A 9-year-old boy presented with a 6-month history of decreased vision in his left eye. The patient’s retinal examination was significant for telangiectatic vessels involving the posterior pole and the superior and inferior post-equatorial regions, circinate intraretinal exudation at the macula, and a full-thickness macular hole that was confirmed by optical coherence tomography. Chronic exudation from the anomalous vessels may be involved in the pathogenesis. Full-thickness macular hole formation may occur in conjunction with Coats’ disease in children, which has not been reported to date, to the best of the authors’ knowledge.
Full-Thickness Macular Hole and Macular Telangiectasia in a Child with Coats’ Disease
From University College of Medical Sciences and GTB Hospital (VK), Delhi; and Guru Nanak Eye Centre (NG, BG, UKR), Maulana Azad Medical College, New Delhi, India.
The authors have no financial or proprietary interest in the materials presented herein.
Address correspondence to Vinod Kumar, MS, MNAMS, FRCS (Glasg), 9/2, Punjabi Bagh Extension, New Delhi - 110026, India. E-mail: email@example.com
Received: June 16, 2009
Accepted: October 21, 2010
Posted Online: December 30, 2010
Coats’ disease has been defined as idiopathic retinal telangiectasia with intraretinal and/or subretinal exudation without appreciable retinal or vitreal traction.1 Although macular telangiectasias are rare, exudation in the presence of peripheral retinal telangiectasias commonly affects the macular region. We report a case of Coats’ disease with macular telangiectasia complicated by a full-thickness macular hole in a child. This association is not commonly recognized. In the absence of vitreomacular traction in Coats’ disease, we hypothesize that chronic macular exudation from telangiectatic vessels may result in cystoid retinal degeneration eventually resulting in a full-thickness macular hole.
A 9-year-old boy presented with the complaint of painless decrease in vision in his left eye for 6 months. There was no history of any trauma. Visual acuity was 20/20 in the right eye and 20/400 in the left eye. Detailed anterior segment examination revealed no abnormality in either eye. The fundus of the right eye was unremarkable.
The fundus of the left eye showed circinate intra-retinal exudation around the macula with a large full-thickness macular hole. There were telangiectatic vessels inferotemporally with pigmentary changes at the temporal aspect of the macula (Fig. A). The superior and inferior post-equatorial regions showed retinal aneurysms, irregularly dilated anomalous retinal vessels, and intraretinal exudation. These findings were confirmed on fundus fluorescein angiography (Figs. B and C). Optical coherence tomography scans were consistent with a full-thickness macular hole. There was no evidence of any vitreomacular traction (Fig. D).
Figure. (A) The Left Eye Shows Circinate Intraretinal Exudation Around the Macula with a Full-Thickness Macular Hole. Telangiectatic Vessels Are Visible Inferiorly and Temporally with Pigmentary Changes. (B) Fluorescein Angiogram Demonstrating Anomalous Dilated Vessels with Saccular Dilatations. Hypofluorescence Demarcates the Area of the Macular Hole. (C) Fluorescein Angiogram Showing Telangiectatic Retinal Vessels with Saccular Dilatations in the Superior Periphery. (D) Vertical Optical Coherence Tomography Scan Through the Center of the Macular Hole Showing Full-Thickness Macular Hole.
The patient was diagnosed as having Coats’ disease. The peripheral anomalous vessels were treated with green laser. The patient’s parents refused surgery in view of the guarded prognosis.
Coats’ disease, first described in 1906,2 is predominantly unilateral and affects males in the first two decades of life. The retinal vascular telangiectasias are more commonly post-equatorial, occur in the inferotemporal quadrant, and are rare in the macular region (1%). However, subretinal and/or intraretinal exudation occurs diffusely; it may be remote from the areas of vascular abnormalities and commonly affects the macula.1 Neovascular glaucoma may complicate chronic exudative retinal detachment and is the most devastating complication. Other posterior segment manifestations include retinal and vitreous hemorrhage, neovascularization of the disc and retina, retinal macrocysts, and secondary vasoproliferative tumors.
Isolated case reports of macular hole (both lamellar and full thickness) associated with Coats’ disease can be found in the literature.3–8 Lamellar macular holes may occur due to chronic macular edema secondary to the chronic macular exudation from the peripheral telangiectatic retinal vessels.7 Only two cases of peripheral retinal telangiectasias with a full-thickness macular hole have been described, and they were both elderly women.8 Although the authors hypothesized that the chronic exudation from the abnormal vessels might cause a change in the vitreous configuration, with secondary vitreomacular traction and full-thickness macular hole formation, the association may be coincidental owing to the frequent occurrence of idiopathic full-thickness macular holes in this demographic profile. Also according to this hypothesis, the simultaneous occurrence of macular holes in Coats’ disease would be much more common than reported, given the frequency of chronic exudation in these cases.
Macular hole formation has been described in conjunction with idiopathic juxtafoveal telangiectasia. Leakage from the abnormal vessels causes intraretinal edema that might induce local destruction of the retina with the formation of cysts and ultimately lamellar holes,9 similar to the mechanism proposed for Coats’ disease. Although full-thickness macular holes have also been associated rarely, they usually have preservation of good visual acuity.10 Absence of structural support afforded by Muller cells in the macula of patients with idiopathic juxtafoveal telangiectasia might lead to lateral separation of photoreceptors resulting in holes. However, lack of photoreceptor atrophy accounts for the good vision even in the presence of full-thickness macular hole.
The occurrence of Coats’ disease with full-thickness macular hole has not been reported in children, irrespective of the presence of macular telangiectasia. In our patient, the hole was large in size and chronic in appearance on clinical examination (Fig. A). With the exception of those consequent to blunt trauma, macular holes are rare in the younger population and thus the association is unlikely to be coincidental. The etiology of macular hole in Coats’ disease has been debated along two general lines: vitreomacular traction versus some form of degenerative dissolution of inner retinal layers in the fovea.11 In the absence of vitreoretinal traction in our case, it seems likely that chronic macular exudation from telangiectatic vessels resulted in cystoid degeneration of the inner retinal layers leading to full-thickness macular hole. As this transformation takes place, the central cystoid spaces disappear and foveal thickness decreases with time; hence thickened retina or macular leakage may not be visible around the hole.
- Shields JA, Shields CL, Honavar SG, Demirci H. Clinical variations and complications of Coats disease in 150 cases: the 2000 Sanford Gifford Memorial Lecture. Am J Ophthalmol. 2001;131:561–571. doi:10.1016/S0002-9394(00)00883-7 [CrossRef]
- Coats G. Forms of retinal disease with massive exudation. Roy Lond Ophthalmol Hosp Rep. 1908;17:440–525.
- Bengisi N. Coats’ disease and a macular hole. Ann Oculist (Paris). 1968;201:158–162.
- Frangieh GT, Green WR, Engel HM. A histopathologic study of macular cysts and holes. Retina. 1981;1:311–336. doi:10.1097/00006982-198101040-00008 [CrossRef]
- Chen C, Wen F, Jiang L, Wu L. A macular hole in a female adult with bilateral coats disease. Yan Ke Xue Bao. 2003;19:168–170.
- Zhao T, Chen Q, Li M, Gu X, Gao R, Wen F. Clinical observation on juvenile macular hole without trauma and hypermyopia. Yan Ke Xue Bao. 2005;21:1–6.
- Ioannidis AS, Liasis A, Sheldrick J, Snead M, Nischal KK. Lamellar macular hole as the presenting feature in a child with Coats’ disease. J Pediatr Ophthalmol Strabismus. 2005;42:378–379.
- Jaycock PD, Thomas D, Zakir R, Laidlaw DA. Retinal telangiectasia in association with macular hole formation. Eye. 2004;18:342–343. doi:10.1038/sj.eye.6700746 [CrossRef]
- 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]
- 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]
- Smiddy WE, Flynn HW Jr, . Pathogenesis of macular holes and therapeutic implications. Am J Ophthalmol. 2004;137:525–537. doi:10.1016/j.ajo.2003.12.011 [CrossRef]