What is Giant Fornix Syndrome?
Giant Fornix Syndrome (GFS) is a rare eye infection that causes inflammation, primarily in the upper parts of your eye (known as superior conjunctival fornices), and less frequently, in the lower parts (inferior conjunctival fornices). Often found in people over 65, GFS typically comes with deep conjunctival fornices (spaces in the eye where your eyelid folds back), recurrent bacterial eye infections, and the weakening of the levator aponeurosis, a muscle that controls eye movement, due to aging.
In healthy South Asian populations, the normal depth of these spaces is 15.3 mm for upper and 10.9 mm for lower fornices. However, with GFS, these depths are abnormally increased.
The condition was first identified in a study by Geoffrey Rose in 2004 using data from 12 elderly patients. Since then, various treatments and results have been documented.
GFS typically affects individuals over 65, with the average and middle age ranging from late seventies to early eighties. Common symptoms include a thick, pus-like discharge from the eye, drooping of the upper eyelid due to inflammation, and long-term eye inflammation, known as chronic conjunctivitis.
The most effective treatment is a combination of different methods, including the use of antimicrobial and antibiotic medicines, steroids, and surgery.
What Causes Giant Fornix Syndrome?
The exact cause of this eye condition is not completely understood. However, it has been suggested that deep pockets in a part of the eye, due to ageing-related changes, might offer a comfortable place for bacteria to live.
Mild eye inflammation, which could be worsened by blocked tear ducts, might result in an excess of bacteria in these pockets, particularly in the top part of the eye. The damage caused by this excess bacteria can lead to an increase in protein-rich discharge from the inflamed eye.
This process can create a cycle where the discharge shelters the bacteria, causing more bacteria to build up. This buildup then causes more inflammation, which results in more discharge. It’s thought that this discharge can shield the bacteria from both cream and pill forms of antibiotics, leading to continued infections that don’t seem to clear up.
Risk Factors and Frequency for Giant Fornix Syndrome
Giant fornix syndrome typically appears in older adults, most commonly in people between 77 and 93 years old. Interestingly, women seem to be more affected by this condition than men. However, it’s important to note that this syndrome is rare and often misdiagnosed, so there isn’t a lot of data about it.
- The age of people developing this syndrome usually ranges from 77 to 93.
- On average, people diagnosed are around 86 years old.
- Women have a higher risk, with studies showing a ratio of ten females to 2 males and in another six females to 2 males.
- While the condition usually affects just one side, it can sometimes affect both.
- Even after treatment, the condition might come back, suggesting that the initial treatment might not have been fully effective.
- Although few cases have been reported, both in Asian and Caucasian populations, more research is needed to understand if other demographic factors can influence the risk of developing the condition.
- One unique case involved a 33-year-old patient who had a history of extensive trauma and surgical intervention.
Signs and Symptoms of Giant Fornix Syndrome
Giant fornix syndrome is marked by prolonged inflammation of the white of the eye (conjunctivitis) and the presence of a lot of yellow, pus-like discharge from the eyes. This is associated with unusually deep spaces in the top or, in some cases, the bottom part of the eye. The patient can experience other symptoms that can include red eyes, pain, unclear vision, drooping eyelids, and a feeling like there is something stuck in the eye.
While the condition usually occurs in one eye, it can affect both eyes. A drooping eyelid might be a result of inflammation in the upper part of the eye. If the condition is not recognized and treated early,
it can lead to severe complications, even if it has gone undiagnosed for a long period. Such complications may include damage to the clear front layer of the eye, scarring of the cornea, entire or partial corneal perforation, reduced eyesight, formation of false membranes, and tiny disease spots on the cornea.
- Prolonged inflammation of the eye whites
- Abundant yellow, pus-like eye discharge
- Unusually deep concave spaces in the eye
- Eye redness
- Eye pain
- Blurred vision
- Drooping eyelids
- Foreign body sensation in the eye
- Corneal damage in severe cases
- Scarring of the cornea
- Corneal perforation
- Reduced eyesight
- Formation of false membranes
- Small disease spots on the cornea
Testing for Giant Fornix Syndrome
CT scans have discovered what seem to be small air pockets in the superior conjunctival fornices, which are the upper corners of the eye where the eyelid and the eyeball meet. Researchers believe this might be a symptom of a condition called giant fornix syndrome. A CT scan can also help determine if there are any foreign objects in the eye or if there’s frontal sinusitis, a type of sinus infection.
In one case, a patient had these air pockets in the eye but didn’t complain of any eye pain, didn’t show any signs of eye inflammation, and didn’t have any other symptoms that might suggest giant fornix syndrome except for the deep corners of the eye. While this individual might have a higher risk of developing giant fornix syndrome, physical examination didn’t provide enough evidence to diagnose them with this condition. This case further shows that, while CT scans can help in diagnosing giant fornix syndrome, they shouldn’t be seen as the ultimate way to confirm this. It’s only when the findings from a physical exam match up with the presence of air pockets detected by a CT scan that there’s strong evidence to diagnose giant fornix syndrome.
Treatment Options for Giant Fornix Syndrome
There are various treatment methods used for certain eye-related diseases, each offering differing levels of successful results. These treatments range from non-invasive methods, like the application of antibiotics and high-dose steroids, to more invasive procedures like surgery. Some treatment methods include:
- Systemically administered antibiotics
- Topically applied antibiotics
- Topically applied steroids
- Diluted antiseptic washes and swabs
- Physical swabbing to reduce number of bacteria
- Injection of antibiotics directly under the conjunctiva (the transparent layer covering the front of the eye)
- Antimicrobial solution rinse
- Eye drops made from a patient’s own blood serum
- Treatment of eyelashes with a weak hypochlorous acid solution
- Surgical removal of part of the diseased tissue
In one study, a condition known as Giant Fornix Syndrome (GFS) was treated using a combination of systemic antibiotics, topical antibiotics, and steroids. Treatment with these types of medicine dramatically prevented reoccurrence of symptoms for the majority of patients, with a few needing to keep using the combined antibiotic/steroid eye drops. A few other patients experienced a reduction in the severity of their condition after the disease was brought under control.
In one case involving a 97-year old woman, the regular application of iodine-based solution (povidone-iodine) using a cotton swab resulted in a significant improvement in her condition. Initially, her condition failed to improve with an antibiotic and steroid-based treatment plan. Even after her treatment plan was modified to include different antibiotics and steroids, her condition got worse. However, after her treatment involved a more aggressive approach using povidone-iodine swabs on the affected area, she was clinically improved and remained infection-free.
Surgery is also an option to manage these types of conditions. They achieve this by removing part of the conjunctiva (the transparent layer covering the front of the eye), thereby reducing the area where bacteria can grow. Results from one study suggest that surgeries resulting in larger resections (removal of tissue) were more successful in completely eliminating symptoms. While there are some concerns about the impact of extensive resection on certain functions of the upper eye lid and tear production, no patients have reported any observable issues post-operation.
What else can Giant Fornix Syndrome be?
Just like Giant Fornix Syndrome, several eye conditions can show up as long-term inflammation of the conjunctiva, also known as conjunctivitis. This can often make it challenging to get the right diagnosis promptly. Here are some conditions that can cause chronic conjunctivitis and might initially look similar:
- Ocular allergies
- Dry eye
- Blepharitis (inflammation of the eyelid)
- Scleritis (inflammation of the white part of the eye)
- Dacryocystitis (infection of the tear sac, usually involving Staphylococcus aureus bacteria)
- Canaliculitis (infection of the tear duct)
- Nasolacrimal duct obstruction (blocked tear duct)
- Lacrimal sac mucocele (cyst in the tear sac)
It might be less likely that the patient is suffering from ocular allergies if they do not have other chronic allergies like asthma or eczema. However, if severe ocular allergies persist, they primarily respond to antihistamine medications. If the treatment isn’t effective, it may exclude allergies, and a bacterial swab test is recommended.
If the swab test comes back showing a substantial presence of bacteria, conditions like Giant Fornix Syndrome and Dacryocystitis should be seriously considered. Dacryocystitis may be ruled out if a patient continues to experience symptoms despite having clear tear ducts after successful tear duct surgery. If the standard treatment for chronic Dacryocystitis doesn’t improve the condition, the patient can then be evaluated for risk factors associated with Giant Fornix Syndrome.
What to expect with Giant Fornix Syndrome
This disease is a recurring, long-term condition that is challenging to manage. How well a patient recovers depends on how early the disease is diagnosed and how well they respond to treatment. If the disease is left untreated, it could lead to serious eye complications such as corneal perforations and ulcers, especially in severe cases.
Your doctor will conduct a comprehensive history taking and physical examination to diagnose this disease. In some instances, CT scans might help confirm the diagnosis, but they cannot be used to exclude conditions like giant fornix syndrome.
For severe cases of the disease, doctors may consider surgery, as it offers the highest chance of symptom relief and the shortest recovery time period. If your symptoms are less severe, your doctor might suggest less invasive procedures to limit potential risks of surgery.
If the disease is mild, it can usually be managed within weeks with a combination of prescribed oral antibiotics and high-dose antibiotic/steroid eye drops. A similar treatment is used for mild to moderate disease, but may require lifelong use of low-dose antibiotic/steroid eye drops to prevent a recurrence. Additionally, cleaning the eyes with povidone-iodine and gently removing clumps with a cotton swab, alongside antibiotics and steroids, can potentially treat or even cure mild, moderate, and potentially severe cases within weeks.
In situations where we resort to surgery, it could involve removing 8-12 mm of the upper eye surface, followed by using a diluted antiseptic and an antibiotic/steroid mix. This surgical procedure has shown high rates of success with a low risk of side effects, based on data from various studies.
Possible Complications When Diagnosed with Giant Fornix Syndrome
Severe cases of eye disease can cause a deep sore, known as a corneal ulcer, and even result in a hole in the cornea. If not caused by an injury, these holes or “corneal perforations” are more challenging to repair successfully. If left unrepaired, these corneal perforations can lead to irreversible eye conditions such as severe glaucoma, a serious eye infection called endophthalmitis, and even blindness.
Problematic Outcomes:
- Deep sore on the cornea (corneal ulcer)
- Hole in the cornea (corneal perforation)
- Challenges in repairing non-injury related corneal perforations
- Unrepaired perforations leading to irreversible glaucoma
- Endophthalmitis (serious eye infection)
- Blindness
Preventing Giant Fornix Syndrome
Giant fornix syndrome is a very rare condition that tends to affect older individuals. Certain physical changes related to the weakening of a muscle in the eyelid, known as the levator aponeurosis, are commonly associated with this condition. Those who have a track record of having infections in the conjunctiva (the clear lining of the inside of your eyelid) should be aware of giant fornix syndrome.
It’s crucial for patients to strictly follow the treatment plan given by their doctor. Regular check-ups are essential to keep track of whether the patient’s condition is improving or worsening. If necessary, additional medical tests can be promptly arranged during these follow-ups. This way, any changes in the patient’s condition will be handled in a timely and effective manner, ensuring the best possible outcome for the patient.
