Cranial cerebrospinal fluid leak and intracranial hypotension syndrome - a case report.

Elena Oana Terecoasa,Iulian Enache,Cristina Tiu,Andreea Nicoleta Marinescu,Razvan Alexandru Radu

doi:10.25122/jml-2021-0090

Abstract

Spontaneous intracranial hypotension is a rare clinical entity caused in most cases by a cerebrospinal fluid leak occurring at the level of the spinal cord. Cranial dural leaks have been previously reported as a cause of orthostatic headaches but, as opposed to spinal dural leaks, were not associated with other findings characteristic of spontaneous intracranial hypotension. We present the case of a male admitted for severe orthostatic headache. The patient had a history of intermittent postural headaches, dizziness, and symptoms consistent with post-nasal drip, which appeared several years after head trauma. Brain imaging showed signs consistent with intracranial hypotension: bilateral hygromas, subarachnoid hemorrhage, superficial siderosis, diffuse contrast enhancement of the pachymeninges, and superior sagittal sinus engorgement. No spinal leak could be identified by magnetic resonance imaging, and the patient had a rapid remission of symptoms with conservative management. Further work-up identified an old temporal bone fracture which created a route of egress between the posterior fossa and the mastoid cells. Otorhinolaryngology examination showed pulsatile bloody discharge and liquorrhea at the level of the left pharyngeal opening of the Eustachian tube. The orthostatic character of the headache, as well as the brain imaging findings, were consistent with intracranial hypotension syndrome caused by a cranial dural leak. Clinical signs and imaging findings consistent with the diagnosis of apparently “spontaneous” intracranial hypotension should prompt the search for a cranial dural leak if a spinal leak is not identified.

Highlights

Orthostatic headache is the hallmark finding of spontaneous intracranial hypotension (SIH)
Superficial siderosis is uncommon in acute cases of SIH, but it can be the consequence of chronic stretching and repeated bleeding from the superficial bridging veins during acute episodes of cerebrospinal fluid (CSF) volume depletion [5, 6]
We did not perform dynamic myelography to rule out a spinal CSF leak, the brain magnetic resonance imaging (MRI) changes together with the findings of the otorhinolaryngology examination strongly point towards a cranial CSF leak leading to intracranial hypotension syndrome in our patient

Summary

INTRODUCTION

Orthostatic headache is the hallmark finding of spontaneous intracranial hypotension (SIH). We report the case of a 76-year-old Caucasian man who presented to our hospital for acute onset epistaxis associated with headache and dizziness He had a history of arterial hypertension, left bundle branch block, dyslipidemia and heart failure, for which he was taking antihypertensive and antiplatelet drugs. Two spinal cord MRI scans aiming to identify a potential CSF leak were performed on separate days, but no signs of dural leakage were identified His headache and dizziness progressively improved, but two days after admission, he complained of left ear fullness. The patient remained symptom-free and was discharged home He was further referred for ENT and neurosurgical follow–up in an experienced center for further assessment of the opportunity of surgical exploration aimed to clearly localize and seal the cranial dural defect and the temporal bone fracture. He did not report any other symptoms in the first month after discharge and refused surgery proposed by a joint neurosurgical-otorhinolaryngology team

Findings

DISCUSSION

CONCLUSION