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The creation of recommendations to guide familial retinoblastoma in the U.S. is an important step bu highlights the challenges of guideline development in the absence of high-quality data 

This article was reviewed by Alison H. Skalet, MD, PhD


	
	When a group of members of the American Association of Ophthalmic Oncologists and Pathologists (AAOOP) was selected in 2015 to develop guidelines for screening children with retinoblastoma, their goal was to develop national guidelines and outline a simple, rational approach that emphasized the importance of genetic testing, according to Alison H. Skalet MD, PhD, ocular oncologist, and director, Retinoblastoma Service at Casey Eye Institute, Oregon Health and Science University, Portland, OR.
	
	Those selected for the consensus panel were from leading centers across North America.
	
	An initial survey of the expert panelists’ screening strategies demonstrated differences in approach, including a lack of agreement about exam frequency and the length of necessary screenings related to retinoblastoma, Dr. Skalet said.
	
	


	
	The results were presented to the AAOOP membership at the annual meeting in 2015 and were followed by an open discussion. Members who were present agreed that the creation of guidelines was a worthwhile endeavor, that screening based upon pretest risk stratification was reasonable, and that genetic testing was important for clarifying risk.
	
	That legwork helped lead to the team publication of nine recommendations in Ophthalmology last year.

 Among the recommendations are the following, according to Dr. Skalet.


	
	Serial dilated eye exams by an ophthalmologist are recommended for children with higher than population risk for retinoblastoma risk due to family history. All children with a family history of retinoblastoma benefit from genetic counseling and testing to clarify their risk.
	
	Children are stratified into high, intermediate, and low-risk categories. Frequency of recommended examinations varies based upon a child’s age and level of risk, decreasing in frequency for all children as they grow older.
	
	The recommendations went through rigorous peer-review and received endorsement from the American Academy of Ophthalmology (AAO), American Academy of Pediatrics, American Association for Pediatric Ophthalmology and Strabismus, and the American Society of Pediatric Hematology/Oncology.
	
	

It takes a village: Vision screening success through collaboration


	
	Yet since publication of the guidelines, there’s been what Dr. Skalet called an “ongoing spirited discussion” about them.
	
	“In particular, it’s been questioned if it’s advisable to differentiate how we are screening children and what method we are using. It has also been questioned whether it is necessary to screen children beyond four or five years,” she said.
	
	Dr. Skalet acknowledged the importance of continued review of the guidelines and pointed out that there was very little level 1 and 2 evidence from which to create the guidelines. Most often, those developing the guidelines had to use expert opinions and noncontrolled studies.
	
	She emphasized that the development of higher-quality data would be beneficial. Dr. Skalet commented that a survey of AAOOP members is planned to gauge awareness of the retinoblastoma screening guidelines and to help define areas of agreement and disagreement. The group also is investigating the use of the AAO’s IRIS Registry for “real-world” data regarding retinoblastoma screening and outcomes in the United States.
	
	“Publication of the U.S. guidelines has been a helpful step in improving care for children at risk for familial retinoblastoma in part because it has stimulated discussion of this important issue among experts not only in the U.S. but around the world,” Dr. Skalet said. “We agree on more than we disagree, and the attention is stimulating research efforts that will allow us to improve upon our current recommendations in the future.”
	
	

1. Skalet AH, Gombos DS, Gallie BL, et al. Screening children at risk for retinoblastoma: Consensus report from the American Association of Ophthalmic Oncologists and Pathologists. 

y. 2018;125:453-458.
	2. Skalet AH. Familial Retinoblastoma Screening: When Eye Cancer Runs in the Family. https://wechope.org/ medical-care/familial-retinoblastoma-screeningapproaches-when-eye-cancer-runs-in-the-family/
	Published April 15, 2019.

Articles

Retinoblastoma screening rules set foundation for care 

Accurate IOL measurements, proper patient selection, and solid surgical technique can help to reduce the number of IOL explantations.

SALT LAKE CITY-Some intraocular lens (IOL) explantations are inevitable for cataract surgeons. However, with good surgical technique, accurate IOL measurements, and proper patient selection and preoperative counseling, surgeons can lower their IOL explantation rate.

, professor of ophthalmology, codirector of the Intermountain Ocular Research Center, and director of ocular pathology, Moran Eye Center, University of Utah, Salt Lake City, reported in presentation of data from the 21st annual survey of 

The survey inquired about patients’ preoperative and postoperative visual acuity, any signs or symptoms and complaints, and complications that required IOL removal, exchange, or secondary intervention.

Surgeons were asked about explantation of the following IOL types: one-piece, plate-type; one-piece IOLs with haptics, three-piece IOLs, multifocal IOLs, and accommodating lenses.

The materials used in the lenses mentioned were silicone, acrylic (hydrophobic), hydrogel (hydrophilic acrylic), and collamer.

As in previous years, the lens had the highest rate of explantation was the one-piece hydrophobic (monofocal) lens. However, this is also the most common type of lens used in the U.S., which would explain why it is removed most frequently, Dr. Mamalis said. Other materials are not removed as often because they are used less often, he said.

Dislocation and decentration were the most common complications associated with one-piece silicone IOLs, followed by glare/optical aberrations and incorrect lens power. Dislocation and decentration also were the most common reasons for removal of three-piece silicone and one-piece acrylic lenses, Dr. Mamalis reported. This complication is usually linked to a surgical complication or weakness in the zonules or the capsular bag, he explained.

Other common reasons for lens removal among various lens types included incorrect lens power and glare/optical aberrations. Incorrect lens power still was a cause of IOL removal in the survey, but this has trended downward in recent years, leading Dr. Mamalis to say that surgeons are getting better at choosing the correct IOL power.

The number of multifocal IOLs that were explanted increased over the past year, and this was most frequently due to glare/optical aberrations. Proper patient selection could help reduce the number of multifocal IOL patients requiring lens removal, he said.

Calcification and opacification were the most common reasons that hydrophobic acrylic IOLs were explanted. The trends seen in the current survey have been consistent over the past five years, Dr. Mamalis said.

In addition to solid surgical technique and appropriate patient collection, surgeons also should aim for continuous curvilinear capsulorhexis with capsular bag fixation of the IOL, Dr. Mamalis added. Quality manufacturing and careful IOL folding and insertion also are key.

The ASCRS and ESCRS survey is an ongoing annual data collection; the survey related to explanted foldable IOLs is available on each society’s respective website, Dr. Mamalis said.

Dr. Mamalis is a consultant for Anew Optics and PerfectLens. He also does research for a variety of ophthalmic companies. This article was adapted from Dr. Mamalis’ presentation at the 2019 American Society of Cataract and Refractive Surgery annual meeting in San Diego.

Annual survey from ASCRS/ESCRS pinpoints trends in explantation

Dislocation and decentration remain common reasons for IOL removal

With planning and strategic use of surgical tips and techniques, physicians can lower the chance for vision loss in advanced glaucoma patients having cataract surgery.


	
	Setting expectations patients and physicians before cataract surgery in advanced glaucoma cases was one of eight pearls shared by 

 associate professor of ophthalmology and co-director of the glaucoma service, 

Department of Ophthalmology, UCSF, San Francisco.


	
	Dr. Ou focused her pearls on advanced glaucoma patients having cataract surgery. “All of us know many of these tips, but taken together, they can help minimize vision loss,” she said.
	
	Here are Dr. Ou’s eight pearls:
	
	

Part of this planning process is deciding whether to do a staged procedure with glaucoma surgery performed first or do a combined procedure.
	
	“Cataract surgery after a filtering procedure may pose a risk to the bleb, but combined surgery also may make some parts of surgery easier,” Dr. Ou said. One option is performing cataract surgery alone, while another pre-surgical plan involves IOL selection.
	
	“Most of us will choose a monofocal IOL, but it’s also worthwhile to consider a toric,” she said.

There is research that shows greater astigmatic correction and good visual outcomes with the use of toric IOLs in this patient group but be careful with this choice in patients who have pseudoexfoliation, Dr. Ou cautioned. Dr. Ou also noted that physicians should avoid multifocal IOLs in advanced glaucoma patients.
	
	


	
	Physicians also should consider consenting for cataract surgery with or without bleb revision. “Patients with well-controlled or low IOP are less likely to have an IOP drop,” Dr. Ou said.
	
	

3. Take steps prophylactically to avoid an IOP spike


	
	Patients with a longer axial length, prior laser trabeculoplasty, or who used a larger number of medications were more likely to have a spike in previous research, Dr. Ou reported. Oral acetazolamide, Miostat, and a topical beta blocker or a combination drop of beta blocker and carbonic anhydrase inhibitor all are options to help protect against IOP spikes.
	
	


	
	“Use all the methods at your disposal for a nice, dilated pupil,” Dr. Ou advised. Efforts to minimize inflammation could include visco dilation with Healon5 or several other iris expansion techniques. Thorough cortex removal also is important.
	
	


	
	Viscoelastic is necessary to maintain the anterior chamber depth throughout the procedure, especially in hyperopic eyes and those with prior filtering surgery. Complete removal can be a challenge, but take the extra time to do it, especially in high myopes, Dr. Ou said. Make sure to also remove viscoelastic material behind the IOL. A technique from 

, called angle sweep shows a way to help remove viscoelastic material and is available on the website 


	Avoid manipulation of the bleb and consider adding a 5-fluorouracil injection at the end of the case and during postoperative visits. To get a sense of bleb function intraoperatively, trypan blue can be used but is not always predictive, Dr. Ou said.
	
	


	
	Spikes in IOP can occur four to six hours after surgery, so consider same-day postoperative visits for at-risk patients or a close follow-up to monitor for IOP spikes.
	
	


	
	The use of ophthalmic steroids is especially key if the surgeon sees early indications of bleb failure, Dr. Ou explained. Ophthalmic steroids, usually eye drops, gels, or ointments, are made to be administered in or around the eye. They also treat inflammation and relieve symptoms that can include pain, swelling, irrigation and redness.

This article was adapted from Dr. Ou’s presentation at the American Glaucoma Society annual meeting. Dr. Ou is a consultant for Merck.

Pearls can ensure physicians get best outcomes, help patients set realistic goals

Setting expectations for glaucoma patients having cataract surgery

Patients with uveitic glaucoma have diverse presentations. Carefully planned cataract surgery can be successful.


	
	There is no longer any reason to dread cataract surgery in eyes with uveitic glaucoma, according to Mark A. Werner, MD, 

. Although there was a time when performing 

 in these patients was a frightening prospect, the prognosis has improved, Dr. Werner said.

“They can do quite well,” he said.
	
	Dr. Werner points to more and better options for steroid delivery and systemic immunosuppression, as well as better knowledge of the importance of aggressively managing inflammation in these patients. In addition to those factors, modern surgical techniques are less traumatic to the eye, he added.

“If patients have poor outcomes now, it is more likely due to posterior segment complications of the underlying uveitis,” Dr. Werner said.
	
	

Potential problems in uveitic glaucoma patients often are linked to their specific disease. For example, a patient with pars planitis is more likely to have 

. A patient with BehÃ§et’s disease may have even more significant posterior segment disease that could limit the visual prognosis.

“It is important to counsel patients on that,” Dr. Werner said. In patients with juvenile idiopathic arthritis, surgeons can improve outcomes with aggressive suppression of inflammation. Glaucoma and hypotony are both concerns, Dr. Werner added.

Anecdotally, Dr. Werner said he has noticed that patients with 

 may have decreased encapsulation of glaucoma drainage implants. “I would advocate for the use of a nonabsorbable suture and to secure your plate well if you are doing a tube shunt,” he said.

Bringing DALK application to forefront for corneal surgeons


	
	Surgeons should also consider how long a patient has gone without inflammation. Dr. Werner said a patient should experience three months with no inflammation before a good outcome is backed by evidence.

, six months may even be better,” he said. It also is preferable that the systemic disease is quiet. Postoperatively, focus on the control of inflammation first, and treat IOP secondarily.
	
	“For anterior intraocular lens opacity or membranes, you can use a YAG laser beam, turn off the retrofocus, avoid the central lens and the iris, and turn the laser energy down, once the eye is quiet,” Dr. Werner said.

Dr. Werner shared information on a patient he treated with rheumatoid arthritis that was reasonably controlled with low-potency steroids. The patient had visually significantly cataracts, posterior synechiae, and IOP that was not well controlled. When evaluating the patient’s optic nerve.

Dr. Werner saw that she had pre-perimetric glaucoma. He performed 

 along with Ahmed tube insertion, a 7-0 Vicryl suture ligation, and an orphan trabeculectomy.

) also was used. She has done quite well with four years of follow up, he said.

Technology assisting surgeons in capturing spectral-domain OCT

This article was adapted from Dr. Werner’s presentation at the American Glaucoma Society annual meeting. Dr. Werner is a speaker for Bausch + Lomb

Physicians also can focus on treating inflammation and IOP in patients

Author | Vanessa Caceres

Articles

Retinoblastoma screening rules set foundation for care

Dislocation and decentration remain common reasons for IOL removal

Setting expectations for glaucoma patients having cataract surgery

Cataract surgery in uveitic glaucoma eye can have good result