Faculty Bibliography

'Vascular anomalies' represents a spectrum of vascular lesions, of unclear etiology and often with unpredictable behavior. Patients with vascular anomalies represent a unique population, in that they have focal aberrations of vascular development (in vascular malformations) or vascular proliferation (in hemangiomas). The etiology of these disorders is unclear, and likely represents a multifactorial process. Vascular anomalies are an attractive model for the study of human disorders of vasculogenesis (development of the vasculature) and angiogenesis (new vessel growth from existing vessels)

BACKGROUND:: Large hemangiomas of the scalp, though uncommon, present unique challenges to the reconstructive surgeon. If not treated early, these lesions can result in large areas of alopecia, distortion of the hairline, or deformation of the ear. Given these potential complications and the relative pliability and redundancy of the infant scalp before 4 months of age, the authors propose early surgical excision. METHODS:: A retrospective review of the senior author's (B.M.Z.) patient records was performed; over a period of 4 years, six infants were identified who underwent resection of a large scalp hemangioma. The surgical planning and execution of each case and follow-up are detailed. RESULTS:: All six hemangiomas were excised completely. In five cases, the excisions were performed in one stage at or before 4 months of age. In a sixth case, a tissue expander was placed before excision and closure in an 18-month-old infant. In three cases, significant ear malposition was corrected by removal of the deforming mass. There were no complications. CONCLUSIONS:: The authors have demonstrated that by taking advantage of the greater elasticity of the infant scalp, large hemangiomas of the scalp can be aggressively and successfully treated with surgical intervention, often in one operation. Beyond the usual indications, early surgical excision of scalp hemangiomas may be advantageous and warranted to prevent the development of large alopecic areas or the permanent distortion of the hairline and aural anatomy

BACKGROUND: Capillary hemangiomas of the eyelids and orbit can cause refractive and occlusive amblyopia. Although oral and intralesional steroid injections are the most common treatment modalities, sometimes they are not successful. There is a paucity of information in the literature on the success of eliminating amblyogenic factors by treating these lesions with surgical resection. METHODS: Retrospective chart review of 10 patients in two pediatric ophthalmology practices who underwent surgical excision of a capillary hemangioma that was causing amblyopia and that had failed to regress with other treatment. RESULTS: Two patients had surgery secondary to pupillary occlusion, which was successful in relieving occlusion. Eight patients had surgery secondary to significant astigmatism. The average preoperative astigmatic difference between the affected and unaffected eye in five of these patients undergoing surgery before the age of 21 months was 2.15 D. The average postoperative astigmatic difference was 0.1 D. The average preoperative astigmatic difference between the affected and unaffected eye in three patients undergoing surgery after 21 months of age was 1.6 D. Surgery completely failed to reduce the astigmatism in two of these patients. The third patient had a decrease of 0.75 D of cylinder but still had a difference of 1.75 D between the two eyes postoperatively. Postoperative complications in this study included wound infection in one patient. CONCLUSIONS: Surgical excision of capillary hemangiomas that were resistant to other modes of treatment was useful in relieving pupillary occlusion and in decreasing the amount of astigmatism if performed before the age of 21 months in our series of patients. Our cases as well as the literature suggest that surgery should be performed at 13 months or earlier to reduce the amount of astigmatism

PURPOSE: Capillary hemangiomas of the eyelid and orbit are treated when amblyopia secondary to anisometropic astigmatism or pupillary occlusion is present or when rapid growth of the hemangioma threatens to occlude the pupil. The goal of this study was to determine whether treatment of hemangiomas resolves or prevents occlusion or results in decrease in astigmatism. METHODS: The records of 54 patients who underwent treatment for reduction in the size of a capillary hemangioma causing amblyopia or threatened amblyopia in two pediatric ophthalmology practices were reviewed. RESULTS: Twenty-eight patients were treated for amblyopia due to anisometropic astigmatism. The average amount of pretreatment astigmatism was 2.71 D, while the average amount of post-treatment astigmatism was 0.46 D. Fifteen of these patients could be tested for optotype visual acuity and all had vision acuity of 20/40 or better. Only 1 of the 15 patients treated for threatened occlusion of the pupil developed occlusion. Six of these patients cooperated with optotype visual acuity and all had vision acuity of 20/30 or better. Eleven patients were treated for pupillary occlusion. Occlusion resolved in all cases. Of the five patients treated for occlusion who cooperated with optotype visual acuity, two had a vision acuity of 20/100 or worse. CONCLUSIONS: Treatment to reduce the size of capillary hemangiomas results in resolution of occlusion, reduction in astigmatism, and prevention of pupillary occlusion. Those with occlusion are at higher risk for severe residual amblyopia and require prompt and definitive treatment

Concomitant hereditary spherocytosis and sickle cell trait, although extremely rare, could potentially lead to splenic sequestration or infarction. We report here the first case of splenic infarction in a child with hereditary spherocytosis and sickle cell trait while flying on a commercial aircraft. The presence of hypoxia, hemoconcentrated erythrocytes, and sickle hemoglobin created the perfect environment for clinical sequelae