Coat’S Disease: Effects, Symptoms, And Therapies Essay

Coat’S Disease: Effects, Symptoms, And Therapies Essay

There are many conditions that can occur within the eye that oftentimes influence an effected person’s everyday life. In understanding these conditions as a nurse, you can provide better care for that patient even if they aren’t there being treated for their eye. This article looked specifically at Coat’s disease.

Coat’s disease has a rare occurrence and is recognized as abnormalities to the normative development of the retinal vasculature. Majority of the time Coat’s disease only affects one eye (Perrone, et al. , 2016). Coat’S Disease: Effects, Symptoms, And Therapies Essay. Pathologically speaking, the disease process of Coat’s disease breaks down the blood-retinal barrier at the endothelium. This results in the leaking of plasma into the vessel wall which then become necrotic and unmanaged. This leakage also results in swelling of the retina. There are pericytes and endothelial cells present that are abnormal in retinal blood vessels. These abnormal cells decline rapidly and lead to retinal vasculature that is abnormal and are seen closing vessels, forming aneurysms, and ischemia.

As a nurse, recognizing the signs and symptoms that Coat’s disease may present can help you identify a need for a referral to an ophthalmologist. If while examining a child for other medical purposes you notice a loss in parallax and depth perception, leukocoria, decline of peripheral or central eyesight, or strabismus it would be good nursing judgement to investigate these symptoms further and possibly further recommend to an ophthalmologist. Recognizing these signs could encourage an early diagnosis, which has a direct correlation on an increasing chance of a positive prognosis.

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The timeline for Coat’s disease is often unpredictable but early and accurate diagnosis and consistent therapy are often related to positive outcomes. If left untreated total retinal detachment and neovascular glaucoma can occur. It is also imperative that a nurse is able to recognize that continued advancement of this disease can result in neovascular glaucoma where certain medications may increase intraocular pressure and cause more harm. When speaking with a patient who has Coat’s disease, it is important to identify which eye is affected and stand on or approach a person on their unaffected eye to allow the patient to see you.

There are a few therapies available for someone managing Coat’s disease. These include, laser photocoagulation, cryotherapy, scleral bulking, vitrectomy, and anti-vascular endothelial growth factor injections. According to the research performed, laser photocoagulation as well as cryotherapy seem to signify the most effective therapy towards decreasing sight-threatening progression related to posterior pole retinal detachment. Coat’S Disease: Effects, Symptoms, And Therapies Essay.

With all therapies offered, long term follow-up is a critical component of managing this progressive disease and reducing further effects on the eye. Complications of this disease can progress from retinal detachment, cataract development, iridocyclitis, rubeosis iridis, and even a blind eye that is painful resulting in enucleation.

Coats disease is a rare eye disorder involving abnormal development of blood vessels in the retina. Located in the back of the eye, the retina sends light images to the brain and is essential to eyesight.

In people with Coats disease, retinal capillaries break open and leak fluid into the back of the eye. As fluid builds up, the retina begins to swell. This can cause the partial or complete detachment of the retina, leading to decreased vision or blindness in the affected eye.

Most of the time, the disease affects only one eye. It’s usually diagnosed in childhood. The exact cause isn’t known, but early intervention may help save your vision.

What are the signs and symptoms?

Signs and symptoms usually begin in childhood. They may be mild at first, but some people have severe symptoms right away. Signs and symptoms include:

  • yellow-eye effect (similar to red eye) that can be seen in flash photography
  • strabismus, or crossed eyes
  • leukocoria, a white mass behind the lens of the eye
  • loss of depth perception
  • deterioration of vision

Later symptoms may include:

  • reddish discoloration of the iris
  • uveitis, or eye inflammation
  • retinal detachment
  • glaucoma
  • cataracts
  • eyeball atrophy

Symptoms usually occur in only one eye, although it can affect both. Coat’S Disease: Effects, Symptoms, And Therapies Essay.

Stages of Coats disease

Coats disease is a progressive condition that’s divided into five stages.

Stage 1

In early-stage Coats disease, the doctor can see that you have abnormal blood vessels, but they haven’t started to leak yet.

Stage 2

The blood vessels have begun to leak fluids into the retina. If the leak is small, you might still have normal vision. With a larger leak, you might already be experiencing severe vision loss. The risk of retinal detachment grows as fluids accumulate.

Stage 3

Your retina is either partially or totally detached.

Stage 4

You’ve developed increased pressure in the eye, called glaucoma.

Stage 5

In advanced Coats disease, you’ve completely lost vision in the affected eye. You may have also developed cataracts (clouding of the lens) or phthisis bulbi (atrophy of the eyeball). Coat’S Disease: Effects, Symptoms, And Therapies Essay.

Who gets Coats disease?

Anyone can get Coats disease, but it’s quite rare. Fewer than 200,000people in the United States have it. It affects males more than females by a ratio of 3-to-1.

Average age at diagnosis is 8 to 16 years. Among children with Coats disease, about two-thirds have had symptoms by age 10. About one-thirdof people with Coats disease are 30 or older when symptoms start. Coat’S Disease: Effects, Symptoms, And Therapies Essay.

It doesn’t appear to be inherited or have any link to race or ethnicity. The direct cause of Coats disease hasn’t been determined.

How is it diagnosed?

If you (or your child) have symptoms of Coats disease, see your doctor right away. Early intervention might save your vision. Also, symptoms can mimic those of other conditions, such as retinoblastoma, which can be life-threatening.

Diagnosis is made after thorough ophthalmic examination, plus review of symptoms and health history. Diagnostic testing may include imaging tests such as:

  • retinal fluorescein angiography
  • echography
  • CT scan
How is it treated?

Coats disease is progressive. With early treatment, it’s possible to restore some vision. Some treatment options are:

Laser surgery (photocoagulation)

This procedure uses a laser to shrink or destroy blood vessels. Your doctor may perform this surgery at an outpatient facility or in an office setting.

Cryosurgery

Imaging tests help guide a needle-like applicator (cryoprobe) that produces extreme cold. It’s used to create a scar around abnormal blood vessels, which helps stop further leakage. Here’s how to prepare and what to expect during recovery. Coat’S Disease: Effects, Symptoms, And Therapies Essay.

Intravitreal injections

Under a local anesthetic, your doctor can inject corticosteroids into your eye to help control inflammation. Anti-vascular endothelial growth factor (anti-VEGF) injections can reduce the growth of new blood vessels and ease swelling. Injections can be given in your doctor’s office.

Vitrectomy

This is a surgical procedure that removes the vitreous gel and provides improved access to the retina. Learn more about the procedure what to do while recovering.

Scleral buckling

This procedure reattaches the retina and is usually performed in a hospital operating room.

Whatever treatment you have, you’ll need careful monitoring.

In the final stage of Coats disease, atrophy of the eyeball may result in surgical removal of the affected eye. This procedure is called enucleation.

Outlook and potential complications

There’s no cure for Coats disease, but early treatment can improve your chances of retaining your eyesight.

Most people respond well to treatment. But about 25 percent of people experience continued progression that leads to removal of the eye. Coat’S Disease: Effects, Symptoms, And Therapies Essay.

The outlook is different for everybody, depending on stage at diagnosis, rate of progression, and response to treatment.

Coats disease is an eye disorder characterized by abnormal development of the blood vessels in the retina (retinal telangiectasia). Most people begin showing symptoms in childhood. Early signs and symptoms vary but may include vision loss, “crossed eyes” (strabismus), and a white mass in the pupil behind the lens of the eye (leukocoria). Over time, Coats disease may also lead to retinal detachment, glaucoma, and clouding of the lens of the eye (cataracts). In most cases, only one eye is affected. Coats disease is not inherited and the underlying cause is not known. It has been theorized that some cases may be due to somatic mutations (acquired, not inherited) in the NDP gene. Treatment depends on the symptoms present and may include cryotherapy and laser photocoagulation (alone or in combination), steroids for inflammation, and/or surgery for retinal detachment.[1][2]  Coat’S Disease: Effects, Symptoms, And Therapies Essay.
Last updated: 8/17/2017

What are the stages of Coats disease?

The most recently proposed staging classification for Coats disease was developed on based in a large series of affected people. This classification may help in choosing the most appropriate course of treatment as well as predicting the long-term outcome (prognosis) for people with Coats disease:[3][4]

  • Stage 1 – retinal telangiectasia only (dilation of capillaries in the retina)
  • Stage 2 – telangiectasia and exudation (escape of fluids and material from blood vessels into surrounding tissues)
    • A – extrafoveal exudation (exudation outside of the fovea, which is a small area in the retina responsible for the acute vision)
    • B – foveal exudation (exudation in the fovea)
  • Stage 3 – exudative retinal detachment
    • A – subtotal (partial) detachment
      • 1 – extrafoveal
      • 2 – foveal
    • B – total retinal detachment
  • Stage 4 – total retinal detachment and glaucoma
  • Stage 5 – advanced end-stage disease (defined as a blind, non-painful eye with a total retinal detachment, often with cataract and phthisis bulbi)

How might Coats disease be treated?

Treatment for Coats disease depends on the severity in each person.[1] The following treatments (used alone or in combination) may be tried: Coat’S Disease: Effects, Symptoms, And Therapies Essay.

  • Laser photocoagulation (uses a laser to shrink or destroy blood vessels)
  • Cryotherapy (a procedure that uses extreme cold to destroy abnormal blood vessels)
  • Intravitreal corticosteroid injections to control inflammation
  • Anti-vascular endothelial growth factor (anti-VEGF) injections

More advanced disease with extensive retinal detachment may also require surgical interventions such as vitrectomy, scleral buckling to correct a detached retina, and external drainage of fluids.[5]

What is the long-term outlook for people with Coats disease?

The prognosis varies in each case. Factors that effect the prognosis include the stage of disease at the time of diagnosis, the age at diagnosis, the rate of disease progression, and the effectiveness of treatment.

In some older children and young adults, spontaneous regression (improvement of symptoms) has been reported. Younger children typically have more aggressive disease and often have a total retinal detachment by the time they are diagnosed; these children usually have a poor visual outcome.[4] Some people present with advanced disease which does not benefit from treatment, while others show disease progression despite treatment.[5] While most people respond well to treatment, approximately 25% will become worse and require removal of the eye.[5][4] An ophthalmologist with knowledge about Coats disease may be able to make a general prediction about the chances of retaining the eye and preserving vision. Coat’S Disease: Effects, Symptoms, And Therapies Essay.

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The extent of visual impairment varies considerably from person to person. Favorable visual outcomes are more likely to be achieved with early detection and treatment, with combined therapies as needed. Even when treatment is not able to restore sight, it is beneficial in saving the eye.[5]

We hope this information is helpful. We strongly recommend you discuss this information with your doctor. If you still have questions, please contact us.

Warm regards,
GARD Information Specialist

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  1. Coats Disease. NORD. 2017; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/837/viewFullReport.
  2. Hubbard B, Laird P. Coats disease. Orphanet. July, 2013; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=190.
  3. Del Longo A. Coats disease. Orphanet Encyclopedia. September 2004; http://www.orpha.net/data/patho/Pro/en/Coats-FRenPro1645.pdf.
  4. Shields JA, Shields CL, Honavar SG, Demirci H, Cater J. Classification and management of Coats disease: the 2000 Proctor Lecture. American Journal of Ophthalmology. May 2001; 131(5):572-583.
  5. Cebeci Z, Bayraktar S, Yilmaz YC, Tuncer S, Kir N. Evaluation of Follow-Up and Treatment Results in Coats’ Disease. Turk J Ophthalmol. October. Coat’S Disease: Effects, Symptoms, And Therapies Essay.
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