
      If complications occur or if conservative treatment does not work, a more aggressive approach may be required, including EBP (an infusion of sterile autologous blood into the epidural space) and, in some cases, percutaneous placement of fibrin sealant and surgical CSF leakage repair. Outcomes have been poorly analyzed, and no management strategies have been studied in properly controlled randomized trials.
      • Sudlow C.
      • Warlow C.
      Epidural blood patching for preventing and treating post-dural puncture headache.
      As has been documented by MRI,
      • Kalina P.
      • Craigo P.
      • Weingarten T.
      Intrathecal injection of epidural blood patch: a case report and review of the literature.
      the EBP resolves the symptoms with the development of a blood clot near the CSF leakage that may stop further CSF leakage. Furthermore, injected blood volume increases the epidural pressure, which aids in the cessation of CSF leakage from the subarachnoid space into the epidural space.
      • Bakir B.
      • Aydin K.
      • Talu G.
      • Ozyalcin S.
      Cranial magnetic resonance imaging in spontaneous intracranial hypotension after epidural blood patch.
      The success rate of the EBP is considerably lower for IHS than for postlumbar puncture headache because the level of the leak is hard to locate, anatomy may be more complicated, and dural defect is often located on the anterior aspect of the dural sac.
      • Schievink W.I.
      Spontaneous spinal cerebrospinal fluid leaks.
      Complications of cervical EBPs include spinal cord and nerve root compression (the risk of which is greater than that associated with lumbar EBPs), chemical meningitis, intrathecal injection of blood, seizures, and neck stiffness. There are few case reports of cervical EBP in the literature, possibly because of its hazardous side effects. In our case, we treated a young patient with a C1–C2 fistula adopting the technique described by Buvanendran et al.:
      • Buvanendran A.
      • Byrne R.W.
      • Kari M.
      • Kroin J.S.
      Occult cervical (C1-2) dural tear causing bilateral recurrent subdural hematomas and repaired with cervical epidural blood patch.
      10 mL of autologous blood injected twice through a cervical epidural catheter left for two days. We modified this technique by using a very low volume of blood only once, reducing the possible side effects. The patient healed completely, and on follow-up, cervical MRI revealed the resolution of the fistula. One year after the procedure, the patient did not report any symptoms.
      With this case report, we want to underline the importance of CT guidance for C1–C2 EBPs, as we can just inject the volume needed to create a tap on the dura hole, minimizing the possible side effects.

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      Linked Article

      • Erratum
        Journal of Pain and Symptom ManagementVol. 40Issue 6
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          Spontaneous Cervical (C1–C2) Cerebrospinal Fluid Leakage Repaired with Computed Tomography-Guided Cervical Epidural Blood Patch. Allegri M, Lombardi F, Scagnelli P, Corona M, Minella CE, Braschi A, Arienta C. J Pain Symptom Manage 2010;40(3):e9-e12.
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