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Orbital cavernous hemangioma


Key points

  • Definition: Orbital cavernous hemangioma is a benign, slow-growing, vascular lesion manifesting as painless, progressive proptosis.

  • Synonyms: Ocular cavernous hemangioma, orbital cavernous malformation, orbital cavmal.

  • Classic clue: Well-circumscribed, enhancing intraconal mass in adult patients.

  • Usual imaging appearance

  • Computed tomography (CT) features:

  • Intraconal mass.

  • Homogeneous, well-circumscribed.

  • Enhancement ↑↑↑↑.

  • Magnetic resonance imaging (MRI) features:

  • T1 isointense.

  • T2 hyperintense.

  • T1 + Gd enhancement.

  • “Blooming” artifact.

  • Most common orbital tumor in adult age group.

  • Usually unilateral.

Imaging

CT findings

  • Precontrast hyperdensity related to microcalcifications.

    • Postcontrast hyperdensity related to enhancement + calcifications.

CT recommendations

  • Many of these lesions are found incidentally when CT is performed for other reasons.

  • CT should not be used as the procedure of choice when MRI can be performed.

  • Radiation dosages should always be kept in mind when imaging orbits.

  • In today’s radiation-conscious climate, MRI’s lack of radiation tops any CT radiation dose, anytime.

  • 3D reconstructions often useful.

MRI findings

  • T1 isointense to muscle.

  • T2 hyperintense to muscle.

  • Hypointense pseudocapsule rim with hemosiderin.

  • Characteristic “mulberry” appearance.

  • Early central, patchy enhancement fills in to a homogeneous pattern over time.

  • “Blooming” related to susceptibility artifact more pronounced with gradient echo techniques.

  • Higher deposition of hemosiderin and longer T Es of T2-weighted images accentuate “blooming.”

MRI recommendations

  • Clearly the modality of choice for imaging the orbit when this type of lesion is suspected or when one is being followed.

  • MRI often characteristic and pathognomonic, precluding the need for additional imaging.

Ultrasound findings

  • Retrobulbar mass with heterogeneous hyperechogenicity.

  • Low resistance, slow flow on Doppler.

Clinical issues

Presentation

  • More common in females than in males, peak incidence in early middle age.

  • Usual presenting symptom: Painless progressive proptosis.

  • Other symptoms: Diplopia and vision loss.

  • Fundoscopic examination:

    • Increased intraocular pressure.

    • Retinal striae.

  • Almost always unilateral.

Natural history

  • Slow progression of enlargement.

  • May eventually cause orbital compression.

  • Recurrent exopthalmos related to puberty, pregnancy and recurrent hemorrhages.

  • Some remodeling of adjacent orbital bone.

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