Surgical Case Discussions from Thought Leaders

Supplement to July/August 2016

Clinical Case Compendium Surgical Case Discussions From Thought Leaders

Supported via advertising by Alcon and Optos LESS IS MORE WHEN IT COMES TO RETINAL TRACTION. SUPERIOR DRIVE TECHNOLOGY IMPROVED PATIENT OUTCOMES.†, 1

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† A clinical study of 120 patients underwent a 3-port pars plana vitrectomy. Main outcome measures were vitrectomy time, induction of posterior vitreous detachment, and intra- and postoperative complications. 1. Rizzo S, Genovesi-Ebert F, Belting C. Comparative study between a standard 25-gauge vitrectomy system and a new ultrahigh-speed 25-gauge system with duty cycle control in the treatment of various vitreoretinal diseases. Retina. 2011;31(10):2007-2013.

MIVS IMPORTANT PRODUCT INFORMATION intravenous connections. Each surgical equipment/component combination may require specific surgical setting adjustments. Ensure that appropriate system settings are used with each product combination. Prior to initial use, contact your Alcon CAUTION: Federal law restricts this device to sale by, or on the order of, a physician. INDICATIONS FOR USE: The CONSTELLATION® Vision System is an ophthalmic microsurgical system that is indicated for both anterior segment (i.e., sales representative for in-service information. Care should be taken when inserting sharp instruments through the valve of the Valved Trocar Cannula. Cutting instrument such as vitreous cutters should not be actuated during insertion or removal phacoemulsification and removal of cataracts) and posterior segment (i.e., vitreoretinal) ophthalmic surgery. The ULTRAVIT® Vitrectomy Probe is indicated for vitreous cutting and aspiration, membrane cutting and aspiration, dissection of tissue to avoid cutting the valve membrane. Use the Valved Cannula Vent to vent fluids or gases as needed during injection of viscous oils or heavy liquids. Visually confirm that adequate air and liquid infusion flow occurs prior to attachment of infusion and lens removal. The valved entry system is indicated for scleral incision, canulae for posterior instrument access and venting of valved cannulae. The infusion cannula is indicated for posterior segment infusion of liquid or gas. WARNINGS AND cannula to the eye. Ensure proper placement of trocar cannulas to prevent sub-retinal infusion. Leaking sclerotomies may lead to post operative hypotony. Vitreous traction has been known to create retinal tears and retinal detachments. Minimize PRECAUTIONS: The infusion cannula is contraindicated for use of oil infusion. Attach only Alcon supplied products to console and cassette luer fittings. Improper usage or assembly could result in a potentially hazardous condition for the patient. light intensity and duration of exposure to the retina to reduce the risk of retinal photic injury. ATTENTION: Please refer to the CONSTELLATION® Vision System Operators Manual for a complete listing of indications, warnings and precautions. Mismatch of surgical components and use of settings not specifically adjusted for a particular combination of surgical components may affect system performance and create a patient hazard. Do not connect surgical components to the patient’s

94732_VIT14177JAD-A.indd 1 7/19/16 12:40 PM LESS IS MORE WHEN IT COMES TO RETINAL TRACTION. SUPERIOR DRIVE TECHNOLOGY IMPROVED PATIENT OUTCOMES.†, 1

In a clinical study, ULTRAVIT® Probes reduced the occurrences of iatrogenic tears by 20% compared to spring-driven probes.†,1

94732_VIT14177JAD-A.indd 1 7/19/16 12:40 PM Surgical Case Discussions From Thought Leaders

This clinical case compendium, written by well-known retina specialists selected by Retina Today, features surgeries related to the posterior segment.

CONTENTS

Intraoperative OCT in a Complex Retinal Detachment Case...... 5

Repair of Progressive Retinal Detachment Associated With Retinoschisis...... 7

Lens-Sparing Vitrectomy for Persistent Fetal Vasculature Syndrome...... 11

En Bloc Resection of Retinal Hemangioma With Combined Tractional and Serous Retinal Detachment...... 14

Inverted Flap Technique for Large Macular Hole Repair. . . . .17

Transvitreal Choroidal Tumor Biopsy Using a 27-Gauge Vitrector...... 19

A digital version of this print supplement, including embedded videos, may be found in the Retina Today app, available at the Apple Store and on Google Play. Surgical Case Discussions From Thought Leaders

Intraoperative OCT in a Complex Retinal Detachment Case Retinal re-detachment caused by a posterior break at the site of regressed melanoma after transvitreal fine-needle aspiration biopsy.

BY ALEKSANDRA V. RACHITSKAYA, MD, AND JUSTIS P. EHLERS, MD

Although its role is still being established, Transvitreal fine-needle aspiration biopsy was performed and intraoperative optical coherence tomogra- radioactive plaque treatment was completed. Subsequently, the phy (iOCT) has been employed in anterior patient developed a right eye rhegmatogenous retinal detachment segment and posterior segment surgeries.1,2 with a retinal tear at 6 o’clock. He underwent retinal detachment In vitreoretinal surgeries, iOCT aids visu- repair with scleral buckle, pars plana vitrectomy, endolaser to the alization of the area of interest, particularly parts of the retinal area of the break and 360 degrees on the scleral buckle, and sili- anatomy that may be difficult to delineate with the en face view cone oil tamponade. through the microscope. The patient’s BCVA postoperatively was 20/100. Subretinal fluid This report describes the use of iOCT in a complex reti- persisted, suggesting a persistent retinal detachment. Although nal detachment case. This case is part of the DISCOVER the initial anterior break at 6 o’clock appeared closed, it was dif- (Determination of Feasibility of Intraoperative Spectral Domain ficult to confirm with certainty on clinical examination. The deci- Microscope Combined/Integrated OCT Visualization During sion was made to take the patient to the operating room. En Face Retinal and Ophthalmic Surgery) study, a single-site, multisurgeon, prospective, consecutive case series, examining SURGERY AND SURGICAL TECHNIQUE microscope-integrated iOCT. Retinal detachment repair in this Pars plana vitrectomy with the 25-gauge Constellation Vision case was accomplished using the 25-gauge Constellation Vision System was performed. The Rescan 700 microscope-integrated System (Alcon). OCT system (Carl Zeiss Meditec) was used for iOCT acquisition. After removal of the silicone oil, scanning of the original peripher- CASE REPORT al inferior break at 6 o’clock confirmed that the break was closed. A 58-year-old man was diagnosed with a choroidal melanoma Imaging of the retina nasal to the regressed melanoma showed located anterior to the inferior arcade of the right eye (Figure 1). subretinal proliferative vitreoretinopathy (PVR) bands. Imaging of the regressed melanoma showed a large posterior break in the temporal aspect of melanoma (Figure 2). Using the iOCT, we established the extent of the break, and we marked it with diathermy. Air-fluid exchange was performed. The iOCT confirmed that the break was closed and the retina

Figure 1. Initial presentation of choroidal melanoma. The patient Figure 2. Intraoperative OCT of the posterior break in the temporal underwent transvitreal biopsy and radioactive plaque therapy. aspect of the regressed choroidal melanoma.

JULY/AUGUST 2016 | SUPPLEMENT TO RETINA TODAY 5 Clinical Case Compendium Surgical Case Discussions From Thought Leaders

break was closed, and we located and established the extent of the posterior break. The subretinal PVR was identified with iOCT and, given its behavior under air, it was deemed not necessary to remove it. We were also able to confirm that the retina was flat after air-fluid exchange, and the break was closed.

CONCLUSION The role of iOCT in vitreoretinal surgery is evolving. In select cases, such as the current complex retinal re-detachment case, iOCT provides additional information that may assist in surgical decision-making. n Figure 3. The iOCT confirmed that the break was closed and the retina 1. Ehlers JP, Kaiser PK, Srivastava SK. Intraoperative optical coherence tomography using the RESCAN 700: preliminary results from was reattached under air. the DISCOVER study. Br J Ophthalmol. 2014;98:1329-1332. 2. Ehlers JP, Goshe J, Dupps WJ, et al. Determination of feasibility and utility of microscope-integrated optical coherence tomography during ophthalmic surgery: the DISCOVER Study RESCAN Results. JAMA Ophthalmol. 2015;133:1124-1132. was reattached under air (Figure 3). The nasal PVR area flattened, 3. Singh AD, Medina CA, Singh N, et al. Fine-needle aspiration biopsy of uveal melanoma: outcomes and complications. Br J and it was deemed not necessary to remove the subretinal bands. Ophthalmol. 2016;100:456-462. 4. Ehlers JP, Ohr MP, Kaiser PK, Srivastava SK. Novel microarchitectural dynamics in rhegmatogenous retinal detachments identified Endolaser was applied, and silicone oil was used as tamponade at with intraoperative optical coherence tomography. Retina. 2013;33:1428-1434. the end of the case. Postoperatively, the retina was attached, and the patient’s BCVA improved to 20/40. Aleksandra V. Rachitskaya, MD DISCUSSION n assistant professor of ophthalmology, Cleveland Clinic Lerner College of Medicine, Cleveland, Ohio Retinal detachment after transvitreal fine-needle aspiration n vitreoretinal surgeon, Cole Eye Institute, Cleveland 3 biopsy is seen in 1% of patients. The diagnostic yield of the trans- n consultant to Allergan 3 vitreal biopsy is reported to be 86%. The case described here is n [email protected] unusual because of the presence of the posterior break at the site of regressed melanoma causing retinal re-detachment and the Justis P. Ehlers, MD closed peripheral break. We postulate that the posterior break n Norman C. and Donna L. Harbert Endowed Chair of Ophthalmic may have been a result of the initial biopsy and subsequent PVR. Research at the Cole Eye Institute of the Cleveland Clinic n In primary rhegmatogenous retinal detachment, iOCT has codirector of intraoperative OCT research at the Ophthalmic Imaging Center of the Cole Eye Institute been shown to identify persistent subretinal fluid and alterations n consultant to Alcon, Alimera Sciences, Bioptigen, Carl Zeiss Meditec, 4 in foveal contour. Studies also suggest that iOCT may provide Leica, Santen, and ThromboGenics; research support including equip- important information during a retinal surgery that may influence ment and/or funding from Bioptigen, Carl Zeiss Meditec, Genentech, surgical decision-making in more than 20% of cases.2 In complex Leica/Surgical One, and Regeneron; intellectual property licensed to retinal detachment, iOCT may be particularly useful to help Bioptigen and Synergetics/Bausch + Lomb identify subclinical retinal breaks and PVR-related membranes. n [email protected] In this case, for example, we confirmed that the initial peripheral

6 SUPPLEMENT TO RETINA TODAY | JULY/AUGUST 2016 Clinical Case Compendium Surgical Case Discussions From Thought Leaders

Repair of Progressive Retinal Detachment Associated With Retinoschisis A complex case requiring surgery after conservative measures, including laser barricade and pneumatic retinopexy, were unsuccessful.

BY ANTHONY JOSEPH, MD, AND CAROLINE R. BAUMAL, MD

Degenerative retinoschisis, a splitting in the layers of the retina, has been classified histopathologically as typical when shallow elevation of the inner retina is present or reticular where bullous elevation of the retina is apparent on clinical examination.1 In some instances, breaks in the outer layer of the retina can occur, which may lead to detachment of the neurosensory layer. These retinal detachments (known as schisis detachments) are typically asymptomatic, confined to the area of retinoschisis, and can be monitored if asymptomatic without progression into the macula.2,3 In contrast, progressive and symptomatic retinal detachment complicating retinoschisis (PSRDCR) is rare, occurring in approximately 0.05% of cases, and requires surgical intervention.2 For limited PSRDCR stemming from a peripherally located outer layer break (OLB), scleral buckling with cryotherapy has shown good results.3,4 If the OLB is larger or more posteriorly located, manage- Figure 1. Spectral domain OCT of the left eye shows adherent ment is less straightforward. posterior hyaloid and vitreoschisis (white arrows) with adjacent Surgical series have reported a lower rate of primary retinal neurosensory detachment (white star). reattachment in eyes with PSRDCR compared with eyes with rhegmatogenous retinal detachment uncomplicated by retinos- retinal detachments in both eyes. He reported a 2-month history chisis.5 This appears to be related to multiple factors, including of bilateral floaters. Anterior segment examination revealed trace tight attachment of the vitreous over the area of retinoschisis, nuclear sclerosis. Dilated fundus examination showed supero- a higher rate of primary proliferative vitreoretinopathy (PVR) in temporal bullous retinoschisis associated with OLBs, and retinal PSRDCR eyes, and the potential for complicated anatomy in these detachments in both eyes. In the left eye, the retinal detachment eyes, including multiple, small inner holes and large irregular outer extended posteriorly, approaching the superotemporal arcade. breaks with posterior and rolled edges. Spectral domain optical coherence tomography (Cirrus HD-OCT; We present a case of recurrent, progressive symptomatic retinal Carl Zeiss Meditec) of the left eye demonstrated retinal detach- detachment associated with retinoschisis repaired with 25-gauge ment associated with retinoschisis and overlying foci of adherent pars plana vitrectomy, internal drainage, C3F8 gas tamponade, posterior hyaloid (Figure 1). After treatment options were dis- and an encircling scleral buckle. Intravitreal triamcinolone aceton- cussed, laser barricade was performed in both eyes. ide (Kenalog; Bristol-Myers Squibb) was used to highlight adher- The right eye remained stable without extension of fluid; how- ent vitreous firmly attached over the retinoschisis cavity and to ever, the subretinal fluid broke through the superotemporal laser facilitate its removal. barricade in the left eye 2 weeks after laser treatment (Figure 2). The patient noted increased floaters in the left eye but no visual CASE REPORT field deficit, and the decision was made to proceed with pneu- A 56-year-old man was referred to the Retina Service of the New matic retinopexy, cryotherapy, and SF6 gas injection. England Eye Center with degenerative retinoschisis associated with The detachment initially improved with complete resorption

JULY/AUGUST 2016 | SUPPLEMENT TO RETINA TODAY 7 Clinical Case Compendium

Figure 3. Intraoperative image of the left eye shows elevation of the posterior hyaloid with the vitreous cutter. Triamcinolone demarcates the hyaloid as it is separated from the underlying schisis cavity. Figure 2. Color fundus photograph mosaic of the left eye shows extension of the retinal detachment (black arrows) beyond the prior OLB, we were able to drain subretinal fluid through an existing laser barricade (white arrows). A large outer layer break in the area of break and flatten the retina under air. We used the curved, illumi- retinoschisis (white stars) is also visible superotemporally. nated endolaser probe to photocoagulate in the area of retinoschisis and the superior and inferior peripheral retina. Following of subretinal fluid, but 6 days following the procedure, the patient air-gas exchange with 15% C3F8 gas, the scleral buckle was adjust- experienced acute worsening of vision with corresponding pro- ed and the conjunctiva closed. gression of the detached retina into the left macula, as well as a new area of inferior subretinal fluid. Accordingly, he was taken to DISCUSSION the operating room for surgical repair. This case highlights some of the major difficulties that exist in managing PSRDCR. These conditions may be asymptomatic SURGICAL TECHNIQUE and nonprogressive2,3; however, our patient’s retinal detachment The patient had a superotemporal area of retinoschisis with in the left eye had extended posteriorly and was in danger of retinal detachment that had extended to involve the macula and encroaching on the macula, leading us to try conservative man- inferior retina. A large posterior OLB was present superotempo- agement with laser barricade. rally with multiple (>6) overlying inner retinal holes, as well as Laser has long been considered a reasonable choice to treat some peripheral retinal holes inferotemporally. Our preoperative subclinical retinal detachment associated with retinoschisis, and it plan was to stain over the region of retinoschisis with an intraop- continues to be an accepted first approach in cases complicated erative adjuvant to ensure complete identification and removal of by a large posterior OLB.6,7 Even so, one must remain cautious in vitreous. Given the peripheral pathology and complex nature of treating asymptomatic retinoschisis, as one case reported devel- the detachment along with the patient’s phakic status, a 41 encir- opment of an OLB and retinal detachment following laser bar- cling band was placed prior to vitrectomy. ricade of retinoschisis.8 Three standard 25-gauge ports were inserted, and a core vitrec- When the detachment progressed beyond the laser barricade, tomy was performed. On preliminary assessment, the posterior we had an extensive discussion with the patient regarding surgi- hyaloid appeared to be elevated, and the vitreous was trimmed cal options. Given the superior location and the extent of the peripherally for 360 degrees. Internal diathermy was used to patient’s pathology, as well as the patient’s phakic status and sta- mark the inner retinal holes over the retinoschisis cavity, and this ble symptoms, a pneumatic retinopexy was deemed a reasonable demonstrated residual hyaloid strands. A small amount of triam- consideration with the hope of decreasing the retinal detachment cinolone was injected over the area of retinoschisis, as well as over enough to augment the prior laser. Although this approach may the optic nerve to delineate residual hyaloid, which was carefully be considered unconventional, Suzuki and colleagues recently elevated from the nerve and gently extended anteriorly (Figure 3). reported successful repair of retinoschisis-related retinal detach- Care was taken to remove all of the hyaloid overlying the schisis ment with pneumatic retinopexy alone.9 cavity and the OLB. After the retinal detachment had extended into the macula Given the posterior location of the inner retinal holes and the and symptoms developed, surgical intervention was necessary.

8 SUPPLEMENT TO RETINA TODAY | JULY/AUGUST 2016 r1_Optos SUPPLEMENT FpAd9x10.75_bmc_072116_9x10.75 7/21/16 4:44 PM Page 1

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Given the posterior location and large size of the causative OLB, When surgery is deemed necessary, vitrectomy can address vitrectomy was appropriate.4 In addition to capturing pathol- posterior OLBs and identify subtle inner retinal holes, while stain- ogy that would be difficult to address with an encircling scleral ing with an adjuvant such as triamcinolone over the area of reti- buckle alone, vitrectomy allows for more exact evaluation of the noschisis is invaluable to identify and elevate residual hyaloid to complex retinal architecture associated with these detachments relieve traction. Addition of an encircling buckle should be con- and, consequently, a more complete retinopexy as well as broad sidered depending on the patient’s lens status, given the high risk tamponade of the offending pathology.7 of PVR. Our patient’s retina remains attached with resolution of In this case, we were able to identify and treat multiple small gas at 6 weeks after surgery and no evidence of early PVR. n inner retinal holes that were not easily discernible on clinical 1. Straatsma BR, Foss RY. Typical and reticular degenerative retinoschisis. Am J Ophthalmol. 1973;75:551-575. examination. We elected to support the vitreous base with an 2. Byer NE. Long-term natural history study of senile retinoschisis with implications for management. Ophthalmology. 1986;93:1127- encircling scleral buckle, because the patient was phakic and there 1137. 3. Byer NE. Perspectives on the management of the complications of senile retinoschisis. Eye (Lond). 2002;16:359-364. was another focus of peripheral pathology in addition to the pri- 4. Gotzaridis EV, Georgalas I, Petrou P, et al. Surgical treatment of retinal detachment associated with degenerative retinoschisis. mary detachment. Indeed, some studies show that an encircling Semin Ophthalmol. 2014;29:136-141. 5. Grigoropoulos VG, Williamson TH, Kirkby GR, Laidlaw DA. Outcomes of surgery for progressive symptomatic retinal detachment scleral buckle alone with external drainage can be sufficient to complicating retinoschisis. Retina. 2006;26:37-43. manage schisis-associated detachments even with OLBs posterior 6. Okun E, Cibis PA. The role of photocoagulation in the management of retinoschisis. Arch Ophthalmol. 1964;72:309-314. 10 7. Hoerauf H, Joachimmeyer E, Laqua H. Senile schisis detachment with posterior outer layer breaks. Retina. 2001;21:602-612. to the equator. 8. Johnson DL, Nieto JC, Ip MS. Retinal detachment due to an outer retinal tear following laser prophylaxis for retinoschisis. Arch One additional consideration in our surgery was whether or Ophthalmol. 2008;126:1775-1776. 9. Suzuki AC, Zacharias LC, Tanaka T, et al. Case report: pneumatic retinopexy for the treatment of progressive retinal detachment in not to resect the inner retinal layer of the retinoschisis cavity dur- senile retinoschisis. Arq Bras Oftalmol. 2015;78:50-52. ing vitrectomy. One report suggests this maneuver may increase 10. Avitabile T, Ortisi E, Scott IU, et al. Scleral buckle for progressive symptomatic retinal detachment complicating retinoschisis the risk for subsequent epiretinal membrane formation.7 After versus primary rhegmatogenous retinal detachment. Can J Ophthalmol. 2010;45:161-165. using triamcinolone to clearly identify the residual posterior hyaloid overlying the OLB, we were confident we had relieved any Caroline R. Baumal, MD n traction with meticulous elevation of the hyaloid. Accordingly, we associate professor of ophthalmology, Tufts University School of Medicine and attending vitreoretinal surgeon, New England Eye Center, elected to leave the retina intact. Boston, Massachusetts n advisory board/speaker: Allergan and Genentech CONCLUSION n [email protected] Management of degenerative retinoschisis and associated retinal detachments can be complex. It is sufficiently rare that Anthony Joseph, MD evidence as to what intervention will provide the best outcome is n fellow, vitreoretinal surgery and disease, New England Eye Center, limited. Observation is warranted with stable peripheral pathol- Boston, Massachusetts n financial interest: none acknowledged ogy, while conservative management with laser barricade is n [email protected] appropriate for posterior but asymptomatic associated retinal detachments.

10 SUPPLEMENT TO RETINA TODAY | JULY/AUGUST 2016 Clinical Case Compendium Surgical Case Discussions From Thought Leaders

Lens-Sparing Vitrectomy for Persistent Fetal Vasculature Syndrome

A two-port vitrectomy technique addresses typical cases characterized by a fibrovascular stalk with little lenticular involvement.

BY ARISTOMENIS THANOS, MD; BOZHO TODORICH, MD, PhD; YOSHIHIRO YONEKAWA, MD; AND KIMBERLY A. DRENSER, MD, PhD

Persistent fetal vasculature syndrome (PFVS) refers to a broad spectrum of disor- ders characterized by incomplete regression of the hyaloid vascular system (HVS). The more accurate term, PFVS, replaced the older term persistent hyperplastic primary vitreous introduced by Reese to accurately reflect the underlying pathophysiology of the disease.1 The HVS is a transient net- work of solely arterial vessels that starts to develop in the fourth or fifth week of gestation and regresses fully by 28 to 30 weeks’ gestational age. Its role is to nourish the developing intraocular components of the crystalline lens and the primary vitreous.2 Clinical findings in PFVS can be mild, ranging from a Mittendorf dot or a Bergmeister papilla, to a dense retrolenticular membrane with associated cataract, microphthalmia, retinal dysplasia, or retinal detachment. PFVS is typically a unilateral condition, and, Figure 1. Anterior segment photos of the right (A) and left (B) eye. A to date, no distinct genetic mutation has been associated with small remnant of hyaloid artery is visible on the posterior capsule of it. The precise molecular events leading to the final programmed the left eye. Both corneas had the same diameter. Fundus photos of the regression of the HVS remain poorly defined, and the predomi- right (C) and left (D) eye demonstrate a fibrovascular stalk extending nant unilaterality of the disease points toward somatic mosaicism. from the inferonasal portion of the optic nerve to the posterior lens Interestingly, persistence of the HVS is seen in 95% of prema- capsule with associated peripapillary retinal traction. ture infants compared with only 3% of full-term infants.3 In addition, accumulating evidence suggests that the Wnt signaling path- deep, and the crystalline lens demonstrated a small remnant of way is involved in the HVS regression.4 This is further supported the hyaloid artery on the posterior capsule (Figure 1B). The cili- by the overlapping clinical phenotypes of patients with PFVS and ary processes did not demonstrate visible elongation. Intraocular familial exudative vitreoretinopathy. In rare cases, PFVS can pres- pressures were within normal limits and symmetrical bilaterally. ent as a bilateral disease, where Norrie disease must be ruled out.5 Fundus examination of the left eye revealed a persistent hyaloid artery with a fibrovascular stalk connecting to the optic disc. CASE REPORT Fundus examination of the right eye was unremarkable (Figure 1C). A 15-month-old boy born full-term was diagnosed by his pedi- A small area of traction retinal detachment in the posterior pole atric ophthalmologist with amblyopia in his left eye secondary was evident, but no peripheral pathology was noted (Figure 1D, to persistent fetal vasculature syndrome and was referred to our arrow). practice for further management. A widefield fluorescein angiogram revealed no areas of capillary An examination under anesthesia revealed that both eyes had nonperfusion or neovascularization in either eye. Given the pres- equal corneal diameters (Figure 1A and B). The anterior segment ence of amblyopia and concurrent retinal traction, we decided of the right eye demonstrated a clear cornea, normal anterior to proceed with lens-sparing vitrectomy and transection of the segment, and clear lens. In the left eye, the anterior chamber was fibrovascular stalk.

JULY/AUGUST 2016 | SUPPLEMENT TO RETINA TODAY 11 Clinical Case Compendium Surgical Case Discussions From Thought Leaders

THE PROCEDURE This case was managed as a two-port lens-sparing vitrectomy. A “In typical cases of PFVS, we prefer a two- conjunctival incision was made at 12 o’clock and extended temporally and nasally at the limbus to 9 o’clock and 3 o’clock. A 20-gauge port technique, because an extensive MVR blade was used to create a sclerotomy temporally 2 mm vitrectomy is not required and the posterior to the limbus (Figure 2A), and a 23-gauge MVR blade infusion provided by the lighted irrigating was used nasally, 2 mm posterior to the limbus at 10 o’clock and 2 o’clock (Figure 2B). A lighted irrigating pick was used temporally, pick is sufficient to maintain a stable and the nasal sclerotomy was used for a 23-gauge vitrectomy. Under intraocular environment.” widefield noncontact microscopy, a core vitrectomy surrounding the stalk tissue was performed to freely release the stalk tissue from surrounding hyaloid attachments (Figure 2C). Using a 23-gauge DISCUSSION vertical scissors (Grieshaber, DSP; Alcon), we incised the stalk after In typical cases of PFVS, such as the case presented here, we identifying the retinal fold and ensuring that only stalk tissue would prefer a two-port technique, because an extensive vitrectomy is be incised (Figure 2D). No bleeding was noted after the incision of not required and the infusion provided by the lighted irrigating the central stalk tissue (Figure 2E). A partial fluid-air exchange was pick is sufficient to maintain a stable intraocular environment. performed and the sclerotomies were closed (Figure 2F). Care should be taken during the transection of the stalk, as a more posterior cut may result in an iatrogenic retinal break, particularly in cases where the peripapillary retinal tissue is drap- ing the stalk tissue (Figure 3). Similarly, care should be taken to not damage the posterior lens capsule around the area where the stalk is attached to the lens, as the stalk does not need to be removed in its entirety. Lowering the infusion pressure to increase perfusion of hyaloidal vessels before the transection will enhance visualization of any patent vasculature within the stalk. Furthermore, fine areas of organized vitreous may be present at the posterior pole, and these should be addressed to prevent the stalk from falling toward the center of the macula. The use of diathermy to cauterize the blood vessels before the transection to decrease the chance of intraoperative hemorrhage

Figure 2. A temporal sclerotomy is made 2 mm posterior to the limbus (A), and a lighted irrigating pick is inserted (B). Using a 23-gauge MVR blade, a nasal sclerotomy is made (C). A core vitrectomy around the stalk tissue is performed (D,E). Vertical scissors are used to dissect the Figure 3. Peripapillary retinal tissue cloaking the stalk tissue, giving fibrovascular stalk, and a fluid-air exchange is performed (F) to prevent it more substance. Great care should be taken to divide only the stalk vitreous incarceration into the sclerotomies. tissue and not the retina or intrinsic retinal vessels.

12 SUPPLEMENT TO RETINA TODAY | JULY/AUGUST 2016 Clinical Case Compendium Surgical Case Discussions From Thought Leaders

3. Jones HE. Hyaloid remnants in the eyes of premature babies. Br J Ophthalmol. 1963;47:39-44. is controversial, as its application may increase vertical traction on 4. Lobov IB, Rao S, Carroll TJ, et al. WNT7b mediates macrophage-induced programmed cell death in patterning of the vasculature. the peripapillary retina and the posterior lens capsule. From our Nature. 2005;437:417-421. 5. Walsh MK, Drenser KA, Capone A Jr, Trese MT. Early vitrectomy effective for bilateral combined anterior and posterior persistent experience, the transection of the stalk rarely results in significant fetal vasculature syndrome. Retina. 2010;30:S2-S8. bleeding, given the minimal patency of the hyaloid artery at the 6. Dass AB, Trese MT. Surgical results of persistent hyperplastic primary vitreous. Ophthalmology. 1999;106:280-284. time of surgery. Two-port vitrectomy is advantageous in PFVS cases with associated microphthalmia by limiting the number of Aristomenis Thanos, MD ports or instruments inside the eye. n vitreoretinal fellow at Associated Retinal Consultants, Oakland University William Beaumont School of Medicine, Royal Oak, Visual outcomes in PFVS cases are variable and depend mostly Michigan on the extent of retinal dysplasia, the timing of surgery, and n financial interest: none acknowledged 6 postoperative amblyopia management. The latter is of particu- n [email protected] lar importance in more complicated cases where lensectomy is performed. Widefield fluorescein angiography is necessary to Bozho Todorich, MD, PhD identify masqueraders, as peripheral retinal nonperfusion may n vitreoretinal fellow at Associated Retinal Consultants, Oakland indicate an asymmetric bilateral condition, specifically familial University William Beaumont School of Medicine, Royal Oak, exudative vitreoretinopathy or Norrie disease. Michigan n financial interest: none acknowledged n [email protected] CONCLUSION This case demonstrates two key take-away points: Yoshihiro Yonekawa, MD • A two-port vitrectomy technique will address typical cases of n vitreoretinal fellow at Associated Retinal Consultants, Oakland PFVS characterized by a fibrovascular stalk with little lenticu- University William Beaumont School of Medicine, Royal Oak, lar involvement. Michigan • Fluorescein angiography is absolutely essential in the diagno- n financial interest: none acknowledged n [email protected] sis and management of patients with PFVS, as it may reveal areas of capillary nonperfusion in the affected eye as well as Kimberly A. Drenser, MD, PhD the nonaffected eye, which may lead to other diagnoses. n n partner at Associated Retinal Consultants, Oakland University William Beaumont School of Medicine, Royal Oak, Michigan 1. Goldberg MF. Persistent fetal vasculature (PFV): an integrated interpretation of signs and symptoms associated with persistent n financial interest: none acknowledged hyperplastic primary vitreous (PHPV). LIV Edward Jackson Memorial Lecture. Am J Ophthalmol. 1997;124:587-626. 2. Saint-Geniez M, D’Amore PA. Development and pathology of the hyaloid, choroidal and retinal vasculature. Int J Dev Biol. n [email protected] 2004;48:1045-1058.

JULY/AUGUST 2016 | SUPPLEMENT TO RETINA TODAY 13 Clinical Case Compendium Surgical Case Discussions From Thought Leaders

En Bloc Resection of Retinal Hemangioma With Combined Tractional and Serous Retinal Detachment This technique may result in successful vision stabilization and globe salvage.

BY PHOEBE LIN, MD, PHD

Retinal capillary hemangiomas (RCH) are common A B findings in von Hippel-Lindau (VHL) disease. They can occur as a single lesion or as multiple lesions associated with complications, such as retinal neovascularization, tractional or exudative retinal detachment, or neovascular glaucoma. Because these tumors produce VEGF, anti-VEGF agents have been attempted as therapy. Other treatments include laser, cryotherapy, radioactive plaques, and photodynamic therapy (PDT). For large tumors associated with retinal detachment, vitreoretinal surgery has been performed with some success.1 Figure 1. Fundus photograph (A) and B-scan ultrasound (B) CASE DESCRIPTION reveal multiple large and small retinal hemangiomas with dilated A 14-year-old girl presented with decreased vision in her left feeder vessels, retinal neovascular membranes, a tractional retinal eye. She had no pertinent family history. Visual acuity was 20/20 detachment, and exudative retinal detachment. OD and counting fingers OS. Anterior segment examination was unremarkable. Dilated fundus examination revealed: 2+ anterior PREOPERATIVE MANAGEMENT vitreous cell; significant proteinaceous haze in the vitreous; a large One day before surgery, intravitreal bevacizumab (Avastin; elevated reddish lesion superotemporally to which a neovascular Genentech) was injected through the superonasal pars plana to membrane extended from the optic nerve; several sets of feeder promote regression of the retinal neovascularization and mini- vessels; numerous smaller reddish retinal lesions; a tractional mize intraoperative bleeding. On the day of surgery, the patient retinal detachment (TRD) involving the macula; a serous retinal was given a retrobulbar block containing 2% lidocaine, 0.75% detachment involving the remaining retina with subretinal exu- bupivacaine, and epinephrine 1:100,000 while under general anes- date nasally (Figure 1A). B-scan ultrasound showed an elevated thesia, also for hemostasis. pulsating mass to which preretinal membranes were attached, causing a TRD (Figure 1B). The right eye was normal. INTRAOPERATIVE MANAGEMENT Intraoperative hemostasis was achieved by high-power dia- MANAGEMENT AND TREATMENT thermy to the dilated feeder vessels. In a different adult patient VHL gene sequencing was positive for the C.227_229delTCT muta- with familial VHL, this approach was successful in the resection of tion. Systemic imaging revealed cerebellar, pulmonary, and adrenal a large retinal hemangioblastoma causing a TRD (Figure 2). In our lesions. Surgical and nonsurgical treatment options were discussed 14-year-old patient, the posterior hyaloid was elevated using the with the patient and her parents over multiple visits. Because it was 23-gauge cutter (Figure 3A). This was followed by segmentation likely that such a severe presentation would progress, the patient of fibrosing neovascular membranes between the optic nerve, and her parents decided to proceed with globe-salvage surgery. She arcades, and temporal macula. Preretinal membrane delamination underwent 23-gauge pars plana vitrectomy (Constellation Vision was aided by bimanual dissection using a 23-gauge lighted pick System; Alcon), membrane peeling, en bloc resection, retinectomy, and nondisposable pick forceps (Figure 3B). A 25-gauge chande- endolaser, and silicone oil tamponade. The principles for a success- lier was placed in the inferonasal pars plana for illumination of the ful treatment include: appropriate indication/goals (requiring a largest superotemporal hemangioma (Figure 3C). Diathermy was detailed discussion), tumor ablation and diagnosis, removal of used to create a border around this lesion after ligating the feeder the tumor vascular source, and repair of the retinal detachment. vessels. Using the 23-gauge pneumatic scissors and pick forceps,

14 SUPPLEMENT TO RETINA TODAY | JULY/AUGUST 2016 Clinical Case Compendium Surgical Case Discussions From Thought Leaders

A B C A B C

Figure 2. Ligation of feeder vessels in a 31-year-old woman with VHL D E F during en bloc resection surgery. Note that the feeder vessels regress in dilation and tortuosity immediately after (B,C) diathermy ligation proximal to the hemangioma (arrow) compared to before ligation (A).

the lesion was resected up to its anterior pars plana attachment (Figure 3D). Intraocular pressure was elevated intermittently for Figure 3. Intraoperative still images showing (A) elevation of posterior additional intraoperative hemostasis. hyaloid; (B) bimanual delamination of membranes using the 23-gauge The superotemporal wound was enlarged using an MVR blade, lighted pick and pick forceps; (C) placement of a 25-gauge chandelier; and the lesion everted through the wound. A pair of nontoothed (D) resection using 23-gauge pneumatic scissors; (E) excision of the forceps and Vanna’s scissors were used to resect the anterior lesion upon eversion through an enlarged sclerotomy; and (F) flatten- border of the lesion (Figure 3E). A 360-degree retinectomy during ing of the retinectomized retina with perfluorocarbon. which I removed several smaller hemangiomas was completed, followed by drainage of subretinal fluid and removal of subretinal exudative material. Perfluorocarbon heavy liquid was used to flatten the retina (Figure 3F), followed by endolaser to the retinec- “A 360-degree retinectomy during which tomy edge, and placement of 5,000 centistoke silicone oil. I removed several smaller hemangiomas POSTOPERATIVE MANAGEMENT was completed, followed by drainage of Gentle handling of the tissue specimen was achieved with non- subretinal fluid and removal of subretinal toothed forceps to transfer tissue with correct orientation onto sterile cardboard and then into a formalin-containing specimen exudative material.” cup. Histopathological evaluation revealed a retinal capillary hemangioma (Figure 4). The patient had a recurrent shallow TRD due to proliferative vitreoretinopathy at 6 weeks, requiring cataract extraction, membrane peel, and replacement of silicone oil. and prevent intraoperative bleeding. At more than 1 year following these two surgeries, the Standard treatment of small to medium RCH (<2 disk diam- patient’s visual acuity remained counting fingers with an eters) is with laser or cryotherapy, while treatment of large RCH, attached retina (Figure 4). She has required three outpatient particularly in the setting of a TRD, usually requires surgery laser procedures for small recurrent hemangiomas at the edge of because laser or cryotherapy alone may not be effective in slowing the retinectomy, and now remains free of retinal hemangiomas disease progression due to early reperfusion of the tumor. Internal in the left eye. The right eye remains normal. en bloc resection of RCH utilizing suture ligation of feeder vessels

DISCUSSION A B C Treatment of large RCH in the setting of retinal neovascular membranes, tractional, and exudative retinal detachments is difficult and requires a detailed discussion of surgical and nonsurgical options with the patient and family members. Although anti-VEGF therapy by systemic and intravitreal routes for D E RCH has been tried, it has been unsuccessful in inducing regression of lesions2,3; Figure 4. Fundus images before (A) and after (B) surgery. Light microscopy image of histopatho- bevacizumab was used preoperatively in logical specimen (C) reveals a retinal capillary hemangioma. Optical coherence tomography shows this case to reduce neovascularization detached retina before (D), and sustained retinal reattachment after surgery (E).

JULY/AUGUST 2016 | SUPPLEMENT TO RETINA TODAY 15 Clinical Case Compendium

detachment, en bloc resection can result in successful vision stabilization and globe salvage. A bimanual technique with chan- “In cases of large or multiple RCH delier illumination, meticulous attention to intraoperative hemo- complicated by retinal neovascularization stasis, removal of membranes, resection of hemangiomas, ligation and combined exudative/tractional retinal of feeder vessels, and long-term tamponade, all using small-gauge instruments, remains extremely valuable in successful surgical detachment, en bloc resection can result management of this blinding retinal condition. n in successful vision stabilization 1. Gaudric A, Krivosic V, Duguid G, et al. Vitreoretinal surgery for severe retinal capillary hemangiomas in von Hippel-Lindau disease. and globe salvage.” Ophthalmology. 2011;118:142-149. 2. Wong WT, Chew EY. Ocular von Hippel-Lindau disease: clinical update and emerging treatments. Curr Opin Ophthalmol. 2008;19:213-217. 4 3. Wong WT, Liang KJ, Hammel K, et al. Intravitreal ranibizumab therapy for retinal capillary hemangioblastoma related to von has been reported by Schlesinger and associates, but because Hippel-Lindau disease. Ophthalmology. 2008;115:1957-1964. of our prior success using endodiathermy alone, the latter was 4. Schlesinger T, Appukuttan B, Hwang T, et al. Internal en block resection and genetic analysis of retinal capillary hemangioblastoma. Arch Ophthalmol. 2007;125:1189-1193. undertaken for the case outlined here. A subsequent case series 5. Alegret A, Cebulla CM, Dubovy SR, et al. Photodynamic therapy and vitrectomy for a large optic nerve hemangioma with neovas- of RCH requiring surgery in 23 eyes over 18 years showed that cularization and retinal detachment: a clinicopathologic correlation. Retin Cases Brief Rep. 2009;3:93-95. 6. Mariotti C, Giovannini A, Reibaldi M, et al. 25-gauge vitrectomy combined with half-fluence photodynamic therapy for the treat- surgery can be successful in globe salvage, but visual prognosis ment of juxtapapillary retinal capillary hemangioma: a case report. Case Rep Ophthalmol. 2014;5:162-167. in complex cases requiring retinectomy is poor.1 Successful treatment with PDT combined with vitrectomy has also been reported.5,6 Phoebe Lin, MD, PhD n assistant professor of ophthalmology, Casey Eye Institute, Portland, CONCLUSIONS Oregon In cases of large or multiple RCH complicated by retinal neo- n financial interest: none acknowledged n [email protected] vascularization and combined exudative/tractional retinal

16 SUPPLEMENT TO RETINA TODAY | JULY/AUGUST 2016 Clinical Case Compendium Surgical Case Discussions From Thought Leaders

Inverted Flap Technique for Large Macular Hole Repair A novel technique to increase closure rates.

BY MARIA H. BERROCAL, MD

The surgical management of macular holes has evolved significantly since its description by Wendell and associates in 1993.1 In my experience, the implementa- tion of dye-assisted internal limiting membrane (ILM) peeling has increased the success rate of the surgery to over 90% in smaller holes. Recent holes also show significant improvements in visual acuity with closure. Despite these advances, however, large holes and macular holes in highly myopic eyes remain a challenge. Nawrocki and associates described in 2010 the inverted flap technique to improve closure rates in large macular holes.2 Their Figure 1. Large, stage 4 macular hole, visual acuity 4/200; OCT shows original technique involved peeling the ILM circumferentially detached posterior hyaloid and cystic spaces on the edge of the hole. around the hole, leaving it attached to the edge of the hole. This attached piece of ILM is inverted to cover the hole. Since then, variations of this technique have been described. In this case report, I present my technique of inverted flap vitrectomy for the management of large macular holes. This technique is particularly useful in the management of macular holes in highly myopic eyes. These eyes have much lower closure rates with conventional surgery, and the inverted flap technique has increased the closure rate in these challenging cases.

CASE REPORT A 70-year-old woman presented with reduced visual acuity of 4/200 in the left eye of more than 1 year’s duration. Optical coherence tomography (OCT) showed detached posterior hyaloid and cystic spaces on the edge of the hole (Figure 1). Fundus photography revealed hypopigmentation of the retinal pigment epithelium in the area of the hole (Figure 2). A pars plana vitrectomy was performed using the 25-gauge valved vitrectomy platform and the 27-gauge Finesse Flex Loop of the Constellation Vision System (Alcon). The core vitrectomy was performed and triamcinolone was injected into the vitreous cavity to help visualize the posterior hyaloid. Active maximum suction with the vitrectomy probe was used to lift and separate the posteri- or hyaloid in the area of the optic nerve. The hyaloid was separated Figure 2. Fundus photograph of the macular hole shows up to the ora serrata. The ILM was stained with brilliant blue, using hypopigmentation of the retinal pigment epithelium in the area of the reflux mode of the Constellation Vision System and the vitrec- the hole. tomy cutter. The dye was aspirated with the vitrectomy probe, and it was painted over the ILM utilizing reflux. This prevents trauma to to remove it from around the macular hole in a circumferential the retina from an excessively forceful injection. manner. A hinge was left on the temporal side, and the flap of The Finesse Flex Loop was used to start a break in the ILM and ILM was positioned over the macular hole. An air-fluid exchange

JULY/AUGUST 2016 | SUPPLEMENT TO RETINA TODAY 17 Clinical Case Compendium

Figure 3. Three weeks postoperatively, visual acuity is 8/200, and the macular hole is closed. Discontinuity of the ellipsoid layer is noted in the area of the hole.

Figure 4. Six months postoperatively, visual acuity is 20/200. OCT shows closed macular hole. Discontinuity of the ellipsoid layer and Figure 5. Fundus photograph shows hypopigmented areas and areas alterations in the choroid in the same area are seen. of pigmentary changes in the macular area.

was performed and the eye was left with 15% C3F8. simple, and elegant tool in the armamentarium of techniques to Postoperative OCT showed a closed macular hole with altera- treat challenging macular hole cases. Closure rates are significantly tion of the ellipsoid layer (Figure 3). The missing ellipsoid layer is increased with this technique despite modest visual acuity gains. a common finding in old and large macular holes and explains the limited visual acuity recovery. In this case, the visual acuity CONCLUSION improved to 20/200 at 6 months’ follow-up (Figures 4 and 5). The inverted flap technique for macular hole closure as described by Nawrocki, as well as its different variations, provides DISCUSSION new tools in the management of challenging macular hole cases. The surgical management of large macular holes remains a Very high macular hole closure rates can be observed with these challenge because anatomical closure rates are low and visual techniques even in very large and highly myopic eyes. n acuity outcomes are suboptimal. Large macular holes, particu- 1. Wendel RT, Patel AC, Kelly NE, et al. Vitreous surgery for macular holes. Ophthalmology. 1993;100:1671-1676. larly in highly myopic eyes, have a poor visual and anatomic 2. Michalewska Z, Michalewski J, Adelman RA, Nawrocki J. Inverted internal limiting membrane flap technique for large macular prognosis. holes. Ophthalmology. 2010;117:2018-2025. Different techniques have been described for the management of these cases, including macular buckling surgeries Maria H. Berrocal, MD combined with pars plana vitrectomy. Buckling techniques are n director of Berrocal & Associates, San Juan, Puerto Rico difficult and have an increased risk of complications both intra- n member of the Retina Today Editorial Board operatively and in the postoperative period, particularly with n consultant to Alcon choroidal flow issues. n (787) 725-9315; [email protected] The inverted flap technique described here offers a new,

18 SUPPLEMENT TO RETINA TODAY | JULY/AUGUST 2016 Clinical Case Compendium Surgical Case Discussions From Thought Leaders

Transvitreal Choroidal Tumor Biopsy Using a 27-Gauge Vitrector

Smaller incisions allow for more stable wounds with less leakage while minimizing ocular trauma and discomfort for the patient.

BY ALI TORAB PARHIZ, MD; KATHLEEN GORDON, MD; AND ODETTE HOUGHTON, MD

Advances in small-gauge instrumentation have significantly improved the safety and efficacy of vitrectomy in recent years.1 “While the role of 27-gauge vitrectomy While the role of 27-gauge vitrec- is expanding in the world of vitreoretinal tomy is expanding in the world of vitreoretinal surgery, we offer surgery, we offer an example of its utility an example of its utility in performing transvitreal biopsy of choroidal lesions. in performing transvitreal biopsy of choroidal lesions.” CASE REPORT A 61-year-old man was referred for evaluation of a suspicious choroidal nevus in the right eye in June 2011. At this time, his visual acuity was 20/20-2 OD. The lesion measured 5 mm x 4 mm Between February 2013 and August 2014, fluctuations in vision in basal diameter and was associated with lipofuscin and subreti- and subretinal fluid volume were observed. In August 2014, the nal fluid. In addition, the lesion was 2 mm from the edge of the patient’s visual acuity was 20/40 OD, and a small amount of optic nerve and lacked both drusen and a halo (Figure 1). Because growth was noted along the superior border of the lesion. There of the high-risk characteristics, the decision was made to monitor was no associated change in the ultrasound characteristics. the patient closely. By February 2016, the patient’s visual acuity had decreased to Over the next 2.5 years, the patient was seen at 2- to 4-month 20/80 OD and was associated with increased subretinal fluid and intervals, and no change was detected in the lesion’s clinical enlarged basal diameter (Figure 3). Ultrasound still showed a highly appearance or the patient’s symptoms. Ultrasound during that reflective lesion with no change in height or basal diameter. Because time found the lesion to be highly reflective with no vascularity of photographic evidence of persistent growth and the significant and a maximum height of approximately 1.46 mm (Figure 2). number of risk factors, we felt the lesion was a small melanoma and

A B

Figure 1. Fundus photograph at presentation showing an elevated, pigmented lesion with overlying lipofuscin pigment deposits (A). OCT at presentation showing disruption of ellipsoid zone, small pockets of subretinal fluid, multifocal elevations in the retinal pigment epithelium. There is a dome-shaped choroidal lesion with hyperreflectivity of the choriocapillaris and choroid at the site of the choroidal lesion (B).

JULY/AUGUST 2016 | SUPPLEMENT TO RETINA TODAY 19 Clinical Case Compendium

“The lateral rectus muscle was imbricated with a 6-0 Vicryl suture on an S29 needle and secured with locking bites.”

PROCEDURE B It was important to expose the sclera overlying the macular lesion. A 360-degree conjunctival peritomy was performed, followed by dissection of the Tenon capsule in all quadrants of the globe. All four recti were isolated and looped with 2-0 silk ties. The lateral rectus muscle was imbricated with a 6-0 Vicryl suture on an S29 needle and secured with locking bites. The lateral rectus was then carefully transected from its insertion. The lesion was identified using a Transilluminator OS3000 (Mira), and the borders were traced onto the sclera with a mark- ing pen. A 12-mm dummy plaque was positioned so that there was a 4-mm margin on the anterior edge of the tumor. The Figure 2. Scan of lesion showing medium to high internal reflectivity plaque was secured to the globe with two 5-0 nylon sutures with a height of 1.40 mm (A). Scan of a dome-shaped, acoustically (RD-1; Ethicon), which served as preplaced sutures for the radio- hollow choroidal lesion (B). active plaque. Once the correct positioning had been confirmed with transillumination, the dummy plaque was replaced with the decided to treat with iodine-125 episcleral brachytherapy. I-125 radioactive plaque using the preplaced 5-0 nylon sutures. At the time of treatment, the lesion measured 6 mm x 6.5 mm The lateral rectus muscle was then secured to the muscle inser- with a height of 1.2 mm. The patient requested cytogenetic analy- tion using the 6-0 Vicryl suture. sis of the tumor for prognostic purposes. Because of the tumor’s The transvitreal choroidal biopsy was performed without small size, we felt a transvitreal approach would be the safest intraocular infusion, using 27-gauge instrumentation and technique with the highest yield. the Constellation Vision System (Alcon). The procedure was

A B

Figure 3. Preoperative fundus photograph revealing a pigmented lesion with overlying orange lipofuscin pigment that has increased in size, compared to that in Figure 1A (A); preoperative OCT showing disruption of ellipsoid zone and increased subretinal fluid compared to Figure 1A, and multifocal elevations of the retinal pigment epithelium. There is a dome-shaped elevation of the choroid and retinal pigment epithelium with hyporeflectivity of the choroid at the site of the choroidal lesion (B).

20 SUPPLEMENT TO RETINA TODAY | JULY/AUGUST 2016 Clinical Case Compendium Surgical Case Discussions From Thought Leaders

TABLE. ALCON ULTRAVIT PROBE DIMENSIONS

Probe Port Port External Port Depth Diameter Probe Diameter to Top (mm) (mm) (mm) (mm)

23 0.22 0.38 0.65 0.29

25+ 0.18 0.38 0.52 0.29

27+ 0.14 0.33 0.42 0.29

performed under a widefield noncontact microscope system (Resight/Lumera 700; Carl Zeiss Meditec). Figure 4. Postoperative photograph of the tumor biopsy site. The 27-gauge cannulas were inserted at a 30-degree angle to Preretinal hemorrhage is overlying the choroidal lesion. the sclera, following conjunctival displacement. The cannulas were placed 4 mm posterior to the limbus at 2:30 o’clock and at 10:30 o’clock. A 27-gauge Ultravit cutter (Alcon) was inserted through the superior temporal cannula, while illuminating through the superior nasal cannula with a 27-gauge endoillumina- tor (Straight Probe; Alcon). The vitrector was advanced through “Although larger gauge needles or cutters the vitreous without cutting. may improve diagnostic yield, precision Upon contact with an extrafoveal, apical portion of the melanoma, a 0.5-mm diameter retinotomy was created. Once the port may be compromised.” of the Ultravit was within the tumor tissue, the probe was rotated while cutting. The vitrector was set to a biased, open duty cycle with 100 cuts per minute and 650-mm Hg vacuum. The port of the vitrectomy probe was withdrawn from the tumor and out of the eye without aspiration. a potentially lower yield when used to biopsy small tumors such The tissue sample was transferred to specimen containers pro- as the one in this case.4-6 vided by Impact Genetics using reflux on the vitrectomy system. One limitation in obtaining adequate tissue for analysis is The presence of pigmented cells was confirmed in the specimen tumor height.7,8 The smaller external dimensions of the 27-gauge container and the biopsy was repeated two more times, using a vitreous cutter make it a more suited for precise sampling of shal- total of two retinotomies over the melanoma. low choroidal lesions compared with the other gauge instruments At the conclusion of the biopsy, mild retinal hemorrhage was (Table). In addition, the use of a cutter enables the surgeon to appreciated on the surface of the lesion and plugging the retinot- collect a larger volume of tissue than the same gauge needle.9 omy sites. The peripheral retina remained attached. The trocars Although larger gauge needles or cutters may improve diag- were removed, and mild pressure was applied to the sclerotomies, nostic yield, precision may be compromised. In addition, evidence which sealed without placement of sutures. suggests that larger gauge instruments increase the risk of tumor Cytogenetic analysis on the collected specimen confirmed a seeding and other intraocular complications.10-12 No evidence of choroidal melanoma with monosomy 3. tumor seeding was found in a large series of patients with choroidal melanoma who underwent FNAB with 27-gauge or 30-gauge DISCUSSION needles at the time of brachytherapy.13 Uveal melanoma is the most common primary intraocular malig- In theory, the cannulas used with 27-gauge instrumentation may nancy in adults. Despite successful treatment of the primary tumor, reduce the risk of transmitting tumor cells to the tissue surrounding approximately 50% of patients succumb to metastatic disease. There the sclerotomy sites. There was one report of extraocular seeding fol- is significant evidence that loss of one copy of chromosome 3 is asso- lowing transvitreal biopsy using a 25-gauge vitrector and cannulas.14 ciated with increased metastatic potential of uveal melanoma.2 In this instance, however, the biopsy was performed 3 weeks prior to Recent advances in cytogenetic analysis have made it possible to proton beam radiotherapy, rather than at the time of radiotherapy. detect chromosomal aberrations using fine-needle aspiration biopsy In summary, our patient with a small melanoma desired cyto- (FNAB), enabling us to provide information about prognosis to all genetic analysis of his tumor in order to plan for the intensity of interested patients, including those not undergoing enucleation.3 metastatic surveillance. A tumor sample was obtained at the time Although FNAB can be performed using a trans-scleral or trans- of treatment with I-125 brachytherapy. A transvitreal biopsy using vitreal approach, a trans-scleral approach carries a higher risk and the 27-gauge vitreous cutter provided easy access to the tumor

JULY/AUGUST 2016 | SUPPLEMENT TO RETINA TODAY 21 Clinical Case Compendium

11. Glasgow BJ, Brown HH, Zargoza AM, Foos RY. Quantitation of tumor seeding from fine needle aspiration of ocular melanomas. tissue and resulted in an adequate specimen for cytogenetic anal- Am J Ophthalmol. 1988;105:538-546. ysis, with minimal disturbance of the overlying retina (Figure 4). 12. Ausburger JJ. Fine needle aspiration biopsy of suspected metastatic cancers to the posterior uvea. Trans Am Ophthalmol Soc. 1988;86:499-560. 13. Shields CL, Ganguly A, Materin MA, et al. Chromosome 3 analysis of uveal melanoma using fine-needle aspiration biopsy at the CONCLUSION time of plaque radiotherapy in 140 consecutive cases. Trans Am Ophthalmol Soc. 2007;105:43-52. 14. Raja V, Russo A, Coupland S, Groenewald C, Damato B. Extraocular seeding of choroidal melanoma after a transretinal biopsy A 27-gauge vitrectomy creates smaller incisions, allowing for with a 25-gauge vitrector. Retin Cases Brief Rep. 2011;5:194-196. more stable wounds with less leakage while causing less ocular trauma and discomfort for the patient. The smaller defect created Ali Torab Parhiz, MD in the retina may decrease the chance of secondary complications n clinical instructor and vitreoretinal fellow at University of North such as tumor seeding, retinal detachment, and hemorrhage, Carolina in Chapel Hill while allowing for more precise biopsy. Continued investigation of n financial interest: none acknowledged FNAB techniques and long-term safety are warranted. n n [email protected]

1. Recchia FM, Scott IU, Brown GC, Brown MM, Ho AC, Ip MS. Small-gauge pars plana vitrectomy: a report by the American Kathleen Gordon, MD Academy of Ophthalmology. Ophthalmology. 2010;117(9):1851-1857. n associate professor of ophthalmology, University of North Carolina in 2 Prescher G, Bornfeld N, Hirche H, et al. Prognostic implications of monosomy 3 in uveal melanoma. Lancet. 1996;347:1222-1225. Chapel Hill 3 Damato B, Dopierala JA, Coupland SE. Genotypic profiling of 452 choroidal melanomas with multiplex ligation-dependent probe amplification. Clin Cancer Res. 2010;16:6083-6092. n medical director of the Kittner Eye Center, University of North 4. Singh AD, Medina CA, Singh N, et al. Fine-needle aspiration biopsy of uveal melanoma: outcomes and complications. Br J Carolina at Chapel Hill Ophthalmol. 2016;100:456-462. n financial interest: none acknowledged 5. Bagger M, Tebering JF, Kiilgaard JF. The ocular consequences and applicability of minimally invasive 25-gauge transvitreal retinochoroidal biopsy. Ophthalmology. 2013;120:2565-2572. n [email protected] 6. Chang MY, McCannel TA. Comparison of uveal melanoma cytopathologic sample retrieval in trans-scleral versus vitrectomy- assisted transvitreal fine needle aspiration biopsy. Br J Ophthalmol. 2014;98:1654-1658. Odette Houghton, MD 7. Cohen VM, Dinakaran S, Parsons MA, Rennie IG. Transvitreal fine needle aspiration biopsy: the influence of intraocular lesion size on diagnostic biopsy result. Eye (Lond). 2001;15:143-147. n associate professor of ophthalmology and program director of the 8. McCannel TA, Chang MY, Burgess BL. Multi-year follow-up of fine-needle aspiration biopsy in choroidal melanoma. Ophthalmol- vitreoretinal surgery fellowship at the University of North Carolina at ogy. 2012;119:606-610. Chapel Hill 9. Bagger M, Andersen MT, Heegaard S, Andersen MK, Kiilgaard JF. Transvitreal retinochoroidal biopsy provides a representative sample from choroidal melanoma for detection of chromosome 3 aberrations. Invest Ophthalmol Vis Sci. 2015;56:5917-5924. n financial interest: none acknowledged 10. Shields JA, Shields CL, Ehya H, Eagle Jr RC, De Potter P. Fine-needle aspiration biopsy of suspected intraocular tumors: the 1992 n [email protected] Urwick Lecture. Ophthalmology. 1993;100:1677-1684.

22 SUPPLEMENT TO RETINA TODAY | JULY/AUGUST 2016 Clinical Case Compendium ™

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Indications for Use: The Finesse™ Flex Loop is a manual ophthalmic surgical instrument intended to aid in ophthalmic surgical procedures. The device may be used in posterior segment surgery to create an edge on a membrane to begin peeling with forceps. The device is provided sterile and is intended for single use. Caution: Federal (USA) law restricts this device to sale by, or on the order of, a physician. Warning and Precautions: • It is not recommended to remove the ILM (internal limiting membrane) with the device. Scraping the retina can cause irreversible damage to the nerve fibers. • Inspect tip for damage (e.g. burrs, bending or loosening), check loop functionality (extendability), and ensure the loop is fully retracted before inserting instrument into the eye. Attention: Reference the Directions for Use for a complete listing of indications, warnings, and precautions.

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