Management and Prognosis

For orbital capillary hemangioma, the treatment options consist of observation, intralesional corticoste-roids, systemic corticosteroids, systemic interferon, and surgical resection. Since capillary hemangioma tends to regress with advancing age, tumors that do not displace the globe or cause refractive errors or am-blyopia can be followed with conservative manage ment. Myopic or astigmatic refractive errors should be corrected, and conventional amblyopia treatment with patching of the controlateral eye should be undertaken.22

For periocular hemangioma, intralesional corticosteroids can be used. The combination is usually a mixture of a long-acting and a rapid-acting corticosteroid such as triamcinolone diacetate (Aristocort) or acetonide (Kenalog) and betamethasone sodium phosphate and betamethasone acetate (Celestone).24 The recommended dose is 40 mg of triamcinolone and 6 mg of betamethasone mixed in a single syringe and injected directly into the mass through a 21- to 30-gauge needle.24-26

During intralesional injection of corticosteroid into a capillary hemangioma, injection pressures generally exceed the systemic arterial pressures. This causes the embolization of corticosteroid particles into the ocular circulation from retrograde arterial flow.25,26 When the drug is injected into a terminal artery, the predominant flow is in the direction of least resistance. The resistance to fluid flow in a terminal artery is much less than the resistance in capillaries. Retrograde flow into an arteriole can also occur if the medication is injected at high pressure into a capillary bed.

There are a number of practical guidelines that should be followed to minimize the risk of emboliza-tion of corticosteroid into the ocular circulation25,26:

1. Before each injection of corticosteroid into the lesion, aspiration into the syringe should be performed to detect the presence of arterial blood. If blood is aspirated into the syringe, the cannula should be withdrawn and repositioned.

2. Multiple areas of the capillary hemangioma should be treated with small volumes of cortico-steroid. The individual treatment sites usually receive 0.1 mL of medication. The total volume of corticosteroid is about 0.8 to 1.5 mL for periocular tumors between 4 and 8 mL in volume. The injection volume accounts for approximately 20% of the tumor volume. This is because the injectable space comprising the interstitium and vasculature are assumed to occupy 20 to 30% of the total tumor volume in capillary hemangiomas.

3. The surgeon injecting the corticosteroids should perform indirect ophthalmoscopy and, if there is central retinal artery enucleation, immediate paracentesis of the anterior chamber should be performed and intravenous carbonic anhydrase inhibitors given. Retinal cells survive ischemia for 90 to 100 minutes. Therefore, immediate action should be taken if retinal arterial occlusion is observed on ophthalmoscopy.

4. After the injection, pressure should not be applied to the tumor, and a pressure patch should not be applied. A shield should be placed around the tumor for 24 hours to prevent inadvertent pressure to the lesion.

Rapid involution of the capillary hemangioma starts in 3 days and continues for about 2 weeks. The involution generally lasts for 6 to 8 weeks (Figures 14.5 and 14.6).24 Many tumors shrink to less than 20% of the original volume, attesting to a successful outcome. If involution is not satisfactory, a second injection can be given 1 to 3 months (average 2 months) after the first injection.24 Many patients show nearly complete involution with two injections and rarely require additional corticosteroid injection. The effect of intralesional corticosteroids on capillary hemangioma seems to be related to a vasoconstricting effect. The blanching of the lesion 1 or 2 days after intralesional injection supports this hypothesis of the action of cor-ticosteroids.24

Potential complications of local corticosteroid injections of capillary hemangioma include subcutaneous fat atrophy,27 eyelid necrosis,28 and retinal artery occlusion.26 Orbital capillary hemangiomas located posterior to the orbital septum should not be injected because of the risk of bleeding or hematoma.24 However, the lesion can be exposed surgically, partially debulked, and injected intralesionally.

Larger tumors that do not respond to periocular cor-ticosteroid injections can be treated using a tapering dose of oral prednisone or prednisolone (2 to 3 mg/kg/day).29 The full dose is given in the morning for 4 to 6 weeks and then tapered slowly. If rebound occurs, the lesion usually responds to a higher dose for 2 weeks before tapering again. In time, an alternate-day regimen may be effective. In the past, radiotherapy was occasionally employed in the management of selected orbital and adnexal capillary hemangiomas.29 Extensive capillary hemangiomas that do not respond to systemic corticosteriods can be treated with systemic interferon alfa 2a or 2b (Figure 14.7).30,31 The recommended dose is 1 to 3 million U/day. Treatment is usually started on a lower dose around 1 million U/day and gradually increased to the upper therapeutic level. The drug is expensive and treatment should continue for months to establish a long-lasting remission. Side effects include increased body temperature, elevated serum transaminases, leukocytosis, delay in motor development, flulike syndrome, and congestive heart failure.

Surgical resection can be used for anteriorly located capillary hemangiomas that do not respond to intrale-sional corticosteroid (Figures 14.3 and 14.4).32 More posterior lesions can be difficult to debulk in large volumes or to excise totally. Embolization treatment of orbital capillary hemangioma as the sole treatment or prior to surgical resection has also been reported.33,34 The tumor is more amenable to surgical excision after em-

FIGURE 14.7. (A) Facial photograph demonstrating nonre-gressing extensive left eyelid capillary hemangioma in a 3-year-old girl after several corticosteroid injections. (B) T1-weighted, contrast-enhanced axial orbital MR image showing the hyperintense capillary hemangioma. Note the signal-void high-flow vessels in the lesion. (C) Facial photograph after alfa-2b interferon treatment showing marked regression of the lesion at 1-year follow-up. (D) Tl-weighted axial orbital MR image at 1-year follow-up demonstrating the marked resolution of the capillary hemangioma. The tumor is isointense to slightly hyperintense to the extraocular muscle and cerebral gray matter.

bolization and closing of the feeder vessels.34 Intrale-sional Nd:YAG laser photocoagulation has also been used in selected cases of periorbital and head and neck hemangiomas and vascular malformations.35'36

0 0

Post a comment