Ophthalmic Surgery, Lasers and Imaging Retina

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Imaging Case Report 

Surgery for Full-Thickness Macular Hole in Patients with Idiopathic Macular Telangiectasia Type 2

Ninel Gregori, MD; Harry W. Flynn Jr., MD

Abstract

The authors report surgical outcomes of full-thickness macular hole repair in two patients with idiopathic macular telangiectasia in a non-comparative case series. Both patients underwent pars plana vitrectomy with indocyanine green-assisted internal limiting membrane peeling and injection of 16% C3F8 gas. Patients were imaged with optical coherence tomography (OCT) before and after surgery. The first patient demonstrated macular hole closure on examination and OCT with visual improvement from 20/50 preoperatively to 20/30 after macular hole surgery and subsequent cataract surgery. The second patient’s hole closed per OCT immediately after surgery but reopened 4 months later, and visual acuity remained 20/70. Macular hole surgery may be an effective treatment in patients with idiopathic macular telangiectasia and full-thickness macular holes and should be further investigated.

Abstract

The authors report surgical outcomes of full-thickness macular hole repair in two patients with idiopathic macular telangiectasia in a non-comparative case series. Both patients underwent pars plana vitrectomy with indocyanine green-assisted internal limiting membrane peeling and injection of 16% C3F8 gas. Patients were imaged with optical coherence tomography (OCT) before and after surgery. The first patient demonstrated macular hole closure on examination and OCT with visual improvement from 20/50 preoperatively to 20/30 after macular hole surgery and subsequent cataract surgery. The second patient’s hole closed per OCT immediately after surgery but reopened 4 months later, and visual acuity remained 20/70. Macular hole surgery may be an effective treatment in patients with idiopathic macular telangiectasia and full-thickness macular holes and should be further investigated.

Surgery for Full-Thickness Macular Hole in Patients with Idiopathic Macular Telangiectasia Type 2

From the Department of Ophthalmology (NG, HWF), Bascom Palmer Eye Institute, University of Miami Miller School of Medicine; and Miami Veterans Affairs Medical Center (NG), Miami, Florida.

Supported in part by an unrestricted grant from Research to Prevent Blindness, New York, New York.

The authors have no financial or proprietary interest in the materials presented herein.

Address correspondence to Ninel Gregori, MD, Bascom Palmer Eye Institute, 900 NW 17th Street, Miami, FL 33136. E-mail: ngregori@med.miami.edu

Received: June 19, 2009
Accepted: April 23, 2010
Posted Online: July 29, 2010

Introduction

Idiopathic macular telangiectasia is a retinal vascular disease that can present in either unilateral or bilateral fashion in three different types, originally defined by Gass and Blodi in 19931 and more recently reclassified by Yannuzzi et al.2 In this recent simplified classification scheme, two distinct types (type 1: aneurysmal telangiectasia; type 2: perifoveal telangiectasia) were described. The third type originally described by Gass and Blodi, occlusive telangiectasia, was omitted based on its association with systemic or cerebral vascular occlusive disorders and the predominant presence of capillary nonperfusion rather than telangiectasis.

Type 1 aneurysmal telangiectasia is generally a unilateral disorder found mostly in men, which presents with leaky telangiectatic vessels, microaneurysms, and macroaneurysms leading to cystoid macular edema and lipid deposition in the macula and sometimes in the retinal periphery.2 Type 2 macular telangiectasia is a bilateral perifoveal telangiectatic disorder without sex predilection that is not associated with aneurysms or cystoid leakage.2 It may present in the nonproliferative or proliferative stages. The nonproliferative stage presents with temporal perifoveal grayish loss of retinal transparency with barely visible telangiectatic vessels and an inner lamellar cyst seen by optical coherence tomography (OCT) in the fovea in more than half of patients with advanced disease.3,4 In the proliferative stage there is subretinal neovascularization and fibrosis.2

Recently, several reports describing the association of lamellar macular hole and full-thickness macular hole with idiopathic macular telangiectasia based on OCT have been published.4–6 To date, no reports of macular hole surgery in this disorder are described in the literature. To our knowledge, ours is the first report of surgical outcomes of full-thickness macular hole repair in patients with idiopathic macular telangiectasia.

Case Report

Case 1

A 64-year-old man with a history of idiopathic macular telangiectasia complained of distorted and decreased vision in his left eye for 6 weeks. Both eyes demonstrated grayish opacification around the fovea and intraretinal staining in the late phases of fluorescein angiogram (Fig. 1). The Stratus OCT III (Carl Zeiss Meditec, Dublin, CA) showed a large cystoid cavitation in the fovea under the intact internal limiting membrane in the right eye and a small full-thickness macular hole in the fovea of the left eye (Fig. 1). Visual acuity was 20/70 in the right eye and 20/50 in the left eye. Anterior segment examination was significant for moderate nuclear sclerotic cataracts in both eyes. The patient believed that his activities of daily living were affected due to recent deterioration of his better seeing eye, and surgical repair of the full-thickness macular hole was recommended.

A 64-Year-Old Man with a History of Idiopathic Macular Telangiectasia Type 2 Complained of Distorted and Decreased Vision in His Left Eye for 6 Weeks. Fundus Photographs Show Grayish Opacification Around the Fovea in the Right (A) and Left (B) Eye. Intraretinal Staining in the Late Phases of Fluorescein Angiogram Is Seen in the Right (C) and the Left (D) Eye. (E) The Stratus OCT III (Carl Zeiss Meditec, Dublin, CA) Showed a Large Cystoid Cavitation Under the Intact Internal Limiting Membrane in the Right Eye. (F) Stratus OCT Taken Preoperatively Shows a Small Full-Thickness Macular Hole in the Left Eye. (G) Spectral-Domain Cirrus OCT of the Right Eye, Taken a Year Later, Shows a Large Foveal Cavitation. (H) Spectral-Domain Cirrus OCT Through the Fovea, Taken 1 Year After the Surgery, Shows Macular Hole Closure in the Left Eye. External Limiting Membrane and Inner–Outer Segment Junction of Photoreceptors Are Preserved.

Figure 1. A 64-Year-Old Man with a History of Idiopathic Macular Telangiectasia Type 2 Complained of Distorted and Decreased Vision in His Left Eye for 6 Weeks. Fundus Photographs Show Grayish Opacification Around the Fovea in the Right (A) and Left (B) Eye. Intraretinal Staining in the Late Phases of Fluorescein Angiogram Is Seen in the Right (C) and the Left (D) Eye. (E) The Stratus OCT III (Carl Zeiss Meditec, Dublin, CA) Showed a Large Cystoid Cavitation Under the Intact Internal Limiting Membrane in the Right Eye. (F) Stratus OCT Taken Preoperatively Shows a Small Full-Thickness Macular Hole in the Left Eye. (G) Spectral-Domain Cirrus OCT of the Right Eye, Taken a Year Later, Shows a Large Foveal Cavitation. (H) Spectral-Domain Cirrus OCT Through the Fovea, Taken 1 Year After the Surgery, Shows Macular Hole Closure in the Left Eye. External Limiting Membrane and Inner–Outer Segment Junction of Photoreceptors Are Preserved.

He underwent a 25-gauge pars plana vitrectomy, membrane peeling with indocyanine green staining, fluid–air exchange, and 16% C3F8 gas injection in the left eye. Postoperative face-down positioning was maintained for 1 week. OCT confirmed that the macular hole closed, but vision was limited by a posterior subcapsular cataract. Four months later, the patient underwent uncomplicated phacoemulsification with posterior chamber intraocular lens implant. Visual acuity improved to 20/30 after cataract surgery. One year after vitrectomy, OCT showed the hole remained closed (Fig. 1). The right eye remained stable visually and did not progress to a macular hole (Fig. 1).

Case 2

A 67-year-old man with idiopathic macular telangiectasia presented with a recent decrease of vision in his right eye. Both eyes demonstrated grayish opacification around the fovea with pigment clumping temporal to the fovea (Fig. 2). The Stratus OCT III showed a small full-thickness macular hole in the right eye and normal foveal appearance in the left eye (Fig. 2). Visual acuity was 20/70 in the right eye and 20/20 in the left eye. The anterior segment examination was significant for mild nuclear sclerotic cataracts in both eyes. The patient desired surgical intervention in hopes of improving his visual acuity.

A 67-Year-Old Man with Full-Thickness Macular Hole and IMT Type 2 Presented with a Recent Decrease of Vision in His Right Eye. (A and B) Fundus Photograph Shows Grayish Opacification Around the Fovea with Pigment Clumping Temporal to the Fovea. (C) Stratus OCT III (Carl Zeiss Meditec, Dublin, CA) Showed a Small Full-Thickness Macular Hole in the Right Eye; Visual Acuity Was 20/70. (D) Stratus OCT Shows Normal Foveal Appearance in the Left Eye. (E) Stratus OCT Radial Scan (representative of 5/6 Radial Scans) Taken 13 Months After Surgery Shows Restored Retinal Anatomy. The Inner-Outer Segment Junction of Photoreceptors Layer Is Interrupted Centrally Indicating Photoreceptor Loss. (F) Horizontal Radial Scan of Stratus OCT Taken 13 Months After Surgery Demonstrates a Prominent Intraretinal Pigment Clump with Shadowing and a Small Full-Thickness Retinal Discontinuity Defect.

Figure 2. A 67-Year-Old Man with Full-Thickness Macular Hole and IMT Type 2 Presented with a Recent Decrease of Vision in His Right Eye. (A and B) Fundus Photograph Shows Grayish Opacification Around the Fovea with Pigment Clumping Temporal to the Fovea. (C) Stratus OCT III (Carl Zeiss Meditec, Dublin, CA) Showed a Small Full-Thickness Macular Hole in the Right Eye; Visual Acuity Was 20/70. (D) Stratus OCT Shows Normal Foveal Appearance in the Left Eye. (E) Stratus OCT Radial Scan (representative of 5/6 Radial Scans) Taken 13 Months After Surgery Shows Restored Retinal Anatomy. The Inner-Outer Segment Junction of Photoreceptors Layer Is Interrupted Centrally Indicating Photoreceptor Loss. (F) Horizontal Radial Scan of Stratus OCT Taken 13 Months After Surgery Demonstrates a Prominent Intraretinal Pigment Clump with Shadowing and a Small Full-Thickness Retinal Discontinuity Defect.

The patient underwent a 25-gauge pars plana vitrectomy, membrane peeling with indocyanine green staining, fluid–air exchange, and 16% C3F8 gas injection in the right eye. Postoperative face-down positioning was performed. Two weeks postoperatively, Stratus OCT III demonstrated hole closure; however, 4 months later OCT revealed reopening of the hole where only 1 of 6 radial scans revealed a small full-thickness discontinuity in the neurosensory retina near a large pigment clump (Fig. 2). Due to the limitations of Stratus OCT, it is difficult to deduce the exact retinal anatomy. Uncomplicated cataract surgery was performed 7 months later; however, visual acuity remained unchanged at 20/70. The OCT appearance remained stable for 1 year after surgery. The patient did not want to undergo further surgery.

Discussion

Several case reports of non-surgical management of macular hole associated with idiopathic macular telangiectasia exist. Patel et al.5 reported a 37-year-old man without diabetes mellitus who had a lamellar macular hole in his eye with unilateral idiopathic macular telangiectasia that fits into the category of Yannuzzi’s type 1, aneurysmal telangiectasia. Visual acuity was 6/18 in that eye. Olson and Mandava published a case report of a 60-year-old woman with diabetes mellitus and a history of bilateral idiopathic macular telangiectasia who had a full-thickness macular hole in the right eye with a visual acuity of 20/200.6

Koizumi et al. reported two cases of full-thickness macular hole in the setting of bilateral idiopathic macular telangiectasia with preservation of good visual acuity. Both patients fit into the category of type 2, perifoveal telangiectasia without proliferative changes.4 One patient had a nearly full-thickness macular hole with preservation of only the internal limiting membrane and a 20/60 visual acuity. The other patient had a large full-thickness macular hole with 20/40 visual acuity, which remained stable during 2-year follow-up. Given their relatively preserved visual acuity, no surgical intervention was undertaken in these patients. The fellow eye of each patient showed a prominent inner foveal cavitation under a thin internal limiting membrane. The authors stated that the preservation of good visual acuity in these two patients implied that the holes were the result of lateral separation of the photoreceptors within the fovea and that there could not have been profound photoreceptor atrophy.4 OCTs of these patients did not reveal any traction on the retina, eliminating traction as a contributing factor in pathophysiology of these holes. The authors speculated that primary failure of Muller cone cells present in the central fovea could be responsible for the retinal cavitation. These Muller cells provide primary structural support of the foveola, and it is conceivable that their loss could play a part in the formation of central cavitation.4

The OCTs of patients in the current report did not show vitreofoveal traction in the eyes with full-thickness macular holes. Spectral-domain OCT of the first patient showed preserved external limiting membrane and inner–outer segment junction of photoreceptors lines (Fig. 1). The second patient demonstrated central loss of photoreceptors, which could have contributed to the lack of visual improvement after surgery (Fig. 2E).

We describe two patients with bilateral idiopathic macular telangiectasia type 2 and a full-thickness macular hole in one eye. Due to acutely decreased vision, pars plana vitrectomy with internal limiting membrane peeling and gas injection was performed in both patients. Complete macular hole closure associated with visual acuity improvement of 2 Snellen lines occurred in one of two patients. Unfortunately, the hole reopened in the second patient and he did not desire further surgery. Macular hole surgery may be a reasonable treatment option in patients with a full-thickness macular hole associated with idiopathic macular telangiectasia and significantly reduced vision.

References

  1. Gass JD, Blodi BA. Idiopathic juxtafoveolar retinal telangiectasisa: update of classification and follow-up study. Ophthalmology. 1993;100:1536–1546.
  2. Yannuzzi LA, Bardal AM, Freund KB, Chen KJ, Eandi CM, Blodi B. Idiopathic macular telangiectasia. Arch Ophthalmol. 2006;124:450–460. doi:10.1001/archopht.124.4.450 [CrossRef]
  3. Paunescu LA, Ko TH, Duker JS, et al. Idiopathic juxtafoveal retinal telangiectasis: new findings by ultrahigh-resolution optical coherence tomography. Ophthalmology. 2006;113:48–57. doi:10.1016/j.ophtha.2005.08.016 [CrossRef]
  4. Koizumi H, Slakter JS, Spaide RF. Full-thickness macular hole formation in idiopathic parafoveal telangiectasis. Retina. 2007;27:473–476. doi:10.1097/01.iae.0000246678.93495.2f [CrossRef]
  5. Patel B, Duvall J, Tullo AB. Lamellar macular hole associated with idiopathic juxtafoveolar telangiectasia. Br J Ophthalmol. 1988;72:550–551. doi:10.1136/bjo.72.7.550 [CrossRef]
  6. Olson JL, Mandava N. Macular hole formation associated with idiopathic parafoveal telangiectasia. Graefes Arch Clin Exp Ophthalmol. 2006;244:411–412. doi:10.1007/s00417-005-0057-9 [CrossRef]
Authors

From the Department of Ophthalmology (NG, HWF), Bascom Palmer Eye Institute, University of Miami Miller School of Medicine; and Miami Veterans Affairs Medical Center (NG), Miami, Florida.

Supported in part by an unrestricted grant from Research to Prevent Blindness, New York, New York.

The authors have no financial or proprietary interest in the materials presented herein.

Address correspondence to Ninel Gregori, MD, Bascom Palmer Eye Institute, 900 NW 17th Street, Miami, FL 33136. E-mail: ngregori@med.miami.edu

10.3928/15428877-20100726-10

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