Recurrent Hyphema in an Aphakic Child

In 1973, Swan1 described 3 patients who developed hyphema months to years after uncomplicated cataract surgery. He noted focal vascularization from an ingrowth of episcleral vessels at the cataract wound site, resulting in recurrent intraocular bleeding. Swan syndrome has been reported following intracapsular cataract extraction, extracapsular cataract extraction (including clear corneal incisions), iridocyclectomy, and glaucoma filtering procedures.2-4 Patients typically present with sudden painless blurred vision, often upon awakening, which may or may not be preceded by physical strain or trauma. Other patients are asymptomatic and diagnosed with hyphema or anterior chamber red blood cells on routine examination.5 The hyphema often resolve spontaneously, making later diagnosis difficult. Gonioscopic visualization of the abnormal wound vessels is necessary for diagnosis. Without active bleeding, however, the fibrovascular tuft may be easily overlooked. We report a case of Swan syndrome in a 16-monthold boy after cataract extraction was performed. To our knowledge, Swan syndrome has not been reported in the pediatric population. Children represent a significant proportion of patients undergoing anterior segment surgery and Swan syndrome should be considered in the differential diagnosis of hyphema in this population. CASE REPORT A 13-month-old Hispanic boy with a history of infantile cataracts underwent bilateral extracapsular cataract extractions, anterior vitrectomy, and peripheral iridectomies performed 2 weeks apart at another institution. The child presented to our facility 3 months later with a painful red left eye and irritability. Details of the surgery were unavailable. Examination revealed a moderately infected left eye with a 50% layered hyphema and an eccentric pupil. Fixation and following movements were poor in the left eye. Intraocular pressure (IOP) was 40 mm Hg, measured with a Tono-Pen on an awake child, and the patient was prescribed Cosopt (Merck & Co, West Point, Pa), Xalatan (Pharmacia and Upjohn, Kalamazoo, Mich), and Pred Forte (Allergan, Inc, Irvine, Calif). Serial examinations showed a persistent hyphema with elevated intraocular pressures despite maximal medical therapy. One week later, an evaluation after induction of anesthesia revealed an IOP of 12 mm Hg in the left eye. The cornea was hazy and the anterior chamber was deep and without hyphema. Findings from funduscopic examination were unremarkable except for optic nerve asymmetry. The cup-to-disc ratios were noted to be 0.20 and 0.35 in the right and left eye, respectively. Gonioscopy was performed, which revealed a visible trabecular meshwork in the left eye with some peripheral anterior synechiae superotemporally. One week later, the hyphema had resolved and the patient was able to fix and follow with his left eye. Ten days later the patient returned with a recurrent hyphema. An evaluation after induction of anesthesia 2 days later revealed an IOP of 28 mm Hg in the left eye. The left cornea was cloudy and a 30% hyphema was present. Corneal diameters were measured to be 12 mm × 11 mm in the right eye and 13 mm × 12 mm in the left eye. Under gonioscopic visualization, fine neovascular vessels were noted at the wound site. When the hyphema did not clear after 10 days, an Iris contact diode laser (Iris Medical Instruments, Mountain View, Calif) was used to ablate the anterior chamber neovascularization with translimbal approach. The superotemporal limbal region was treated with 17 spots of 1250 mW for 3000 ms. After laser treatment, the hyphema did not recur. The glaucoma became refractory to medical treatment several months later and the patient underwent a trabeculectomy with mitomycin. DISCUSSION