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Cavernous Hemangioma Clinical Presentation

  • Author: Adam J Cohen, MD; Chief Editor: Hampton Roy, Sr, MD  more...
 
Updated: Sep 01, 2015
 

History

Patients who present with clinically significant cavernous hemangiomas usually are middle-aged. Some of the more salient clinical symptoms are listed below.

Patients commonly describe a painless, slowly progressive protrusion or bulging of their globe accompanied by mild eyelid fullness.

A change in visual acuity secondary to induced hyperopia or reduction of the myopic refractive error can result from an anteriorly directed mass effect. In some cases, a compressive optic neuropathy can be the etiological basis for the visual acuity or field disturbance.

Extraocular muscle dysfunction and the resultant diplopia secondary to extraocular muscle impingement can cause a patient to seek consultation.

Some patients may describe the feeling of something next to or behind their eye and describe swelling or fullness of their upper lid.

Rarely, a patient harboring a cavernous angioma may describe gaze-evoked amaurosis fugax or headache.

Zauberman and Feinsod described a pregnancy-induced increase in symptomatology.[3]

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Physical

Performing a complete ophthalmologic examination on patients is beneficial.

As with any examination, a thorough history and review of symptoms is paramount in formulating a comprehensive list of differential diagnoses.

Examination of patients should commence with an observation of facial features, noting any asymmetry or scarring. Palpation of the lids and globes allows one to assess differences in lid fullness and increased resistance to retropulsion. Hertel exophthalmometry can detect axial proptosis and should be documented for comparison on follow-up visits.

Visual and color acuities, as well as visual fields, should be assessed, followed by testing of pupillary and extraocular muscle function. Decreased color vision, visual field deficits, and relative afferent pupillary defects warrant immediate imaging to rule out a compressive optic neuropathy. Additionally, any extraocular motility disturbance should be quantitated with prismatic measurements.

Rarely, slit lamp or penlight evaluation may find dilated and tortuous epibulbar vessels, an epibulbar cherry-red spot, or a darkening over insertions of extraocular muscles.

Dilated funduscopic examination may elucidate choroidal folds secondary to compression of the globe by the mass. If the tumefaction is in close proximity to the optic nerve, visible changes may include edema, elevation, pallor, and even atrophy in severe cases.

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Contributor Information and Disclosures
Author

Adam J Cohen, MD Assistant Professor of Ophthalmology, Section Director of Oculoplastic and Reconstructive Surgery, Rush Medical College of Rush University Medical Center

Adam J Cohen, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Ophthalmic Plastic and Reconstructive Surgery, American College of Surgeons

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: BioD, Poferious<br/>Serve(d) as a speaker or a member of a speakers bureau for: IOP<br/>Received income in an amount equal to or greater than $250 from: IOP for speaking.

Coauthor(s)

Michael Mercandetti, MD, MBA, FACS Private Practice

Michael Mercandetti, MD, MBA, FACS is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, Sarasota County Medical Society, American Academy of Ophthalmology, American College of Surgeons, American Society for Laser Medicine and Surgery, American Society of Ophthalmic Plastic and Reconstructive Surgery, Association of Military Surgeons of the US

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Hampton Roy, Sr, MD Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy, Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Additional Contributors

Andrew W Lawton, MD Neuro-Ophthalmology, Ochsner Health Services

Andrew W Lawton, MD is a member of the following medical societies: American Academy of Ophthalmology, Arkansas Medical Society, Southern Medical Association

Disclosure: Nothing to disclose.

Acknowledgements

David A Weinberg, MD, FACS Director, Oculoplastic and Orbital Surgery, Assistant Professor of Neurology and Ophthalmology, Department of Surgery, Division of Ophthalmology, Fletcher Allen Health Care

Disclosure: Nothing to disclose.

References
  1. Henderson GW. Vascular hamartomas, hyperplasias, and neoplasms. Henderson GW, ed. Orbital Tumors. New York: Raven Press; 1994. 94-100.

  2. Harris GJ, Jakobiec FA. Cavernous hemangioma of the orbit: a clinicopathologic analysis of sixty-six cases. Jakobiec, ed. Ocular and Adnexal Tumors. Birmingham, Ala: 1978. 741-81.

  3. Zauberman H, Feinsod M. Orbital hemangioma growth during pregnancy. Acta Ophthalmol (Copenh). 1970. 48(5):929-33. [Medline].

  4. Ko F, Dibernardo CW, Oak J, Miller NR, Subramanian PS. Confirmation of and differentiation among primary vascular lesions using ultrasonography. Ophthal Plast Reconstr Surg. 2011 Nov. 27(6):431-5. [Medline].

  5. Boari N, Gagliardi F, Castellazzi P, Mortini P. Surgical treatment of orbital cavernomas: clinical and functional outcome in a series of 20 patients. Acta Neurochir (Wien). 2011 Mar. 153(3):491-8. [Medline].

  6. Cho KJ, Paik JS, Yang SW. Surgical outcomes of transconjunctival anterior orbitotomy for intraconal orbital cavernous hemangioma. Korean J Ophthalmol. 2010 Oct. 24(5):274-8. [Medline]. [Full Text].

  7. Liu X, Xu D, Zhang Y, Liu D, Song G. Gamma Knife surgery in patients harboring orbital cavernous hemangiomas that were diagnosed on the basis of imaging findings. J Neurosurg. 2010 Dec. 113 Suppl:39-43. [Medline].

  8. Dortzbach RK, Kronish JW. Orbital disease. Dortzbach RK, ed. Ophthalmic Plastic Surgery Prevention and Management of Complications. New York: Raven Press; 1994. 312-25.

  9. Harris GJ, Jakobiec FA. Cavernous hemangioma of the orbit. J Neurosurg. 1979 Aug. 51(2):219-28. [Medline].

  10. Mercandetti M, Cohen AJ. Exophthalmos. Medscape Reference [serial online]. February 7, 2007. [Full Text].

  11. Mercandetti M, Cohen AJ. Tumors, orbital. Medscape Reference [serial online]. February 7, 2007. [Full Text].

  12. Rodgers IR, Grove AS. Vascular lesions of the orbit. Principles and Practice of Ophthalmology. Philadelphia: WB Saunders; 1994. 1970-1.

  13. Shields JA, Shields CL. Vascular and hemorrhagic lesions. Atlas of Orbital Tumors. Philadelphia: Lippincott, Williams and Wilkins; 1999. 50-56.

  14. Yan J, Wu Z. Cavernous hemangioma of the orbit: analysis of 214 cases. Orbit. 2004 Mar. 23(1):33-40. [Medline].

 
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Extirpation of an orbital cavernous hemangioma. Note en bloc removal and preservation of capsule. Courtesy of Robert Alan Goldberg, MD.
MRI demonstrates enhancing mass in apex of left orbit. White arrow points to the superior portion of the optic nerve, showing its deviation. Mass was pressing on superotemporal optic nerve and displacing it inferomedially at apex. Patient had 6 months of progressive decreased vision and visual field loss. Courtesy of M. Duffy, MD, PhD.
In A, final preoperative visual field of same patient as in Media file 2, demonstrating significant inferior altitudinal field loss; in B, postoperative visual field at approximately 3 weeks after orbital apex decompression and removal of mass; and in C, postoperative visual field at approximately 6 months.
Intraoperative photo of same patient as in Media file 2. Neurosurgical service performed craniotomy and decompression of the superior orbital fissure and optic canal (yellow arrows) at request of ophthalmology service. Orbital surgery service then opened the periorbita over a bulge (double black arrows) between optic nerve and cranial nerves (single black arrow) and bluntly dissected out mass. Pathology confirmed mass as a cavernous hemangioma. Cranial nerves V and IV were adhered, and careful blunt separation was performed. Postoperatively, a small left hypertropia resolved over 6 weeks. Courtesy of M. Duffy, MD, PhD.
 
 
 
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