Perspective
Cavernous Hemangioma of the Orbital Apex: Pathogenetic Considerations in Surgical Management

https://doi.org/10.1016/j.ajo.2010.07.027Get rights and content

Purpose

To consider the pathogenesis and growth of cavernous hemangioma, particularly within the crowded orbital apex, in decisions regarding surgical indications, timing, and technique.

Design

A perspective based on analysis of the microanatomic relationships and growth potential of apical cavernous hemangiomas, with representative case studies illustrating management recommendations.

Methods

Analysis of microscopic findings in typical and vision-loss cases; review of tumor growth patterns as reported in observational and interventional studies; consideration of surgical approaches and reported functional outcomes.

Results

An ongoing, local hemodynamic imbalance may drive the proliferation of a cavernous hemangioma. Extension into neighboring tissue induces a fibrous capsule, which is continually reconstituted as the lesion expands, and which may incorporate visually critical structures in the confines of the apex. The extent of this microanatomic intimacy is not detectable preoperatively. The tumor's remaining growth potential at the time of diagnosis or following incomplete resection is not predictable.

Conclusions

Patients without significant vision deficits should be observed for progression. Those with significant deficits or signs of progression should be offered timely surgery, with recognition of the risks. The surgical approach should be individualized based on macroanatomic relationships. The decision to intervene should not be a commitment to complete resection at any cost; intraoperative recognition of “inoperable” attachments may dictate modifications in order to preserve vision.

Section snippets

Management Recommendations

Considering the risk of unchecked tumor growth into apical structures—and the risk of tumor removal from them—each case requires thoughtful analysis.

If a lesion with imaging features typical for hemangioma is serendipitously found in a patient with normal ophthalmic findings, then continued observation is indicated. At the time of discovery, it is unknown whether the lesion is actively growing or has been stable for years, and without vision impairment, the risk of surgery outweighs any

Case 1

A 51-year-old woman had been aware of blurred vision in the left eye for 2 years, orbital asymmetry for 18 months, and left-sided headache for 2 weeks. Best-corrected vision was 20/25 OD and 20/80 OS, with a 2+ left relative afferent pupillary defect (RAPD). The left globe was laterally displaced, relatively proptotic by 4 mm, and fully mobile. Orbital imaging showed a large tumor wedged between the optic nerve and the medial and inferior rectus muscles, with pressure expansion of the apical

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