University of Edinburgh
 

Medical Information on Persistent Hyperplastic Primary Vitreous (PHPV)

by Dr Andrew Blaikie for VI Scotland

This document is written with the minimum use of medical terms and jargon. It is impossible to avoid all medical terms but where we have used them we have attempted to explain them as clearly as we can. Although the information is intended to describe most aspects of the condition each child is different and there will always be exceptions to the rule. As far as we can determine these pages are true and accurate and have been written in good faith.

What this information is not for?

This document is not a substitute for a consultation with a Health Professional and should not be used as a means of diagnosing a condition.

We hope the information will help you:

  • Have a better understanding of the condition
  • Know what tests and treatments are normally available
  • Know when to seek professional advice
  • Be able to discuss the condition in a more informed way
  • Make the most of consultations with carers, teachers and health professionals
  • Be reassured and more able to cope

Due to staffing limitations we are not able to offer telephone or email advice to parents of children.

Medical Information on Persistent Hyperplastic Primary Vitreous (PHPV)

What we see is made in the brain from signals given to it by the eyes. What we see is in fact made in the brain. The brain makes sight from signals given to it by the eyes.

What is the normal structure of the eye?

The eye is made of three parts.

  • A light focussing bit at the front (cornea and lens).
  • A light sensitive film at the back of the eye (retina).
  • A large collection of communication wires to the brain (optic nerve).

A curved window called the cornea first focuses the light. The light then passes through a hole called the pupil. A circle of muscle called the iris surrounds the pupil. The iris is the coloured part of the eye. The light is then focused by a lens through a space filled with clear jelly to the back of the eye. The jelly is called the vitreous. The back of the eye is covered with tiny light sensitive patches (photoreceptors). These photoreceptors collect information about the visual world. The covering of photoreceptors at the back of the eye forms a thin film known as the retina. Each photoreceptor sends its signals down very fine wires to the brain. The wires joining each eye to the brain are called the optic nerves. The information then travels to many different special 'vision' parts of the brain. All parts of the brain and eye need to be present and working for us to see normally.

What is Persistent Hyperplastic Primary Vitreous (PHPV)?

The space at the back of the eye, behind the lens, is normally filled with a clear jelly. This jelly is called the vitreous. Children with PHPV are born with a hazy, scarred vitreous. The hazy vitreous blocks light passing to the back of the eye. This leads to blurred vision.

PHPV can lead to other eye problems developing

The scarred vitreous is often stuck to the back of the lens and areas of the retina. This can damage these parts of the eye as well and lead to other eye conditions:

  • The lens may become hazy (cataract)
  • The retina may peel off the back of the eye (retinal detachment)
  • The pressure in the eye can rise (glaucoma)
  • The eye can be smaller than usual (microphthalmia)

If you would like a VI Scotland information document on any of these conditions please ask us.

Why are some children born with PHPV?

Sometimes when an unborn child is growing in the womb not all parts of the eye develop normally. In children with PHPV the first (primary) vitreous that grows in the eye fails to become clear. It grows too much (hyperplastic) and becomes hazy and scarred. Usually it would disappear and become clear but instead it stays (persists). This is why the condition is called Persistent Hyperplastic Primary Vitreous. This leads to blurred vision and other eye conditions sometimes developing.

Nobody really knows why the vitreous sometimes does not develop correctly. Most cases of PHPV occur by chance although some cases are known to run in families. Usually the condition only affects one eye.

How is PHPV first suspected?

PHPV can present in different ways. These include:

  • White pupil (leucocoria)
  • Squint (when both eyes don't appear to point in the same direction)
  • Painful red eye
  • Blurred vision
  • Nystagmus (fast to and fro movements of the eyes)

The most common way PHPV presents is when the normally black pupil in a child's eye looks pale or white. Parents may notice this around the time of birth or sometimes later in photographs of their child. The medical word for white pupil is Leucocoria. Leuco means white. Coria means pupil. The pupil looks white because the scarred and hazy vitreous behind the lens is white. A scan of the affected eye can help confirm the diagnosis. Other eye conditions can present as Leucocoria. It is important to be able to tell the difference. Different conditions need different treatment.

An eye with poor vision may start to squint. This is when one eye does not appear to point in the same direction as the other.

Sometimes an eye affected with PHPV can become painful and red. This may mean that the pressure has become high and has developed glaucoma. If drops do not make the eye comfortable an operation may be needed.

Sometimes parents also notice (by the way their child acts) that their child's vision is reduced. If they discuss their concerns with their Family Doctor an assessment can be arranged.

Usually children with PHPV develop fast to-and-fro movements of the eyes (nystagmus). Again parents often notice this. It is a common sign seen in children with blurred vision. If parents discuss their concerns with their Family Doctor an assessment can be arranged.

How does PHPV affect a child's vision?

PHPV can affect different children in different ways. PHPV usually causes blurring of vision. The more hazy the vitreous is the more blurred the vision will be.

Most children with PHPV in only one eye usually have good vision in the other. These children do not normally complain of reduced vision. If both a child's eyes suffer from PHPV then they are much more likely to have serious visual impairment.

Most young children who are born with PHPV in both eyes however feel their vision to be 'normal'. At first they assume that everyone else has vision the same as their own, as they have never known anything else but their own visual world. They do not realise that other people see things differently.

If only one eye is slightly affected then the child may have near normal vision. If PHPV affects both eyes then the child may be more severely visually impaired. They may only see bright lights and large shapes. This usually allows the child to get around but may not notice kerbs and smaller objects on the floor. They are likely to recognise faces from close up and be able to play with large toys.

How can doctors help?

Treatment depends on:

  • Whether both eyes are affected or not and
  • What other eye conditions develop

If only one eye is affected operations are avoided. The main aim is to try to preserve the eye. Improving vision in an eye with PHPV is difficult and usually needs several operations. If the other eye has good vision it is often best not to try operations that are unlikely to offer an affected child any overall visual benefit. It is also important to ensure the child is happy with the appearance of the affected eye. Sometimes wearing glasses or a contact lens can improve the appearance.

If both eyes are affected operations can be done to try to improve vision. If an eye becomes red and painful due to high pressure operations may also be needed. An operation normally tries to take away the hazy vitreous and usually the lens as well. This would let light travel through to the back of the eye without being blocked. It also helps to reduce pressure in the eye.

The retina can detach in eyes with PHPV. The retina is the light sensitive film that covers the back of the eye. This can be very difficult to reattach and fix despite 2 or 3 operations. If the central part of the retina is detached then vision in that eye will be poor.

Children need frequent eye drops after an operation. The operated eye may benefit from wearing a contact lens and patching of the other eye. The child will also need to attend the hospital regularly for check ups. It can be a demanding time. Sometimes the operations are not successful. Both the child and parents need to know before starting on treatment that it will be hard work with no guarantee of improvement in vision or reduction in pain.

If treatment is not successful sometimes it is better for the child to have the eye removed (enucleation). Prosthetic (artificial) implants can be placed in the bony orbital cavity that normally holds the eye. The implant helps the bony orbit grow fully, to the same size as the other side. An imitation eye, that matches the other eye, can then sit comfortably upon the implant.

How can parents, family, friends and teachers make a difference?

Many things can be done to help children with PHPV see better.

We use our vision to get around, learn new things and to meet other people and make friends.

Most children with PHPV give the impression that their vision is normal. It is important however to consider what your child's particular problems with vision might be.

If your child has been prescribed spectacles, contact lenses or a Low Visual Aid (LVA) it is important that they are encouraged to wear and use them. This will help your child see more clearly and ensure the vision parts of the brain grow and develop correctly. Wearing a hat and tinted glasses can also help reduce problems from bright light. If your child has been prescribed drops they should be used regularly as advised by the nurses and doctors at the eye clinic.

Problems at school may be due to some of the reading books being hard to see. This often means it takes longer and more effort to do the work. If the size of print is increased and letters and words spaced more widely most children will find schoolwork easier. Good bright lighting and crisp black print on a clean white background will also make things easier.

It is also worth watching carefully to find out what the smallest toys are that a child can see and play with. Then try to only play with toys that are the same size or bigger. Placing one toy on a plain background will often help children see it more readily. Placing lots of toys of different size and colour close together on a patterned background can make them 'invisible' to some children.

Recognising facial expressions can often be difficult. It is worth trying to find out at what distance facial expressions can be seen and responded to. Then always try to talk and smile from within this distance. This helps a child to learn what facial expressions mean and to copy them.

Infants and young children need to learn about the world around them. Home visiting teachers, physiotherapists and occupational and speech therapists may all add to the child's care and education. It is important to continue the programmes that they recommend. If the child is involved in family activities vision can improve and new skills can develop.

Even if a child has very poor vision many useful and practical things can be done to improve the ability of the child to get around, interact with other children and learn.

Who wrote these documents?

These pages are the consensus of opinion of many different people. They include parents of visually impaired children, visually impaired children themselves, Community Paediatricians, Ophthalmologists, Educationalists and Psychologists.

The main author and person responsible for their content is Dr Andrew Blaikie who was an Ophthalmology Research Fellow with Visual Impairment Scotland and is a member of the Royal College of Ophthalmologists.

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