Efficacy of Macular Hole Surgery in Patients with Idiopathic Macular Telangiectasia Type 2

Efficacy of Macular Hole Surgery in Patients with Idiopathic Macular Telangiectasia Type 2

Journal Pre-proof Efficacy of Macular Hole Surgery in Patients with Idiopathic Macular Telangiectasia Type 2 Alexander G. Miller, Rohit Chandra, Charl...

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Journal Pre-proof Efficacy of Macular Hole Surgery in Patients with Idiopathic Macular Telangiectasia Type 2 Alexander G. Miller, Rohit Chandra, Charles Pophal, Jerome P. Schartman, Joan H. Hornik, David G. Miller PII:

S2468-6530(19)30651-7

DOI:

https://doi.org/10.1016/j.oret.2019.11.017

Reference:

ORET 668

To appear in:

Ophthalmology Retina

Received Date: 10 September 2019 Revised Date:

7 November 2019

Accepted Date: 22 November 2019

Please cite this article as: Miller A.G., Chandra R., Pophal C., Schartman J.P., Hornik J.H. & Miller D.G., Efficacy of Macular Hole Surgery in Patients with Idiopathic Macular Telangiectasia Type 2, Ophthalmology Retina (2019), doi: https://doi.org/10.1016/j.oret.2019.11.017. This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. © YEAR Published by Elsevier Inc. on behalf of American Academy of Ophthalmology

Macular hole surgery in macular telangiectasia type 2

Efficacy of Macular Hole Surgery in Patients with Idiopathic Macular Telangiectasia Type 2 Alexander G. Miller1,2 Rohit Chandra 1,2 Charles Pophal 1,2 Jerome P. Schartman1 Joan H. Hornik1 David G. Miller1 1

Retina Associates of Cleveland, Cleveland, OH, United States; 2Northeast Ohio Medical University, Rootstown, OH, United States; Corresponding author: David G. Miller, MD Retina Associates of Cleveland 3401 Enterprise Parkway, Suite 300 Cleveland, Ohio 44122 Phone 216-831-5700 Email [email protected] This material was presented in part at the 2019 meeting of the Association for Research in Vision and Ophthalmology. Financial support: None No conflicting relationship exists for any author. Running head: Macular hole surgery in macular telangiectasia type 2 Address for reprints: Retina Associates of Cleveland, 3401 Enterprise Parkway, Suite 300, Cleveland, Ohio 44122

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Macular hole surgery in macular telangiectasia type 2 1

Abstract

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Objective: To compare visual acuity (VA) and optical coherence tomography (OCT) outcomes in

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idiopathic macular telangiectasia Type 2 (IMT Type 2) patients who had pars plana vitrectomy

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(PPV) surgery for full-thickness macular holes (FTMH) versus those who elected to manage

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medically (MM) without surgery.

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Design: Comparative retrospective case series.

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Subjects: Patients with IMT Type 2 and FTMH.

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Methods: We reviewed records within an 11-year period and collected data on visual acuity

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(VA), optical coherence tomography (OCT) changes, development of choroidal

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neovascularization (CNV), and length of follow-up. VA measurements were standardized from

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Snellen to LogMAR units for statistical analysis. Two-sample t-tests were used to analyze VA

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data. OCT changes were assessed by a single masked retinal specialist.

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Results: There were 12 eyes in the PPV group and 26 eyes in the MM group. There was no

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statistically significant VA improvement in either group between initial VA recording and last

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follow-up. The PPV group had no significant change in VA between the pre-operative visit and

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visits at 3 or 12 months. OCT scans improved by 1 step in 10 PPV patients. None of the MM

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patients had OCT improvement. CNV developed in 1 eye in the PPV group and 5 eyes in the MM

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group.

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Conclusions: There was no significant change in visual acuity in patients who opted to have PPV

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to treat their IMT Type 2 and FTMH compared with those who did not have surgery. OCT scans

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improved by qualitative judgment in patients who had surgery compared with those who opted

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for medical management.

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Macular hole surgery in macular telangiectasia type 2 24

Idiopathic macular telangiectasia type 2 (IMT Type 2) is a disease that can affect both eyes

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with aberrant blood vessel growth and neurodegeneration of the macula.1 It is estimated that

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almost 0.1% of people in certain populations have this disease.2 There currently is no definitive

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treatment for IMT Type 2.

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Full-thickness macular holes (FTMH) are a prominent cause of vision loss. The standard of

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care for FTMH is pars plana vitrectomy (PPV) to help restore the anatomy and visual acuity

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(VA). IMT Type 2 is an infrequent cause or association of FTMH.3,4 There have been few reports

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evaluating outcomes with PPV versus observation of these patients. We performed this study

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to compare visual acuity (VA) and optical coherence tomography (OCT) outcomes in IMT Type 2

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patients who had surgery for FTMH (PPV) versus those who elected to manage medically (MM)

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without surgery.

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Methods

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Sterling Internal Review Board (IRB) determined that the study was exempt from IRB

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review. Using the practice management software, we retrospectively gathered all patient

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records with an International Statistical Classification of Diseases and Related Health Problems

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(ICD 9 and 10) of FTMH and IMT Type 2 over an 11-year period (January 2007‒December 2018)

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in a single retinal specialty practice comprised of 8 – 13 retinal physicians over that time period.

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The charts were reviewed for consistency of the diagnosis of IMT Type 2 and FTMH by

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reviewing the color fundus photos, OCT, and fluorescein angiogram. Patients with the following

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were included: coincident diseases that did not affect visual acuity (VA), intraoperative

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complications such as retinal tears, and postoperative complications. Data collected included

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Macular hole surgery in macular telangiectasia type 2 45

age, sex, surgeon in PPV cases, VA, OCT findings, development of choroidal neovascularization

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(CNV), and length of follow-up.

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VA data were collected for surgical patients preoperatively, at 3 months and 12 months

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postoperatively, and at most recent follow-up. VA data were collected for MM patients at initial

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diagnosis of concurrent FTMH and IMT type 2 and most recent follow-up. VA measurements

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were standardized from distance habitual correction with pinhole Snellen to LogMAR units for

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statistical analysis. Change in LogMAR units from initial diagnosis to most-recent follow up was

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compared between the 2 groups using 2-sample t-tests. 2-sample t-tests were also conducted

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to analyze for change in VA from initial diagnosis to milestone visits at 3 and 12 months in PPV

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group.

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The same masked retinal specialist graded OCT scans at first diagnosis and most recent

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follow-up on a 3-point scale, where 1 was typical-appearing open FTMH, 2 was flat edges of

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FTMH, and 3 was a closed FTMH with opposed edges. The change in grades on the OCT scans

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between initial diagnosis and last follow-up were tabulated.

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CNV development was also recorded.

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Results

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Patient demographics are shown in Table 1. Four different retinal surgeons performed the

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PPVs with similar techniques, with one surgeon performing 8 of the cases, and the remaining 3

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surgeons performing the other 4 cases.

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There was no statistically significant VA improvement in either group between initial VA

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recording and last follow-up. In addition, there was no significant change in VA for the PPV

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group between pre-op recording and visits at 3 or 12 months.

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Macular hole surgery in macular telangiectasia type 2 67

OCTs were available to read in all patients at initial data point and last follow-up. The OCT

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scans improved by 1 step (using the 3-point grading system) in 10 PPV patients; there was no

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improvement in the 2 remaining patients (Figure 1). The OCT scans of 4 patients showed closed

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or opposed edges (grade 3) at last follow up. None of the MM patients showed any

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improvement on OCT grading (Figure 2).

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CNV developed in 1 of 12 eyes in the PPV group and 5of 26 eyes in the MM group. In the

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PPV group, post-op complications were seen in 2 patients: 1 repeat macular hole surgery for

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enlarging FTMH following initial surgery and 1 iridotomy for acute angle closure glaucoma.

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Discussion

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The prognosis for stage 2-4 FTMH is poor, with central visual acuity deteriorating to the

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range of 20/100 to 20/400 over time.5 The Vitrectomy for Macular Holes Study Group in the

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1990s reported that, compared with observation, surgery for stage 2 holes resulted in a

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significantly lower incidence of hole enlargement; however, there was no significant difference

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in ETDRS visual acuity measurements.6 The group’s study of stage 3 and stage 4 macular holes

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found a significant benefit in the rate of hole closure and a marginally significant benefit in

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ETDRS VA in the surgery group.7 Since those reports, advances in surgical techniques and

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instrumentation have led to reported rates of hole closure rates of 91% to 98% and median

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postoperative VA of approximately 20/40.5

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There are few reports in the literature of FTMH in patients with IMT Type 2 and even

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fewer of surgical intervention in such patients. 3,4,8-13 Previous publications have only compared

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surgery as a treatment modality between patients with IMT Type 2 and FTMH versus those with

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FTMH only or only reported outcomes of surgery for these patients without a comparison

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Macular hole surgery in macular telangiectasia type 2 89

group of no surgery.4,6 In this study, there does not appear to be any advantage to surgery in

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patients with FTMH associated with IMT Type 2. Even though the OCT did show some

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anatomical improvement in most patients who underwent surgery, there was no accompanying

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VA improvement. Certainly these patients are quite dissimilar to typical FTMH patients, and the

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accompanying IMT Type 2 is likely limiting the improvement in VA and OCT anatomical results.

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One can hypothesize that since IMT Type 2 pathophysiology in creating a macular hole is very

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dissimilar to typical FTMH created by abnormal vitreoretinal traction, the results of surgery

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would also be different. In IMT Type 2, there is loss of the ellipsoid zone, development and

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coalescing of cystic cavities, and ultimately a dissolution of the neurosensory retina.4, 13 While

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the hole maybe closed with surgery in patients with IMT Type 2, there is not a regeneration of

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the neurosensory retina, which results in lack of improvement in visual acuity. In contrast for

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FTMH, the neurosensory retinal tissue is still present, and surgical repair places the tissue back

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into a normal alignment with resultant significant improvement in visual acuity. Due to cost of

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surgery and risk of surgical complications, patients FTMH associated with IMT Type 2 may

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benefit more with observation than surgical intervention.

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This study is limited by several weaknesses including that it was a retrospective study in

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only one private practice, follow-up was variable, and the small patient population. Further

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randomized prospective studies could be more definitive in conclusion.

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Macular hole surgery in macular telangiectasia type 2 107

References

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1. Charbel Issa PS, Gillies MC, Chew EY, et al. Macular telangiectasia Type 2. Prog Retin Eye Res.

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2013;34:49-77.

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2. Klein R, Blodi BA, Meuer SM, Myers CE, Chew EY, Klein BE. The prevalence of macular

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telangiectasia Type 2 in the Beaver Dam Eye Study. Am J Ophthalmol. 2010;150:55-62.

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3. Charbel Issa P, Scholl HP, Gaudric A, et al. Macular full-thickness and lamellar holes in

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association with type 2 idiopathic macular telangiectasia. Eye (Lond). 2009;23:435-441.

114

4. Karth PA, Raja SC, Brown DM, Kim JE. Outcomes of macular hole surgeries for macular

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telangiectasia type 2. Retina. 2014;34:907-915.

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5. American Academy of Ophthalmology Retina/Vitreous Panel. Preferred Practice Pattern®

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Guidelines. Idiopathic Macular Hole. San Francisco, CA: American Academy of Ophthalmology;

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2017. Available at www.aao.org/ppp. Accessed 15 August 2019.

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6. Kim JW, Freeman WR, Azen SP, et al, Vitrectomy for Macular Hole Study Group. Prospective

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randomized trial of vitrectomy or observation for stage 2 macular holes. Am J Ophthalmol

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1996;121:605-614.

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7. Freeman WR, Azen SP, Kim JW, et al, The Vitrectomy for Treatment of Macular Hole Study

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Group. Vitrectomy for the treatment of full-thickness stage 3 or 4 macular holes: results of a

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multicentered randomized clinical trial. Arch Ophthalmol. 1997;115:11-21.

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8. Jaycock PD, Thomas D, Zakir R, Laidlaw DA. Retinal telangiectasia in association with macular

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hole formation. Eye (Lond). 2004;18:342-343.

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9. Koizumi H, Slakter JS, Spaide RF. Full-thickness macular hole formation in idiopathic

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parafoveal telangiectasis. Retina. 2007;27:473-476.

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10. Olson JL, Mandava N. Macular hole formation associated with idiopathic parafoveal

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telangiectasia. Graefes Arch Clin Exp Ophthalmol. 2006;244:411-412

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11. Rishi P, Kothari AR. Parafoveal telangiectasia (PFT) has been associated with changes in

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macular architecture and macular holes (lamellar and full thickness). Retina. 2008;28:184-185.

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12. Gregori N, Flynn HW Jr. Surgery for full-thickness macular hole in patients with idiopathic

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macular telangiectasia type 2. Ophthalmic Surg Lasers Imaging. 2010;41 Online:1-4.

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13. Shukla D. Evolution and management of macular hole secondary to type 2 idiopathic

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macular telangiectasia . Eye (Lond). 2011;25:532-533.

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Figure legends

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Figure 1a. 71-year-old male in the PPV group presented with VA of 20/100 in right eye with

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macular hole and IMT Type 2. OCT graded as open edges (1).

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Figure 1 b. Last follow up was 86 months post PPV. VA was 20/100. OCT showed 1- step

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improvement to flat edges (2) but also highly irregular retinal pigment epithelium (RPE)

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changes.

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Figure 2a. 61-year-old female in the MM group presented with IMT Type 2 and macular hole in

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left eye. VA was 20/200. OCT has open edges, or grade 1.

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Figure 2b. Last follow-up was 49 months later. VA was 20/80 with persistent macular hole. OCT

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remained graded as open edges (1) with no change.

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Macular hole surgery in macular telangiectasia type 2

Table 1. Patient demographics Mean age (years)

Males

Females

Mean follow-up (months)

PPV

71 +/- 10

4

8

44 +/- 31

MM

69 +/- 10

16

10

22 +/- 15

PPV = pars plana vitrectomy group MM = medical management group

Macular hole surgery in macular telangiectasia type 2 Table 2. Mean visual acuity, PPV group

Snellen

LogMAR

P (LogMAR)

Pre-op

20/114

0.756 +/- 0.332

3 months post-op

20/113

+0.751+/- 0.332

0.717

12 months post-op

20/94

+0.670 +/- 0.298

0.546

Macular hole surgery in macular telangiectasia type 2

Table 3. VA change from diagnosis to last visit Initial

Last

Change

PPV LogMAR VA

+0.756 +/- 0.332

+0.732 +/- 0.236

-0.013 +/- 0.2

MM LogMAR VA

+0.481 +/- 0.345

+0.477 +/-0.321

+0.004 +/- 0.2

P (PPV vs MM)

0.830