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Annual exam reveals hyphema secondary to iris neovascularization
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A 48-year old white female presented for her annual eye exam through our primary care service. She was severely disabled (both mentally and physically), which left her confined to a wheelchair and completely nonverbal. She lived in a group home where a caretaker could tend to her needs around the clock.
Her previous ocular history was positive for severe mixed cataracts in both eyes; however, her power of attorney (her grandmother) had declined any further ocular intervention other than yearly eye exams since she began her care at our institution several years prior.
Her medical history was extensive, but the primary medical disorders (beyond her mental and physical limitations) were: gastric reflux disease, muscle contractures, constipation and a seizure disorder. Appropriately, her medication list included the following: omeprazole, bisacodyl, cyclobenzaprine and levetiracetam. Notably, she was not diabetic nor hypertensive. Her social history was otherwise unremarkable, and she had no known drug allergies.
The entering examination revealed stable visual acuities (as best determined for the nonverbal status). She would turn her head away when the binocular indirect ophthalmoscope was shone in her right eye, but, noticeably, she did not shift her gaze away from the light when presented to her left eye. Extraocular motilities were grossly normal without obvious restrictions in either eye. Pupils were small and equal in both eyes, but the right eye responded to light significantly, whereas the left eye did not. Additionally, there was a relative afferent pupillary defect in the left eye at this visit that was not noted previously.
Retinoscopy and refraction were unable to be performed due to the severity of the patient’s cataracts (as had been noted at prior evaluations). Handheld slit lamp anterior segment examination revealed a grossly normal anterior segment in the right eye, but a clear 10% to 15% hyphema was noted in the left eye. Upon closer inspection, there was evident neovascularization of the iris (NVI) present in the left eye as well. Goldmann tonometry was unable to be performed due to the patient’s poor cooperation as well as being confined to her wheelchair, but iCare tonometry was performed and showed IOPs of 18 mm Hg OD and 21 mm Hg OS.
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The differential diagnoses considered in this case were based on the most commonly known causes of NVI/neovascular glaucoma (NVG) and included the following:
- Proliferative diabetic retinopathy is the most common cause of NVI/NVG; however, this patient was not diabetic and, therefore, this was ruled out quickly based on her medical history.
- Retinal vein occlusion (RVO) is another common cause of NVI/NVG, but the patient had no typical known risk factors for RVO such as diabetes mellitus or hypertension. Additionally, RVOs typically do not lead to the retinal detachment/retinal traction noted on the B-scan in this case, so this was considered less likely.
- The patient had no chronic iritis or subclinical iritis signs such as keratic precipitates nor posterior synechiae at any of her previous exams at our clinic over several years.
- Trauma was ruled out by history; follow-up questioning of care givers confirmed a negative history of trauma. In addition, the patient being confined to her wheelchair due to her physical disability made this unlikely.
- Carotid occlusive disease is another known cause of NVI/NVG; however, the clinical findings in this case with B-scan (retinal detachment and/or retinal traction) are not typical of carotid occlusive disease findings in most cases. Also, this typically happens most commonly in adults who are older than this patient. However, carotid occlusive disease could be investigated further in the future with carotid Doppler ultrasound testing.
- Sickle cell retinopathy is uncommon in Caucasian populations. This patient’s case history and family history were negative for any form of sickle cell disease or thalassemias.
- Chronic retinal detachment is a known cause of NVI/NVG and can often be found on B-scan, as in this case. It fits the overall clinical presentation the best, given the limited views of the posterior pole secondary to the patient’s advanced cataracts.
Further clinical information
Gonioscopy was unable to be performed due to patient cooperation at this visit and subsequent visits. The patient was dilated in hopes of examining the posterior segment; however, this was unable to be performed due to the denseness of the patient’s cataracts in both eyes. Additionally, a posterior segment ultrasound (B-scan) showed a suspected retinal detachment and/or retinal traction in the left eye.
I immediately contacted one of our fellowship-trained retinal specialists (who conveniently also performs cataract surgeries routinely) at our local ophthalmology referral partner here in Memphis who happily agreed to see the patient promptly.
Discussion
Neovascularization of the iris is an ominous sign and typically indicates a poorly controlled underlying disease process. The most dreaded issue with NVI is the development of NVG, which can lead to extremely elevated IOPs that can be difficult to control (even with surgery in some cases), pain, headaches, loss of vision or even blindness.
Commonly, NVI and NVG occur simultaneously in patients, so for the remainder of this article both will be used synonymously as (NVI/NVG) for simplicity. NVG occurs in up to 4% to 6% of all glaucoma cases (Liao et al., Havens et al.) There are three main stages of NVG that are outlined in the accompanying table. More than 76% of patients are reported to be at least 60 years old, and 97% of cases are linked to some form of retinal ischemia, most commonly diabetic retinopathy, as shown in the other table.
The underlying problem of retinal ischemia leads to large amounts of vascular endothelial growth factor (VEGF) being released from the ischemic retinal tissues. The primary source of VEGF liberation are the Muller cells in the retina, but other studies have also shown heavy release of VEGF from ciliary body tissues as well. Other chemical mediators also play a role, but VEGF seems to be the most prominent (Chalam et al., Chen et al.). NVI begins with endothelial “budding” of the major and minor arterial circles of the iris vasculature secondary to the extensive VEGF release from the posterior segment. Approximately 6% to 12% cases of NVG have neovascularization of the angle (NVA) but no obvious signs of NVI (Havens et al.). Hence, attentive gonioscopy is imperative in suspicious cases of NVG or in any case of elevated IOP. In cases of suspicious or difficult-to-diagnose NVI, a useful test can be iris angiography (intravenous fluorescein is injected in the same manner as retinal angiography, but photos are focused on the iris instead of the retinal vasculature), which shows NVI in 97.2% of cases (Duh et al.).
A new and exciting development along these same lines, but less invasive and easier to perform, is anterior segment (OCTA). Updated OCTA software now allows anterior segment scanning of the iris and cornea blood vessels, which might prove quite useful in the future for cases of suspected NVI, as OCT is generally more readily available for most eye care providers.
Patients may complain of the following symptoms in cases of NVG: redness, pain, photophobia, severe headaches, decreased vision/blindness, nausea and vomiting. Clinical signs of NVG typically include the following: variable visual acuity (20/20 to no light perception possible), NVI/NVA, peripheral anterior synechiae, elevated IOP, anterior chamber reaction, corneal edema, ectropion uveae, hyphema, glaucomatous optic nerve cupping and visual field loss.
Management
Treatment and management of cases of NVI/NVG center around two main principles: control the underlying retinal ischemia and control the IOP. Retinal ischemia control is generally performed under a retinal specialist and includes panretinal photocoagulation and anti-VEGF injections. Both of these treatments are aimed at reducing or eliminating the underlying retinal ischemia responsible for the underlying disease process at this level.
Usually secondary to controlling the retinal ischemia is controlling the elevated IOP. This can be done medically with topical medications (ie, beta-blockers, alpha-agonists, carbonic anhydrase inhibitors), but approximately 80% of cases fail and require some form of surgical intervention (Havens et al.). Surgical intervention typically results in trabeculectomy surgery and/or tube shunts in order to control IOP; however, there are still high failure rates with both of these procedures in controlling IOP long-term.
Currently, there are no randomized clinical trials to guide surgical decision-making in regard to trabeculectomies vs. tube shunts in NVG. Therefore, typically this decision is left up to the discretion of the glaucoma surgeon based on his/her surgical experience and expertise in general. Generally, minimally invasive glaucoma surgery (MIGS) is not a great option in most NVG cases because of the poor access to the trabecular meshwork due to the extensive peripheral anterior synechiae present in most cases.
The OD’s role
The optometrist’s role in NVG cases has not changed much in several decades. Optometrists should perform their usual astute clinical exam with emphasis on the presence of NVI/NVA in suspicious cases followed by careful gonioscopy to identify the sometimes subtle peripheral anterior synechiae, accurate IOP measurements and appropriate medical management of elevated IOP until the patient can get to the office of a retinal and/or glaucoma specialist. An important note, aside from the visually devastating effects of NVI/NVG as a whole, is the increased ischemic stroke risk in NVI/NVG patients compared to a normal individual. Therefore, another appropriate referral is efficiently guiding the patient back to his or her primary care physician for maximal control of their underlying systemic conditions.
Follow-up for this patient
In this patient’s case, the hyphema had completely resolved with topical medical treatment (prednisolone acetate 1% four times daily and atropine twice daily in the left eye) by the time the patient was able to see the retinal specialist approximately 1 week later. He declined further surgical intervention at that visit for the left eye given the unlikely chance of regaining useful vision in that eye. He did recommend cataract surgery in the patient’s right eye for potential, yet cautious, visual restoration and discussed it with the patient’s grandmother (power of attorney). Ultimately, the grandmother refused any further surgical management of the patient’s eyes, but did consent to medically managing the patient’s comfort levels with topical and/or oral medications that might be needed.
The patient was followed at monthly intervals for 3 months and is currently on a 3-month follow-up schedule monitoring closely for elevated IOP levels and worsening of her overall ocular condition. The patient continues to remain stable over a 9-month follow-up period at the time of this article. Thankfully, her IOP has never climbed above 21 mm Hg to 23 mm Hg at any of her follow-ups. We have simply maintained her current treatment of topical prednisolone acetate 1% once daily and atropine 1% once daily in the left eye as well for comfort per her power of attorney’s wishes. If there is any further worsening in this patient’s ocular status (ie, increased pain, IOP elevation, etc.), the patient will be promptly referred back to our local ophthalmology colleagues for further surgical intervention.
Another potential consideration worth discussing in this case is to rule out a clear traumatic cause, as hyphemas are most commonly associated with some form of ocular trauma. Given the patient is nonverbal, confined to a wheelchair and lives in a group home, physical abuse must be ruled out in all cases like this. As such, I spoke with the patient’s grandmother, the caretaker, as well as the group home’s on-staff nurse about the potential of abuse. All three denied vehemently the possibility of abuse or trauma. Clearly, after examining the patient and seeing the extensive NVI and retinal detachment on the B-scan, abuse seemed unlikely. However, eye care providers can never be too careful and should always rule out any form of abuse in odd or unexpected presentations of ocular diseases. Naturally, we will continue to monitor the patient for any odd presentations in the future that may suggest abuse as a possible cause.
In the end, NVI/NVG are typically signs of a poorly controlled ocular and/or systemic disease. This visually devastating disease needs to be caught as early as possible to have the best chance of salvaging patients’ vision. We need to continue to keep this disease in the back of our minds as we examine our patients and institute treatment and/or referral quickly to have the best chance of getting the best outcomes for these unfortunate patients.
- References:
- Aref AA. Curr Opin Ophthalmol. 2016;doi:10.1097/ICU.0000000000000239.
- Chalam KV, et al. JAMA Ophthalmol. 2014;doi: 10.1001/jamaophthalmol.2014.2356.
- Chen S, et al. Acta Ophthalmol. 2015;doi.org/10.1111/aos.12624.
- Duh EJ, et al. JAMA Ophthalmol. 2000;118:1296-1298.
- Havens SJ, et al. Dev Ophthalmol. 2016;doi:10.1159/000431196.
- Kolomeyer AM, et al. Retina. 2015;doi:10.1097/IAE.0000000000000235.
- Liao N, et al. BMC Ophthalmol. 2016;doi:10.1186/s12886-016-0190-8.
- Olmos LC, et al. Eye. 2016;doi:10.1038/eye.2015.259.
- Roberts PK, et al. Curr Eye Res. 2017;doi:10.1080/02713683.2017.1293113.
- Rodrigues GB, et al. Int J Retin Vitr. 2016;2:26.
- Su CW, et al. J Ophthalmol. 2017;doi.org/10.1155/2017/1851568.
- For more information:
- Chris Borgman, OD, is an assistant professor at the Southern College of Optometry in Memphis. He can be reached at: cborgman@sco.edu.
- Edited by Leo P. Semes, OD, FAAO, a Primary Care Optometry News Editorial Board Member and Professor Emeritus in the Department of Optometry and Vision Science at the University of Alabama at Birmingham. He can be reached at: leopsemes@gmail.com.