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What is Peters anomaly?

What is Peters anomaly?

20 June 2024

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Peters anomaly is a rare congenital eye disease characterized by abnormal development of the cornea, lens, and, in some cases, the iris. This condition can vary in severity and affect one or both eyes. It is named after German ophthalmologist Alfred Peters, who first described the condition in 1906.

Characteristics:

People with Peters anomaly have a number of distinctive characteristics, which may include:

1. Corneal opacity: one of the most common manifestations is the presence of a central opacity in the cornea, which can significantly affect vision. This opacity is caused by a failure in adequate separation between the corneal endothelium and the lens during fetal development.

2. Iridocorneal adhesions: In many cases, the iris may be adhered to the cornea in the area of ​​the opacity, a condition known as synechiae.

3. Lens anomalies: there may be dislocation or malformation of the lens. In some cases, the lens may be absent or present with congenital cataracts.

4. Congenital glaucoma: A significant percentage of patients with Peters anomaly also have congenital glaucoma, a condition that can lead to increased intraocular pressure and damage to the optic nerve.

Causes and genetics

Peters anomaly is a genetically heterogeneous disorder. It has been associated with mutations in several genes that are crucial for eye development, however, in many cases a specific genetic cause cannot be identified. The condition can occur in isolation or as part of more complex syndromes, such as Peters plus syndrome, which includes other congenital anomalies.

Diagnosis

Diagnosis of Peters anomaly is primarily made through a complete eye examination. This may include:

  • Biomicroscopy: allows us to observe the structure and clarity of the cornea, as well as the presence of synechiae.
  • Optical coherence tomography (OCT): helps obtain detailed images of the different layers of the cornea and the iridocorneal angle.
  • Ocular ultrasound: useful to evaluate the presence and position of the lens and the anatomy of the eyeball in general.

Treatment

Treatment of Peters anomaly depends on the severity of the manifestations and the presence of additional complications such as glaucoma.

The prognosis for patients with Peters anomaly varies widely. In some cases, vision can be significantly improved with early intervention and proper management. However, in severe cases, vision may be severely compromised despite interventions. Long-term follow-up with a specialized ophthalmologist is crucial for the management of this condition.

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