Surgical Management Of Congenital Anterior Segment Dysgenesis

Congenital anterior segment dysgenesis, such as Peters anomaly, is frequently associated with refractory pediatric glaucoma and poor visual prognosis. Surgical management is complex due to corneal opacity, abnormal anatomy, and high failure rates of conventional glaucoma procedures. This case report aims to describe the anatomical and functional outcomes of a combined surgical approach using penetrating keratoplasty and implantation of a Paul glaucoma drainage device in the vitreous cavity in a pediatric patient with congenital anterior segment dysgenesis and uncontrolled glaucoma.

Department of Cornea and Ocular Surface, Barraquer Eye Center, Barcelona, Spain

A 7-year-old girl with bilateral Peters anomaly and congenital glaucoma presented with diffuse corneal opacity in both eyes. Previous interventions included bilateral trabeculectomy and transscleral cyclophotocoagulation. At presentation, intraocular pressure was 30 mmHg bilaterally, with best corrected visual acuity of 0.0063 in the right eye and 0.01 in the left eye. Electrophysiological testing showed better function in the right eye, which underwent combined penetrating keratoplasty, cataract extraction without intraocular lens implantation, pars plana vitrectomy, and implantation of a Paul glaucoma drainage device in the vitreous cavity.

Surgery was completed without intraoperative complications. One month postoperatively, the corneal graft was clear and intraocular pressure was 4 mmHg under topical hypotensive therapy. At three months of follow-up, best corrected visual acuity in the right eye improved from 0.0063 preoperatively to 0.03. In addition, the parents reported a noticeable improvement in the patient’s ability to perform daily activities at home, along with improved object fixation. No postoperative complications were observed.

In pediatric patients with congenital anterior segment dysgenesis, achieving and maintaining corneal transparency is essential for visual rehabilitation. Combined penetrating keratoplasty with appropriate intraocular pressure control using a glaucoma drainage device, rather than repeat filtering surgery, may contribute to improved graft survival and stable visual outcomes. Careful selection and positioning of drainage devices can help preserve endothelial integrity and support long-term corneal clarity in complex congenital cases.