PHTVL/PHPV in King Charles Spaniels

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Persisting Hyperplastic Tunica Vasculosa Lentis and Primary Vitreum

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What is PHTVL/PHPV?
PHTVL/PHPV is a hereditary eye disease affecting the lens of the eye. To the affected dog, this is a serious condition that may entail complications demanding veterinary care, and regular eye tests during the whole life of the dog.

Early in the foetal stage, there is a system of blood vessels coating the lens. These blood vessels normally regress before the puppy is born. But in a dog with PHTVL/PHPV this has not occurred the way it is supposed to, and also the vitreous body of the eye, has not developed normally. In its mildest form PHTVL/PHPV causes small pigmented dots in the posterior lens capsule. In more severe cases the dog has got fully developed blood vessels in the rear face of the lens and maybe a deformed lens. Then there is a high risk of cataracts (clouding of the lens) even young puppies can go blind.

What does it mean to the affected dog?
PHTVL/PHPV is sometimes graded on a scale from 1 to 6. The mildest form does not affect the eyesight. In grade 2 to 5 there is increasingly poor vision, and grade 6, the most severe form, means total cataract, i.e. the eye is completely blind.

Another complication that might occur is a haemorrhage inside the eye. This is rather difficult for the dogs’ owner to discover, but when it happens it is necessary with immediate treatment that is continued over a long period of time.

How is it diagnosed?
PHTVL/PHPV is not visible to the naked eye, but can be seen from the age of 8 weeks in an ophthalmic examination carried out by an ophthalmologist. The examination is completely painless. This disease is congenital and will not appear later in life. However, the gene carrying the disease can exist in a dog that has been tested clear.

Is it common in King Charles Spaniels?
In Sweden there are now 14 officially recognised cases of PHTVL/PHPV, and 1 born in Norway and 1 Finnish bred exported to Norway. 
For pedigrees of these affected dogs – please click here »

In 1998-99 the Swedish Kennelclub Veterinarian did a questionnaire regarding eye anomalies in King Charles Spaniels. This was completed by the Swedish ophthalmologists. The result of the questionnaire showed that from a total of 116 examined King Charles Spaniels, 23 showed symptoms that may be related to PHTVL/PHPV. By this time 4 had been diagnosed as having PHTVL/PHPV. Out of the 23 dogs with symptoms, 3 were born in the 1970’s, 6 in the 1980’s and 13 in the 1990’s.

Is it hereditary?
Yes! A dog diagnosed with PHTVL/PHPV or one that is a known carrier of the disease should not be bred from. Litter brothers and sisters should not be bred from either. A dog that has been tested clear can however, still carry the gene and through it to his or her offspring.

PHTVL/PHPV is a hereditary disease that mainly has been studied in Dobermans and Staffordshire Bull terriers. Frans Stades in the Netherlands has investigated the inheritance in Dobermans. He believes that the inheritance is by a dominant gene, but other genes are also involved determining the expression of the disease (i.e. individuals can be affected to varying degrees) and thereby the symptoms.

What the Swedish Kennel Club and the breed club have done
The breed club (King Charles Alliansen) published in its newsletter ”Charlieposten” no 1-2 1994, an article on the first known case in Sweden, Ramblers Annie. Since 1996 Charlieposten have published the eye examination results of totally 86 dogs – both clear tests and dogs with diagnosed eye disease.

At the Annual General Meeting of King Charles Alliansen the members voted for the introduction of a set of breeding recommendations. The vote was unanimous. Regarding eyes these recommendations say that all puppies should be examined by an ophthalmologist before they are delivered to their new homes. Also all breeding stock must be examined by an ophthalmologist and be free of hereditary eye diseases. The examination result must be no older than one year at the time of mating. The reason for the examinations of adult breeding stock is to check for other hereditary diseases such as cataracts and RD, which sometimes appear in the breed. A dog with a hereditary eye disease shall of course not be bred from. 

The KCA (King Charles Alliansen) puppy register should only recommend litters, where both the sire and the dam have tested clear as stated in the breeding recommendations.

In August 2000 the Swedish Kennel Club decided to centrally register all eye examination results in King Charles Spaniels after the KCA had applied for this. The reason for this was the occurrence of PHTVL/PHPV in the breed. 

What is the situation like today?
Five years have passed since the breed club introduced the breeding recommendations. Since then 33 litters have been registered. Out of these litters 12  live up to the recommendations.
See all litters here »

Breeders have continued breeding from dogs that have produced offspring with PHTVL/PHPV. Full- and half brothers and sisters of affected dogs have been bred from. As recent as in 2003 two littersisters of a dog with PHTVL/PHPV had had a litter each.

In France there is a Swedish export that is a daughter of Ch Ramblers Anchor, affected by PHTVL/PHPV. This bitch in France has produced 20(!) puppies, which in their turn have produced 26 puppies, and many of those are also being bred from.

What should the breeders do?
As an absolute minimum the breeders should at least follow the breeding recommendations! But breeding King Charles Spaniels is not just about eyes. A good background regarding heart condition is of prime importance, as the hereditary heartdisease MVD is the most common hereditary disease in the breed.  
Read MVD in King Charles Spaniels
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It should be in every breeders own interest to be aware of where the dangerous defects are and to try and avoid them as much as possible in their breeding program. 

Is it too much of a risk to buy a King Charles Spaniel?
No, definitely no, but one should be very careful with exactly what puppy to buy. Luckily enough to the prospective buyer, PHTVL/PHPV is a disease that can be diagnosed already when the puppy is about 8 weeks. Therefore noone has to buy an affected dog without knowing it. This is on the condition that the breeder follows the breeding recommendation to have every puppy examined by an ophthalmologist before it is delivered to its new home. As a buyer you should always demand that your puppy is examined before you pick it up. You should also demand a copy of the parents’ heartcertificates and eyecertificates! And I hope that nobody pays 10.000  SEK or more for a puppy unless the litter is bred in accordance to the breeding recommendations! 

A good advise to anyone planning to buy a puppy (indifferent what breed) is to find out as much as possible about the ancestors of the prospective puppy. The most important thing is after all to get a healthy dog..
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Reviewed by Kristina Narfström, Professor of Veterinary Ophthalmology, 2001.
November 2001, updated Nov. 2006
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