Intracranial hypotension syndrome in a pediatric patient with Marfan syndrome
DOI:
https://doi.org/10.23938/ASSN.1031Keywords:
Orthostatic headache, Intracranial hypotension, Fistula, Cerebrospinal fluid, Marfan syndromeAbstract
Intracranial hypotension syndrome (IHS) is attributed to reduced cerebrospinal fluid (CSF) pressure. It may be spontaneous or secondary to a history of trauma or systemic disease.
We present the case of an 11-year-old boy, with medical history of Marfan syndrome, with orthostatic headache and persistent vomiting (12 hours) following a fall on the sacrococcygeal region. Magnetic resonance showed extradural fluid collections at dorsal and lumbosacral levels, compatible with CSF leak. The condition was resolved with treatment, but the patient had two new episodes during the follow-up period. Thus, an epidural blood patch was performed two years after the first episode.
Although HIS is uncommon in children, it should be suspected in patients with orthostatic headache, particularly if the patient presents a connectivopathy. Few studies have assessed the management of HIS in paediatric age. The case presented here and the reviewed available literature provides further data for these type of cases.
Downloads
References
RODRÍGUEZ A, CASTRO D, MIÑAMBRES J. Cefalea por hipotensión del líquido cefalorraquídeo. Rev Clin Med Fam 2008; 2(4): 181-183. https://doi.org/10.4321/S1699-695X2008000200009
LIN J, ZHANG S, HE F, LIU M, MA X. The status of diagnosis and treatment to intracranial hypotension, including SIH. J Headache Pain 2017; 18(4): 1-8. https://doi.org/10.1186/s10194-016-0708-8
CABREA BUENO F, GALLEGO GARCÍA DE VINUESA P, EVANGELISTA MASIP A. Nuevos criterios diagnósticos en el síndrome de Marfan. Cardiocore 2011; 46(3): 85-88. https://doi.org/10.1016/j.carcor.2011.05.003
PICHOTT A, BERNSTEIN T, GUZMAN G, FARIÑA G, AGUIRRE D, ESPINOZa A. Ectasia dural e hipotensión endocraneal en síndrome de Marfán. Rev Chil Pediatr 2020; 91(4): 591-596. https://doi.org/10.32641/rchped.v91i4.1233
DEPUTY SR, TILTON AH. New-onset headache in an adolescent with MASS syndrome. Semin Pediatr Neurol 1999; 6(3): 177-181. https://doi.org/10.1016/S1071-9091(99)80010-2
MILLEDGE JT, ADES LC, COOPER MG, JAUMEES A, ONIKUL E. Severe spontaneous intracranial hypotension and Marfan syndrome in an adolescent. J Paediatr Child Health 2005; 41(1-2): 68-71. https://doi.org/10.1111/j.1440-1754.2005.00541.x
ROSSER T, FINKEL J, VEZINA G, MAJD M. Postural headache in a child with Marfan syndrome: case report and review of the literature. J Child Neurol 2005; 20(2): 153-155. https://doi.org/10.1177/08830738050200021701
CHEURET E, EDOUARD T, MEJDOUBI M, ACAR P, PIENKOWSKI C, CANCES C et al. Intracranial hypotension in a girl with Marfan syndrome: case report and review of the literature. Childs Nerv Syst 2008; 24(4): 509-513. https://doi.org/10.1007/s00381-007-0506-3
PUGET S, KONDAGESKI C, WRAY A, BODDAERT N, ROUJEAU T, DI ROCCO F et al. Chiari-like tonsillar herniation associated with intracranial hypotension in Marfan syndrome. Case report. J Neurosurg 2007; 106(1): 48-52. https://doi.org/10.3171/ped.2007.106.1.48
CHUNG SJ, KI CS, LEE MC, LEE JH. Fibrillin-1 gene analysis of Korean patients with spontaneous CSF hypovolemia. Headache 2007; 47(1): 111-115. https://doi.org/10.1111/j.1526-4610.2006.00635.x
VOERMANS NC, DIJK KG, BOS MM, GEUS-OEI LF, VERRIPS A, LINDERT EJ. Postural headache in marfan syndrome associated with spinal cysts and liquor hypotension. Neuropediatrics 2009; 40(4): 201-204. https://doi.org/10.1055/s-0029-1243164
ROSDY B, KOLLÁR K, MÓSER J, VÁRALLYAY G, KORDAS M. [Severe intracranial hypotension in an adolescent with Marfan syndrome. Case report]. Ideggyogy Sz 2009; 62(5-6): 181-184.
ABU LIBDEH A, MATSUMOTO JA, DHAMIJA R. Teaching NeuroImages: Intracranial hypotension in a patient with Marfan syndrome. Neurology 2016; 87(4): e40-e41. https://doi.org/10.1212/WNL.0000000000002896
MIRCHI A, SAINT-MARTIN C, MYERS KA. Spontaneous multilevel cerebrospinal fluid leak in Marfan syndrome. Ann Neurol 2020; 88(4): 855-856. https://doi.org/10.1002/ana.25837
BEZOV D, LIPTON RB, ASHINA S. Post-dural puncture headache : Part I Diagnosis, epidemiology, etiology and pathophysiology. Headache 2010; 50: 1144-1152. https://doi.org/10.1111/j.1526-4610.2010.01699.x
MOKRI B. Spontaneous CSF leaks: low CSF volume syndromes. Neurol Clin NA 2014; 32(2): 397-422. https://doi.org/10.1016/j.ncl.2013.11.002
Headache Classification Committee of the International Headache Society (HIS). The international classification of headache disorders 3rd edition. Cephalalgia 2018; 38(1): 1-211. https://doi.org/10.1177/0333102417738202
AKBAR JJ, LUETMER PH, SCHWARTZ KM, HUNT CH, DIEHN FE, ECKELHUNT LJ. The role of MR myelography with intrathecal gadolinium in localization of spinal CSF leaks in patients with spontaneous intracranial. AJNR Am J Neuroradio. 2012; 33: 535-540. https://doi.org/10.3174/ajnr.A2815
TYAGI A. Management of spontaneous intracranial hypotension. Pract Neurol. 2016; 16(2): 87-88. https://doi.org/10.1136/practneurol-2015-001343
SCHIEVINK WI, MAYA MM, LOUY C, MOSER FG, SLONINSKY L. Spontaneous intracranial hypotension in childhood and adolescence. J Pediatr 2013; 163(2):5 04-510. https://doi.org/10.1016/j.jpeds.2013.01.055
COHEN-GADOL AA, MOKRI B, PIEPGRAS DG, MEYER FB, ATKINSON JLD. Surgical anatomy of dural defects in spotaneous spinal cerebrospinal fluid leaks. Neurosurgery 2006; 58: 238-245. https://doi.org/10.1227/01.NEU.0000204712.16099.FB
Downloads
Published
How to Cite
Issue
Section
License
This work is licensed under a Creative Commons Attribution-ShareAlike 4.0 International License.
La revista Anales del Sistema Sanitario de Navarra es publicada por el Departamento de Salud del Gobierno de Navarra (España), quien conserva los derechos patrimoniales (copyright ) sobre el artículo publicado y favorece y permite la difusión del mismo bajo licencia Creative Commons Reconocimiento-CompartirIgual 4.0 Internacional (CC BY-SA 4.0). Esta licencia permite copiar, usar, difundir, transmitir y exponer públicamente el artículo, siempre que siempre que se cite la autoría y la publicación inicial en Anales del Sistema Sanitario de Navarra, y se distinga la existencia de esta licencia de uso.
