Journal of Pediatric Ophthalmology and Strabismus

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Short Subjects 

Spontaneous Regression in Two Unusual Cases of Advanced Coats’ Disease

Sengul C. Ozdek, MD; Tugba Erdinc, MD; Burak Kagnici, MD

Abstract

The authors present two cases of advanced Coats’ disease having total exudative retinal detachment, in which the disease regressed spontaneously. The patients were a 4-year-old girl and 3-year-old boy with total retinal detachment with widespread vascular anomalies. In the follow-up, fundus examination revealed spontaneous regression. At this stage, photocoagulation and cryotherapy were applied. In case 1, the eye was stable during 2 months but recurrence started thereafter, which ended with phthisis. In case 2, the retina was still attached 6 months following surgery. The authors emphasize the possibility of spontaneous regression, even in advanced Coats’ disease.

Abstract

The authors present two cases of advanced Coats’ disease having total exudative retinal detachment, in which the disease regressed spontaneously. The patients were a 4-year-old girl and 3-year-old boy with total retinal detachment with widespread vascular anomalies. In the follow-up, fundus examination revealed spontaneous regression. At this stage, photocoagulation and cryotherapy were applied. In case 1, the eye was stable during 2 months but recurrence started thereafter, which ended with phthisis. In case 2, the retina was still attached 6 months following surgery. The authors emphasize the possibility of spontaneous regression, even in advanced Coats’ disease.

From Gazi University, School of Medicine, Ophthalmology Department, Ankara, Turkey.

Presented as a free paper at the 42nd National Conference of the Turkish Ophthalmology Society, November 19–23, 2008, Antalya, Turkey.

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

Address correspondence to Sengul Ozdek, MD, 30. cad. No: 20/4 Doga sitesi, 100.YIL 06530, Ankara, Turkey.

Received: January 07, 2009
Accepted: October 08, 2009
Posted Online: May 21, 2010

Introduction

Coats’ disease is a distinct clinical entity characterized by idiopathic retinal telangiectasia and retinal exudation. It is usually unilateral, occurs mostly in boys (mainly in the first decade of life), and can cause severe visual loss resulting from exudative retinal detachment.1–4 As the disease advances, accumulation of cholesterol crystals starts and exudative retinal detachment develops. Also, rubeosis iridis and secondary neovascular glaucoma may worsen the clinical progress of the disease. Shields et al. classified Coats’ disease into 5 stages, with stage 1 involving retinal telangiectasia only and stage 5 being the most complicated form of the disease, with total retinal detachment, cataract, and phthisis bulbi.4 Therapy in the early stages includes laser photocoagulation and cryotherapy. Therapy in the end stages involves enucleation if severe glaucoma and pain are present.1,2,4 The result of the natural progression in advanced Coats’ disease is the development of a blind, painful eye. Although rare, some cases with spontaneous regression have been reported in the literature.5,6 Coats’ disease in older children and young adults is generally less aggressive and has better prognosis than usual.4 That is why a spontaneous regression can be expected in such cases. We report two cases of unilateral stage 4 and 3B Coats’ disease having a total exudative retinal detachment in whom retinal detachment regressed spontaneously.

Case Reports

Case 1

A 4-year-old girl presented to our clinic with red eye, leukocoria, and loss of vision in her left eye that was noticed by her family for the past 2 months. Visual acuity was 20/20 in the right eye and hand motions in the left eye. Intraocular pressure (IOP) was measured to be 14 mm Hg in the right eye and 32 mm Hg in the left eye with Goldmann applanation tonometer. There was an afferent pupillary defect in the left eye. Slit-lamp examination revealed conjunctival hyperemia, one quadrant of rubeosis iridis on the pupillary margin, and a highly elevated exudative retinal detachment that could easily be seen behind the lens (Fig. 1A). There were widespread vascular anomalies including telangiectasia, tortuosity, venous beading, sausage-like segmental dilatations, and subretinal yellow exudation in all quadrants of the highly elevated retina. The fundus examination of the right eye was normal. No mass lesion or calcification was noted with the B-mode ultrasonography. A clinical diagnosis of stage 4 Coats’ disease that was thought to be inoperable was made. The family was informed about the disease prognosis and enucleation was proposed later when a disturbing ocular pain started. Topical antiglaucoma drops (dorzolamide-timolol combination) were prescribed to control IOP and cyclopentolate and dexamethasone were used to prevent any inflammation, which could be induced by rubeosis iridis.

(A) Fundus Photography of the Left Eye of Case 1 at Presentation. The Retina is Just Behind the Lens. Severe Vascular Anomalies (multiple Telangiectasia, Vascular Tortuosity, Venous Beading, Sausage-Like Segmental Dilatations, and Subretinal Yellow Exudation in All Quadrants) are Evident. (B) Fundus Photography of the Left Eye 3 Months Later. Retina was Reattached with Limited Shallow Retinal Detachment and Aneurysms Left only in the Inferior Temporal Quadrant. There was Widespread Subretinal Yellow Hard Exudate and Normal Caliber Vessels.

Figure 1. (A) Fundus Photography of the Left Eye of Case 1 at Presentation. The Retina is Just Behind the Lens. Severe Vascular Anomalies (multiple Telangiectasia, Vascular Tortuosity, Venous Beading, Sausage-Like Segmental Dilatations, and Subretinal Yellow Exudation in All Quadrants) are Evident. (B) Fundus Photography of the Left Eye 3 Months Later. Retina was Reattached with Limited Shallow Retinal Detachment and Aneurysms Left only in the Inferior Temporal Quadrant. There was Widespread Subretinal Yellow Hard Exudate and Normal Caliber Vessels.

After a 3-month follow-up period, the patient returned with a quiet eye for the control visit. The visual acuity was increased to counting fingers from 7 feet, IOP was normalized to 19 mm Hg with medication, and there was no conjunctival hyperemia and rubeosis iridis on slit-lamp examination. Examination of the fundus revealed spontaneous regression of the retinal detachment with resolution of almost all of the subretinal exudative fluid with limited shallow retinal detachment left only in the inferior temporal quadrant. There was widespread subretinal yellow hard exudate and normal caliber vessels (Fig. 1B).

The patient was thought to benefit from ablation of the leaking telangiectatic vessels at this stage, and panretinal photocoagulation (to the attached retinal areas) and cryotherapy (to the inferior temporal quadrant where the retina is shallowly detached) were applied to the left eye under general anesthesia at the same session. The eye was stable during the first 2 months following surgery, but exudation started to increase and caused exudative retinal detachment to recur slowly thereafter, which resulted in phthisis bulbi 6 months postoperatively. We could not perform any more surgery because the family preferred to wait for a new spontaneous resolution. There was no light perception with severe ocular hypotonia (could not be measured), ectropion uvea, evident rubeosis iridis, and exudative retinal detachment during the last examination.

Case 2

A 3-year-old boy was referred to our clinic because of an exudative retinal detachment. The parents’ chief complaint was exodeviation of the right eye. He had no significant medical or surgical history; even his family history was unremarkable. On examination, the patient’s best-corrected visual acuity was light perception in the right eye and 20/20 in the left eye. Light reflexes were normal and there was no afferent pupillary defect in the right eye. He had 30 prism diopters of sensory exotropia of the right eye without light fixation. Extraocular movements of the eyes were normal. Slit-lamp examination was within normal limits. IOP was measured to be 14 mm Hg in the right eye and 17 mm Hg in the left eye. Dilated fundus examination of the left eye was normal. Evaluation of the right eye revealed a highly elevated total retinal detachment that could be seen immediately posterior to the lens with slit-lamp biomicroscopy, with characteristic telangiectatic tortuous vessels and several intraretinal hemorrhages (Fig. 2A).

(A) Initial Presentation of the Right Eye in Case 2. Total Retinal Detachment that can be Seen Immediately Posterior to the Lens, Vascular Anomalies, and Yellow Subretinal Fluid. (B) Fundus Photography of the Right Eye 6 Months Later. The Retina was Almost Totally Attached. Limited Shallow Exudative Retinal Detachment at the Superotemporal Quadrant was Seen. (C) Fundus Photography of the Right Eye 6 Months Postoperatively. The Retina was Totally Attached. Note the Laser Scars and Few Subretinal Exudates.

Figure 2. (A) Initial Presentation of the Right Eye in Case 2. Total Retinal Detachment that can be Seen Immediately Posterior to the Lens, Vascular Anomalies, and Yellow Subretinal Fluid. (B) Fundus Photography of the Right Eye 6 Months Later. The Retina was Almost Totally Attached. Limited Shallow Exudative Retinal Detachment at the Superotemporal Quadrant was Seen. (C) Fundus Photography of the Right Eye 6 Months Postoperatively. The Retina was Totally Attached. Note the Laser Scars and Few Subretinal Exudates.

An extensive medical work-up including consultations to pediatric oncology, orbital ultrasonography, and computed tomography did not reveal any evidence of retinoblastoma. He was diagnosed as having stage 3B Coats’ disease and observed without treatment because the family did not favor any surgical intervention. There was no change in the findings until the sixth month, when almost total regression of the retinal detachment occurred. There was only a limited shallow exudative retinal detachment at the superotemporal quadrant and some subretinal exudation associated with limited abnormal vessels superiorly (Fig. 2B). At this stage, panretinal laser photocoagulation was offered. Laser photocoagulation was applied to the attached retinal areas of the right eye under general anesthesia. The retina was still attached at the 6-month visit following surgery (Fig. 2C).

Discussion

Coats’ disease is a relatively uncommon disease and the degree of clinical involvement varies from case to case. Shields et al. defined Coats’ disease as idiopathic retinal telangiectasia with intraretinal and/ or subretinal exudation without appreciable retinal or vitreal traction in a review of 150 consecutive patients.3 They classified Coats’ disease into stage 1, telangiectasia only; stage 2, telangiectasia and exudation (2A, extrafoveal exudation; 2B, foveal exudation); stage 3, exudative retinal detachment (3A, subtotal; 3B, total); stage 4, total detachment and secondary glaucoma; and stage 5, advanced end-stage disease.4 This classification is useful in forming a treatment plan and prognosis prediction. They reported the risk factors predictive of poor visual outcome (20/200 or worse) as postequatorial, diffuse, or superior location of the telangiectasias and exudation, failed resolution of subretinal fluid after treatment, and presence of retinal macrocysts. Patients with stages 4 and 5 were reported to have a poor visual prognosis. The main risk factors for enucleation were elevated IOP (greater than 22 mm Hg) and iris neovascularization.4

Our two cases were classified as stage 4 and 3B disease, and many cases with these stages (78% of stage 4 and 11% of stage 3B) have been reported to need enucleation.4 Although there are five reported cases with spontaneous regression in the literature, they were thought to have had a form of Coats’ disease that had better prognosis than usual.5–7 Deutsch et al. documented spontaneous regression of the telangiectasias in older children and young adults.5 Coats’ disease generally has a more aggressive clinical course in younger children.3,8

Our cases seem to be unique because they presented with advanced diseases in young children that regressed spontaneously without treatment. Exudative retinal detachment recurred in case 1 and progressed to phthisis even after treatment. Because we were not able to perform fluorescein angiography before or during surgery, panretinal photocoagulation was considered as a treatment modality. We performed panretinal photocoagulation together with direct ablation of the aneurysmal vessels instead of only direct ablation of the aneurysmal vessels because of possible existing diffuse capillary nonperfusion areas. Even in case 2 when abnormal vessels were confined to superior quadrants, we performed panretinal photocoagulation due to similar reasons and an expectation of poor prognosis. Although we did not hesitate while planning cryotherapy in case 1, we wonder what the natural course would have been if we had not performed the surgery.

Similarly, Gass described an adult patient who developed total retinal detachment and massive peri-retinal proliferation after cryotherapy to peripheral telangiectasis in an eye with 20/20 visual acuity initially.9 The recurrence of the telangiectasia and retinal exudation was reported in 7% of cases after a mean of 10 years preceding successful treatment.3 For this reason, it is important to realize that patients undergoing treatment need close follow-up and may require additional treatments, depending on clinical progress.

We would like to emphasize that, although rare, the possibility of spontaneous regression should be kept in mind in the end stages of the Coats’ disease, even in young children, and should be considered before performing enucleation. However, this regression may be transient, as in our first case.

References

  1. Mandava N, Yannuzzi LA. Miscellaneous retinal vascular conditions. In: Regillo CD, Brown GC, Flynn HW, eds. Vitreoretinal Disease, The Essentials. New York: Thieme; 1999:196–200.
  2. Haller JA. Coats’ disease. In: Ryan SJ, ed. Retina, 3rd ed. St. Louis: Mosby; 2001:1441–1448.
  3. 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]
  4. Shields JA, Shields CL, Honavar SG, Demirci H, Cater J. Classification and management of Coats disease: the 2000 Proctor Lecture. Am J Ophthalmol. 2001;131:572–583. doi:10.1016/S0002-9394(01)00896-0 [CrossRef]
  5. Deutsch TA, Rabb MF, Jampol LM. Spontaneous regression of retinal lesions in coats disease. Can J Ophthalmol. 1982;17:169–172.
  6. Wolfe JD, Hubbard GB III, . Spontaneous regression of subretinal exudate in Coats’ disease. Arch Ophthalmol. 2006;124:1208–1209. doi:10.1001/archopht.124.8.1208 [CrossRef]
  7. Campbell FP. Coats’ disease and congenital vascular retinopathy. Trans Am Ophthalmol Soc. 1976;24:365–372.
  8. Char DH. Coats’ syndrome: long term follow up. Br J Ophthalmol. 2000;84:37–39. doi:10.1136/bjo.84.1.37 [CrossRef]
  9. Gass JDM. Stereoscopic Atlas of Macular Disease: Diagnosis and Treatment, 4th ed. St. Louis: Mosby; 1997:500.
Authors

From Gazi University, School of Medicine, Ophthalmology Department, Ankara, Turkey.

Presented as a free paper at the 42nd National Conference of the Turkish Ophthalmology Society, November 19–23, 2008, Antalya, Turkey.

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

Address correspondence to Sengul Ozdek, MD, 30. cad. No: 20/4 Doga sitesi, 100.YIL 06530, Ankara, Turkey.

10.3928/01913913-20100318-05

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