title: Transclival cerebrospinal fluid fistula in a patient with Marfan's syndrome.
abstract: Marfan's syndrome is a disease associated with reduced structural integrity of connective tissues. We report a 36-year-old patient with Marfan's syndrome who presented with rhinorrhoea, occipital headache and vomiting. Physical examination revealed typical Marfan's syndrome features including dolicocephalous, mandibular micrognathia, tall stature, disproportionately long limbs and digits, and hypermobility of the joints. A high-resolution CT scan demonstrated pneumoencephalous, cerebrospinal fluid (CSF) filling the sphenoidal sinus, and a small bone defect of the clivus. Surgery performed through a transsphenoidal approach revealed the sphenoid sinus to be filled with CSF and a small fenestration in the clivus. The arachnoid diverticulum and the fenestration were repaired and covered with a graft of abdominal fat. In this patient, a deficiency in bone development associated with Marfan's syndrome gave rise to a clival fenestration and a transclival CSF fistula. Although abnormalities of the spinal meningeal membranes have been reported in Marfan's syndrome, to our knowledge, this is the first report of a fistula located in the cranial base in this condition.
authors.affiliation: Department of Internal Medicine, Hospital Universitario Puerta de Hierro, Universidad Autónoma de Madrid, Madrid, Spain. antrammar@yahoo.es
authors.forename: A
authors.initials: A
authors.lastname: Ramos
authors.forename: J
authors.initials: J
authors.lastname: García-Uría
authors.forename: L
authors.initials: L
authors.lastname: Ley
authors.forename: G
authors.initials: G
authors.lastname: Saucedo
mesh_terms: D000328:Adult; D016080:Arachnoid Cysts / Q000150:complications / Q000473:pathology* / Q000601:surgery; D002559:Cerebrospinal Fluid Rhinorrhea / Q000209:etiology / Q000473:pathology* / Q000503:physiopathology; D003388:Cranial Fossa, Posterior / Q000002:abnormalities* / Q000473:pathology / Q000601:surgery; D005260:Female; D005402:Fistula / Q000209:etiology / Q000473:pathology* / Q000503:physiopathology; D006261:Headache / Q000209:etiology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008382:Marfan Syndrome / Q000150:complications*; D019635:Neurosurgical Procedures; D013101:Sphenoid Sinus / Q000473:pathology / Q000503:physiopathology / Q000601:surgery; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014839:Vomiting / Q000209:etiology