Clinical Course of Retrobulbar Hemangioblastomas in von Hippel-Lindau Disease

Catherine Meyerle, Sam S. Dahr, Nicholas M. Wetjen, Guy V. Jirawuthiworavong, John A. Butman, Russell R. Lonser, Edward Oldfield, Hanna Rodriguez-Coleman, Wai T. Wong, Emily Y. Chew

Research output: Contribution to journalArticle

Abstract

Objective: To report clinical findings of rare retrobulbar optic nerve hemangioblastomas associated with von Hippel-Lindau disease (VHL). Design: Retrospective observational case series. Participants: Nine patients with VHL. Methods: The clinical course and magnetic resonance imaging findings of patients with VHL and hemangioblastomas affecting the anterior visual pathway from the intraorbital optic nerve to the optic chiasm are reviewed. Main Outcome Measure: Clinical course of retrobulbar optic nerve hemangioblastomas. Results: The mean age of VHL diagnosis was 24±14 years, and mean follow-up was 5±4 years. All had other CNS lesions and retinal hemangioblastomas. Approximately 50% (5/9) had a previous enucleation or had visual acuity loss (4/9), some due to other VHL ocular complications. Four patients underwent surgical resection of an intracranial hemangioblastoma. Growth patterns and pathology are similar to those of other hemangioblastomas in the CNS. Conclusions: Although these lesions are rare, patients with VHL who present with signs of optic neuropathy should be evaluated for anterior visual pathway hemangioblastomas impinging on the optic nerve from the orbit to the chiasm. On neuroimaging, the hemangioblastomas may demonstrate chiasmal or optic tract edema, associated cysts, and T2 flow voids. Lesions may remain radiologically and clinically stable, evolve radiographically with no visual or neurological progression, or progress clinically and radiographically. Patients at risk for visual loss should be considered for surgical resection. Close coordination among neuroradiology, neurosurgery, and ophthalmology patient care teams is advised for optimal management of these patients.

Original languageEnglish (US)
Pages (from-to)1382-1389
Number of pages8
JournalOphthalmology
Volume115
Issue number8
DOIs
StatePublished - Aug 2008
Externally publishedYes

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Hemangioblastoma
von Hippel-Lindau Disease
Optic Nerve
Visual Pathways
Optic Chiasm
Optic Nerve Diseases
Patient Care Team
Neurosurgery
Orbit
Ophthalmology
Neuroimaging
Visual Acuity
Cysts
Edema
Magnetic Resonance Imaging
Outcome Assessment (Health Care)
Pathology

ASJC Scopus subject areas

  • Ophthalmology

Cite this

Meyerle, C., Dahr, S. S., Wetjen, N. M., Jirawuthiworavong, G. V., Butman, J. A., Lonser, R. R., ... Chew, E. Y. (2008). Clinical Course of Retrobulbar Hemangioblastomas in von Hippel-Lindau Disease. Ophthalmology, 115(8), 1382-1389. https://doi.org/10.1016/j.ophtha.2008.01.027

Clinical Course of Retrobulbar Hemangioblastomas in von Hippel-Lindau Disease. / Meyerle, Catherine; Dahr, Sam S.; Wetjen, Nicholas M.; Jirawuthiworavong, Guy V.; Butman, John A.; Lonser, Russell R.; Oldfield, Edward; Rodriguez-Coleman, Hanna; Wong, Wai T.; Chew, Emily Y.

In: Ophthalmology, Vol. 115, No. 8, 08.2008, p. 1382-1389.

Research output: Contribution to journalArticle

Meyerle, C, Dahr, SS, Wetjen, NM, Jirawuthiworavong, GV, Butman, JA, Lonser, RR, Oldfield, E, Rodriguez-Coleman, H, Wong, WT & Chew, EY 2008, 'Clinical Course of Retrobulbar Hemangioblastomas in von Hippel-Lindau Disease', Ophthalmology, vol. 115, no. 8, pp. 1382-1389. https://doi.org/10.1016/j.ophtha.2008.01.027
Meyerle C, Dahr SS, Wetjen NM, Jirawuthiworavong GV, Butman JA, Lonser RR et al. Clinical Course of Retrobulbar Hemangioblastomas in von Hippel-Lindau Disease. Ophthalmology. 2008 Aug;115(8):1382-1389. https://doi.org/10.1016/j.ophtha.2008.01.027
Meyerle, Catherine ; Dahr, Sam S. ; Wetjen, Nicholas M. ; Jirawuthiworavong, Guy V. ; Butman, John A. ; Lonser, Russell R. ; Oldfield, Edward ; Rodriguez-Coleman, Hanna ; Wong, Wai T. ; Chew, Emily Y. / Clinical Course of Retrobulbar Hemangioblastomas in von Hippel-Lindau Disease. In: Ophthalmology. 2008 ; Vol. 115, No. 8. pp. 1382-1389.
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abstract = "Objective: To report clinical findings of rare retrobulbar optic nerve hemangioblastomas associated with von Hippel-Lindau disease (VHL). Design: Retrospective observational case series. Participants: Nine patients with VHL. Methods: The clinical course and magnetic resonance imaging findings of patients with VHL and hemangioblastomas affecting the anterior visual pathway from the intraorbital optic nerve to the optic chiasm are reviewed. Main Outcome Measure: Clinical course of retrobulbar optic nerve hemangioblastomas. Results: The mean age of VHL diagnosis was 24±14 years, and mean follow-up was 5±4 years. All had other CNS lesions and retinal hemangioblastomas. Approximately 50{\%} (5/9) had a previous enucleation or had visual acuity loss (4/9), some due to other VHL ocular complications. Four patients underwent surgical resection of an intracranial hemangioblastoma. Growth patterns and pathology are similar to those of other hemangioblastomas in the CNS. Conclusions: Although these lesions are rare, patients with VHL who present with signs of optic neuropathy should be evaluated for anterior visual pathway hemangioblastomas impinging on the optic nerve from the orbit to the chiasm. On neuroimaging, the hemangioblastomas may demonstrate chiasmal or optic tract edema, associated cysts, and T2 flow voids. Lesions may remain radiologically and clinically stable, evolve radiographically with no visual or neurological progression, or progress clinically and radiographically. Patients at risk for visual loss should be considered for surgical resection. Close coordination among neuroradiology, neurosurgery, and ophthalmology patient care teams is advised for optimal management of these patients.",
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AU - Wetjen, Nicholas M.

AU - Jirawuthiworavong, Guy V.

AU - Butman, John A.

AU - Lonser, Russell R.

AU - Oldfield, Edward

AU - Rodriguez-Coleman, Hanna

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AU - Chew, Emily Y.

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N2 - Objective: To report clinical findings of rare retrobulbar optic nerve hemangioblastomas associated with von Hippel-Lindau disease (VHL). Design: Retrospective observational case series. Participants: Nine patients with VHL. Methods: The clinical course and magnetic resonance imaging findings of patients with VHL and hemangioblastomas affecting the anterior visual pathway from the intraorbital optic nerve to the optic chiasm are reviewed. Main Outcome Measure: Clinical course of retrobulbar optic nerve hemangioblastomas. Results: The mean age of VHL diagnosis was 24±14 years, and mean follow-up was 5±4 years. All had other CNS lesions and retinal hemangioblastomas. Approximately 50% (5/9) had a previous enucleation or had visual acuity loss (4/9), some due to other VHL ocular complications. Four patients underwent surgical resection of an intracranial hemangioblastoma. Growth patterns and pathology are similar to those of other hemangioblastomas in the CNS. Conclusions: Although these lesions are rare, patients with VHL who present with signs of optic neuropathy should be evaluated for anterior visual pathway hemangioblastomas impinging on the optic nerve from the orbit to the chiasm. On neuroimaging, the hemangioblastomas may demonstrate chiasmal or optic tract edema, associated cysts, and T2 flow voids. Lesions may remain radiologically and clinically stable, evolve radiographically with no visual or neurological progression, or progress clinically and radiographically. Patients at risk for visual loss should be considered for surgical resection. Close coordination among neuroradiology, neurosurgery, and ophthalmology patient care teams is advised for optimal management of these patients.

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