Disease overview

Primary lens luxation (PLL), also known as isolated canine ectopia lentis, is a painful genetic disease that causes the lens of the eye to spontaneously detach from its normal position and become displaced. Lens displacement can be posterior (lens falls backwards) or anterior (lens falls forward). A single base change mutation in the ADAMST17 gene results in PLL, an inherited condition that is painful and can lead to inflammation and glaucoma. If left untreated, this disease can lead to blindness.

Gene name

ADAMTS17

Inheritance

Autosomal dominant with incomplete penetrance

Signs and symptoms

Signs of PLL begin around 3-8 years of age. An affected dog may exhibit watery, red, teary eyes or other signs of ocular irritation in one or both eyes. The luxated lens will be visible when looking through the pupil in the eye and look like a clear half-moon (either in front or behind the iris).

How is it diagnosed

A veterinarian will review health history, conduct a thorough exam, and note any clinical signs. A careful examination of the eye by a veterinarian or a veterinary ophthalmologist can determine if there is an anterior or posterior luxation. Genetic testing can assist veterinarians with diagnosis and help breeders identify affected and carrier dogs.

Breeds at higher risk

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Citations

Oliver JAC, Rustidge S, Pettitt L, Jenkins CA, Farias FHG, Giuliano EA, Mellersh CS. Evaluation of ADAMTS17 in Chinese Shar-Pei with primary open-angle glaucoma, primary lens luxation, or both. Am J Vet Res. 2018 Jan;79(1):98-106. doi: 10.2460/ajvr.79.1.98. PMID: 29287154.

Farias FH, Johnson GS, Taylor JF, Giuliano E, Katz ML, Sanders DN, Schnabel RD, McKay SD, Khan S, Gharahkhani P, O'Leary CA, Pettitt L, Forman OP, Boursnell M, McLaughlin B, Ahonen S, Lohi H, Hernandez-Merino E, Gould DJ, Sargan DR, Mellersh C. An ADAMTS17 splice donor site mutation in dogs with primary lens luxation. Invest Ophthalmol Vis Sci. 2010 Sep;51(9):4716-21. doi: 10.1167/iovs.09-5142. Epub 2010 Apr 7. PMID: 20375329.

Disclaimer

Disease diagnosis and treatment should always be performed by a veterinarian. The following information is for educational purposes only.