ReviewChild's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery2022
Gorham-Stout disease of skull base leading to cranial settling and rhinorrhea: a case-based review.
Review in Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 6 papers.
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6 citing papers in PubMed, 6 citations in OpenAlex.
- Repeated transitions between intracranial hypotension and intracranial hypertension in Gorham-Stout disease: illustrative case.Journal of neurosurgery. Case lessons · 2026Article
- Gorham-Stout disease of the craniovertebral junction presenting with bow hunter syndrome and cerebellar infarction treated by occipitocervical fusion: illustrative case.Journal of neurosurgery. Case lessons · 2026Article
- Clavicular Gorham-Stout disease: a rare case report and literature review.Frontiers in endocrinology · 2026Review
- Gorham-Stout disease of the craniovertebral junction causing basilar impression and Chiari malformation type I: illustrative case.Journal of neurosurgery. Case lessons · 2025Article
- Gorham-Stout disease affecting the spine with cerebrospinal fluid leakage and Chiari-like tonsillar herniation: a rare case report and review of literature.BMC neurology · 2023Review
- The molecular mechanism of Gorham syndrome: an update.Frontiers in immunology · 2023Review
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5 authors at 2 institutions in 1 country.
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No grant is acknowledged in the PubMed record.
Abstract
purposeGorham-Stout disease (GSD) is a rare progressive osteolytic disorder, theoretically caused by lymphovascular endothelial proliferation. Spinal involvement carries a dismal prognosis because of neurological consequences. Lesions of the skull base are extremely rare and entail even more devastating prognosis due to cervical instability and cerebrospinal fluid (CSF) leakage. Due to scarcity of this condition, the aim of this study was to give an overview of skull base GSD and review the cases with such condition reported in the literature.
methodsIn this case-based review, different aspects of skull base GSD are discussed, and a sample clinical case of GSD leading to cranial settling and rhinorrhea is presented. The characteristics, symptoms, and managements of all English-language PubMed-reported cases were reviewed, and different features of presentation and methods of treatments were analyzed.
resultsBased on the literature review, most of the cases encountered serious problems in the course of the disease. Meningitis/CSF leakage was detected in 12 of 26 collected cases, followed by hearing loss/tinnitus/otitis media in 10 cases, headache in 8, and neck pain/stiffness in 8 patients. Despite a variety of treatments, improvement was only observed in 8 of 26 collected cases. The reminders showed either stable condition or worsening and death.
conclusionAll cases of GSD of the skull base should be evaluated for rhinorrhea/otorrhea and cranial settling, both of them being among the most life-threatening conditions. Since definite treatment, in order to stop disease progression, is sometimes impossible, symptomatic and supportive treatment should be started as possible.
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