Infantile Glaucoma and Corneal Opacity

CASE PRESENTATION A 2-day-old Hispanic female was referred to us by her pediatrician for an evaluation of her hazy corneas and to rule out glaucoma. The patient was born by uncomplicated cesarean section at 37 weeks, at which time she weighed 7 pounds, 4 ounces. There were no exposures or complications during pregnancy, and the patient had no family history of childhood ocular problems. Upon initial examination, the patient had mild blepharospasm and winced with both eyes when exposed to a bright light. The patient’s lids and conjunctiva were normal, but her corneas showed diffuse haze (greater centrally than peripherally). Her irides and lenses were difficult to examine secondary to the corneal haze, but the anterior chambers seemed to be formed without any gross iris-to-cornea or lens-to-cornea adhesions. While she slept, we measured her IOP with a Tono-Pen XL (Medtronic Xomed Ophthalmics, Inc., Minneapolis, MN), at 42 mm Hg OD and 40 mm Hg OS. We prescribed acetazolamide 5 mg/kg t.i.d. and Trusopt (Merck & Co., Inc., West Point, PA) t.i.d. OU. The patient was admitted to the OR when she was 5 days old. Her IOP measured 30 mm Hg OD and 34 mm Hg OS. Both corneas had a diameter of 12 mm and exhibited 4+ microcystic edema. Pachymetry measured 974 to 978 μm OU. The anterior chambers were deep, the irides showed mild ectropion uvea, and the pupils were dilated to 8 mm, despite preoperative pilocarpine. Axial length measured 20.5 mm OD and 20.3 mm OS. The B-scan ultrasound showed no retinal detachment or mass. Because we were unable to view the trabecular meshwork with gonioscopy, we performed bilateral temporal trabeculotomies using the metal trabeculotome (Storz Ophthalmics, St. Louis, MO) without complication (Figures 1 through 4). The patient’s corneas began to clear peripherally 2 weeks postoperatively. In the office, her IOP measured 15 mm Hg OU, and there appeared to be central Haab’s striae in both eyes with overlying corneal edema. Timoptic 0.25% (Merck & Co., Inc.) was added to the patient’s drug regimen. At 6 weeks postoperatively, her IOP increased to 38 mm Hg OD and 32 mm Hg OS. Lumigan (Allergan, Inc., Irvine, CA) lowered her IOP to 22 mm Hg OD and 16 mm Hg OS. The crystalline lens became visible in each eye, and there appeared to be mild lamellar opacities bilaterally. The optic nerves were not visible. At 5 months old, the patient’s IOP increased to 40 mm Hg OD and 32 mm Hg OS despite treatment with Cosopt (Merck & Co., Inc.), Lumigan, and Diamox (Wyeth Pharmaceuticals, Philadelphia, PA). We performed nasal trabeculotomies on both eyes. This intervention failed to decrease IOP, so we placed an Ahmed Glaucoma Valve (model S3; New World Medical, Inc., Rancho Cucamonga, CA) in her right eye when she was 6 months old and in her left eye