Spontaneous dural tear leading to intracranial hypotension and tonsillar herniation in Marfan syndrome: a case report

Aqueel H Pabaney,Humera Ahsan,Farhan A Mirza,Nadir A Syed

doi:10.1186/1471-2377-10-54

Abstract

BackgroundWe describe the case of a 38 year old male with Marfan syndrome who presented with orthostatic headaches and seizures.Case PresentationThe patient was diagnosed with Spontaneous Intracranial Hypotension secondary to CSF leaks, objectively demonstrated by MR Myelogram with intrathecal contrast. Epidural autologus blood patch was administered at the leakage site leading to significant improvement.ConclusionOur literature search shows that this is the second reported case of a Marfan patient presenting with symptomatic spontaneous CSF leaks along with tonsillar herniation.

Highlights

We describe the case of a 38 year old male with Marfan syndrome who presented with orthostatic headaches and seizures.Case Presentation: The patient was diagnosed with Spontaneous Intracranial Hypotension secondary to Cerebrospinal Fluid (CSF) leaks, objectively demonstrated by MR Myelogram with intrathecal contrast
Spontaneous intracranial hypotension (SIH) is an under diagnosed entity that was first described by Schaltenbrand in 1938 [1]; a more objective description of SIH was later proposed as a "decrease in CSF pressure to less than 60 mm H2O associated with occipital headaches [2,3]"
Among the connective tissue disorders (CTDs), Marfan,[6,7,8] Ehlers-Danlos type II [9] and autosomal dominant polycystic kidney disease [10] are associated with spontaneous CSF leaks leading to SIH

Summary

Background

Spontaneous intracranial hypotension (SIH) is an under diagnosed entity that was first described by Schaltenbrand in 1938 [1]; a more objective description of SIH was later proposed as a "decrease in CSF pressure to less than 60 mm H2O associated with occipital headaches [2,3]". Case Presentation Our patient, a 38 year old male, diagnosed case of Marfan's syndrome, presented with complaints of orthostatic headaches and one episode of seizure. His current illness started 15 days ago with bilateral neck pain, which progressed to a holocranial headache which would get markedly worse on sitting, standing or bending forward and relived by lying down. In the first 48 hours after the procedure, strict recumbent position was maintained He was discharged with instructions to gradually resume sitting posture from complete bed rest by adding one to two pillows under his head every day. He was followed up regularly as an outpatient for the 6 months with no evidence of any complications or recurrence and returned to normal baseline activity and lifestyle

Discussion

Findings

Conclusion

Schaltenbrand V