Multiple congenital ocular anomalies syndrome in a family of Shetland and Deutsches Classic ponies in Belgium

The multiple congenital ocular anomalies (MCOA) syndrome has been associated with the Silver phenotype only in a few equine breeds. This report describes the phenotypic and genotypic characteristics of MCOA in a family of Silver‐coated Shetland ponies including a 20‐year‐old stallion, 17‐year‐old mare and their 1.5‐year‐old female offspring. Another 7‐year‐old Silver female Deutsches Classic Pony descending from the same dam but from a different sire, was also examined. Each pony underwent a complete ophthalmic examination, tonometry, ocular ultrasonography and genotyping for the silver coat colour. The stallion had a thickened iris, temporal retinal atrophy and bilateral iridociliary and peripheral retinal cysts. All females presented more severe anomalies: cornea globosa, iridocorneal adhesions, miosis, hypoplastic granula iridica and poorly responsive pupils to light and to pharmacological mydriasis. Iris hypoplasia, anterior cortical cataracts and temporal retinal atrophy were detected in 2 mares. One female presented bilateral lens subluxation. Supero‐temporal cystic structures were confirmed ultrasonographically in all ponies. The stallion was heterozygous for the Silver mutation, whereas all females were homozygous. This is the first report of the MCOA syndrome in a family of Shetland ponies and a Deutsches Classic Pony in association with the Silver phenotype, in Europe.     

Post mortem magnetic resonance imaging of multiple congenital ocular anomalies in a Comtois mare

Multiple congenital ocular anomalies syndrome consists of several abnormalities mainly localised to the anterior segment of the eye. This condition, comprising iridal or ciliary cysts with or without iridal hypoplasia, an excessively protruding cornea (‘cornea globosa’), miotic pupils, retinal dysplasia, cataract, and other lesions, has been reported in several breeds and is strongly related to the silver coat colour, as a result of a dominant mutant allele at the PMEL17 locus. This report describes the macroscopic, ultrasonographic, histological and magnetic resonance imaging findings of the ocular abnormalities in a Comtois mare with multiple congenital ocular anomalies syndrome.     

Multiple ciliary body cysts and secondary glaucoma in the Great Dane: a report of nine cases

Between 1989 and 1995 nine Great Danes were presented to the small animal clinic of the University of Zurich Teaching Hospital with glaucoma and multiple cysts in the anterior and posterior chamber. In four of the nine dogs cysts were present in both eyes; however, bilateral glaucoma was seen in one case only. Mean intraocular pressure (IOP) at initial presentation was 42 mmHg measured by applanation tonometry. With the exception of one dog, all animals were treated medically for a minimum of 13 days. Two animals were subsequently lost to follow up. Two dogs underwent evisceration with implantation of a silicone prosthesis. The glaucomatous globe was enucleated in three dogs. One owner declined surgery and the dog remained buphthalmic with a poorly controlled IOP. One animal remained visual after trans-scleral diode laser cyclophotocoagulation. Histopathology of the three enucleated globes showed multiple cysts originating from the ciliary body epithelium. The mechanism of IOP elevation is probably by anterior displacement of the iris with narrowing of the angle and collapse of the ciliary cleft. A delicate pre-iridal fibrovascular membrane was also seen in each case, which could have also contributed to the elevation of IOP. The high odds ratio of 2.23 (CI 95% 1.14, 3.99) for glaucoma and 37.01 (CI 95% 16.42, 77.81) for ciliary body cysts suggests they are both inherited in this breed. Pedigree analysis of the affected dogs failed to definitely reveal the mode of inheritance.     

Comparative Doppler imaging of the ophthalmic vasculature in normal Beagles and Beagles with inherited primary open-angle glaucoma

The objective of this study was to compare orbital and ocular vasculature velocity, measured by Doppler imaging, in normal Beagles and Beagles with inherited primary open-angle glaucoma. Eight normal Beagles and 13 Beagles with different stages of primary open-angle glaucoma were evaluated twice with a 2–4-week period between measurements. Doppler imaging was performed with the dogs anesthetized, and the Doppler transducer applied directly on the corneal surface. The majority of the orbital vasculature (external ethmoidal artery; internal ophthalmic artery and vein; and external ophthalmic artery and vein) and ocular blood vessels (anterior ciliary artery and veins; long posterior ciliary arteries; short posterior ciliary arteries; primary retinal arteries; and the vortex veins) were identified and Doppler blood velocity parameters were determined. The glaucomatous dogs demonstrated significant differences in the Doppler velocity parameters of several orbital vessels (external ethmoidal, external ophthalmic, and internal ophthalmic arteries), and several ocular vessels (anterior ciliary, short posterior ciliary, and long posterior ciliary arteries). These differences included decreased blood velocities, and increased pulsatility and resistive indexes. The Doppler blood flow velocities of the primary retinal arteries were unchanged between the normal and glaucomatous dogs. In the glaucomatous dogs, the Doppler imaging suggests increased vascular resistance downstream in both the orbital and ocular vasculature. These blood velocity parameter changes may be primary or secondary, and may offer therapeutic opportunities to increase perfusion, prolong the retina and optic nerve head function, and maintain vision in the canine glaucomas.     

Intercellular junctions in rabbit eye ora serrata

Summary The aim of this study was to describe and localize the intercellular junctions in the ora serrata region of albino and pigmented rabbit eyes. Eyes of albino and pigmented rabbits were fixed and processed for transmission electron microscopy. Light and electron microscope examination was carried out on semithin and ultrathin sections. The ora serrata region showed adherens, gap and tight junctions in the retinal and ciliary margins of albino and pigmented rabbit eyes. In the retinal margin, zonulae adherens between Müller cells and photoreceptors are associated with tight junctions. In the ciliary margin, epithelial cells are joined by adherens, gap and tight junctions localized between apical and apicolateral cell membranes. Tight junctions appear as zonulae occludens in the non-pigmented apicolateral cell membranes and as tight focal junctions between pigmented and non-pigmented apical cell membranes. Between the ciliary and retinal margins there are adherens and tight focal junctions which attach pigmented apical cell membranes to adjacent cells. There were no differences in the distribution of intercellular junctions between albino and pigmented rabbits.     

Microvasculature of the hamster eye: scanning electron microscopy of vascular corrosion casts

The architecture of the retina, choroid and anterior eye segment was investigated in 12 Syrian hamsters using scanning electron micrographs of methylmethacrylate corrosion casts. The hamster eye receives its primary blood supply from the long posterior ciliary artery, which directly enters the optic nerve head, and divides into three branches: the central retinal artery and medial and lateral long posterior ciliary arteries. In the retina the central retinal artery divides into six radiating branches. Retinal arterioles form a slender and long course to capillaries. Retinal capillaries are extremely thin in diameter and form a sparse capillary network. The choroid is supplied by the long posterior ciliary arteries. Choroidal arterioles exhibit a thick and short course to the choriocapillaris. The choriocapillaris is thick and sinusoid-like, forming a dense network in the choroid. The ciliary body, iris and bulbar conjunctiva are supplied by the anterior ciliary arteries, which are branches of the long posterior ciliary arteries. Capillaries supplying the anterior margin of the ciliary process are large in diameter with an irregular bore, forming a thoroughfare channel draining blood in the ciliary arterioles into the pars plana vessels. Blood from the retina is drained by the central retinal veins. Venules from the anterior eye segment empty into the vortex veins via the pars plana vessels. Venous blood from the choroid is drained only by vortex veins via the choroidal veins. The functional significance of the vascular architecture and species differences are discussed.     

Ocular lesions of bovine malignant catarrhal fever

The ocular lesions of bovine malignant catarrhal fever were characterized in 15 naturally occurring and eight experimentally induced cases of the disease. Consistent findings included: lymphocytic vasculitis of retinal, scleral, posterior ciliary, and uveal vessels; uveitis, especially involving ciliary processes, ciliary body, and iris; and keratitis with corneal edema, neovascularization, and epithelial and endothelial degeneration. Lymphocytic ciliary neuritis and optic meningitis were found less frequently. Ultrastructural examination of the ciliary body and iris from one experimental calf confirmed that most infiltrating mononuclear cells were lymphocytes. The uveitis, vasculitis, and keratitis of malignant catarrhal fever were probably immune-mediated.     

Glaucoma associated with uveal cysts and goniodysgenesis in American Bulldogs: a case series

We present a series of three American Bulldogs with clinical signs of glaucoma and intraocular inflammation accompanied by bilateral uveal cysts and abnormal gonioscopic findings. All dogs proved refractory to medical management and were enucleated. Histopathologic findings were similar in all three and included significant preiridal fibrovascular membranes and mononuclear inflammatory infiltrates in the anterior uvea. On microscopic evaluation, cysts appeared to arise primarily from the ciliary body and iridociliary sulcus, with smaller cysts also budding from the posterior iris. Pigment dispersion was variable but consistent, involving deposition of a small number of pigment‐laden cells in the dependent trabecular meshwork. Cataract formation was not noted. Glaucoma associated with uveal cysts has been described previously in Golden Retrievers and Great Danes, although clinical and histopathologic findings in those breeds are not identical to those described here. American Bulldogs with uveal cysts should have gonioscopy performed and should be monitored carefully for signs of increased intraocular pressure and intraocular inflammation. Furthermore, documentation of cyst‐associated glaucoma in a third breed suggests clinicians should exercise caution in dismissing uveal cysts in dogs as incidental findings.     

Ultrastructural ocular lesions of 6-aminonicotinamide toxicosis in rabbits

6-Aminonicotinamide, given by intraperitoneal injection to male and female Dutch belted rabbits, produced swelling and vacuolation of ciliary and iridal epithelium plus vacuolation of the retinal pigment epithelial and outer plexiform layers of the retina. By transmission electron microscopy, inner and outer ciliary epithelial cells and inner iridal epithelial cells contained numerous coalescing, membrane-bound vacuoles of the cytocavitary network. These vacuoles were viewed as numerous interconnecting, intracytoplasmic cavities in scanning electron micrographs. Swelling of vacuolated epithelial cells and the presence of fibrin and proteinaceous fluid in the ciliary stroma resulted in thickening of the anterior ciliary processes with the formation of surface alterations detectable by scanning electron microscopy. In transmission electron micrographs the vacuoles in the retinal pigment epithelium were large, electron-lucent spaces and the vacuoles in the outer plexiform layer of the retina appeared to be intracytoplasmic spaces in axons of photoreceptor cells. Distention of cytocavitary structures has been reported in glial cells of animals given 6-aminonicotinamide and this change was apparently due to alterations in ion and water movement across cellular membranes that resulted in intracellular edema.     

The causes of glaucoma in cats

The cause of glaucoma in 131 enucleated eyes from 128 cats was determined in a retrospective histologic study. Obliteration of the ciliary cleft by diffuse iridal melanoma (38 eyes), or other neoplasms (14 eyes), or by the presence of idiopathic lymphocytic-plasmacytic anterior uveitis (53 eyes) were the most frequent lesions likely to explain the development of glaucoma. Secondary changes of inner retinal atrophy, optic disc cupping, scleral thinning with megaglobus, and atrophy of ciliary processes were similar to those described in dogs and human beings with chronic glaucoma. In light of the duration and severity of the glaucoma, the degree of inner retinal atrophy was often less than expected. Diffuse corneal edema and breaks in Descemet's membrane, changes typical of glaucoma in other species, were rarely detected. Eyes with chronic uveitis and glaucoma had collapsed ciliary clefts, iridoscleral adhesions, and posterior displacement of the iris. We were unable to determine whether these changes were consequences of the uveitis and thus responsible for the development of glaucoma, or if they were merely the result of the chronic glaucoma itself.     

Unilateral ocular subalbinism in a laboratory Beagle dog

Blue discoloration of the iris was found in the left eye of a male laboratory Beagle dog, which had a normal tricolor coat and clinically showed no visual impairment. Ophthalmoscopically, the affected eye revealed red-colored tigroid fundus, in which no tapetum was present. The retinal vasculature and the optic disc showed no noticeable changes. Histopathologically, in the left eye melanocytes had extremely few melanin granules in the anterior segment, including the anterior border layer, in the stroma and sphincter muscle of the iris and in the stroma of the ciliary body and choroid. However, the posterior pigment epithelium of the iris, the pigment epithelium of the ciliary body and the retinal pigment epithelium showed normal pigmentation. The tapetal elements were completely absent. Number and distribution of the S-100 protein-positive melanocytes with or without melanin granules in the iris, ciliary body and choroid of the left eye were similar to those of the normal right eye. Ultrastructurally, melanocytes in the anterior segment of the affected iris possessed no or few melanosomes which were incompletely melanized. In the right eye, no abnormal features were observed. Based on these results, the present case was diagnosed as unilateral ocular subalbinism with tapetal aplasia in a Beagle dog.     

Scanning electron microscopy of corrosion casts of the optic nerve microcirculation in dogs

Scanning electron microscopy of vascular corrosion casts of the optic nerve region in normal and glaucomatous Beagles demonstrated that the blood supply to the laminar optic nerve is derived from short posterior ciliary arteries, cilioretinal arteries, and longitudinal pial vessels. The short posterior ciliary arteries formed a ring of striated pillars around the scleral canal. The central retinal artery was not present in the dog. Differences between the casts in normal and glaucomatous dogs were not detected.     

The pathogenesis and significance of pre-iridal fibrovascular membrane in domestic animals

Histologic examination was made of 1,419 globes from domestic animals (964 dogs, 374 cats, 41 horses, and 40 cattle) with ocular disease; pre-iridal membranes (rubeosis iridis) were found in 98. The membranes originated as endothelial budding from the anterior iridal stroma and seemed to mature into fibrous or fibrovascular membranes that were often followed by hyphema or, occasionally, glaucoma. Pre-existent disease in the 98 affected globes included chronic endophthalmitis (27/98), chronic glaucoma (24/98), anterior uveal melanoma (15/98), ciliary body adenoma (14/98), neoplasms metastatic to the eye (8/98), and chronic retinal detachment (6/98). In terms of likelihood of occurrence, pre-iridal membranes seen in 21% (6/21) of globes with retinal detachment, 19% (14/75) of those with ciliary body adenomas, 14% (24/167) of those with chronic glaucoma, and 10% (15/158) of those with anterior uveal melanoma. They were detected with greatest relative frequency in horses (9/41) followed by dogs (83/964), cats (5/374) and cattle (1/40). These membranes, which are rarely detected by clinical examination, probably form in response to angiogenic factors released by ischemic retina, by neoplasms, or by leukocytes involved in ocular inflammation.     

A histopathological study of iridociliary cysts and glaucoma in Golden Retrievers

This is a retrospective histopathological study of archive slides from the Comparative Ocular Pathology Laboratory of Wisconsin from 1981 to 1997. Reports on eyes or ocular contents from 2176 dogs were reviewed. Five-hundred and thirty out of 2176 (24%) of the cases had a clinical or histologic diagnosis of glaucoma. Twenty-five out of 530 (5%) of the canine cases of glaucoma were in Golden Retrievers. Thirteen out of 25 (52%) of the Golden Retriever cases of glaucoma had iridociliary cysts. Iridociliary cysts in Golden Retrievers may lead to the development of glaucoma. Histologically all 13 of the cases of glaucoma and iridociliary cysts had large thin-walled cysts lined with attenuated cuboidal epithelium filling most of the posterior chamber. The following histologic features were also present: thick walled cysts containing hyaluronic acid (8/13; 62%), a solid cellular proliferation (2/13; 15%), a retrocorneal membrane associated with a defect in Descemet's membrane (7/13; 54%), iris bombé (5/13; 38%), a preiridal fibrovascular membrane (4/13; 31%), hemorrhage (4/13; 31%), a cellular membrane on the anterior lens surface (6/13; 46%), retinal detachment (5/13; 38%), peripheral anterior synechia (5/13; 38%), and posterior synechia (6/13; 46%). Complete follow up was obtained on 11/13 (85%) of the Golden Retrievers with glaucoma and iridociliary cysts. Two/11 (18%) of the dogs were euthanized due to intractable glaucoma. Eight/13 (62%) of the dogs had problems in the other eye. The other eye was diagnosed with uveitis 5/11 (45%), glaucoma 2/11(18%), pigment in the anterior chamber 3/11 (27%), and iridociliary cysts 4/11 (36%).     

Ocular albinism and hypopigmentation defects in Slc24a5-/- mice

As part of a high-throughput mutagenesis and phenotyping process designed to discover novel drug targets, we generated and characterized mice with a targeted mutation in Slc24a5, a gene encoding a putative cation exchanger. Upon macroscopic examination, Slc24a5-/- mice were viable, fertile, and indistinguishable by coat color from their heterozygous and wild-type litter mates. Ophthalmoscopic examination revealed diffuse retinal hypopigmentation, and a histologic examination of the eye confirmed the presence of moderate-to-marked hypopigmentation of the retinal pigmented epithelium (RPE), ciliary body, and iris pigment epithelium (IPE). Hypopigmentation was most severe in the anterior layer cells of the IPE, where melanosomes were smaller, paler, and more indistinct than those of the anterior stroma and posterior IPE. The pigment granules of the posterior IPE appeared to be nearly as dark as those in stromal melanocytes; however, both cell layers were thinner and paler than corresponding layers in wild-type mice. Ultrastructural analysis of the RPE, IPE, and ciliary body pigmented cells confirmed that mutation of Slc24a5 results in marked hypopigmentation of melanosomes in optic cup-derived pigmented neuroepithelium in the eyes. Milder reductions in melanosome size and pigmentation were noted in neural crest-derived melanocytes. The severe hypopigmentation of neuroepithelium-derived cells in the eyes resulted in a novel form of ocular albinism in Slc24a5-/- mice. Our findings suggest that SLC24A5 may be a candidate gene for some forms of ocular albinism and for the BEY1/EYCL2 locus previously associated with central brown eye color in humans.     

Ocular manifestations of a metastatic pulmonary adenocarcinoma in a cat

A primary pulmonary bronchogenic adenocarcinoma originating from an intramediastinal accessory lung was diagnosed in a 14.5-year-old cat. The cat had been admitted because of a cloudy right eye. Physical examination revealed a thin cat with severe iritis, aqueous flare, and a fibrin clot in the anterior chamber of the right eye. Right fundic examination revealed bullous retinal detachment superior to the optic disc. Euthanasia and necropsy were requested when FeLV test results were positive. Metastatic neoplastic cells similar to those of the primary tumor were detected in the choroid, ciliary body, and ciliary processes of the right eye. Ciliary and iridic stromal necrosis attributable to neoplastic embolization of uveal vessels had led to severe uveitis. Foci of metastasis were also in the heart, kidney, and cerebral meninges.     

Congenital ocular abnormalities of Rocky Mountain Horses

Objective: To determine the incidence and describe ocular abnormalities in a cross-section of the population of Rocky Mountain Horses.
Design: Prospective study.
Animals: Five-hundred and fourteen Rocky Mountain Horses.
Procedure: Ophthalmic examinations were performed using a slit-lamp biomicroscope and an indirect ophthalmoscope. Intraocular pressures were measured by applanation tonometry. Eyes from six horses were obtained for histologic examination.
Results: Cysts of the posterior iris, ciliary body, and peripheral retina were detected most frequently (249 horses), and were always located temporally. Curvilinear streaks of retinal pigmented epithelium extending from the peripheral temporal retina marked the boundary of previous retinal detachment in 189 horses. Retinal dysplasia was detected in 125 horses. Multiple ocular anomalies were evident in 71 horses and were always bilateral and symmetrical. Affected eyes had a large, clear cornea that protruded excessively and had an apparent short radius of curvature, a deep anterior chamber, miotic and dyscoric pupil, and iris hypoplasia. Pupillary light responses were decreased or absent and pupils failed to dilate after repeated instillation of mydriatic drugs in horses with multiple ocular anomalies. Less frequently encountered abnormalities included peripheral iridocorneal adhesions and goniosynechiae. Congenital cataract was always present in eyes with multiple abnormalities. Intraocular pressures did not differ among horses with normal eyes and horses with multiple ocular abnormalities. Histologic examination of eyes corroborated the clinical appearance.     

Bilateral typical complete colobomas in a donkey: Retinographic and fluorangiographic description

An 18-year-old male donkey was referred with a history of blindness. On ocular examination a complete bilateral typical colobomatous defect involving iris, ciliary body, lens, choroid, retina and optic nerve was observed. An optic pit was presented at the temporal side of the right optic head. Ophthalmoscopic and fluorangiographic characteristics are described.