What is Uveitis Glaucoma Hyphema Syndrome?
Uveitis Glaucoma Hyphema (UGH) syndrome, also known as Ellingson syndrome, is a condition that can happen after getting an implant put inside your eye (intraocular implant). The implant can rub against and damage the iris, which is the colored part of your eye. This can lead to a range of effects, including internal bleeding in your eye (hyphema) and increased pressure inside your eye. UGH syndrome involves three aspects: uveitis, glaucoma, and hyphema.
Uveitis is inflammation or swelling of the uvea, which includes the iris, ciliary body, and choroids – various components of your eye. Infections, autoimmune diseases, and injuries can all potentially cause uveitis. Hyphema and uveitis can both result from high pressure inside the eye. Glaucoma often results from increased eye pressure, and it can lead to loss of vision, damage to the optic nerve (the nerve that connects the eye to the brain), and thinning of the retinal nerve fiber layer, which helps send visual information to the brain.
Complications from uveitis can lead to secondary glaucoma, which can contribute to UGH Syndrome. Hyphema is when there’s a buildup of blood in the front part of the eye, and it can be a side effect of an eye injury or conditions like glaucoma or uveitis.
The mechanics of UGH syndrome involve interplay between uveitis, glaucoma, and hyphema. The inflammation caused by uveitis can interfere with how eye fluids drain, leading to more pressure inside the eye. Hyphema only increases this pressure because the blood can block the outflow path and worsen the inflammation. Certain implants can cause UGH syndrome more frequently because of their shape and placement.
UGH syndrome can happen right away or develop over several years, causing the vision in the affected eye to decrease. It can lead to temporary or slowly declining vision loss. The eye might look swollen and have increased pressure. Blood or blood clots might be visible in the lower front part of the eye, and the eye’s response to inflammation may also be seen. If left untreated, it may cause optic nerve damage due to glaucoma.
To diagnose UGH syndrome, doctors will consider your medical history, conduct an examination, and perform certain tests. The most reliable test for detecting UGH syndrome is ultrasound biomicroscopy. Other methods used may include a slit-lamp exam to get a closer look at your eye, tests to measure your eye pressure, and ultrasound imaging to visualize any bleeding in the eye. They may also use a technique known as optical coherence tomography to examine the front and back parts of your eye. The treatment options include removing or replacing the implant or using anti-inflammatory eye drops.
What Causes Uveitis Glaucoma Hyphema Syndrome?
UGH syndrome is a condition that can cause bleeding in the front part of the eye, inflammation of the middle layer of the eye, and a type of eye disease known as glaucoma due to a rise in eye pressure. It’s often linked to issues that crop up after eye surgery, particularly cataract removal. This surgery can cause minor trauma to the iris and ciliary body (part of the eye that produces eye fluid), leading to inflammation. This can break the barrier between the blood and eye fluid, resulting in inflammation of the eye’s middle layer (uveitis). Hyphema, or bleeding in the front of the eye, can also happen due to mechanical stress caused by an artificial lens, especially if it is incorrectly positioned or rubs against the iris. All these factors can contribute to an increase in eye pressure, which can lead to glaucoma. Variations in the front part of the eye and certain anatomical predispositions may also be connected to UGH syndrome.
Dealing with UGH syndrome requires a comprehensive approach that considers both pressure and inflammation-related issues. Anti-inflammatory drugs, glaucoma medications, and surgical treatments are used to restore the health of the eye.
Most commonly, UGH syndrome is caused by the rubbing of the iris from the lens placed in the front part of the eye after cataract surgery. It can also happen due to any artificial lens. Problems often arise when the edge of the lens rubs against the root of the iris, causing repeated inflammation, high eye pressure, and bleeding in the front part of the eye. Removing the lens usually solves the problem. Some types of lenses have been associated with UGH syndrome due to their design and manufacturing issues.
Different artificial lenses, including those supported by the iris with metal loops, can cause UGH syndrome. Instances occur when lenses are wrongly made, incorrectly placed, or poorly fitted, leading to rubbing that causes the syndrome. Other surgical devices have also been found to cause mechanical irritation leading to UGH syndrome.
Almost all kinds of lenses or implants inside the eye can be linked with UGH syndrome. Conditions such as pseudoexfoliation syndrome (looseness and flaking of lens fibre due to age) or use of blood thinners may increase the chance of developing UGH syndrome. Some reports suggest that late-onset of UGH syndrome can be caused by a cataract condition that leads to a shift of the implanted lens, leading to it touching the iris. However, other studies show that placing a specific type of lens in the eye may result in good vision outcomes and acceptable complication rates.
Risk Factors and Frequency for Uveitis Glaucoma Hyphema Syndrome
The number of people experiencing UGH syndrome has gone down because of better control of the quality of Intraocular Lenses (IOLs), improved materials used in manufacturing, and advancements in manufacturing techniques and lens designs. The UGH syndrome is linked to the touch of the iris-ciliary by parts of the IOLs, which is believed to trigger the syndrome by releasing certain inflammatory substances.
UGH syndrome is mostly found in adults and children after they’ve had cataract surgery. Over years, the number of UGH syndrome cases dropped from 2.2% to 1.2% within a year. It’s usually connected with the use of first-generation IOLs that are placed in the anterior chamber, although it can also be caused by a single-piece IOL in the capsular bag. There was a case of a child with an anterior migration of the PCIOL optic that led to UGH syndrome; however, it vanished after the PCIOL was removed.
Signs and Symptoms of Uveitis Glaucoma Hyphema Syndrome
The UGH syndrome is a problem that can occur after cataract surgery. You might experience episodes of blurry vision, eye pain, light sensitivity, a change in color perception, and red eyes. These episodes of blurred vision or pain tend to recur. However, it’s worth noting that this condition does not usually cause complete vision loss if no severe glaucoma is present.
There are a few variations of the UGH syndrome, such as UGH plus and IPUGH syndrome. UGH plus can result in bleeding in the vitreous part of the eye. IPUGH syndrome involves bleeding into the chamber behind the iris without causing inflammation.
With the help of a slit-lamp exam, the doctor might see some symptoms like redness of the conjunctiva, cells in the anterior chamber of your eye, and microscopic amounts of blood in your eye’s anterior chamber. If you have UGH syndrome, your doctor may also see corneal edema, or corneal swelling, usually caused by the irritated contact with an intraocular lens (IOL).
- Redness of the conjunctiva
- Presence of cells in the anterior chamber of the eye
- Microscopic blood in the anterior chamber (hyphema)
- Corneal edema (swelling of the cornea)
Count on your doctor to conduct a comprehensive examination to accurately diagnose UGH syndrome. They would use a slit-lamp examination to see and evaluate anterior chamber cells, flare, vitreous cells, or any deposits over the IOL. Also, tell your doctor about your symptoms and previous ocular surgeries you’ve had, as this information helps in determining the presence of UGH syndrome and assessing possible complications.
Testing for Uveitis Glaucoma Hyphema Syndrome
The eye health assessments can involve many different methods to diagnose a disease and monitor its progress. One of these methods is ‘anterior segment photography’, which helps to document the illness, track the response to the chosen treatment, and explain the disease and treatment to the patient.
There is also a technique called ultrasound biomicroscopy (UBM), which is handy to diagnose UGH syndrome, a condition linked to trauma in the eye due to an artificial lens. This method can be especially useful for understanding the placement of certain parts of the lens and how they interact with the parts of the eye that surround it.
In addition, a non-invasive assessment called anterior segment optical coherence tomography allows doctors to visualize front chamber of eye structures. If this method doesn’t provide the necessary views, UBM could be used again.
Certain decorative iris implants can lead to severe conditions like UGH syndrome or corneal decompensation, where the transparent front part of the eye (cornea) starts to lose its function. ‘Specular microscopy’ is a useful diagnostic tool for examining the corneal cells and understanding the health status of the cornea. The thickness of the central cornea can also be observed to quantify swelling, which is often associated with UGH syndrome.
Checking for glaucoma, a group of eye conditions that damage the optic nerve, can involve several methods, such as examining the front of the eye chamber, checking the central corneal thickness, mapping the field of vision, and studying images of the optic nerve.
When diagnosing a condition like UGH, it’s critical to rule out all other causes of inflammation in the eye, and the approach to investigate them would differ depending on the individual. The usual tests can include a complete blood count, an erythrocyte sedimentation rate (measuring how fast red blood cells sink in a test tube, indicating the presence of inflammation), testing for the HIV virus, carrying out a skin test for tuberculosis (Mantoux test), autoimmune antibody tests, X-rays of the chest and the joint at the very base of the spine, and looking at the eye from side-to-side and front-to-back.
Optical coherence tomography is also used for the macula, the part of the eye responsible for sharp, central vision. Looking at the macula can help identify a type of eye condition called cystoid macular edema, and a technique called fundus fluorescein angiography tests for other causes of bleeding in the eye or abnormal new blood vessels in the iris. For cases linked to recurring hyphema, or bleeding in the eye, it could be important to research medication history and have a coagulation profile to understand how well the blood clots.
Treatment Options for Uveitis Glaucoma Hyphema Syndrome
If you have UGH Syndrome, doctors commonly reduce the internal eye pressure using medicines both applied directly to the eye and taken by mouth. There are two main types of medications used: antiglaucoma drugs and corticosteroids. The former helps decrease the pressure inside the eye, while the latter helps control inflammation in the front part of the eye.
To help reduce inflammation, topical steroids (medicines applied directly to the eye) are used. The potency and dosage of these are chosen based on how well they can penetrate the cornea (the clear, outer layer of the eye). Additional treatments may include non-steroidal anti-inflammatory drugs and cycloplegics. These medications can relieve symptoms by easing spasms in the eye’s ciliary (muscles that help the eye focus) and reducing inflammation.
It’s advised to avoid miotics (like pilocarpine), as they can increase the irritation of the iris. In some specific cases, like when the implanted lens is causing UGH Syndrome due to pupillary blockage, a laser procedure called peripheral iridotomy may help. But remember, the treatment method may differ from person to person, depending on what’s causing the UGH Syndrome.
In cases where the cause of issues can be pinpointed, more specific treatments may be possible. For instance, in folks with express shunts, laser iridoplasty (a procedure to reshape the iris) might help manage the irritation of the iris. Occasionally, if the doctors identify a blood vessel in the iris causing bleeding, they may use lasers to halt the bleeding.
If standard medical treatments don’t sufficiently calm the inflammation and vision becomes reduced, more direct intervention may be needed. This could involve modifying the implanted lens, repositioning it, or in some cases, removing it entirely. In fact, lens exchange is the definitive treatment for UGH Syndrome when no other conditions explain the patient’s symptoms.
There are also other innovative treatment options. For example, stitching the “arms” of the lens implant to the iris may alleviate UGH Syndrome in patients who experience shaking of the artificial lens. An anti-growth drug called bevacizumab can also be injected into the eye to reduce the frequency of acute UGH Syndrome attacks, regress iris neovascularization (an abnormal growth of blood vessels in the iris), and lessen macular edema (swelling in the macula, an area in the retina).
What else can Uveitis Glaucoma Hyphema Syndrome be?
When determining the cause of UGH syndrome, other medical conditions that can have similar symptoms are often considered. These include:
- Trauma: UGH syndrome can be mistaken for a blunt trauma to the eye.
- Inflammatory Glaucoma: Signs of inflammation (uveitis) are usually present and there may be a history of surgery.
- Hyphematous conditions: Conditions causing abnormal bleeding in the eye such as vascular abnormalities, iris tufts or varices may be confused with UGH syndrome. Herpes zoster and herpes simplex uveitis may also be associated with these conditions.
- Retinal Vascular Occlusions: Doctors should rule out conditions like central retinal venous occlusion if there are new blood vessels growing in the iris and this results in neovascular glaucoma.
- Coagulation disorders and sickle cell disease: These should be ruled out for patients with UGH syndrome.
- Uveitis: Can also mimic UGH syndrome but reactions to this are usually well-controlled with the use of corticosteroids and cycloplegics.
- Retinoblastoma: Some late-stage cases can present as hyphema.
- Chronic postoperative endophthalmitis: Patients may present with similar symptoms but corticosteroids can initially alleviate symptoms.
The presence of hyphema and the touching of the uveal implant by the defect in the iris can favor the diagnosis of UGH syndrome as opposed to these other conditions.
What to expect with Uveitis Glaucoma Hyphema Syndrome
UGH syndrome can often be managed initially by non-surgical methods. However, removing the implanted lenses through surgery is typically the best way to cure it. It’s important to note though that surgery does not always guarantee an end to UGH syndrome.
‘Polymethyl methacrylate’, ‘acrylic’, and ‘silicone’ refer to different types of lens materials. Improvements in the design and quality of these lenses have helped to reduce complications associated with UGH syndrome.
In a large study of 71 patients with UGH syndrome, those who underwent surgical treatment saw better final vision and lower eye pressure. However, patients who were treated non-surgically didn’t enjoy the same improvements in vision or reductions in eye pressure.
Possible Complications When Diagnosed with Uveitis Glaucoma Hyphema Syndrome
UGH syndrome can cause various complications, such as:
- Corneal staining: Long-term hyphema, which is bleeding in the anterior chamber of the eye, can lead to staining of the cornea. It usually begins at the edge of the cornea and expands towards the center.
- Chronic inflammation: This could result in posterior synechia or peripheral anterior synechia, which are abnormal connections between different parts of the eye.
- Pseudophakic bullous keratopathy: This is a condition where the cornea becomes flooded with fluid due to damage to the endothelium, the inner layer of the cornea. This can be caused by consistently high intraocular pressure (IOP), damage from the implant, and inflammation. In severe cases, it may require a corneal transplant.
- Vitreous hemorrhage: In some cases of UGH syndrome, there may be bleeding in the vitreous, the gel-like substance that fills the inside of the eye. This typically happens when there’s a hole or tear in the posterior capsule of the lens.
- Glaucomatous nerve damage: If the intraocular pressure is constantly high, it could damage the optic nerve and eventually lead to blindness.
- Cystoid macular edema: In some cases, this eye condition, which is the presence of multiple cyst-like spaces within the macula, the sensitive area of the retina, might occur.
Preventing Uveitis Glaucoma Hyphema Syndrome
Patients dealing with UGH syndrome should learn about their condition,
including the effects on the eyes, the different treatments available, and
possible complications. Damage caused by complex surgery for cataracts (cloudy lens in the eye) can lead to significant issues regarding eyesight and mental wellness. It’s important for patients to be actively involved and informed about their condition and the advantages of getting treatment in a timely manner. Careful guidance is crucial in these cases, as the method of treatment depends on what exactly is causing the current issue.
Possible treatment options include the removal of the Intraocular Lens (IOL, an artificial lens implanted in the eye), or other surgeries aimed at minimizing the harm. However, all factors must be evaluated beforehand, such as the patient’s ability to lie flat on their back and any additional health conditions that could potentially worsen complications. This helps in strategizing the most effective treatment plan.
