Hemangioma, a disorder characterized by an abnormal proliferation of capillary endothelium, is the most common tumor of infancy, sometimes with fatal consequences.1 Most hemangiomas are small, harmless lesions that appear soon after birth in various parts of the body, including the eyelid and orbit. However, in rare cases, hemangiomas can be life-threatening in 10% to 20% of cases. Hemangiomas that grow to a large size and proliferate simultaneously can distort adjacent structures, as well as be function- and life-threatening through bleeding, heart failure, acute respiratory distress, and sepsis. Only a third of these life-threatening hemangiomas respond to corticosteroide and the mortality rate is 40% to 60%.2"* In 1992, Alan et al5 reported that recombinant interferon alfa-2-a is the drug of choice in corticosteroid resistant hemangiomas.
We examined a pretenu infant weighing 1800 g who was born with right ear hemangiomas and slight unilateral proptosis of 2.0 mm. Computed axial tomography revealed hypervascular mass of the right orbit, brain, and liver. Clinical diagnosis of diffuse neonatal hemangiomatosis syndrome with visceral involvement was made. In addition, the infant had congestive heart failure, hypertension, thrombocytopenia, and bilateral inguinal hernia. The family history was unremarkable.
Six weeks later, exophthalmometry revealed a severe proptosis of 4.00 mm and nasal displacement of the right eye was noted due to the orbital mass. Neither anterior nor posterior segment abnormalities were detected. The pupil reacted to light both directly and consensually. High intraocular pressure of 30 mm Hg was measured, and treated with antiglaucoma drugs. Cycloplegic retinoscopy revealed astigmatism of 5.00 diopters. The left eye was normal.
At this stage, repeated computed axial tomography of the brain and orbit disclosed a hypervascular mass filling most of the right cavernous sinus, invading the upper orbital fissure, and occupying most of the retrobulbar space (Figure).
The multiple hemangiomas enlarged rapidly and at the age of 7 weeks the child was treated with 5 mg/kg/d of prednisone for 3 months, with no improvement. The proptosis persisted, and secondary exposure keratitis was noted.
The steroids were considered to be ineffective, and daily subcutaneous treatment of 500,000 units interferon alpha2-a was started. At the same time a lateral tarsorrhaphy was performed to reduce secondary corneal damage. Following this systemic treatment, the generalized and orbital hemangiomas decreased in size, and the exposure keratitis and the large degree of astigmatism disappeared.
In the past, treatment of selected periorbital hemangiomas have included intralesional injection of corticosteroide, systemic corticosteroide, cytotoxic drugs, radiation therapy, and surgical excision. Of the few cases which have been described in the literature concerning interferon alfa-2-a5 and 2-b6 therapy for life-threatening hemangiomas of infancy, only some cases involved periorbital hemangioma. To the best of our knowledge, systemic treatment with interferon alfa-2-a for retrobulbar hemangioma in combination with partial tarsorrhaphy for secondary exposure keratitis has not been reported yet in cases where steroid therapy failed to cause regression of proptosis.
FIGURE: Computed tomography shows a hypervascular mass involving the retrobulbar space of the right eye with extension back to the cavernous sinus.
The recommended mode of administration is daily subcutaneous injection of up to 3 million unite per square meter of body surface area. The duration of treatment ranges from 9 to 13 months. Earlier withdrawal is associated with regrowth of the hemangioma. Apart from mild fever, no toxic effects were reported.1
We recommend that in cases where life or vision are threatened and the hemangiomas are resistant to corticosteroids, interferon alfa-2-a is a viable treatment alternative.
1. Folkman J. Toward a new understanding of vascular ptoliferative disease in children. Pediatrics. 1984; 74:850-856.
2. Enjolras O, Rich MC, Merland JJ, Escarde JP. Management of alarming hemangiomas in infancy. A review of 25 cases. Pediatrics. 1990;85:491-498.
3. Herman B, Lim HW-P. Concurrent cutaneous and hepatic hemangiomata in infancy. Report of a case and a review of the literature. Dermatol Surg Oncol. 19 78 i4:869-873.
4. El-Dessouky M, Azmy AF, Raine PAM, Young DG. Kasabach-Merritt syndrome. J Pediatr Surg. 1988;23 : 109-111.
5. Alan R, Ezekowitz B, MuUiken JB, Folkman J. Interferon alfa-2-a therapy for life-threatening hemangiomas of infancy. N Engl J Med, 1992;326:1456-1463.
6. Loughnan MS, Elder J, Kemp A. Treatment of massive orbitalcapillary hemangioma with Interferon alfa-2-b: short-term results. Arch Ophthalmol. 1992;110: 1366-1367.