Peters anomaly is a rare developmental condition presenting with an annular corneal opacity (leukoma) in the central visual axis (Fig 4-11), often accompanied by iris strands that originate at the iris collarette and adhere to the corneal opacity. The leukoma corresponds to a defect in the corneal endothelium and underlying Descemet membrane and posterior stroma. The lens may be in its normal position, with or without a cataract, or the lens may be adherent to the posterior layers of the cornea. Patients with corneolenticular adhesions have a higher likelihood of other ocular abnormalities, such as microcornea and angle anomalies, and of systemic abnormalities, including those of the heart, genitourinary tract, musculoskeletal system, ear, palate, and spine.
Table 11-9 Differential Diagnosis for Axenfeld- Rieger Syndrome
Figure 11-4 Photograph of an eye with Peters anomaly exhibiting central leukoma, which can be confused for corneal edema.
(Courtesy of JoAnn A. Giaconi, MD.)
Peters anomaly is usually sporadic, although autosomal dominant and autosomal recessive forms have been reported. Most cases are bilateral, and angle abnormalities leading to glaucoma occur in approximately 50% of affected patients. As previously mentioned, glaucoma is thought to result from a malformed trabecular meshwork or Schlemm canal. Development of glaucoma is more common in patients with cataracts or corneolenticular adhesions. Elevated IOP typically presents in infancy but can also arise later in life.
Treatment of glaucoma associated with Peters anomaly can be difficult because of the iridocorneal dysgenesis. If possible, angle surgery is performed; alternative treatments include medications, trabeculectomy, tube shunt surgery, and cyclodestructive procedures.
Excerpted from BCSC 2020-2021 series: Section 10 - Glaucoma. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.