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Extradural Compressive Spinal Cerebrospinal Fluid Leak in Ehlers-Danlos Syndrome
Clinical Images
Extradural Compressive Spinal Cerebrospinal Fluid Leak in Ehlers-Danlos Syndrome Laura Pradini-Santos, Claudia L. Craven, Parag P. Sayal
Key words Cerebrospinal fluid - Connective tissue disorder - Epidural injection - Spine -
Abbreviations and Acronyms CSF: Cerebrospinal fluid EDS: Ehlers-Danlos syndrome ICP: Intracranial pressure MRI: Magnetic resonance imaging Victor Horsley Department of Neurosurgery, National Hospital for Neurology and Neurosurgery, Queen Square, London, England To whom correspondence should be addressed: Claudia L. Craven, M.R.C.S. [E-mail: [email protected]]
Ehlers-Danlos syndrome can be associated with cerebrospinal fluid (CSF) disturbances including recurrent CSF leak and Chiari I malformations. Persistent pseudomeningoceles are known to be associated with raised intracranial pressure. We present an unusual case of a compressive epidural CSF collection occurring after a computed tomographyLguided L5 nerve root block and describe an effective management strategy.
11 days later. She underwent a second repair, with the assistance of a lumbar drain placed above the CSF collection. Due to her Chiari-I malformation, the lumbar drain was connected to Liquoguard 7 (Moller Medical, Fulda, Germany), a pressure- and volume-controlled
CSF drain that enables intracranial pressure (ICP) monitoring with a safety mechanism preventing overdrainage. Initial median ICP post repair was 24 mm Hg. Over 7 days the ICP gradually reduced to <12 mm Hg when the drain was removed. A 3-month MRI scan confirmed
Citation: World Neurosurg. (2019) 132:67-68. https://doi.org/10.1016/j.wneu.2019.08.163 Journal homepage: www.journals.elsevier.com/worldneurosurgery Available online: www.sciencedirect.com 1878-8750/$ - see front matter Crown Copyright ª 2019 Published by Elsevier Inc. All rights reserved.
A 29-year-old female with Ehlers-Danlos syndrome (EDS) type 3 underwent a transforaminal computed tomography guided L5 nerve root block for radiculalgia caused by grade 1 spondylolisthesis. A 22-gauge spinal needle was positioned adjacent to the left L5 nerve root, and 1.5 mL of 0.25% bupivacaine and 4 mg dexamethasone were instilled. The procedure was not complicated, and the patient went home the same day. Five weeks later she presented to her local emergency department with a leftsided foot drop, with Medical Research Council grade 1 power in ankle dorsiflexion. Her magnetic resonance imaging (MRI) revealed an epidural compressive cerebrospinal fluid (CSF) collection from L4 extending to S2 (Figure 1). She underwent urgent decompression (L5 and S1 fenestration) and repair of the punctuate dural lesion causing the CSF leak (Figure 2), leading to complete recovery of ankle power. Despite being managed on bed rest, she developed a further symptomatic pseudomeningocele
Figure 1. Magnetic resonance imaging demonstrating an epidural compressive cerebrospinal fluid collection extending from L4 down to S2.
WORLD NEUROSURGERY 132: 67-68, DECEMBER 2019
www.journals.elsevier.com/world-neurosurgery
67
CLINICAL IMAGES LAURA PRADINI-SANTOS ET AL.
CSF LEAK IN EHLERS-DANLOS SYNDROME
REFERENCES 1. Reinstein E, Pariani M, Bannykh S, Rimoin DL, Schievink WI. Connective tissue spectrum abnormalities associated with spontaneous cerebrospinal fluid leaks: a prospective study. Eur J Hum Genet. 2013;21:386-390. 2. Milhorat TH, Bolognese PA, Nishikawa M, McDonnell NB, Francomano CA. Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and chiari malformation type I in patients with hereditary disorders of connective tissue. J Neurosurg Spine. 2007;7:601-609.
Figure 2. Intraoperative photo of the punctuate lesion in the dura.
resolution of the pseudomeningocele (Figure 3). Patients with EDS are at risk of developing plexopathies, diskopathy, and degenerative spondylotic disease due to ligamentous laxity. Nerve root injections are considered routine, and the risk of CSF leaks causing neural compromise is rare. However, aforementioned risks may be higher in EDS because such patients have inherently more friable dura, in addition to commonly having
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Figure 3. The 3-month magnetic resonance imaging scan showing resolution of the pseudomeningocele.
disturbances of CSF dynamics (including recurrent CSF leaks and Chiari-I malformations1,2). Raised ICP is a risk factor for persistent pseudomenginoceles.3 In conclusion, nerve root blocks in patients with EDS should be considered with caution. EDS in the presence of raised ICP is uniquely challenging as the propensity for CSF leaks is exacerbated by the raised pressure driving the CSF leak.
3. Craven C, Toma AK, Khan AA, Watkins LD. The role of ICP monitoring in patients with persistent cerebrospinal fluid leak following spinal surgery: a case series. Acta Neurochir (Wien). 2016;158: 1813-1819.
Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. Received 16 July 2019; accepted 22 August 2019 Citation: World Neurosurg. (2019) 132:67-68. https://doi.org/10.1016/j.wneu.2019.08.163 Journal homepage: www.journals.elsevier.com/worldneurosurgery Available online: www.sciencedirect.com 1878-8750/$ - see front matter Crown Copyright ª 2019 Published by Elsevier Inc. All rights reserved.
WORLD NEUROSURGERY, https://doi.org/10.1016/j.wneu.2019.08.163
Extradural Compressive Spinal Cerebrospinal Fluid Leak in Ehlers-Danlos Syndrome Laura Pradini-Santos, Claudia L. Craven, Parag P. Sayal
Key words Cerebrospinal fluid - Connective tissue disorder - Epidural injection - Spine -
Abbreviations and Acronyms CSF: Cerebrospinal fluid EDS: Ehlers-Danlos syndrome ICP: Intracranial pressure MRI: Magnetic resonance imaging Victor Horsley Department of Neurosurgery, National Hospital for Neurology and Neurosurgery, Queen Square, London, England To whom correspondence should be addressed: Claudia L. Craven, M.R.C.S. [E-mail: [email protected]]
Ehlers-Danlos syndrome can be associated with cerebrospinal fluid (CSF) disturbances including recurrent CSF leak and Chiari I malformations. Persistent pseudomeningoceles are known to be associated with raised intracranial pressure. We present an unusual case of a compressive epidural CSF collection occurring after a computed tomographyLguided L5 nerve root block and describe an effective management strategy.
11 days later. She underwent a second repair, with the assistance of a lumbar drain placed above the CSF collection. Due to her Chiari-I malformation, the lumbar drain was connected to Liquoguard 7 (Moller Medical, Fulda, Germany), a pressure- and volume-controlled
CSF drain that enables intracranial pressure (ICP) monitoring with a safety mechanism preventing overdrainage. Initial median ICP post repair was 24 mm Hg. Over 7 days the ICP gradually reduced to <12 mm Hg when the drain was removed. A 3-month MRI scan confirmed
Citation: World Neurosurg. (2019) 132:67-68. https://doi.org/10.1016/j.wneu.2019.08.163 Journal homepage: www.journals.elsevier.com/worldneurosurgery Available online: www.sciencedirect.com 1878-8750/$ - see front matter Crown Copyright ª 2019 Published by Elsevier Inc. All rights reserved.
A 29-year-old female with Ehlers-Danlos syndrome (EDS) type 3 underwent a transforaminal computed tomography guided L5 nerve root block for radiculalgia caused by grade 1 spondylolisthesis. A 22-gauge spinal needle was positioned adjacent to the left L5 nerve root, and 1.5 mL of 0.25% bupivacaine and 4 mg dexamethasone were instilled. The procedure was not complicated, and the patient went home the same day. Five weeks later she presented to her local emergency department with a leftsided foot drop, with Medical Research Council grade 1 power in ankle dorsiflexion. Her magnetic resonance imaging (MRI) revealed an epidural compressive cerebrospinal fluid (CSF) collection from L4 extending to S2 (Figure 1). She underwent urgent decompression (L5 and S1 fenestration) and repair of the punctuate dural lesion causing the CSF leak (Figure 2), leading to complete recovery of ankle power. Despite being managed on bed rest, she developed a further symptomatic pseudomeningocele
Figure 1. Magnetic resonance imaging demonstrating an epidural compressive cerebrospinal fluid collection extending from L4 down to S2.
WORLD NEUROSURGERY 132: 67-68, DECEMBER 2019
www.journals.elsevier.com/world-neurosurgery
67
CLINICAL IMAGES LAURA PRADINI-SANTOS ET AL.
CSF LEAK IN EHLERS-DANLOS SYNDROME
REFERENCES 1. Reinstein E, Pariani M, Bannykh S, Rimoin DL, Schievink WI. Connective tissue spectrum abnormalities associated with spontaneous cerebrospinal fluid leaks: a prospective study. Eur J Hum Genet. 2013;21:386-390. 2. Milhorat TH, Bolognese PA, Nishikawa M, McDonnell NB, Francomano CA. Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and chiari malformation type I in patients with hereditary disorders of connective tissue. J Neurosurg Spine. 2007;7:601-609.
Figure 2. Intraoperative photo of the punctuate lesion in the dura.
resolution of the pseudomeningocele (Figure 3). Patients with EDS are at risk of developing plexopathies, diskopathy, and degenerative spondylotic disease due to ligamentous laxity. Nerve root injections are considered routine, and the risk of CSF leaks causing neural compromise is rare. However, aforementioned risks may be higher in EDS because such patients have inherently more friable dura, in addition to commonly having
68
www.SCIENCEDIRECT.com
Figure 3. The 3-month magnetic resonance imaging scan showing resolution of the pseudomeningocele.
disturbances of CSF dynamics (including recurrent CSF leaks and Chiari-I malformations1,2). Raised ICP is a risk factor for persistent pseudomenginoceles.3 In conclusion, nerve root blocks in patients with EDS should be considered with caution. EDS in the presence of raised ICP is uniquely challenging as the propensity for CSF leaks is exacerbated by the raised pressure driving the CSF leak.
3. Craven C, Toma AK, Khan AA, Watkins LD. The role of ICP monitoring in patients with persistent cerebrospinal fluid leak following spinal surgery: a case series. Acta Neurochir (Wien). 2016;158: 1813-1819.
Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. Received 16 July 2019; accepted 22 August 2019 Citation: World Neurosurg. (2019) 132:67-68. https://doi.org/10.1016/j.wneu.2019.08.163 Journal homepage: www.journals.elsevier.com/worldneurosurgery Available online: www.sciencedirect.com 1878-8750/$ - see front matter Crown Copyright ª 2019 Published by Elsevier Inc. All rights reserved.
WORLD NEUROSURGERY, https://doi.org/10.1016/j.wneu.2019.08.163