Lynda Charters

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The occurrence of headache in conjunction with visual loss is a scenario that requires an in-depth examination including evaluation of the visual fields, pupils, and fundus to gather clues to the diagnosis. Imaging is a must for patients who describe a “thunderclap” onset of headache, according to Kathleen Digre, MD, speaking during the virtual American Academy of Ophthalmology (AA0) 2020 annual meeting.

Dr. Digre, a Distinguished Professor of Neurology and Ophthalmology at the John A. Moran Eye Center at the University of Utah in Salt Lake City, advised that physicians SNOOP to uncover 

ostural involvement (worse when upright)/


A 79-year-old man presented with transient monocular blindness and new holocranial headaches. He had a bilateral visual acuity (VA) of 20/20 and no afferent pupillary defect, but visual field evaluation showed an enlarged blind spot in the right eye. 

His history was especially noteworthy for fatigue and recent weight loss, previous hypertension, and no previous headaches.

New headaches accompanied by visual loss in older patients always require further evaluation, Dr. Digre advised.

“Remember to snoop for clues in the patients’ history, including age, fatigue, weight loss, new-onset headache, although the headache type may not be helpful,” she said. Clues may be found in the VA, visual fields, pupils, and fundus. Fluorescein is a valuable tool.

She noted that amaurosis fugax is an important early visual symptom of giant cell arteritis and an ominous sign of impending blindness.

A pitfall is overlooking an underlying cause of the headaches even in the presence of good vision. In this patient, the erythrocyte sedimentation rate and the C reactive protein, respectively, were 60 and 10. The biopsy diagnosis was giant cell arteritis.


A 58-year-old woman who reported no previous headaches presented with the complaint of new dull continuous right-sided headaches. However, she described episodes of severe headache with very sharp pain in the right frontotemporal area that lasted 10 to 15 minutes and recurred 5 times daily in addition to tearing and painful eyes and blurry vision.

The examination was essentially normal with 20/20 vision, full visual fields to confrontation, slight ptosis in the right eye, no pupillary defect but anisocoria with the smaller pupil on the right, and a normal fundus.

The combination in this case of homonymous hemianopia, Horner’s syndrome, and cluster-like headache pointed to a pituitary lesion. The diagnosis was a pituitary adenoma.

Dr. Digre commented, “The phenotype can often be a new headache and can be trigeminal autonomic cephalgia, like cluster headaches. This can be accompanied by tearing and Horner’s syndrome.” 

In cases in which the patients report unilateral continuous headaches, she advised looking for a Horner’s syndrome. Imaging, especially magnetic resonance imaging (MRI), is paramount in cases in which the headache is side-locked to one side.


A 31-year-old pregnant woman with 2 children reported a sudden headache onset and a seizure accompanied by visual loss. Her blood pressure was 150/100; the patient was lethargic with briskly reactive pupils, and reported counting fingers vision. All extremities were able to move.

“In severe pre-eclampsia and eclampsia, the sites of the lesions that can affect vision include serous retinal artery detachment, hypertensive retinopathy, vasospasm, retinal artery occlusion, choroidal infarction, and anterior ischemic optic neuropathy. But don’t forget that it can also cause cerebral blindness,” Dr. Digre said.

This patient was diagnosed with Posterior reversible encephalopathy syndrome, which is characterized by sudden-onset headaches, visual loss, confusion, and seizure. 

This syndrome, which often is associated with pregnancy, can cause blindness. It can be overlooked during evaluation. One of the keys to the diagnosis was that the patient’s pupillary constriction was brisk and intact. 

Another closely linked condition that requires consideration is reversible cerebrovascular constriction syndrome. This is characterized by thunderclap-onset repeated headaches, which patients can describe as the worst headache. 

The segmental vasoconstriction can lead to stroke. Diagnosis of both of these conditions requires MR imaging and referral to a neurologist.

“Always ‘snoop’ if a patient presents with visual loss and headache, Dr. Digre concluded. “Call into play examination skills and tools in the evaluation of the visual fields, pupils, and fundus.

Patients with a side-locked headaches or those with a thunderclap onset should always undergo MRI unless the physician suspects an acute bleed.”


Dr. Digre has no financial interest in this subject matter.

Articles

During AAO 2020, Kathleeen B. Digre, MD, details how a headache in conjunction with visual loss is a scenario that requires an in-depth examination, including evaluation of the visual fields, pupils, and fundus to gather clues to the diagnosis.

AAO: A headache with visual loss may require honing of detective skills

Physicians at Kaiser Permanente have been using a prophylactic drop-free drug regimen before cataract surgery for more than a decade. 

Their regimen is so streamlined that the only drop that patients receive in anticipation of cataract surgery is a supplemental dilation drop instilled preoperatively while they are still in the holding area, according to Neal H. Shorstein, MD, who discussed the topic at the American Academy of Ophthalmology’s virtual 2020 annual meeting.

Dr. Shorstein is an ophthalmologist, researcher and associate chief of quality at Kaiser Permanente Medical Center in Walnut Creek, CA.

This more efficient approach seems highly beneficial for patients because drop instillation is associated with a few risks, i.e., prescription error, failure to pick up the prescription, failure to adhere to the instillation instructions, and stopping the drops prematurely. 

If only the correct instillation is considered, 93% of drop-naive patients have been reported to instill drops incorrectly. In addition, the bottle tip can become contaminated and it can cause microtrauma. 

The resistance of bacteria to topical antibiotics is another issue, such that instillation periods that are too short or too long or repeated over time can result in bacterial resistance. Finally, and not to be overlooked, the drugs can be expensive.


The regimen begins with an injection of LidoPhen 1%/1.5% (cyclopentolate/tropicamide, VNH Pharmaceuticals Pvt, Ltd.) at the beginning of the surgery.

The surgery ends with an injection of at least 0.5 ml of intracameral moxifloxacin 0.1%. 

However, 0.1 ml of cefuroxime 1% can be substituted in place of the moxifloxacin. Moxifloxacin also can be used for stromal hydration because recent data have indicated that the antibiotics remain in the wound for about 24 hours.

Finally, at least 4 mg of subconjunctival triamcinolone acetonide is injected at least 5 mm inferior to the inferior limbus. No perioperative topical nonsteroidal anti-inflammatory drug (NSAID) or steroid is used, Dr. Shorstein explained.


The use of moxifloxacin in the manner described for stromal hydration and in an intracameral injection has been associated with low endophthalmitis infection rates. 

This was supported by a meta-analysis that concluded that there was no supporting evidence that any postoperative use of topical antibiotics lowered the risk of endophthalmitis. 

Another study found no difference in the endophthalmitis rates with or without the use of short-term topical antibiotics in conjunction with a regimen of intracameral antibiotics, according to Dr. Shorstein.

A look at the studies that evaluated triamcinolone injections during cataract surgery showed that similar or better control of inflammation was achieved with subconjunctival injection compared with topical steroid instillation or with a steroid plus an NSAID in the 2017 PREMED Study. 

In the latter, the inflammation was well controlled in patients with diabetes who did not develop cystoid macular edema, but the intraocular pressure (IOP) was higher because of a higher subconjunctivally injected steroid dose, he pointed out.

That result leads to consideration of the importance of dose and injection site.

“Location is everything,” Dr. Shorstein said. “Injecting steroid closer to the limbus produces a higher concentration of the steroid in the aqueous and a higher mean IOP at 3 months compared with the same dose injected more posteriorly resulting in a lower aqueous concentration and a lower mean IOP.”

There is a tradeoff when injecting the drug more anteriorly. Pain and iritis are addressed but it carries the increased risk of higher IOP. Injecting more posteriorly should be more beneficial to the macula with less chance of IOP spikes ,but perhaps at the expense of enhanced pain and iritis control.

Initially, Dr. Shorstein reported, the drop-free regimen included the concentrated form of triamcinolone that resulted in some IOP spikes; with the same dose but injecting a greater volume (0.4 mL) of the dilute form of drug (10 mg/mL), fewer IOP spikes occurred and the physicians adhere to this practice.

“The most common problem associated with steroid injections is conjunctival hemorrhage that results from nicking a blood vessel during the injection,” he said. “This can be minimized by visualizing the needle tip and avoiding large vessels.”

Rarely, self-limited late hemorrhages months after the injections have been observed.

Drug reflux is another problem that can be avoided by tunneling the needle 2 to 4 mm under the conjunctiva and injecting slowly.

Dr. Shorstein also advises physicians to educate patients about the presence of the depot white spot and that it generally resolves in about 1 to 2 months and that, while IOP spikes occur infrequently, patients should have a checkup 1 month postoperatively.

Finally, when adhering to the dropless regimen, Dr. Shorstein noted the importance of instilling povidone-iodine 5% solution for 3 to 5 minutes, careful wound construction, leaving a relatively firm eye at the end of the surgery, and use of stromal hydration with an antibiotic.

Dr. Shorstein has no financial interest in this subject matter. The drugs mentioned are used off-label.

During AAO 2020, Neal H. Shorstein, MD, explains how physicians at Kaiser Permanente have a streamlined regimen using a prophylactic drop-free drug before cataract surgery for more than a decade. 

AAO: Drop-free prophylaxis regimen before cataract surgery lowers treatment burden with caveats

The first-line treatment for multiple sclerosis (MS), fingolimod 0.5 mg (Gilenya, Novartis), reduces both the number of relapses and disease progression in patients with relapsing-remitting MS and may reduce the incidence of uveitis, which is higher in this patient population compared with the general population. 

However, the downside is that the drug can cause fingolimod-associated macular edema (FAME), and patients require close observation.

The drug works by blocking movement of central memory T-cells from the lymph nodes to the blood stream and has been shown to have a favorable effect on murine models of experimental autoimmune uveitis, according to Bradley Smith, MD, and Scott Sonne, who discussed the topic during a presentation at the American Academy of Ophthalmology’s virtual 2020 annual meeting.

Dr. Smith is a physician partner at The Retina Institute in St. Louis and an instructor in Clinical Ophthalmology & Visual Sciences at the Washington University School of Medicine in St. Louis, MO. Sonne is from the St. Louis University School of Medicine in St. Louis.

The investigators conducted a retrospective analysis

 to determine the incidence of uveitis and macular edema in patients with MS who were treated with fingolimod 0.5 mg. The study patients were identified in the electronic health record at The Retina Institute.


A total of 188 patients were included in the study; they had been followed for from 1 to 104 months (mean, 60.9) after treatment with fingolimod.

The authors reported that no patient had or developed uveitis. Acute macular neuroretinopathy developed in 1 patient. Twenty-six (13.83%) patients had a history of optic neuritis and 7 (3.7%) had a recurrence during the study. Fingolimod was discontinued in 3 patients after 57, 43, and 13 months for nonophthalmic reasons.

FAME developed in 3 (1.6%) patients, none of whom had intraocular inflammation or another identifiable cause of the ME.

The authors also reported good visual acuity (VA) results in most patients, i.e., 20/40 or better in 93% of eyes; 5% had a VA ranging from 20/50 to 20/200, and 2% had a VA below 20/200.

“The VA in 2 eyes was worse than 20/400 as a result of optic neuritis. Additional causes of visual loss in the patients with worse than 20/100 were optic neuritis, ischemic optic neuropathy, ruptured globe, keratoconus, macular hole, and amblyopia,” they commented.

It is recognized that uveitis occurs more frequently in patients with MS and develops in from 3.6 to 9 years on average. By contrast, uveitis did not develop in this patient cohort after a mean follow-up exceeding 5 years.

According to the investigators, fingolimod was well tolerated.

“Our retrospective review suggests possible efficacy against the development of uveitis in an at-risk population that is consistent with fingolimod’s anti-inflammatory effect in animal models,” they said. 

However, the investigators concluded that more work will need to be done to confirm or deny this possibility.

“Although there was a higher incidence of FAME, all patients were treated successfully with topical therapy,” they said.


Dr. Smith has no financial interest in this subject matter.

1. Sonne SJ, Smith BT. Incidence of uveitis and macular edema among patients taking fingolimod 0.5 mg for multiple sclerosis. 

 2020 Sep 21;10(1):24; https://doi.org/10.1186/s12348-020-00215-1

Bradley Smith, MD, presents at AAO 2020 on how fingolimod 0.5 mg, a first-line treatment for multiple sclerosis, may reduce both the number of relapses and disease progression in patients with relapsing-remitting MS as well as the incidence of uveitis.

AAO: Fingolimod for MS offers disease control with no development of uveitis

Autologous retinal transplant (ART) for macular holes, a relatively new procedure, is providing anatomic hole closure in the vast majority of cases of large macular holes as well as increases in visual acuity (VA). 

In addition to the macular hole closure, the keys to better final outcomes were ellipsoidal band reconstitution and alignment of the neurosensory layers.

In this procedure, which was developed by Tamer Mahmoud, MD, PhD, an autologous neurosensory retinal free flap is harvested and moved over the hole to cover the hole gap and connect to surrounding macular tissue for large holes in which the mean minimal linear diameter exceeded 800 μm in the study.

, a professor of ophthalmology at Oakland University William Beaumont School of Medicine, and Associated Retinal Consultants in Royal Oak, MI

, presented the information at the American Academy of Ophthalmology’s virtual 2020 annual meeting on behalf of the ART Team Study. 


In a multicenter, international, case series in which 33 surgeons participated, 130 eyes of 130 patients (women, 58%; mean age, 63±6.3 years) underwent ART to treat their macular holes. All patients underwent a pars plana vitrectomy and ART during the study from January 2017 through December 2019. 

Dr. Mahmoud and his colleagues sought to determine the anatomic and functional outcomes of ART for the surgical repair of primary and refractory macular holes and macular hole retinal detachment (MHRD). This study is in press in 

Of the 130 patients, 35 (27%) had primary macular holes and 76 (58%) had refractory macular holes. Among the patients, 19 (15%) also had a MHRD, that was recurrent in 13 cases, and 12 of the 19 patients had a recurrent MHRD and had undergone previous internal limiting membrane (ILM) peeling. The mean number of surgeries that the patients had undergone previously was 1.6±0.2 (ILM peeling and flaps).

Dr. Mahmoud recounted that the patients’ mean preoperative axial length was 24.6 ± 3.2 mm, the mean maximal hole diameter 1,470 ± 160 μm, and the mean minimal hole diameter 840 ± 94 μm. The preoperative logarithm of the minimum angle of resolution best-corrected VA (logMAR BCVA) was 1.37 ± 0.12 (~20/500).

The graft type was a 90% neurosensory retina without the choroid that was harvested in 45% of cases superiorly, 17% inferonasally, 11% superotemporally, 8% each superonasally, and inferiorly, 7% temporally, and 4% inferotemporally. 

In 84% of cases, the harvest site was posterior to the equator and in 16% anterior to the equator. Most grafts (70%) ranged in size from 0 to 1 disc diameter (DD), 29% from 1 to 2 DD, and 1% 2 to 3 DD.

When the grafts were positioned in the eyes, 81% were placed preretinally and the remainder subretinally. The mean follow-up time was 8.6 ± 0.8 months.

The reconstitution of the external limiting membrane and the ellipsoidal zone is important to the success of the procedure.

“In this study, we introduced the term “ANL” or alignment of the neurosensory layers,” Dr. Mahmoud said. “When the graft is first moved to the hole area, vertical lines are seen on optical coherence tomography [OCT] between the graft and surrounding macular tissue.”

Dr. Mahmoud noted that within the first few weeks, those lines gradually fade and details of graft layers can be detected and those align with similar layers in the surrounding host macular tissue, plexiform to plexiform, nuclear to nuclear, etc.

“We feel this is remarkable as the macular tissue recognizes the retinal tissue and may try to connect to respective layers creating that integration of the retinal transplant, which reflects on better visual outcomes,” he said.

The final postoperative logMAR BCVA improved significantly (p<0.001) to 1.05 ± 0.09 (~20/225), with 29% of patients gaining more than 5 lines of vision and 43% gaining 3 lines of vision.


Fifteen (12%) patients had a final VA of 20/50 or better; among these, a third had a primary macular hole and two-thirds a refractory macular hole. 

The mean preoperative BCVA in this subgroup was 0.848 ± 0.219 (~20/125) and the mean final BCVA was 0.308±0.079 (~20/40), a difference that reached significance (p<0.001). 

Two-thirds gained more than 3 lines and 40% more than 5 lines; all of these holes closed. In addition, two-thirds had a complete ellipsoidal zone.

According to Dr. Mahmoud, the global study reflects the initial experience worldwide, with most surgeons sharing their initial experiences.

“With better understanding of prognostic factors and refinement of surgical techniques developed throughout this collaboration, anatomic closure can be achieved in most large macular holes, which would reflect on better visual outcomes,” he said. “This also paves the way to further research the role of peripheral retina into acquiring macular function and its potential in many macular diseases.”

The postoperative complications included 5 cases of retinal detachment, 5 cases of ART graft dislocation, and 2 cases of subretinal perfluoro-N-octane. Endophthalmitis was suspected in 1 case. There were 5 cases of ART graft dislocation (3.9%).

Dr. Mahmoud enumerated the main study points, including that the preoperative diagnosis (p = 0.026) and preoperative VA were associated significantly (p < 0.001) with the postoperative VA, and that the macular hole closure (p < 0.001), reconstitution of the ellipsoid zone band (p=0.02), and alignment of neurosensory layers (p = 0.01) on OCT were associated with better final VA.

The results showed a high rate of closure (89%) of the macular holes and 95% in MHRD. The gains in VA were substantial, in that 29% of cases had at least a 5-line gain in visual acuity and 43% had at least a 3-line gain in visual acuity.

“The higher final levels of VA were associated significantly with macular hole closure (p < 0.001), reconstitution of the ellipsoid zone band (p = 0.02), and alignment of the neurosensory layers (p = 0.01) on OCT images,” Dr. Mahmoud concluded.

Dr. Mahmoud has no financial interest in this subject matter.


1. Moysidis SN, Koulisis N, Adrean SD, et al. Autologous retinal transplantation for primary and refractory macular holes, and macular hole retinal detachments: The Global Consortium. 

 2020; Published online: October 9, 2020.

Tamer Mahmoud, MD, PhD, discusses autologous retinal transplant (ART) for macular holes, a procedure he developed, which is providing anatomic hole closure in the vast majority of cases as well as increases in visual acuity. 

Author | Lynda Charters

Articles

AAO: Drop-free prophylaxis regimen before cataract surgery lowers treatment burden with caveats

AAO: Fingolimod for MS offers disease control with no development of uveitis

AAO: Autologous retinal transplant for larger macular holes gaining traction

AAO: A headache with visual loss may require honing of detective skills