During Episode 142, Jay was joined by Dr. Ed Ryan of Minneapolis, MN to discuss the design of devices to improve surgical techniques, advances in vitrectomy, and scleral buckling. Central to this discussion was the idea that while scleral buckling may be falling out of favor, it is a technique that must not be forgotten and should be considered as the primary intervention in certain cases. Today’s blog post is from a new name on our team: Amy Kloosterboer, a third-year medical student at the University of Miami’s Miller School of Medicine. Today, we present the history of scleral buckling and some of the basic principles behind this surgery.  

Retinal detachment occurs when the retina separates from the underlying retinal pigment epithelium (RPE) and choroid. This can occur actively, as seen with diabetic traction retinal detachment, or passively due to accumulation of fluid between the two layers. The inner layers depend on the outer layers for delivery of nutrients and oxygen, and so this detachment can lead to damage to and even death of the cells in the neurosensory retinal layer. 

Image Credit:

(Left) http://www.shreeramkrishnanetralaya.com/retinal_detachment.html

(Top Right) https://www.lehp.org.au/Training%20Course/pages/conditions.html

(Bottom Right) http://www.fairvieweyecenter.com/Education/Conditions/RetinalDetachment/tabid/3103/Default.aspx

One of the options to treat retinal detachment is a scleral buckle. The principle behind this surgery is to collapse the space created between the detached retinal layer and its supporting layers. This is done by applying a band-like device onto the exterior of the globe, which causes inward indentation of the sclera to create a ridge (or buckle) that functionally closes the breaks. This allows the separated layers to come together once more.

Image Credit: https://www.texomaretina.com/services-2/common-treatments/scleral-buckle/

The first successful treatment of retinal detachment occurred in 1920 by Swiss ophthalmologist, Dr. Jules Gonin. His technique involved closure of the break using trans-scleral cauterization. Since the patient subsequently recovered vision, this showed that retinal breaks were the main cause of retinal detachments, and that successful treatment involved sealing those breaks. Many different techniques were developed after 1920, but it was not until 1949 that the first scleral buckle surgery was reported. Dr. Erns Custodis, a German ophthalmologist and professor, performed the first scleral buckling procedure that included a retained exoplant. He sutured a polyviol material to the sclera, and effectively indented the eye wall at the area of the break to close the gap between the retinal layer and the RPE. In 1956, Dr. Charles L. Schepens, inventor of the binocular indirect ophthalmoscope, modified this technique to use an encircling polyethylene tube. With the help of the indirect ophthalmoscope, Dr. Schepens was able to localize the retinal break and correctly position this tube to seal the break between the layers. Today, there are a variety of materials can be used as a buckle, and the most common is silicone as it is nontoxic, nonallergenic, and can be molded as needed by the surgeon. This last property allows for great variability between operations, giving the surgeon the flexibility to adapt the technique to the patient and to choose between a localized scleral buckle to an encircling circumferential buckle. 

-Amy Kloosterboer

Last week, we began a new blog feature with one of our medical students offers his or her perspective on the current week’s episode. For this week’s podcast episode, we were joined by Dr. Audina Berrocal for a discussion on a number of topics in pediatric retinal surgery. Of these, one in particular that has received special attention in the media and in ophthalmology communities has been the use of gene therapy to treat children with hereditary retinal diseases such as Leber congenital amaurosis (LCA). The most common hereditary retinal disease is retinitis pigmentosa (RP) and there are current trials ongoing in the United States looking at gene therapy for RP. For our listeners and readers who may be patients, parents, or simply curious, we wanted to provide a basic overview of RP and how this treatment can be used to save some patients’ sight.

Retinitis Pigmentosa: Background

RP is a clinically and genetically heterogenous group of inherited (can be passed down from parent to child) retinal disorders, meaning that there is a good amount of variability between patients with respect to how they inherited the disease and how the disease affects their vision. In general, however, RP involves a progressive (worsening) retinal dysfunction, usually starting with the rod photoreceptors (the “night-vision” part of the retina) and later affecting the cones (for “day-vision”) and retinal pigment epithelium. Thus, patients with RP generally first experience night blindness (difficulty seeing in low-light settings), followed by progressive visual field loss (a sort of “tunnel vision” as shown below).

Image Credit: https://www.mesvision.com

Retinitis Pigmentosa: Genetics

There are no known risk factors for RP other than genetic predisposition. So what does this mean? We mentioned earlier that RP is “genetically heterogeneous” - indeed, RP can be inherited as an isolated sporadic disorder, or in an autosomal dominant, autosomal recessive, or X-linked pattern.

• Isolated Sporadic: a genetic mutation can be passed on, but in order for one to be present and available to be passed on, the mutation has to occur for the first timein some individual of a family. This is called “sporadic” since the mutation occurs, essentially, randomly.

• Autosomal Dominant: for most of your DNA, there exists a second “backup copy” for each gene (one from Mom, one from Dad). For autosomal dominant inheritance patterns, if one of the two copies is “bad,” the disease can occur. In general, the purpose of each gene is to make a certain protein that your body needs to function. Think of this as a two-person team at work; if one of your team-members refuses to do his/her work, you may not meet that deadline. In the same way, if one member of the two-protein team (two copies: one from Mom, one from Dad) isn’t cooperating, a disease process can occur.

• Autosomal Recessive: in this case, both copies need to be “bad” for the disease state to occur. In other words, one good copy is sufficient to prevent disease. Think of this as being like transportation to work: if your car is broken down but your spouse’s car is running, you can borrow the car and still make it to work on time. For autosomal recessive diseases, if one copy of the gene is “bad” and the other is “normal,” the patient will not have the disease (see image below).

• X-Linked: for these diseases, the important gene is located on a particular chromosome called the X-chromosome. For baby boys, they only receive an X-chromosome from their mother. For baby girls, they receive an X-chromosome from both their mother and their father. Since the boy would only have one X-chromosome, he wouldn’t have a “backup” copy in case the copy he received is “bad,” and so he would be more likely to be affected.

Image Credit: https://ghr.nlm.nih.gov/primer/inheritance/inheritancepatterns

Retinitis Pigmentosa and Gene Therapy

Now that we’ve finished our genetics lesson for the day, how does this relate to “gene therapy” treatments for RP? In the episode, Jay and Dr. Berrocal discuss use of RPE65 therapy for some children with LCA. Since this is an autosomal recessive disease, that means that both copies of the gene are “bad” and the so both copies of the protein that they are (supposed to be) making are “bad.”

Gene therapy is a way to allow the patient to create the “good” protein. A special virus (which carries a “good copy” of the RPE65 gene) is used to insert the “good” gene into cells of the patient’s retina. After that, the cells are are able to make the “good” protein, which helps preserve or improve vision!

Image Credit: http://www.retina-specialist.com/article/gene-therapy-the-new-frontier-for-inherited-retinal-disease

Image Credit: https://www.semanticscholar.org/paper/The-gene-therapy-revolution-in-ophthalmology.-Al-Saikhan/065a764404bda4440485b3f07a3109cffdd75e2e/figure/1

Looking To The Future

Although Jay and Dr. Berrocal only talked about one specific gene that is being targeted with gene therapy, there are others that are being targeted in current clinical trials (e.g. Dr. Berrocal’s patient with X-linked RP who received gene therapy at Bascom Palmer) or will be in the future. Gene therapy is a bright light for the future, and hopefully will improve the lives of countless patients with a number of ophthalmologic and non-ophthalmologic conditions.

-Michael Venincasa

#genetherapy #retinitispigmentosa #leberscongenitalamaurosis #rpe65 #ophthalmology

Dr. Joseph I. Maguire, a vitreoretinal surgeon at Mid-Atlantic Retina and Wills Eye Hospital and one of my fellowship attendings and role models, passed away over the Thanksgiving weekend after a long battle with cancer. I wanted to write this post not only to honor a man who meant so much to many, but also to pay tribute to a few of the lessons he taught me and countless fellows and residents over the years at Wills (as an aside, the name, Straight from the Cutter’s Mouth, was actually inspired by Dr. Maguire’s love for idioms):

1) Take time: In his own practice and in his teachings, Dr. Maguire emphasized the importance of taking the necessary time to sit down with patients and explain. Explain what is going on, why it is happening, and what the goals of therapy are. I remember once presenting to him a patient with a diabetic tractional retinal detachment from the fellow clinic. Dr. Maguire, despite being very busy in his own private clinic, took the time to sit down with this patient to go over her blood sugar and insulin regimen, emphasizing the impact that her systemic disease was having not just on her vision, but also her life as a whole. That experience has always stuck with me and since then, I always try to make enough time for my initial encounters with patients suffering from significant diabetic eye disease. 

2) Be a doctor, not just an eye specialist: Dr. Maguire had almost an encyclopedic knowledge of systemic diseases with retinal findings, whether it was Purtscher-like retinopathy or crystalline retinopathy. It reflected in his day to day patient care; he took thorough, comprehensive medical histories that put me, as the fellow working in his clinic, to shame on more than one occasion. As he liked to say to me, ‘the ‘MD’ stands for ‘medical doctor’ for a reason’.

3) Movement equals error: One of Dr. Maguire’s favorite expressions in the operating room was ‘movement equals error’. It was his caution for the excited, rapidly improving surgical fellow that being an efficient and skilled surgeon is not about how fast you move while operating, but about preparing and planning in advance, understanding surgical principles, and avoiding wasted movement. There were surgical specifics that I personally learned first from him, like how to imbricate sutures on a scleral buckle or the concept of ‘oar-locking’ instruments in vitrectomy cannula, but the concept that moving faster is not better will stick with all of his former fellows.  

4) Give feedback: Dr. Maguire gave direct, honest, and constructive feedback frequently to me and I appreciated every bit of it. Early on in fellowship I remember doing (what I thought was) a thorough retinal examination on a patient with new floaters on call and finding no issues. Dr. Maguire saw the patient two days later in follow-up and found a retinal tear. He picked up the phone and called me, not to berate me or scold me, but to simply tell me what he had found and where so I could learn. Giving feedback seems like a simple thing to do, but for many of us it can be difficult to tell someone to improve in a compassionate enough way to avoid hurt feelings. He also would give positive feedback unsolicited. When he once called me after a long day in the OR together, I assumed it would be about a patient-related medication prescription or paperwork that I had forgotten to fill out. Instead, he simply told me that I had done a great job and that he was very proud of me. We cannot forget to let our trainees and colleagues know when they are doing well.

5) Pick up the phone: The examples in #4 above were classic Dr. Maguire because he was ‘old school,’ and he picked up the phone and called you when he needed to talk. We live in the digital age of text messages, Instagram DMs, and retweets, but so much can be misconstrued when sent in a few words without any sense of inflection or context. If a conversation is important, pick up the phone. Two minutes of talking can get a lot more across than fifteen minutes of back and forth cryptic emojis. 🤨

6) Be loyal to your team: No one would stand up for his fellows, residents, and staff more than Dr. Maguire. No matter what his schedule commitments were like, he always came to fellow presentations and conferences. He was generous to all those around him; in fact, the last time I saw him in person he quietly picked up the check for fifteen former and current fellows out for lunch after a reunion in Philadelphia. I also remember once there was a patient being extremely rude to one of the front desk staff. Before anyone else could intervene, Dr. Maguire arrived at the scene and quietly but concisely reminded the patient his responsibility as a patient of the practice to be as respectful to the staff as he would be to any of the doctors. Being loyal to the people around you is not only the right thing to do, but it inspires loyalty back that will build priceless relationships and an A+ working environment. 

7) Be honest: I remember as a fellow writing a research paper with several attending surgeons including Dr. Maguire as a co-author. When I emailed a draft to him, he called me (see point #5 above!) and asked to not be listed as an author, not because he was not keen in supporting me, but because he felt that he had not contributed enough to merit a spot on the authorship docket. While I explained and eventually convinced him that the research would have been impossible without his help, I was always struck about how principled he was about academic honesty that his initial instinct was to call and ask not to be included. Let’s all be honest with ourselves, because in the end it is more important we respect the person we see in the mirror every morning than to have a couple extra lines on a CV. 

8) Be a good person: Dr. Maguire was an exceptional doctor and surgeon, but more than that, he was one of the best people I have ever met. The first word that comes to mind when his colleagues and fellows think of him is ‘gentleman.’ He was respectful and kind to everyone,without any ulterior motives. It was simply the way he was built. 

I will miss him tremendously and I know I am not alone among my friends and family from Wills. I feel for his family, and I hope that they can take solace that Dr. Maguire was regarded by all who worked with him as an amazing husband, father, doctor, surgeon, mentor, role model, and friend. RIP Dr. M. We all love you.

In the true spirit of learning lessons from our pupils starting this week we are starting a new blog feature with one of our stellar medical students offering their perspective on the week’s episode

-JS

In this week’s episode, Jay was joined by Drs. Daniel Chao and M. Ali Khan for another journal club installment (Link) – this time, the topics of discussion were outcomes of K grant awardees in ophthalmology; medical student involvement with ophthalmology and professional ophthalmology organizations; and the accuracy of online, freely-available medical information for patients. 

As a fourth-year medical student currently applying for an ophthalmology residency, the middle segment of this episode resonated with me. Fortunately, ophthalmology was one of my first exposures to the medical field, as a high school student shadowing to explore the option of medical school. Although I did not understand everything (or, rather, almost anything) I saw during my encounters, those experiences placed ophthalmology on my mind early on. At the University of Miami, our medical school is fortunate enough to be affiliated with the Bascom Palmer Eye Institute. As a result, we have a 1-week ophthalmology course built into our pre-clinical curriculum, and there are a number of ophthalmology electives for those interested in exploring the field.

However, as discussed in this week’s episode, many students have a difficult time “finding” ophthalmology when the field is not part of the required, pre-clinical curriculum, or when elective options are limited or even absent. While on residency interviews this year, I have met a few students who had no ophthalmology department at their institution, and thus had to resort to external sources for shadowing and mentorship purposes. Although this can certainly be done for those who are motivated, it does limit the students’ initial exposure to the field. To me, the simplest start for schools is to add a lecture of introductory ophthalmology (we had one such lecture during our Neurology block), complete with information relevant for the non-ophthalmologist and the ophthalmologist alike. Jay, Louie, and I discussed this in a previous blog post (Link), but when it comes to clinical electives, my most educational moments were those where a teaching scope was available and the clinician talked through their thought process, which allowed me to build some pattern recognition skills. Regardless of the specifics, early exposure would help medical students to place ophthalmology on their radar for the future.

This episode also discussed the role medical students play in the various professional ophthalmology associations, including whether or not students can be members, can serve on the board, or receive discounted registration fees. I recently attended my first ophthalmology meeting, the American Academy of Ophthalmology’s 2018 Annual Meeting, to present the results of our one-year anniversary survey from last year. As a medical student, I greatly enjoyed my time there. I was immediately struck by how busy the venue was, with healthcare professionals abound, pharmaceutical advertisements lining the entrance’s large staircase and hanging banners, and security at every door. Lectures were plentiful, and I was able to tailor my attendance toward those that would offer the most benefit for my level and my interests. I am not a committee member for any of these organizations and so cannot comment on the value of that specific experience, but I would certainly advocate for financial support for medical students to attending meetings. Although ophthalmology is a relatively small medical community, attendance allows one to appreciate its worldwide presence and to get excited about advancing this presence in the years to come.

-Michael Venincasa

During this year’s American Academy of Ophthalmology Annual Meeting, Dr. Jay Sridhar worked with Dr. Usiwoma “MentorMeMD” Abugo, Dr. Steven “EyeSteve” Christiansen, Dr. Rahul Khurana, and Dr. Matthew Weed to deliver a course entitled “Online Content Creation for Ophthalmology Education and Marketing.” Throughout this talk, each panelist discussed their take on the use of social media in the medical field, including tips and tricks to maximize benefit and avoid pitfalls. Today, we wanted to discuss a few of the most salient points from the course, so that you may also enjoy the world of social media:

1.    Social media presence is an important consideration for all medical professionals.

I always thought of social media as a dichotomy between two realms: (1) personal use to keep in touch with family, friends, and colleagues, and (2) professional use for business advertisement and “promoting” to generate advertising revenue. I participate in both of these realms to some degree, with a personal presence to interact with old friends and fellow classmates and a professional presence posting updates for Straight from the Cutter’s Mouth. However, I never thought of myself as a “promoter” and I never considered how both realms apply to all individuals, even those not involved with a medical podcast.

During the course, the panelists discussed the importance of social media for all medical providers. One does not need to be the producer of a podcast or a medical blog for this to be true. Instead, the simple creation of a Google+ profile (and updating your current location of practice on this profile) allows your patients to find your correct contact information when they search for your name. Setting up a Google Alert for your name notifies you when a new post is made about you online, and allows you to respond as needed. Finally, your current place of employment may have a large online presence, but growing your individual presence (if not contractually prohibited) can benefit you if you move to another practice or university.

2.    Private and professional social media accounts should remain separate. 

Although a “personal” touch can add a lot to your professional postings, one’s private and professional lives should generally remain separate on social media. Your patients certainly may enjoy seeing pictures from your recent vacation and your family may look forward to posts about medical topics, but separation of these realms allows one to be selective about what is shared to either group. Along those lines…

3.    When sharing a post, you can be selective about whose feeds it will reach.

For example, Facebook allows one to create “Friend Lists” – subgroups from your overall friends list – that make it simple to select who will see your post. Prior to publishing a post it can be helpful to check its privacy settings, choosing whether to share this to your entire friends list or only to one of your “Lists”. Dr. Weed uses this tactic on his personal Facebook; although he enjoys sharing interesting medical findings, papers, or current events, he feels that these may not appeal to all of his high school friends and so chooses to limit these to a “List” containing only his ophthalmology colleagues.

4.    The best social media post takes into account its audience.

A social media post should always consider its intended audience. Some content creators choose to cater their posts to patients only, others to medical professionals, and others to a mixture of the two. Although it may seem difficult to create a post that is interesting to a medical professional and the general public alike, this can help to broaden the reach of your online presence. By combining correct medical terminology (which allows the professional to think “this person knows what he/she is talking about”) with simple explanations (which exposes the patient to terms he/she may hear in the clinic or online), a post can benefit both groups. Since audience attention span may be limited, posts should be short and should include multimedia whenever possible. Finally, Dr. Weed’s recommendation was to keep an “eye” on the media: whenever a topic arises in popular culture (such as the “what color dress is this?” viral post) that can apply to ophthalmology, this is a perfect topic for a post!

5.    Finally, always remain cognizant of HIPAA and the legal aspects of what you post.

Fortunately, ophthalmology is a field where the part we care about the most does not require an identifiable image, with a full view of a patient’s face. For Dr. Abugo, social media is important because many of her patients desire “before & after” images to see a sample of her work. Even in these cases where the image is not identifiable, Dr. Christiansen suggests making a habit of obtaining patient consent before posting. When he sees a medical post on social media, he feels much more comfortable when he sees “posted with patient permission” within the description, as this demonstrates respect for the patient. Even better, he enjoys posts made by patients, such as post-op photos with the physician that thank him/her for a job well-done. When a patient chooses to post something on their own social media accounts, not only are you reaching your followers who already know and like you but, perhaps more importantly, you are reaching his/her family and friends who are unknown of yet to you.

- Michael Venincasa

Dr. Usiwoma “MentorMeMD” Abugo: http://www.mentormemd.com

Dr. Steven “EyeSteve” Christiansen: http://www.eyesteve.com

Dr. Rahul Khurana: https://www.aao.org/clinical-education

Dr. Matthew Weed: http://www.mattweedmd.com