Peter Anomoly Diagnosis

Peters anomaly: Anterior Chamber Cleavage syndrome

Peters anomaly was first described in 1906 by a German Ophthalmologist, Dr Alfred Peters. The anomaly affects the eyes of people of both genders and from all ethnic groups. Peters anomaly is a developmental error of early pregnancy (10-16 weeks). Normally, the cornea, which is the transparent 'window' of the eye, focuses light through the lens onto the retina (a light sensitive film at the back of the eye). Signals are then sent by the optic nerve to the brain for interpretation. The cornea, lens, retina and optic nerve need to work perfectly in harmony for clear vision. In Peters anomaly the central part of the cornea is hazy and white. This may affect one or both eyes. The corneal opacity is the obvious feature that Dr Peters described but this is now known to be part of a spectrum of abnormal development of the front of the eye. The eye may be abnormal in other ways including the drainage angle of the eye which may be underdeveloped so there is a risk of glaucoma and the lens of the eye may be cloudy. The fellow eye may have a milder developmental anomaly or be more severely affected where only a rudimentary small eye has developed.

* What are the symptoms?

A number of features will lead to the actual way the child is affected: If the centre area of the cornea is white or cloudy, the cornea will not allow the eye to obtain a clear picture of the world. Light enters the eye but, if both eyes are affected, the child will not be able to clearly see what an object is. They will just be aware that something is there and aware of colours. Peters anomaly can be associated with other eye problems that contribute further to reduced vision including glaucoma, nystagmus, microphthalmia, cataracts and retinal detachment. As the cloudy area usually affects the centre of the cornea, then even if the cornea is later grafted with a clear donor cornea the eye will be amblyopic (lazy eye). The area of the developing infant brain that responds to signals from the eyes needs to be given information of good quality about the world very early in life for normal eyesight to develop. In one study, sixty per cent individuals with Peters anomaly of the eye had abnormalities of other organs, in particular the heart or central nervous system. Twenty per cent of cases had developmental delay. Some of the anomalies associated with Peters anomaly occur in a particular pattern and form a recognisable syndrome such as Peters Plus syndrome.

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How is it diagnosed?

A diagnosis of Peters anomaly will be made by examination by an ophthalmologist. Usually, the eye abnormality is detected soon after birth but there may be a delay before the correct diagnosis is made. The clouding of the front of the eye may spontaneously improve over the first few months but it is most important that the child is examined as soon as possible by an ophthalmologist. It is very rare for an ophthalmologist to require a baby to have a general anaesthetic to make a diagnosis but occasionally this may be necessary to make a thorough examination. It is difficult for both parents and specialists to predict how well the child will see. This will become more apparent as the child develops and interacts with their environment.