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Retinal disease and glaucoma can be overlapping conditions which present opportunities for collaboration among subspecialties.
Anita Agarwal, MD, discussed diagnostic and management considerations for patients with hypertensive uveitis, central retinal vein occlusion (CRVO), narrow angles, and retinal dystrophy, and the need for pars plana tube shunt surgery. She outlined these opportunities at the 23rd annual Glaucoma Symposium, held during the 2019 Glaucoma 360 meeting.
Hypertensive uveitis
Elevated intraocular pressure (IOP) is a feature of certain types of uveitis.
Reminding glaucoma specialists to conduct a thorough examination to establish an accurate diagnosis that will guide appropriate treatment, Dr. Agarwal underscored her point with several case presentations.
One case involved a HIV-positive patient who presented with elevated IOP and anterior uveitis.
“Cytomegalovirus was suspected to be the cause of the uveitis because the patient was HIV-positive, but the posterior segment examination showed widespread Toxo-plasma retinitis,” said Dr. Agarwal, West Coast Retina Group, San Francisco, and adjoint professor of ophthalmology, Vanderbilt University, Nashville, TN. “This patient needed treatment for the ocular inflammation and elevated IOP, but also needed systemic treatment for the toxoplasmosis.”
Another case involved a female patient presenting with hypertensive anterior uveitis in the left eye due to herpes zoster, based on the presence of vesicular cutaneous lesions over a V1 distribution.
The patient was treated with topical corticosteroids, pressure-lowering medication, and oral acyclovir, which she was taking infrequently. She returned after two weeks complaining of blurred vision in the right eye.
On exam-nation, the right eye was found to have keratitic precipitates, 2+ anterior chamber cells, significant vitritis, and acute retinal necrosis. “In patients with anterior uveitis, it is important to think about the potential for involvement of the posterior segment and also the fellow eye,” Dr. Agarwal said.
To prevent complications that can cause permanent vision loss, treatment for patients with hypertensive uveitis requires aggressive intervention to control both the inflammation and elevated IOP. Corticosteroid therapy remains the mainstay for suppressing inflammation, and in certain chronic situations, such as sarcoidosis, it has been revolutionized by the availability of long-acting intraocular implants, Dr. Agarwal said.
“Treat the inflammation fully and don’t worry about an IOP-steroid response,” Dr. Agarwal said. “If using a steroid implant, advise patients that they have a 100% chance of developing a cataract and persistently elevated IOP that may require cataract surgery and a glaucoma tube shunt.”
Changing landscape
The risks of iris neovascularization and neovascular glaucoma (NVG) developing in eyes suffering an ischemic CRVO have been reduced because of the advent of effec-tive pharmacotherapy. Current CRVO management involves intravitreal, anti-VEGF injections and steroids as needed to reduce or resolve cystoid macular edema (CME).
Patients are carefully monitored for natural evolution to development of collaterals and for the development of iris neovascularization and NVG. However, there is no clearly defined treatment endpoint, and iris neovascularization and NVG can develop as patients fail to return for follow-up.
Dr. Agarwal discussed a patient who eventually returned with count-fingers vision and was found to have massive CME, an atrophic retina, and iris neovascularization. Treatment at this stage was to restart the anti-VEGF injections and perform panretinal photocoagulation.
Retinal dystrophy
Autosomal recessive bestrophinopathy, a macular dystrophy caused by a mutation of the Bestrophin gene (VMD2) gene, is something to think about in a relatively young patient who presents with narrow angles and elevated IOP, said Dr. Agarwal.
First described in 2008, age of onset for the condition ranges from 4 to 40 years. The retinal findings include multiple yellow flecks, increased fundus autofluorescence from lipofuscin deposition, islands of retinal pigment epithelium loss, and intraretinal fluid accumulation. Many patients also have elevated IOP that is related to a narrow angle.
In affected children, elevated IOP may be the initial manifestation.
“Because the mutation is a null defect and the protein coded for by the gene is absent, the consequences are severe,” Dr. Agarwal said. "Since it is a recessive condition, patients may not have a positive family history.”
Surgical comanagement
There are several situations where glaucoma and retinal specialists may meet in the operating room.
Patients needing pars plana placement of a tube shunt is one setting. Indications for a pars plana shunt include NVG, uveitic glaucoma, traumatic aphakia with glaucoma, pediatric aphakic glaucoma, aniridia, corneal scarring, and the post-corneal transplant state.
Depending on the indication, pars plana placement may be preferred to achieve more durable success or for greater safety, Dr. Agarwal explained.
Retinal disease and glaucoma can be overlapping conditions which present opportu-nities for collaboration among subspecialties.