scholarly journals 075 A case of spontaneous CSF rhinorrhea in a patient with marfan syndrome

2019 ◽  
Vol 90 (e7) ◽  
pp. A23.3-A24
Author(s):  
Viral Upadhyay ◽  
Salman Khan
Keyword(s):  
Cerebrospinal Fluid ◽  
Marfan Syndrome ◽  
Spontaneous Intracranial Hypotension ◽  
Cerebrospinal Fluid Leak ◽  
Bed Rest ◽  
Connective Tissues ◽  
Csf Leakage ◽  
Hereditary Disorders ◽  
History Of ◽  
Fluid Leak

IntroductionSpontaneous cerebrospinal fluid leak is uncommon condition and frequently associated with hereditary disorders of connective tissues. Nasal CSF leakage is extremely rare.1Methods and resultsWe present the case of a 40-year-old woman presented to hospital for few days history of postural headache associated with clear intermittent discharge from right nostril without any signs of meningism. There was no history of trauma. She has a background history of Marfan syndrome with associated complications of ASD repair at age 2, mechanical Aortic and Mitral valve replacement, aortic root repair, previous ST elevation MI with LV dysfunction, automated implantable cardioverter-defibrillator in situ, atrial fibrillation, and Hashimoto’s thyroiditis. Her regular medications are warfarin, bisoprolol and thyroxine. The clear nasal discharge was positive for β-2 transferrin confirming cerebrospinal fluid. Her CT Brain did not reveal any clear site of CSF leak. She had a flexible nasendoscopy which showed normal nasal passageway, no defect in nasal mucosa and no active CSF leakage. She was managed conservatively with strict bed rest and advised to minimise strenuous activity and heavy lifting.ConclusionSpontaneous cerebrospinal fluid leak is uncommon condition and frequently associated with hereditary disorders of connective tissues. Nasal CSF leakage is extremely rare.1 Testing β-2 transferrin has high sensitivity and specificity.2Initial treatment may include bed rest, oral or intravenous hydration, oral caffeine or corticosteroids.3 4 If conservative therapy fails, surgical repair with nasal endoscopic approach is recommended.2 5ReferencesOmmaya A, Di Chiro G, Baldwin M, Pennybacker J. Non-traumatic cerebrospinal fluid rhinorrhoea. Journal of Neurology, Neurosurgery & Psychiatry 1968;31(3):214-–225.Wang E, Vandergrift W, Schlosser R. Spontaneous CSF Leaks. Otolaryngologic Clinics of North America 2011;44(4):845–856.Milledge J, Ades L, Cooper M, Jaumees A, Onikul E. Severe spontaneous intracranial hypotension and Marfan syndrome in an adolescent. Journal of Paediatrics and Child Health 2005;41(1–2):68–71.Placantonakis D, Bassani L, Graffeo C, Behrooz N, Tyagi V, Wilson T, et al. Noninvasive diagnosis and management of spontaneous intracranial hypotension in patients with marfan syndrome: Case Report and Review of the Literature. Surgical Neurology International 2014;5(1):8.Spontaneous cerebrospinal fluid rhinorrhea. Medicine 2018;97(7):e9954.

scholarly journals Ehlers Danlos, POTS, and Occult Cerebrospinal Fluid Leak: A Case Report

2017 ◽  
Vol 2 (2) ◽  
Keyword(s):  
Cerebrospinal Fluid ◽  
Spontaneous Intracranial Hypotension ◽  
Cerebrospinal Fluid Leak ◽  
Csf Leak ◽  
Postural Orthostatic Tachycardia Syndrome ◽  
Mri Imaging ◽  
Epidural Blood Patching ◽  
Blood Patch ◽  
History Of ◽  
Fluid Leak

Ehlers Danlos syndromes (EDS) are associated with spontaneous intracranial hypotension (SIH) and postural orthostatic tachycardia syndrome (POTS). We hypothesized that some POTS patients might in fact have occult SIH due to unappreciated EDS. We describe a 26-year-old female with a history of POTS and headache who presented to us with negative imaging for cerebrospinal fluid leak (CSF). Upon examination with clinical evidence of EDS and supportive history, we performed an epidural blood patch despite repeat imaging evidence negative for CSF leak resulting in resolution of the patient’s POTS, headache, and cognitive symptoms. Patients with POTS and connective tissue disorders associated with dural weakness may be suffering from occult chronic CSF leaks. MRI imaging may be falsely reassuring and dissuade clinicians from performing epidural blood patching among patients who can be cured.

Ossified ligamentum flavum of the thoracic spine presenting as spontaneous intracranial hypotension: case report

2018 ◽  
Vol 28 (4) ◽  
pp. 401-405 ◽  
Author(s):  
Mazda K. Turel ◽  
Mena G. Kerolus ◽  
John E. O’Toole
Keyword(s):  
Back Pain ◽  
Thoracic Spine ◽  
Intracranial Hypotension ◽  
Ligamentum Flavum ◽  
Radiological Finding ◽  
Spontaneous Intracranial Hypotension ◽  
Cerebrospinal Fluid Leak ◽  
Accurate Diagnosis ◽  
History Of ◽  
Fluid Leak

Ossification of the ligament flavum in the thoracic spine is an uncommon radiological finding in the Western population but can present with back pain, varying degrees of myelopathy, and even paraplegia on occasion. The authors here present the case of a 50-year-old woman with a history of progressive back pain and symptoms of spontaneous intracranial hypotension who was found to have an ossified ligamentum flavum of the thoracic spine resulting in a dural erosion cerebrospinal fluid leak. Surgery involved removal of the ossified ligament flavum at T10–11, facetectomy, ligation of the nerve root, and primary closure of the dura, which resulted in complete resolution of the patient’s symptoms. Radiological, clinical, and intraoperative findings are discussed to assist surgeons with an accurate diagnosis and treatment in the setting of this unusual presentation.

Sonography of the optic nerve sheath diameter before and after microsurgical closure of a dural CSF fistula in patients with spontaneous intracranial hypotension – a consecutive cohort study

Cephalalgia ◽  
2018 ◽  
Vol 39 (2) ◽  
pp. 306-315 ◽  
Author(s):  
Jens Fichtner ◽  
Christian T Ulrich ◽  
Christian Fung ◽  
Debora Cipriani ◽  
Jan Gralla ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Optic Nerve ◽  
Intracranial Hypotension ◽  
Spontaneous Intracranial Hypotension ◽  
Cerebrospinal Fluid Leak ◽  
Optic Nerve Sheath Diameter ◽  
Optic Nerve Sheath ◽  
Cerebrospinal Fluid Leakage ◽  
Nerve Sheath ◽  
Fluid Leak

Objective Spontaneous intracranial hypotension is caused by spinal cerebrospinal fluid leakage. Patients with orthostatic headaches and cerebrospinal fluid leakage show a decrease in optic nerve sheath diameter upon movement from supine to upright position. We hypothesized that the decrease in optic nerve sheath diameter upon gravitational challenge would cease after closure of the leak. Methods We included 29 patients with spontaneous intracranial hypotension and refractory symptoms admitted from 2013 to 2016. The systematic workup included: Optic nerve sheath diameter sonography, spinal MRI and dynamic myelography with subsequent CT. Microsurgical sealing of the cerebrospinal fluid leak was the aim in all cases. Results Of 29 patients with a proven cerebrospinal fluid leak, one declined surgery. A single patient was lost to follow-up. In 27 cases, the cerebrospinal fluid leak was successfully sealed by microsurgery. The width of the optic nerve sheath diameter in supine position increased from 5.08 ± 0.66 mm before to 5.36 ± 0.53 mm after surgery ( p = 0.03). Comparing the response of the optic nerve sheath diameter to gravitational challenge, there was a significant change from before (−0.36 ± 0.32 mm) to after surgery (0.00 ± 0.19 mm, p < 0.01). In parallel, spontaneous intracranial hypotension-related symptoms resolved in 26, decreased in one and persisted in a single patient despite recovery of gait. Conclusions The sonographic assessment of the optic nerve sheath diameter with gravitational challenge can distinguish open from closed spinal cerebrospinal fluid fistulas in spontaneous intracranial hypotension patients. A response to the gravitational challenge, that is, no more collapse of the optic nerve sheath while standing up, can be seen after successful treatment and correlates with the resolution of clinical symptoms. Sonography of the optic nerve sheath diameter may be utilized for non-invasive follow-up in spontaneous intracranial hypotension.

Correlations among brain and spinal MRI findings in spontaneous intracranial hypotension

Cephalalgia ◽  
2018 ◽  
Vol 38 (14) ◽  
pp. 1998-2005 ◽  
Author(s):  
Jr-Wei Wu ◽  
Yen-Feng Wang ◽  
Jong-Ling Fuh ◽  
Jiing-Feng Lirng ◽  
Shih-Pin Chen ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Cerebrospinal Fluid ◽  
Magnetic Resonance ◽  
Intracranial Hypotension ◽  
Spontaneous Intracranial Hypotension ◽  
Brain Magnetic Resonance Imaging ◽  
Cerebrospinal Fluid Leak ◽  
Resonance Imaging ◽  
Imaging Signs ◽  
Fluid Leak

Objectives Several brain and spinal magnetic resonance imaging signs have been described in spontaneous intracranial hypotension. Their correlations are not fully studied. This study aimed to explore potential mechanisms underlying cerebral neuroimaging findings and to examine associations among spinal and brain magnetic resonance imaging signs. Methods We conducted a retrospective review of magnetic resonance myelography and brain magnetic resonance imaging records of patients with spontaneous intracranial hypotension. Categorical principal component analysis was employed to cluster brain neuroimaging findings. Spearman correlation was employed to analyze associations among different brain neuroimaging findings and between brain and spinal neuroimaging findings. Results In patients with spontaneous intracranial hypotension (n = 148), categorical principal component analysis of brain neuroimaging signs revealed two clusters: Cerebral venous dilation and brain descent. Among all brain magnetic resonance imaging signs examined, only midbrain-pons angle associated with anterior epidural cerebrospinal fluid collection length (surrogate spinal cerebrospinal fluid leak severity) (n = 148, Spearman’s ρ = −0.38, p < .001). Subgroup analyses showed that the association between midbrain-pons angle (within brain descent cluster) and spinal cerebrospinal fluid leak severity was presented in patients with convex margins of the transverse sinuses (n = 122, Spearman’s ρ = −0.43, p < .001), but not in patients without convex margins (n = 26, Spearman’s ρ = −0.19, p = .348). The association between severity of transverse sinus distension and spinal cerebrospinal fluid leak severity was only presented in patients without convex margins (n = 26, Spearman’s ρ = 0.52, p = .006). Conclusion This study indicates that there are two factors behind the brain neuroimaging findings in spontaneous intracranial hypotension: Cerebral venous dilation and brain descent. Certain brain neuroimaging signs correlate with spinal cerebrospinal fluid leakage severity, depending on different circumstances.

Symptomatic postsurgical cerebrospinal fluid leak treated by aspiration and epidural blood patch under ultrasound guidance in 2 adolescents

2013 ◽  
Vol 11 (1) ◽  
pp. 87-90 ◽  
Author(s):  
Jared S. Fridley ◽  
Andrew Jea ◽  
Chris D. Glover ◽  
Kim P. Nguyen
Keyword(s):  
Cerebrospinal Fluid ◽  
Adolescent Girls ◽  
Epidural Blood Patch ◽  
Cerebrospinal Fluid Leak ◽  
Symptomatic Patient ◽  
Cerebrospinal Fluid Leakage ◽  
Blood Patch ◽  
Csf Leakage ◽  
Unique Method ◽  
Fluid Leak

Cerebrospinal fluid leakage causing a pseudomeningocele is a well-recognized complication after spine surgery. The repair of pseudomeningocele in a symptomatic patient is usually accomplished with direct open surgical repair of the durotomy, prolonged lumbar drainage, and/or placement of an epidural blood patch. The authors highlight a unique method of pseudomeningocele repair by presenting 2 cases of adolescent girls with symptomatic lumbar pseudomeningoceles. In both cases ultrasound was used to guide the aspiration of CSF from each pseudomeningocele and to apply the epidural blood patch. Both patients had complete and immediate resolution of symptoms. The authors found ultrasound to be a useful tool to assess the extent of the CSF leakage, to determine the degree of aspiration of the extradural CSF, and to confirm the injection of the blood into the epidural space and the space created by the pseudomeningocele.

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