Eye: persistent hyperplastic primary vitreous
Synonym(s): PHPV, Persistent hyperplastic tunica vasculosa lentis (PHTVL), PHTVL/PHPV
Introduction
- Cause: developmental ocular disorder.
- Signs: there is a spectrum of effects, from no visible outward signs to intraocular hemorrhage, cataracts and blindness.
- Diagnosis: ophthalmoscopy, ocular ultrasonography.
- Treatment: none unless vision impairment is caused by cataracts, which can be surgically removed by modified phacoemulsification.
- Prognosis: more severely affected dogs have poor vision. Phacoemulsification is more complicated in these cases and so the risks are greater.
Presenting signs
- The severity of the clinical findings of PHTVL/PHPV vary on a spectrum and a grading system of grades 1-6 developed by Stades has been internationally adopted.
- Grade 1 - Limited posterior capsular cataract with multifocal pigment or white dots on the capsule or retrolental.
- Grade 2 - More extensive central posterior capsular cataract with yellow-brown capsular/retrolental fibrous tissue. Multiple pigment dots found in both the central and peripheral portions of the posterior lens capsule. Cataract may be dense. Persistent pupillary membrane (PTVL anterioris) may also be apparent.
- Grade 3 - PTVL - hyaloid system vessels visible retrolentally as a meshwork, associated with Grade 2 abnormalities to a greater or lesser extent.
- Grade 4 - Posterior lenticonus of varying extent and associated Grade 2 abnormalities.
- Grade 5 - Combination of Grade 3 and Grade 4 abnormalities.
- Grade 6 - The severest manifestation, a combination of the other grades, plus abnormalities such as lens coloboma Lens: coloboma, microphakia, microphthalmos and intraocular hemorrhage.
Age predisposition
- Congenital condition which should be visible by six weeks of age.
Breed/Species predisposition
- Dobermann (inherited) Dobermann.
- Staffordshire Bull Terrier (inherited) Staffordshire Bull Terrier.
- Bloodhound Bloodhound.
- Bouvier des Flandres Bouvier des Flandres.
- Samoyed Samoyed.
- Siberian Husky Siberian Husky.
- Spanish Pachon.
Special risks
- Cataract surgery is more challenging when there is a patent hyaloid artery, which will require cautery. A planned posterior continuous curvilinear capsulorrhexis is usually required. An anterior vitrectomy may be required.
- Potential complications include vitreal hemorrhage with the formation of traction bands that could result in retinal detachment.
Pathogenesis
Pathophysiology
- As a result of the anomalous development of the primary vitreous, it persists during formation of the secondary vitreous, when it would normally regress.
- It is associated with hyperplasia of the mesodermal elements contained in the primary vitreous and hyaloid artery system, combined with subsequent incomplete regression.
- In the Dobermann, the inheritance pattern is thought to be incomplete dominance.
- Similarly, genetic studies in the Staffordshire Bull Terrier suggest that PHPV is either caused by a single mutation with a dominant or co-dominant mode of inheritance, or is a more complex condition with perhaps several genes involved. Further studies are on-going at the Animal Health Trust, UK.
Diagnosis
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Treatment
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Prevention
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Outcomes
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Further Reading
Publications
Refereed Papers
- Recent references from PubMed and VetMedResource.
- Gemensky-Metzler A J, Wilkie D A (2004) Surgical management and histologic and immunohistochemical features of a cataract and retrolental plaque secondary to persistent hyperplastic tunica vasculosa lentis/persistent hyperplastic primary vitreous (PHTVL/PHPV) in a Bloodhound puppy. Vet Ophthalmol 7, 369-375 PubMed.
- Pollard Z F (1997) Persistent hyperplastic primary vitreous: diagnosis, treatment and results. Transactions of the American Ophthalmological Society 95, 487-549 PubMed.
- Leon A, Curtis R, Barnett K C (1986) Hereditary persistent hyperplastic primary vitreous in the Staffordshire bull terrier. JAAHA 22 (6), 765-774 VetMedResource.
- Stades F C (1983) Persistent hyperplastic tunica vasculosa lentis and persistent hyperplastic primary vitreous in doberman pinschers. Genetic aspects. JAAHA 19 (6), 957-964 VetMedResource.
- Stades F C (1980) Persistent hyperplastic tunica vasculosa lentis and persistent hyperplastic primary vitreous (PHTVL/PHPV) in 90 closely related Doberman Pinschers: clinical aspects. JAAHA 16 (5), 739-751 VetMedResource.
- Reese A B (1955) Persistent hyperplastic primary vitreous. American Journal of Ophthalmology 40 (3), 317-331 PubMed.
Other sources of information
- Veterinary Ophthalmology (2013) 5th edn. Edited by Gelatt K N, Gilger B C & Kern T J. John Wiley and Sons, Ames, Iowa. ISBN 978-0-470-96040-0
Organisation(s)
- Animal Health Trust UK www.aht.org.uk/cms-display/research_phpv.html