Successful treatment of juvenile xanthogranuloma using bevacizumab Noy Ashkenazy, MS, a Christopher R. Henry, MD, a Ashkan M. Abbey, MD, b Craig A. McKeown, MD, a Audina M. Berrocal, MD, a and Timothy G. Murray, MD, MBA c Juvenile xanthogranuloma (JXG) is an uncommon non-Langerhans cell histiocytic disorder that occurs predominantly in infants. Traditional treatment of ocular JXG involves the administration of topical or local corticosteroids. We treated 2 children with JXG refractory to local corticosteroid therapy with off-label intraocular bevacizumab. To our knowledge, this is the first report of successful use of bevacizumab for ocular JXG. Case 1 A 4-year-old boy presented with complaints of pain and decreased vision in his right eye. On examina- tion, visual acuity was 20/30 in the right eye and 20/20 in the left eye. Pupils were equal, round, and reactive to light. Extraocular motility was full; there was no evidence of strabismus or nystagmus. Intraocular pressure (IOP) was 17 mm Hg in the right eye and 14 mm Hg in the left eye. Anterior segment examination revealed a 1 mm boat-shaped hyphema in the right eye. The patient was initially managed with topical atropine 1% eyedrops twice daily in the right eye, and the hyphema improved. One week later, the patient returned with visual acuity decreased to 20/200 and pain in his right eye. Microcystic corneal edema and 3 1 cells were noted in the anterior cham- ber. An irregular elevation of the iris was identified supero- temporally, and gonioscopy revealed the presence of a yellow-white mass on the surface of the iris (Figure 1A). Anterior segment optical coherence tomography revealed a hyperreflective, heterogenous mass on the surface of the pe- ripheral iris and abutting the trabecular meshwork (Figure 1B). Topical prednisolone acetate 1% eyedrops 4 times daily was added. During serial examinations under anesthesia, he was noted to have resolution of anterior cham- ber cellular reaction and stabilization of the iris lesion; visual acuity improved to 20/20 in the right eye. A taper of prednis- olone acetate and atropine eyedrops was attempted. One month after discontinuation of topical steroids, the patient again presented with decreased visual acuity (20/ 80) in the right eye. A recurrent, spontaneous hyphema was noted. Growth of the hypopigmented iris mass with intrinsic vascularity and overlying focal hemorrhage was noted (Figure 1C). Repeat examination under anesthesia was performed, and a fine-needle aspiration biopsy of the iris lesion was obtained using a 27-gauge needle on a syringe. Intravitreal bevacizumab (1.25 mg/0.05 mL) was in- jected via the pars plana using a 30-gauge, half-inch needle. Prednisolone acetate eyedrops were resumed. Fine-needle aspiration specimens revealed granulomatous inflammation comprising a mixture of histiocytes and lymphocytes consis- tent with a diagnosis of JXG. Over the ensuing months, prednisolone acetate eyedrops were tapered. Visual acuity at 18 months’ follow-up was 20/20 in the right eye and IOP was 15 mm Hg; there was complete involution of the lesion, without recurrence of hyphema or iritis (Figure 1D). Case 2 A 6-month-old girl with a history of multifocal cutaneous lesions on her forehead and scalp was referred to our insti- tute with a history of recurrent subconjunctival hemorrhages in the right eye. Visual acuity was 9.8 cycles/cm at 55 cm in each eye. Cycloplegic refraction was 10.75 1 1.00 Â 080 in the right eye and 11.50 1 0.50 Â 115 in the left eye. Pupils were equal, round, and reactive to light, extraocular motility was full, and there was no evidence of strabismus or nystagmus. The patient had a 4 mm yellow-orange, elevated, inferotemporal episcleral lesion that was concerning for JXG (Figure 2A). On gonioscopy, during examination under anesthesia, the lesion was noted to involve the posterior peripheral aspect of the cornea, angle, and iris (Figure 2B). B-scan ultrasonography revealed abnormal thickening of the iris and ciliary body complex. The patient underwent initial treatment with a sub-Tenon’s injection of triamcino- lone acetonide (40 mg/1 ml) and was started on topical prednisolone acetate 1% eyedrops 4 times daily in the right eye. Examination under anesthesia was repeated 3 weeks later and revealed enlargement of the lesion. A sub-Tenon’s injection of triamcinolone acetonide was repeated. She was observed for an additional month, with no noted improvement of the lesion. Treatment with intracameral bev- acizumab (1.25 mg/0.05 mL), using a 30-gauge needle, was instituted. The patient was started on topical dorzolomide 2% with timolol 0.5% eyedrops twice daily in the right eye due to an elevated IOP of 22 mm Hg. Topical steroid eyedrops were tapered. Three months following bevacizumab treatment, flattening and reduced vascularity of the epibulbar component of the JXG lesion was observed (Figure 2C). Go- nioscopy revealed significant involution and reduced Author affiliations: a Bascom Palmer Eye Institute, Department of Ophthalmology, University of Miami Miller School of Medicine, Miami, Florida; b Associated Retinal Consultants, William Beaumont Hospital, Royal Oak, Michigan; c Murray Ocular Oncology and Retina, Miami Submitted November 1, 2013. Revision accepted January 12, 2014. Published online April 24, 2014. Correspondence: Christopher R. Henry, MD, Bascom Palmer Eye Institute, 900 N.W. 17th Street, Miami, Florida 33136 (email: chenry2@med.miami.edu). J AAPOS 2014;18:295-297. Copyright Ó 2014 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2014.01.007 Journal of AAPOS Volume 18 Number 3 / June 2014 Ashkenazy et al 295