Capillary hemangiomas are the most common periocular and orbital tumors of childhood that typically arise in infancy. In the vast majority of cases these tumors undergo spontaneous involution without sequelae. Capillary hemangiomas can present as small isolated lesions, or large masses that can cause visual impairment, systemic effects, or exist as part of a syndrome.
Capillary hemangioma is a form the capillary unit structure of endothelial cells surrounded by pericytes develops at early childhood with no clear etiology
Historically, females are three times as likely as males to be affected. Capillary hemangiomas are more frequent in premature or low-birth-weight infant. There is a large increase, approximately 10-fold, in infants born to women who underwent chorionic-villus sampling during pregnancy. There is no definitive association between capillary hemangiomas and race, ethnicity, or geographic location. There is no clear hereditary pattern or increased incidence among siblings.
Histopathology of capillary hemangionas depends on the stage of evolution of the tumor. Early stage may be very cellular with nets of solid plump endothelial cell and little vascular lumen. Established lesion show a well formed, flattened, endothelial lined capillary lumen arranged in a lobular fashion. Involuting lesions show lumen occlusion with fibrosis and hyalinization of capillary walls.
Capillary hemangioma is considered as a congenital vascular tumor. There is no established preventive measure for this disease entity.
- Kasabach-Merrit syndrome characterized by thrombocytopenia, hemolytic anemia and coagulopathy and is associated with large visceral hemangioma. The thrombocytopenia occurs because if platelet sequestration withen the tumor's vascular channels.
- Microangiopathic hemolytic anemia (MAHA) was also described with periocular, gastrointestinal and liver hemangiomas. In MAHA erythrocytes are destroyed from coagulation, or are sheared or fragmented by high pressure forcing them through the abnormally small vessels of the hemangioma.
- PHACES syndrome is characterized by posterior fossa malformations, hemangiomas, arterial anomalies, cardiac defects, eye abnormalities, sternal cleft and supraumbilical raphe syndrome and is associated with an increased risk of amblyopia and more likely to require treatment.
- Periocular capillary hemangiomas may also occur simultaneously with oral, nasal, subglottic, and paratracheal hemangiomas.
Typically capillary hemangiomas are not present at birth, but appear in the first few months of life, continue to enlarge over the first 6-12 months after the first year, with 75% resolution during the first 4-5 years of life. The lesion may start as a small macular red spot known as herald patch. Hemangiomas may be cutaneous with bright red in color (strawberry nevus) or subcutaneous with bluish color visible through overlying unaffected skin. Hemangiomas located deeper within the orbit may present as progressive proptosis with no skin involvement or discoloration
Capillary hemangioma is nearly always diagnosed clinically. Radiological testing may be used to help distinguish capillary hemangioma from other vascular tumors, to know the extent of the lesion and to monitor the tumor growth.
- Plain radiography is not particularly helpful in the diagnosis of capillary hemangiomas.
- Ultrasonography scan may show an irregular mass with variable reflectivity.
- Computed tomography (CT) is another modality for monitoring tumor growth. Capillary hemangiomas often show as a homogenous soft tissue mass without adjacent bone destruction. With the use of contrast the lesion will enhance helping to identify the border and relation of the tumor to optic nerve, extraocular muscles and orbital structure.
- On Magnetic Resonance Imaging (MRI) studies, capillary hemangiomas may appear as well-circumscribed densely lobulated tumors. On T1-weighted MRI, the capillary hemangioma is of intermediate signal intensity, but it is moderately hyperintense on T2-weighted imaging due to the slow blood flow through the vascular channels. The tumor mass may enhance following gadolinium contrast injection, so images should have fat suppression on T1 weighted images to better visualize the tumor.
- Angiography is rarely used as a diagnostic tool for capillary hemangiomas.
The differential diagnosis for capillary hemangioma includes rhabdomyosarcoma, lymphangioma, chloroma, neuroblastoma, orbital cysts, and cellulitis
Treatment of capillary hemangioma is indicated in patients with visual complications which include occlusion of the visual axis, amblyogenic anisometropia, optic nerve compression, or significant proptosis causing exposure keratopathy.
If treatment is indicated, medical therapy consists of steroids orally with adobe of 1-2 mg/ kg/ day of pridnisolone, regression most frequently was noted in the first two weeks of treatment. However, rebound growth was seen in some cases, this together with the side effected of systemic steroids including growth delay, adrenal suppression, cushingoid features, hyperglycemia, delayed wound healing, arterial hypertension, hypertrophic cardiomyopathy, and susceptibility to infection decrease the popularity of this method of treatment. Direct administration of steroids into the lesion has been used more frequent and used to be the mainstay of treatment but the side effects which include Atrophy of the skin, necrosis, risk of hemorrhage, growth retardation and occlusion of the central retinal artery, with resultant blindness made it suboptimal.
Recombinant interferon alfa, and angiogenesis inhibiting agent, has been used successfully as a secondary agent for life-threatening hemangiomas that have failed corticosteroid therapy. Both interferon alfa-2a and 2b have been used as subcutaneous injections. Common side effects include irritability, neutropenia, and abnormalities of liver enzymes.
Propranolol, a non-selective beta blocker, can be used both topically and systemically with high efficacy. There is a low incidence of side effects and decreased risk of recurrence after therapy which seems to be inversely related to the age. The suggested dosage of oral propranolol is 2 to 3 mg/kg/day, divided twice daily, and adjusted for weight changes during growth of the infant. Beta blockers side effects include exacerbating reactive airway disease (hence oral propranolol therapy is contraindicated in asthma patients), bradycardia, systemic hypotension, and masking of symptoms of hypoglycemia in the infant population. It is suggested that the patient undergo cardiac evaluation prior to treatment and monitoring of the blood pressure, heart rate and blood sugar during the first 24 hours of treatment. Some patients may be good candidates for outpatient propranolol treatment which is often slowly increase in dosage in order to avoid systemic complications. Topical timolol gel has now been utilized with good success for smaller eyelid hemangiomas with negligible complications. Topical therapy does not typically treat deeper orbital lesions.
Surgical removal is usually reserved for cases where conservative therapy has failed, and tumor removal is necessary. The highly vascular tumor may create a significant higher risk of intraoperative bleeding. Surgery can reduce tumor size by compromising the tumor feeder vessels, constriction and sclerosing of the small vascular channels, or primary excision of the entire mass can be performed.
Laser therapy can also be applied to capillary hemangioma. Carbon dioxide, argon, neodymium-YAG, and flash-lamp pumped-dye laser have all been utilized in the treatment of capillary hemangiomas. Laser treatments have been found to be more effective for early and superficial lesions and less effective in deeper orbital lesions. Radiation therapy can be effective in the management of certain capillary hemangiomas. Microembolisms can be created in the tumors by low-level radiation, which can speed the regression of the mass.
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