CC BY-NC-ND 4.0 · Asian J Neurosurg 2024; 19(02): 179-185
DOI: 10.1055/s-0044-1779447
Research Article

Atypical Presentation of Idiopathic Intracranial Hypertension: A Case Series and Literature Review

1   Neurosurgery Division, Department of Surgery, College of Medicine, King Saud University, Riyadh, Saudi Arabia
Aued I. Alanazi
2   Department of surgery, College of Medicine, King Saud University, Riyadh, Saudi Arabia
Sherin Hamad Alokayli
2   Department of surgery, College of Medicine, King Saud University, Riyadh, Saudi Arabia
Sarah Maghrabi
2   Department of surgery, College of Medicine, King Saud University, Riyadh, Saudi Arabia
Sherif M. Elwatidy
1   Neurosurgery Division, Department of Surgery, College of Medicine, King Saud University, Riyadh, Saudi Arabia
› Author Affiliations
Funding The authors extend their appreciation to the Deputyship for Research & Innovation, Ministry of Education in Saudi Arabia, for funding this research through Project No. (IFKSURG-1423).


Idiopathic intracranial hypertension (IIH) is a condition in which intracranial pressure (ICP) increases without an apparent cause. Typically, patients present with headaches, dizziness, pulsatile tinnitus, visual disturbances, blurred vision, diplopia, photophobia, visual field defects, and papilledema on fundoscopy. The association between IIH, spontaneous cerebrospinal fluid (CSF) rhinorrhea, and arachnoid cysts has been discussed in the literature; however, there is no clear explanation for this association. We aimed to present a series of four patients with a confirmed diagnosis of IIH with atypical presentations, discuss the management of each case, and provide an explanation for this association to alert clinicians to the atypical presentation of IIH and facilitate early diagnosis and proper treatment of this condition by CSF diversion. This was a retrospective case series of all patients who were diagnosed with IIH and showed improvement after ventriculoperitoneal shunt insertion after failure of at least one operative intervention resulting from primary radiological and clinical findings in 2001 to 2022. Data on demographics, clinical presentation, radiological findings, surgical management, and diagnostic criteria for IIH were recorded. We identified four patients with a confirmed diagnosis of IIH who presented with atypical presentations as follows: intracranial arachnoid cyst, cervical spine arachnoid cyst, giant Virchow perivascular space, and spontaneous CSF (CSF) rhinorrhea. All patients responded to CSF diversion after failure of surgical treatment targeting the primary pathology. IIH should be suspected after the failure of primary surgical treatment in cases of spontaneous CSF rhinorrhea, spinal and cranial arachnoid cysts, and symptomatic ventriculoperitoneal shunt. Treatment in such situations should be directed toward IIH with CSF diversion.

Ethical Approval

The manuscript does not contain patient data or prospective clinical studies.

Authors' Contributions

F.S.A. helped in conceptualization, methodology, software, validation, visualization, writing, review and editing. A.I.A. was involved in conceptualization, data curation, formal analysis, investigation, methodology, project administration, resources, validation, visualization, writing original draft, review and editing. S.A. and S.M. helped in data curation, investigation, resources, software, validation, and visualization. S.M.E. was involved in conceptualization, data curation, methodology, software, supervision, validation, visualization, writing review and editing.

Publication History

Article published online:
27 May 2024

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