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

Abstract

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.