What is Schwartz-Matsuo Syndrome?
Schwartz-Matsuo syndrome is a rare eye condition where there’s increased pressure within the eye, known as intraocular pressure (IOP), and issues with the retina, the innermost layer of the eye, detaching. The retina is made up of many complex cells.
This condition is believed to be caused by a separation of the retina – specifically, a rhegmatogenous retinal detachment, where the retina breaks, typically at the edge, near the ora serrata or non-pigmented epithelium of the pars plana or pars plicata. This broken retina allows fluid and the outer parts of photoreceptor cells to move into the front part of the eye. This blocks the flow of the eye’s natural fluid at the trabecular meshwork, a network of tiny channels that drain these fluids from the eye, and as a result, increases the pressure within the eye.
The symptoms of this syndrome can range from pain in one eye and a decrease in clear vision, to losing peripheral (side) vision because of the increased eye pressure and retinal detachment. For a diagnosis of Schwartz-Matsuo syndrome to be made, three conditions need to be met: there are cells in the front chamber of the eye, the retina is detaching, and the pressure inside the eye is elevated.
What Causes Schwartz-Matsuo Syndrome?
Schwartz-Matsuo syndrome is a condition that occurs when the retina, a layer at the back of the eye, detaches. This detaching action puts pressure on the eye (increases IOP) in part because light receptors (photoreceptor outer segments) move from the break into the front of the eye (anterior chamber). Why it happens often relates to what can cause retinal detachments.
Several common factors can contribute to a retinal detachment. These include:
* Injury to the eye
* Having had eye surgery that involves the retina
* Tears or breaks in the peripheral part of the retina
* A degenerative eye condition known as “lattice degeneration”
* Extreme nearsightedness, also known as “pathological myopia”
* Having undergone cataract surgery, which is an operation to treat clouding of the eye lens
* If someone in one’s family has a history of retinal detachment.
Risk Factors and Frequency for Schwartz-Matsuo Syndrome
Schwartz-Matsuo syndrome involves a series of events that are set off by a condition known as RRD. While RRD is quite common and typically occurs in two age groups (20 to 30-year-olds with severe nearsightedness and older people due to a condition called posterior vitreous detachment), it doesn’t always result in Schwartz-Matsuo syndrome. Schwartz-Matsuo syndrome itself is fairly rare and information about it is typically gathered from individual case studies.
Signs and Symptoms of Schwartz-Matsuo Syndrome
Schwartz-Matsuo syndrome is a condition where a person might experience symptoms like eye pain, sensitivity to light (photophobia), blurred vision, headaches, nausea, or vomiting, typically due to increased internal eye pressure, or IOP. Other symptoms tied to retinal detachment might include a history of eye injuries, recent eye surgery, seeing floaters or flashing lights (photopsia), loss of peripheral vision, or a decrease in overall sight.
Mechanical eye trauma is a common reason for retinal detachment, leading to Schwartz-Matsuo syndrome. Retinal breaks, or tears in the retina, after such trauma can often cause this type of retinal detachment. There are two primary ways that such trauma can lead to retinal detachment:
- A direct hit to the eyeball causing a tear or break in the retina
- A penetrating injury to the back of the eye
The former often leads to retinal dialysis, a type of tear in the retina, and other irregular breaks. However, retinal detachment can also occur due to a severe penetration injury to the back of the eye, which would likely require immediate medical attention.
It’s important to note that trauma-related retinal detachment often does not show immediate signs, making it hard to diagnose early. Late symptom presentation, delayed medical assistance, or other distracting injuries can cause a delay in treatment. That’s why it’s crucial to communicate all the conditions and symptoms to the healthcare provider as soon as possible.
Additionally, the medical history should also capture potential other causes of increased eye pressure such as a family history of glaucoma, a history of ocular hypertension, anterior uveitis, inflammations inside the eye, or other relevant health issues, including cardiovascular diseases, high blood pressure, diabetes, and prolonged use of certain medications.
Diagnosing Schwartz-Matsuo syndrome might entail ruling out medical conditions leading to ocular inflammation and eye pain, such as inflammatory bowel disease, ankylosing spondylitis, reactive arthritis, psoriatic arthritis, sarcoidosis, systemic lupus erythematosum, and several infections including HIV/AIDS.
Infected eyes are generally marked by the presence of white blood cells and protein accumulation in the eye’s anterior chamber. This condition resembles Schwartz-Matsuo syndrome but usually has additional symptoms that are typically not seen in Schwartz-Matsuo syndrome. Increased eye pressure is found in several such conditions.
Consequently, it’s vital to check for other possible causes of increased eye pressure in patients presenting with symptoms tied to anterior uveitis, as it is a crucial differential diagnosis of Schwartz-Matsuo syndrome. The examination might include measuring the eye pressure, conducting a thorough eye examination, and checking for retinal detachment. It’s also crucial to rule out other causes of high eye pressure. Notably, both retinal detachment and changes in the eye’s angle – due to blunt trauma to the eye – can happen at the same time, leading to Schwartz-Matsuo syndrome.
The diagnostic criteria for Schwartz-Matsuo syndrome typically consists of having increased eye pressure, retinal detachment, and having a type of white blood cell called an aqueous cell in the anterior chamber of the eye.
Testing for Schwartz-Matsuo Syndrome
Schwartz-Matsuo syndrome is a condition that doctors typically diagnose by looking for certain symptoms. However, for a definite diagnosis, they need to check for specific cells using a technique called transmission electron microscopy (or TEM). This method involves viewing tiny parts of your body’s cells. In Schwartz-Matsuo syndrome, doctors will check your eyes (specifically the front part called the anterior chamber) for a certain type of cell, known as photoreceptor outer segments.
As the name suggests, photoreceptors are cells in your eyes that take in light and help you see. An electron micrograph (a sort of image made using the TEM) in Schwartz-Matsuo syndrome often shows tiny bits of pigment and cell fragments within a part of your eye called the trabecular meshwork.
These photoreceptor cells have different inner structures, depending on whether they are rods or cones (the two types of photoreceptors). The TEM image of rod cells show small component parts (or ‘lobules’) containing structures called rod sacs- these have two thick membranes with a less packed space inside. However, TEM images of cone cells don’t show these lobules, instead, we see a continuous fold of the membrane that looks like a stack.
However, not all practices and hospitals can access the TEM, as it is an advanced piece of equipment. In such situations, doctors use a device known as a slit lamp with special color filters to help make a diagnosis. These filters help to identify different types of cells that might show up because of the condition- this includes white blood cells, red blood cells, pigment cells, and photoreceptors.
A commonly used option is the ‘green’ filter. This filter uses a specific light wavelength to make it easier to see blood and blood vessels against the background. It is particularly useful in diagnosing Schwartz-Matsuo syndrome. The green filter allows doctors to see white blood cells in the anterior chamber, while reducing the visibility of red blood cells and pigmented cells, which might interfere with the analysis. Photoreceptor cells also blend into the background under the green filter because of the pigments in them.
Treatment Options for Schwartz-Matsuo Syndrome
For those suffering from Schwartz-Matsuo syndrome, the definitive solution is a surgical procedure that involves reattaching the retina. This can be achieved through a process called retinopexy, using a device called a scleral buckle, or via a method known as pars plana vitrectomy. Doctors will also clean out debris from the front part of the eye during this procedure.
Since this disease is rare, there isn’t much information on how patients fare after the treatment. To manage the symptoms, doctors may recommend a variety of medications to lower the pressure inside the patient’s eye. These medications include prostaglandin agonists, beta-blockers, selective alpha-2 receptor agonists or topical carbonic anhydrase inhibitors. These medications are used to control the pressure in the eye while waiting for surgery, but their effectiveness is still unclear.
However, surgery to reattach the retina has been proven to normalize the pressure within the eye and prevent further vision loss for those with Schwartz-Matsuo syndrome. It’s also important to note that corticosteroids, a type of medication often used to reduce inflammation, have no role in the treatment of Schwartz-Matsuo syndrome.
What else can Schwartz-Matsuo Syndrome be?
When trying to diagnose Schwartz-Matsuo syndrome, doctors need to distinguish it from a few different conditions:
- Open-angle glaucoma (a type of eye disease)
- Anterior uveitis (eye inflammation)
- Posner-Schlossman syndrome (rare eye condition)
- Uveitic glaucoma (a kind of eye disorder)
- Cluster headache (severe headaches that occur in clusters)
Therefore, it’s important to accurately identify the condition to ensure the suitable treatment.
What to expect with Schwartz-Matsuo Syndrome
Finding out about a disease early and quickly managing high eye pressure (IOP) and a detached retina is crucial for a good outcome. If a retina is successfully reattached through surgery, it can help lower high eye pressure. This happens because it prevents further interaction between the underneath part of the retina (subretinal space) and the fluid in the eye (aqueous humor), which can lead to a better prognosis. Generally, if surgery is done soon enough, the prognosis is good.
Possible Complications When Diagnosed with Schwartz-Matsuo Syndrome
In Schwartz-Matsuo syndrome, problems may occur due to sudden increases in eye pressure and a separation of the retinal layer from the back of the eye. Serious rise in this pressure can lead to loss of sight and even blindness. This can get even worse due to a condition called glaucoma, needing more medical treatment or surgeries.
Potential Complications of Schwartz-Matsuo Syndrome:
- Sudden increases in eye pressure
- Separation of the retinal layer from the back of the eye
- Loss of sight
- Blindness
- Complications due to glaucoma
- Requirement for further medical treatment or surgeries
Recovery from Schwartz-Matsuo Syndrome
After surgery for Schwartz-Matsuo syndrome, which is similar to procedures for repairing a retinal detachment, it’s important to closely keep track of your eye pressure (IOP). Once the surgery is finished, doctors often provide eye-drop steroids, antibiotics, and cycloplegics (medicine to relax your eye muscles) to prevent any increase in inflammation and infection. Any signs of worsened vision, eye pain, headache, nausea, or vomiting could mean that your eye pressure is increasing. This is a serious situation and you should see a doctor right away.
Preventing Schwartz-Matsuo Syndrome
It’s crucial for patients to understand the risks of delaying treatment for Schwartz-Matsuo syndrome. Delayed treatment can lead to further complications, including permanent vision loss caused by optic nerve damage or detachments in the retina (the part of your eye that sends images to your brain) that are not repaired. Following surgery, patients need to make regular visits to their eye doctor to check for any new instances of the retina detaching.
