From The Eye Institute, Medical College of Wisconsin, Milwaukee, Wisconsin.
Drs. Wirostko and Connor are supported in part by an unrestricted grant from Research to Prevent Blindness, Inc., New York New York.
The authors have no financial or proprietary interest in the materials presented herein.
Address correspondence to William J. Wirostko, MD, 925 N. 87th Street, Milwaukee, WI 53226.
Coats disease is a well-described, idiopathic condition featuring peripheral vascular telangiectasis, and exudative leakage.1–5 The male to female ratio is 3:1, and patients are typically diagnosed in the first two decades.2,4,5
Coats disease is diagnosed clinically with support from imaging studies. Findings include characteristic, “light bulb” aneurysms on fluorescein angiography (FA); telangiectasis with associated exudative detachments; and yellow, subretinal deposits.2–4 Often, retinoblastoma must be ruled out, especially in younger patients. In these cases, FA can help accentuate the vascular abnormalities typical of Coats disease, whereas ultrasound and CT scans can demonstrate calcifications typical of retinoblastoma.1–4,6
Coats disease typically requires laser or cryotherapy to the regions of abnormal retinal vasculature to avoid disease progression to total retinal detachment.1–5 The RetCam II (Clarity Medical Systems, Pleasanton CA) is a wide-field, fundus imaging system that also allows intraoperative FA imaging to facilitate diagnosis and treatment. Prior reports have described RetCam-obtained FA images in ROP;7–9 to our knowledge this is the first report of its use in Coats Disease.
A 5-year-old girl who failed a pediatric vision screening was sent to a community ophthalmologist, who referred her to our institution for evaluation of Coats disease versus retinoblastoma in her left eye. She had no significant past or family medical history, nor did she have any prior ophthalmic history or symptoms.
On examination her acuity without correction was 20/40 in the right eye and count fingers at three feet in the left eye. Pupil, motility, and slit lamp examinations were normal. Indirect ophthalmoscopy in the clinic was normal for the right eye. The left eye had a large macular detachment with a prominent, yellow, submacular, nodular lesion. In clinic, limited views of the periphery suggested peripheral telangiectasis and exudates. A B-scan ultrasound showed the macular detachment and no calcification in the submacular lesion.
An examination under anesthesia (EUA) was arranged to allow a more thorough examination as well as intraoperative FA images with the RetCam II. Figure 1 shows color photographs obtained with the RetCam II, and Figure 2 shows demonstrative FA images. Not all regions with abnormal vasculature and ischemia were readily apparent on clinical examination or color photography. These regions of latent pathology were clearly seen on the FA, however. In particular, note the region inferior to the cluster of dilated vessels seen temporally in Figure 1. The FA in Figure 2 clearly shows dilated vessels with associated telangiectatic vessels and ischemia in this region. The FA made this region much more apparent intraoperatively. Scatter laser photocoagulation was performed using a diode green laser with treatment boundaries determined by the vascular abnormalities seen on the FA.
Figure 1. RetCam II Color Photographs Obtained During the Examination Under Anesthesia (EUA) Show the Yellow, Submacular Nodule and the Peripheral, Telangiectatic Vessels.
Figure 2. RetCam II Flurescein Angiography (FA) Images Obtained During the Examination Under Anesthesia (EUA) Clearly Demonstrate the Full Extent of Peripheral Telangiectasis, Light Bulb Aneurysms, and Regions of Ischemia.
The patient’s most recent follow-up was 5 months after the procedure. Her examination showed her visual acuity to be unchanged. The submacular exudate was of smaller size, and the peripheral vascular abnormalities had regressed. Figure 3 shows the macula; more peripheral views could not be obtained. The detachment was improved and no further treatment was felt necessary at that time.
Figure 3. Clinical Photo Taken 5 Months After Treatment. The Macular Detachment and Submacular Nodule are Reduced, Though Still Present.
This case demonstrates the intraoperative utility of RetCam II FA imaging to facilitate diagnosis and treatment of Coats disease. Coats disease has well described features to help distinguish it from retinoblastoma, toxocariasis, persistent fetal vasculature, and other disease entities; in younger and more severe presentations, however, these differences may be obscured.2–6,10,11 In this case the patient presented with a large submacular nodule and macular detachment, raising concerns for retinoblastoma. Similar presentations of Coats disease have been previously described, but they are not the classic presentation.3,10 The patient’s age, the yellow color of the nodule, and the peripheral telangiectatic vessels made the diagnosis of Coats disease seem more likely in clinic. In contrast, retinoblastoma classically has a white coloration, with enlarged vessels that may penetrate an endophytic or exophytic mass. However, the clear, intraoperative demonstration of leaking, telangiectatic peripheral vasculature with classic “light bulb” aneurysms allowed certainty in the diagnosis and the ability to avoid serial monitoring with EUA. Such vascular features have been reported in retinoblastoma, but they are atypical.12
The severity of Coats disease is variable. Younger patients and those with macular exudates or large areas of detachment tend to have a more dismal long-term prognosis. Particularly in these severe cases, the primary goal is to prevent total retinal detachment and painful neovascular glaucoma, with vision preservation a secondary goal. Primary treatment with cryotherapy and photocoagulation are advocated, with minimal surgical intervention for retinal detachment, if needed to apply ablative therapies.1,2,4 Multiple treatments may be needed initially, with recurrences possible up to 35 years later in up to a third of patients.2,11,13
More complete initial treatment of abnormal vasculature may decrease the need for initial retreatment. Unfortunately, this proposal would be difficult to test with a prospective study because of the low incidence of Coats disease. Avoiding retreatment would be especially useful in younger patients who require treatment under anesthesia. In this case report, there were large peripheral regions with ischemia and abnormal vasculature that were not readily apparent on clinical examination or in color RetCam photographs. The FA images clearly showed these regions, allowing a much more complete treatment.
The RetCam is a well-established device in pediatric ophthalmology, allowing wide-angle fundus photographs to be captured easily. It can be used in both the clinic and the operating room. The RetCam II, with the addition of FA capability, is a useful aid, both for confirming the diagnosis and for ensuring complete treatment of Coats disease in pediatric patients.
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