Please log in.

Lost your password?

Create an account

Diagnosis and Treatment

How is Peters Anomaly diagnosed?

Doctors examine the eyes of newborns to see if they are normal, and an eye doctor is called to perform further tests if any problems are noticed. However, it is difficult to examine a newborn’s eyes closely, and the problem may be overlooked. Parents may notice a problem with their baby’s eyes by their appearance or the baby’s actions. They can discuss the problem with clinicians to arrange an assessment [3]. Peters anomaly is clinically diagnosed with a physical examination.

Follow-up Testing

It has been suggested that all patients diagnosed with Peters anomaly after birth should undergo chromosomal analysis and molecular genetic testing [1]. If chromosomal translocations or deletions exist, or other patterns of Peters anomaly are found, parents should see a genetic counselor to determine the risk in future pregnancies.

Patients with Peters anomaly are likely to have systemic malformations, especially if they have the bilateral condition, so careful evaluation should be done to ensure appropriate intervention [1].

Commonly Associated Conditions

Glaucoma occurs in up to 90% of cases of Peters anomaly. Microcornea, cornea plana, sclerocornea aniridia, colobomas of the iris and the choroid, and PHPV may also be associated with Peters anomaly. Optic nerve hypoplasia or atrophy can also occur. Systemic abnormalities, Krause-Kivllin, and Peters plus syndrome are observed with Peters anomaly [2].

Other conditions may be present, especially if both eyes are affected [1]:
Problems of the heart
Problems of parts of the brain
Abnormal appearance of the face
Short stature
Learning difficulties

Surgical Treatment

Penetrating keratoplasty

In penetrating keratoplasty, the thick cornea is removed and replaced by transparent corneal tissue. The goal of this surgical intervention is to maximize vision and minimize amblyopia [4]. Eyes send signal to parts of the brain responsible for vision, which become ‘lazy’ in Peters anomaly because of the reduced quality of signals. The brain is only able to ‘see’ as clear an image as given to it by the eyes. The hazy cornea prevents the formation of sharp, clear images, so these parts of the brain do not develop properly, leading to progressive visual degradation [1].

The success rate for penetrating keratoplasty lies between 22% and 83%. Such a large range is likely due to the broadness of the spectrum of disease labeled as Peters anomaly. In one study, the success rate in patients with Type I Peters anomaly was 83%. It is suggested that children with Type I Peters anomaly be considered for penetrating keratoplasty within the first year of life to prevent amblyopia [3].

Because there is an increased incidence of glaucoma in these patients, a glaucoma specialist, a pediatric ophthalmologist, and a corneal specialist should be included in the care group for all cases [3].

 

References:
1. Andrew Blaikie. Medical Information on Peter’s Anomaly. Retrieved from http://www.ssc.education.ed.ac.uk/resources/vi&multi/eyeconds/PetAno.html
2. Guruswami Arunagiri. (2009). Peters Anomaly. Retrieved from http://emedicine.medscape.com/article/1200372-overview
3. Bhandari et al. (2011). Peters Anomaly: Review of the Literature. Cornea. 2011 Apr 20.
4. Yang L.L.H. and Lambert S.R. (2011). Peters’ Anomaly A Synopsis of Surgical Management and Visual Outcome. Ophthalmol Clin North Am. 2001 Sep;14(3):467-77.
5. To read more about Peters-plus syndrome: http://omim.org/entry/261540