Doberman Eye Diseases:

Editors Comments: The following information was abstracted from an email from Dr. Mervi Ihantola of Nastola, Finland, a veterinarian and Doberman breeder. We are fortunate here in the United States that very few Dobermans are affected with this disease. However, with the larger numbers of dogs of European bloodlines being imported recently into this country we must maintain a high degree of vigilance. Here we have an expert resource in CERF-certified veterinary ophthalmologists. We strongly recommend that all dogs be examined and registered with CERF to avoid the dilemma now faced by our colleagues in Europe.

Dr. Ihntolas comments: The problem we have in European bloodlines is called PHTVL/PHPV (Persistent Hyperplastic Tunica Vasculosa Lentis and Persistent Hyperplastic Primary Vitreous). In 1980 and 1983, this anomaly first found in the Dutch Dobermann population was described in detail in the veterinary literature. PHTVL/PHPV is a congenital eye anomaly, which in most cases leads to formation of a cataract. It is present at birth and can be detected in young pups. The problem in the eye goes back to the time of embryonic development when the growing lens needs a blood supply. A. hyaloidea comes from the retina through the primary vitreous to the posterior area of the lens. From the gestation day 45 onwards, these blood vessels should start to disappear; this is not the case in affected Dobermanns. There remains fibrotic tissue left from the vessel system attached to the lens capsule and sometimes other problems. The spots are situated behind the lens, on the posterior lens capsule. They are remnants of the hyaloid artery, and when grade 1, ONLY SMALL SPOTS ranging from five to one hundred can be seen; these will be scattered in the periphery of the lens if only a few are present. Normally spots are found in both eyes. If there are only a few and in only one eye, it is considered a questionable or borderline case. When spots are more numerous, they are also evident in the central area, and are also easier to detect. Dr. Stades, a prominent researcher into the disorder, says it looks like a sky with stars. In grade 2 eyes, the spots form more clear plaques and little cataracts can be seen. In grade 3 eyes, the plaque is combined with persistent parts of the hyaloid vascular system. In grade 4, plaques are also present and the lens formation itself is abnormal (lenticonus posterior). In Grade 5 there are both severe type 3 and 4 changes. Grade 6 is a totally blind animal with severe changes of the lens shape, free blood in the eye, etc. There is a strong suspicion that the disease had existed for sometime before veterinarians and breeders recognized it. We know previous stories about blind Dobermanns from Germany. At about the same time it was recognized in the Netherlands, PHTVL was found in Dobermanns in Sweden and in Finland. First only some individuals with PHTVL were found. As veterinarians became more expert, it was discovered that grade 1 was as common in Finland as in Holland. (At the beginning in Holland -51
Breed01-1.p65 Feb. 26, 2001

it was nearly 30%!!). The severe cases were fewer (10%). Bad cases are very few, Grade 1 is more typical - as long as the vets know what they are looking for and dare to say it to their clients. Grade 1 is VERY difficult to see at first and can be seen only with a microscope. There may be only five to ten spots and maybe only in one eye (borderline case). The good thing here is that Grade 1 doesnt progress, (Im quite convinced that grade 2 or 3 will not cause blindness either, but further grades surely cause problems. If not outright blindness, then at least they can make the dog unsure and frightened because of the impaired vision). One other good point is that PHTVL is CONGENITAL. It can be found by a skilled ophthalmologist at a very early age!! So normally a breeder can have the eyes of his puppies examined before they are sold. Grade 1 is so difficult to detect that a free puppy may later be found to have grade 1. Grade 1 however, never seems to progress further. Because of the problems with the results and the large number of otherwise excellent grade 1, animal breeders have decided in Finland (and in Holland as well) to breed with them.

The hereditary basis has been studied in Holland by Dr. Stades using test matings who has concluded that it is autosomal incomplete dominant. If this conclusion is correct, an animal affected at any grade will always pass the defect to its offspring. The offspring will be affected to varying degrees however. The obvious conclusion that affected animals should not be bred has some practical difficulties resulting from difficulties in reliably detecting Grade 1. Furthermore, there are now additional doubts as to the significance of Grade 1. What is grade 1? Stades himself was a little unsure some years ago when he reported that they had a lot of peculiar results in Holland when mating grade 1x grade 1. More healthy animals that appear clear were produced than could be expected. Why? He couldnt tell. Maybe not all grades 1 were important; why the heredity didnt work. So at the moment there are open questions. Until then, breeders in Finland still use grade 1 for breeding, because the dogs in question are too valuable to be discarded only because of the eyes but try not to do combinations grade 1 x grade 1. All breeding material in Finland has to be examined for PHTVL but only once in their lifetime. That is because PHTVL is seen from the puppyhood. At the moment, Dobermanns in Europe do not suffer from other eye problems (PRA, RD, HC).

-52

Breed01-1.p65 Feb. 26, 2001

EYE PROBLEMS We recommend that all breeding stock be examined on an annual basis by a CERF (Canine Eye Registration Foundation) -certified veterinary ophthalmologist. Although most of the eye diseases found in dobermans are rare, there is one which has been found on a consistent basis, especially in dogs of close European ancestry: PHTVL/PHPV In the Doberman Pinscher, persistent hyperplastic primary vitreous (PHPV) is one part of a dysplastic disease process of the lens and posterior lense capsule of the eye. Persistent hyperplastic tunica vasculosa lentis (PHTVL) is also an integral part of this syndrome. The condition is referred to as PHTVL/PHPV. PHTVL/PHPV is an ocular defect in the Doberman Pinscher, inherited through an incomplete dominant gene. To date, almost all cases in Dobes have been restricted to the Netherlands [editorial commentof course weve learned since 1986, when this was published, that this is no longer true]. Both eyes are usually affected. In most cases, it becomes progressively more severe. PHTVL/PHPV begins during the 25th to 35th day of fetal development, occurring equally in both sexes. A defect in the formation of the covering of the lens in the eye is followed by a failure in the regression of fetal eye lens blood vessels. The severity is graded from 1 to 6. Grade 1 is very slight and consists of a slight posterior lens capsule cataract with small pigmented dots in the same region. At the other end of the scale, grade 6 consists of a combination of findings present in lower grades including severe degeneration of the lens, the presence of a great deal of pigment on the lens, and the presence of fetal vascular structures around the lens. Dobes having grade 1 PHTVL/ PHPV are able to see well. Those affected with grades 3 to 6 have increasingly poor vision sometimes manifested by increased aggressiveness. As vision decreases, fear of being out of control causes increased reactions to stimuli. Generally specialized ophthalmic examination equipment is required to assess the degree of involvement. The clinical signs are only apparent to the naked eye when the eyes are severely affected. The pupil of the eye looks white, and the eye itself may be smaller than normal. In some Dobes, the presence of a cataract keeps the ophthalmologist from seeing the pigmented dots on the posterior lens capsule (covering). In most dogs, the degree of blindness is progressive. Only those dogs with Grade 1 and very slight cataract involvement may not progress with age. The white pupil associated with PHTVL/PHPV must be distinguished from intraocular cancers, congenital cataracts, retinal dysplasia, and retinal detachment. If treatment is attempted, the lens must be removed. In cases where the lens was removed, other problems related to the lens removal have appeared several months later. Since PHTVL/PHPV is a hereditary eye defect in the Doberman Pinscher, affected stock should not be used for breeding. Carriers with no visible defects do occur and should also be eliminated from breeding programs. All possibly affected dobes should be examined by -53
Breed01-1.p65 Feb. 26, 2001

a competent veterinary ophthalmologist any time after four weeks of age.

-54

Breed01-1.p65 Feb. 26, 2001