Peters anomaly

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Peters Anomaly

  • Affects 1 person in a million.
  • There are 2 types of Peters' anomaly, depending on severity.
  • Causes more than 40% of congenital corneal opacities.

What is Peters Anomaly?

Peters anomaly is a congenital condition that occurs between 10 and 16 weeks of gestation, affecting the cornea, the clear window at the front of the eye. This anomaly results in central corneal opacity, where the central part of the cornea becomes opaque (white), leading to vision loss.

It can affect one or both eyes and is typically not detected through prenatal screening. The condition can be genetic and may appear as a one-off abnormality in a family, though it can sometimes affect more than one family member.

Peters-plus syndrome occurs when this condition is linked with other systemic issues such as heart disease, deafness, learning difficulties, cleft lips, and cleft palates due to genetic mutations affecting more than just the eyes.

Causes and Symptoms

  • The central cornea becomes opaque, leading to visual impairment.
  • Severity of the opacity impacts vision, with a greater degree of opacity leading to more severe loss of vision.
  • If both eyes are affected, the child may not be able to recognize objects but might still perceive shapes and recognize colors.
  • The condition is associated with other abnormalities in the front part of the eye, such as:
    • Glaucoma (increased ocular pressure)
    • Nystagmus (involuntary eye movements)
    • Cataracts (lens opacity)
    • Retinal detachment
  • In the most severe cases, the eye may be smaller than usual (microphthalmia) and underdeveloped.

How is Peters Anomaly Diagnosed?

  • Peters anomaly is often detected by a paediatrician shortly after birth.
  • Diagnosis is confirmed by an ophthalmologist, who may need to conduct a thorough eye examination under general anesthesia to assess the extent of the anomaly.
  • In some cases, central corneal opacity improves spontaneously in the first months of life, but the extent of vision recovery can vary.

Treatments

  • Corneal transplantation is the primary treatment when both eyes are affected to improve visual acuity.
    • Surgery is performed on an outpatient basis under general anesthesia.
  • If glaucoma is present, it must be treated to prevent damage to the optic nerve. Treatment may involve eye drops or, in some cases, surgery.
  • For acute photophobia (light sensitivity), wearing sunglasses may help alleviate discomfort.

Associated Treatments for Peters' Anomaly:

  • Corneal transplant to improve vision.
  • Glaucoma management with eye drops or surgery.
  • Sunglasses for light sensitivity (photophobia).