Orbital Vascular Tumors
There was an excellent session on orbital lymphovascular tumors,for which there are many more treatment options than we have had in the past. The speakers during this session discussed debulking and percutaneous drains in conjunction with using sclerosing agents for lymphangioma and lymphovenous malformations.
Different sclerosing agents are now available, and low-dose bleomycin seems to work better than in the past with other sclerosing agents.
Deborah Alcorn reported that sildenafil may even be effective in lymphangioma treatment, but more research is needed in this area. The mechanism of action of sildenafil in this situation is not known. Sildenafil works by relaxation of smooth muscle, so when the venous channels become decongested, it may allow the cystic components in the lymphangioma to decompress. Or it may cause cyst compression from the vasodilation.
In the same session, Gregg Lueder presented an update on infantile capillary hemangioma. In the past, steroids were the mainstay of treatment. But in recent years, propranolol has become the first-line agent for vision-threatening hemangiomas. It has been used in children for more than 40 years for cardiovascular indications. In hemangioma, propranolol has an advantage over steroids of having an incredibly rapid onset with a low-risk profile.
I followed Dr. Lueder with a discussion of PHACE syndrome, which is a neurocutaneous disorder, comprising posterior fossa malformations, segmental hemangiomas, arterial anomalies, cardiac defects, eye anomalies, and sternal raphae. Because of the increased risk for stroke and cardiovascular complications, propranolol should be used with caution in patients with PHACE syndrome, and initiation of treatment should take place in the hospital.
Arif Khan reported in a poster at the Academy that a defect in the fibrillin gene was found in patients with isolated subluxated lenses at his institution, suggesting that these patients may have forme fruste Marfan syndrome, and cardiology consultation may be considered. This is very important now, because there is an effective treatment with losartan, an angiotensin-converting enzyme inhibitor, which can prevent or even reverse the aortic dilation that is very common in Marfan syndrome.
These are some of the pediatric ophthalmology highlights from this year's Academy meeting. Thanks for listening. This is Evelyn Paysse for Medscape Ophthalmology Insights.
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Cite this: What's New in Pediatric Ophthalmology - Medscape - Dec 12, 2013.