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Keratoglobus continues to be a somewhat mysterious disease, but it can be successfully managed with a variety of clinical and surgical techniques. The patient is at risk for globe perforation because the thinned out cornea is extremely weak.
The center of the cornea shows normal thickness, with an intact central epithelium, but the inferior cornea exhibits a peripheral band of thinning, to about 1–2 mm. The portion of the cornea that is immediately adjacent to the limbus is spared, usually a strip of about 1–2 mm. In PMD we can see high against the rule astigmatism along with horizontal bow ties. The inferior peripheral thinning is seen between the 4 o'clock and 8 o'clock positions.
PMD lacks apical corneal scarring, Rizutti's phenomenon, Munson's sign, and the central corneal thickness is usually normal.
The gold standard diagnostic test for PMD is corneal topography. However, it may not as specific as corneal pachymetry, because corneal topography only evaluates the degree and distribution of surface irregularities on the cornea, not the thickness of the cornea. Corneal topography may show a "crab claw-like" appearance, a finding that is seen in both keratoconus and in pellucid marginal degeneration. Thus, if corneal topography is used for diagnosis, it should be in conjunction with clinical findings of peripheral, inferior corneal thinning.
DLK is usually seen after refractive surgery. Neutrophils infiltrate the corneal stroma in a diffuse, multifocal pattern. Infiltration is confined to the surgical flap interface with no posterior or anterior extension, and overlying epithelium most often remains intact. As it is a sterile process, cultures based on swab tests are negative.
Pterygium (conjunctiva) can be diagnosed without need for a specific exam, however corneal topography is a practical test (technique) as the condition worsens.
The erosion may be seen by an eye doctor using the magnification of a biomicroscope or slit lamp. Usually fluorescein stain must be applied first and a cobalt blue-light used, but may not be necessary if the area of the epithelial defect is large. Optometrists and ophthalmologists have access to the slit lamp microscopes that allow for this more-thorough evaluation under the higher magnification. Mis-diagnosis of a scratched cornea is fairly common, especially in younger patients.
The diagnosis of episcleritis is based upon the history and physical examination. The history should be explored for the presence of the diseases associated with episcleritis, and the symptoms they cause, such as rash, arthritis, venereal disease, and recent viral infection. Episcleritis may be differentiated from scleritis by using phenylephrine or neosynephrine eye drops, which causes blanching of the blood vessels in episcleritis, but not in scleritis. A blue color to the sclera suggests scleritis, rather than episcleritis.
After anesthetizing the eye with medication, the conjunctiva may be moved with a cotton swab to observe the location of the enlarged blood vessels.
The incidence and prevalence of PMD are unknown, and no studies have yet investigated its prevalence or incidence. However, it is generally agreed that PMD is a very rare condition. Some uncertainty regarding the incidence of PMD may be attributed to its confusion with keratoconus. PMD is not linked to race or age, although most cases present early in life, between 20 and 40 years of age. While PMD is usually considered to affect men and women equally, some studies suggest that it may affect men more frequently.
Several diseases have been observed in patients with PMD. However, no causal relationships have been established between any of the associated diseases and the pathogenesis of PMD. Such diseases include: chronic open-angle glaucoma, retinitis pigmentosa, retinal lattice degeneration, scleroderma, kerato-conjunctivitis, eczema, and hyperthyroidism.
NK is diagnosed on the basis of the patient's medical history and a careful examination of the eye and surrounding area.
With regard to the patient's medical history, special attention should be paid to any herpes virus infections and possible surgeries on the cornea, trauma, abuse of anaesthetics or chronic topical treatments, chemical burns or, use of contact lenses. It is also necessary to investigate the possible presence of diabetes or other systemic diseases such as multiple sclerosis.
The clinical examination is usually performed through a series of assessments and tools:
- General examination of cranial nerves, to determine the presence of nerve damage.
- Eye examinations:
1. Complete eye examination: examination of the eyelids, blink rate, presence of inflammatory reactions and secretions, corneal epithelial alterations.
2. Corneal sensitivity test: performed by placing a cotton wad or cotton thread in contact with the corneal surface: this only allows to determine whether corneal sensitivity is normal, reduced or absent; or using an esthesiometer that allows to assess corneal sensitivity.
3. Tear film function test, such as Schirmer's test, and tear film break-up time.
4. Fluorescein eye stain test, which shows any damage to the corneal and conjunctival epithelium
The diagnosis of Reis-Bücklers corneal dystrophy is based on the clinical presentation, rather than labs or imaging. Sometimes it is difficult to distinguish the disease from honeycomb dystrophy.
Treatment includes the use of protective eye glasses. A number of surgical options are also available.
Further progression of the disease usually leads to a need for corneal transplantation because of extreme thinning of the cornea. Primarily, large size penetrating keratoplasty has been advocated.
Recent additions of techniques specifically for keratoglobus include the "tuck procedure", whereby a 12 mm corneo-scleral donor graft is taken and trimmed at its outer edges. A host pocket is formed at the limbal margin and the donor tissue is "tucked" into the host pocket.
A pinguecula is one of the differential diagnoses for a limbal nodule. It may have an increased prevalence in Gaucher's disease.
Corneal and Retinal Topography: computerized tests that maps the surface of the retina, or the curvature of the cornea.
Fluorescein Angiogram: evaluation of blood circulation in the retina.
Dilated Pupillary Exam: special drops expand the pupil, which then allows doctors to examine the retina.
Slit-Lamp Exam: By shining a small beam of light in the eye, eye doctors can diagnose cataracts, glaucoma, retinal detachment, macular degeneration, injuries to the cornea, and dry eye disease.
Ultrasound: Provides a picture of the eye’s internal structure, and can evaluate ocular tumors, or the retina if its suffering from cataracts or hemorrhages.
Since the condition appears to slowly subside or diminish on its own, there are no specific treatments for this condition available.
Some precautions include regular visits to an ophthalmologist or optometrist and general testing of the pupil and internal eye through fundamental examinations (listed below). The examinations can determine if any of the muscles of the eye or retina, which is linked to the pupil, have any problems that could relate to the tadpole pupil condition.
As it is associated with excessive sun or wind exposure, wearing protective sunglasses with side shields and/or wide brimmed hats and using artificial tears throughout the day may help prevent their formation or stop further growth. Surfers and other water-sport athletes should wear eye protection that blocks 100% of the UV rays from the water, as is often used by snow-sport athletes. Many of those who are at greatest risk of pterygium from work or play sun exposure do not understand the importance of protection.
Pingueculae may enlarge slowly over time, but are a benign condition, usually requiring no treatment. Artificial tears may help to relieve discomfort, if it occurs. If cosmesis is a concern, surgical excision is sometimes done. Occasionally, a pinguecula may become inflamed, a condition called pingueculitis. The cause of pingueculitis is unknown and there are no known infectious agents associated with it. If an inflamed pinguecula is causing discomfort or cosmetic concerns, it may be treated with an anti-inflammatory agent, such as prednisolone drops.
Conjunctival concretions can be seen easily by everting the eyelid. The projecting concretions should be removed. Removal is easily performed by a doctor. For example, using needles or sharp knife removes the concretion, under a local light anesthesia of the conjunctiva.
Depending on severity, therapies may range from topical or oral anti-inflammatories to irrigation and surgical repair.
Episcleritis is a benign, self-limiting condition, meaning patients recover without any treatment. Most cases of episcleritis resolve within 7–10 days. The nodular type is more aggressive and takes longer to resolve. Although rare, some cases may progress to scleritis. However, in general, episcleritis does not cause complications in the eye. Smoking tobacco delays the response to treatment in patients with episcleritis.
The diagnosis is clinical. The intraocular pressure (IOP) can be measured in the office in a conscious swaddled infant using a Tonopen or hand-held Goldmann tonometer. Usually, the IOP in normal infants is in the range of 11-14 mmHg. Buphthalmos and Haab's striae can often be seen in case of congenital glaucoma.
Given that episodes tend to occur on awakening and managed by use of good 'wetting agents', approaches to be taken to help prevent episodes include:
- Environmental:
- ensuring that the air is humidified rather than dry, not overheated and without excessive airflow over the face. Also avoiding irritants such as cigarette smoke.
- use of protective glasses especially when gardening or playing with children.
- General personal measures:
- maintaining general hydration levels with adequate fluid intake.
- not sleeping-in late as the cornea tends to dry out the longer the eyelids are closed.
- Pre-bed routine:
- routine use of long-lasting eye ointments applied before going to bed.
- occasional use of the anti-inflammatory eyedrop FML (prescribed by an ophthalmologist or optometrist) before going to bed if the affected eye feels inflamed, dry or gritty
- use of a hyperosmotic (hypertonic) ointment before bed reduces the amount of water in the epithelium, strengthening its structure
- use the pressure patch as mentioned above.
- use surgical tape to keep the eye closed (if Nocturnal Lagophthalmos is a factor)
- Waking options:
- learn to wake with eyes closed and still and keeping artificial tear drops within reach so that they may be squirted under the inner corner of the eyelids if the eyes feel uncomfortable upon waking.
- It has also been suggested that the eyelids should be rubbed gently, or pulled slowly open with your fingers, before trying to open them, or keeping the affected eye closed while "looking" left and right to help spread lubricating tears. If the patient's eyelids feel stuck to the cornea on waking and no intense pain is present, use a fingertip to press firmly on the eyelid to push the eye's natural lubricants onto the affected area. This procedure frees the eyelid from the cornea and prevents tearing of the cornea.
Treatment options include contact lenses and intrastromal corneal ring segments for correcting refractive errors caused by irregular corneal surface, corneal collagen cross-linking to strengthen a weak and ectatic cornea, or corneal transplant for advanced cases.
Prior to any physical examination, the diagnosis of keratoconus frequently begins with an ophthalmologist's or optometrist's assessment of the person's medical history, particularly the chief complaint and other visual symptoms, the presence of any history of ocular disease or injury which might affect vision, and the presence of any family history of ocular disease. An eye chart, such as a standard Snellen chart of progressively smaller letters, is then used to determine the person's visual acuity. The eye examination may proceed to measurement of the localized curvature of the cornea with a manual keratometer, with detection of irregular astigmatism suggesting a possibility of keratoconus. Severe cases can exceed the instrument's measuring ability. A further indication can be provided by retinoscopy, in which a light beam is focused on the person's retina and the reflection, or reflex, observed as the examiner tilts the light source back and forth. Keratoconus is amongst the ophthalmic conditions that exhibit a scissor reflex action of two bands moving toward and away from each other like the blades of a pair of scissors.
If keratoconus is suspected, the ophthalmologist or optometrist will search for other characteristic findings of the disease by means of slit lamp examination of the cornea. An advanced case is usually readily apparent to the examiner, and can provide for an unambiguous diagnosis prior to more specialized testing. Under close examination, a ring of yellow-brown to olive-green pigmentation known as a Fleischer ring can be observed in around half of keratoconic eyes. The Fleischer ring, caused by deposition of the iron oxide hemosiderin within the corneal epithelium, is subtle and may not be readily detectable in all cases, but becomes more evident when viewed under a cobalt blue filter. Similarly, around 50% of subjects exhibit Vogt's striae, fine stress lines within the cornea caused by stretching and thinning. The striae temporarily disappear while slight pressure is applied to the eyeball. A highly pronounced cone can create a V-shaped indentation in the lower eyelid when the person's gaze is directed downwards, known as Munson's sign. Other clinical signs of keratoconus will normally have presented themselves long before Munson's sign becomes apparent, and so this finding, though a classic sign of the disease, tends not to be of primary diagnostic importance.
A handheld keratoscope, sometimes known as "Placido's disk", can provide a simple noninvasive visualization of the surface of the cornea by projecting a series of concentric rings of light onto the cornea. A more definitive diagnosis can be obtained using corneal topography, in which an automated instrument projects the illuminated pattern onto the cornea and determines its topography from analysis of the digital image. The topographical map indicates any distortions or scarring in the cornea, with keratoconus revealed by a characteristic steepening of curvature which is usually below the centreline of the eye. The technique can record a snapshot of the degree and extent of the deformation as a benchmark for assessing its rate of progression. It is of particular value in detecting the disorder in its early stages when other signs have not yet presented.
Once keratoconus has been diagnosed, its degree may be classified by several metrics:
- The steepness of greatest curvature from 'mild' ( 52 D);
- The morphology of the cone: 'nipple' (small: 5 mm and near-central), 'oval' (larger, below-center and often sagging), or 'globus' (more than 75% of cornea affected);
- The corneal thickness from mild (> 506 μm) to advanced (< 446 μm).
Increasing use of corneal topography has led to a decline in use of these terms.
Prophylaxis consists of periodic administration of Vitamin A supplements. WHO recommended schedule, which is universally recommended is as follows:
- Infants 6–12 months old and any older children weighing less than 8 kg - 100,000 IU orally every 3–6 months
- Children over 1 year and under 6 years of age - 200,000 IU orally every 6 months
- Infants less than 6 months old, who are not being breastfed - 50,000 IU orally should be given before they attain the age of 6 months
There are a number of different treatments to deal with TSPK. Symptoms may disappear if untreated, but treatment may decrease both the healing time and the chances of remission.
- PRK laser eye surgery may cure this disease (NOTE: A full clinical study has not been done, but a case study of one person was reported in 2002 PRK-pTK as a treatment).
- Artificial tear eye-drops or ointments may be a suitable treatment for mild cases.
- Low-dosage steroidal eye-drops, such as prednisone, fluorometholone, loteprednol (Lotemax 0.5%) or rimexolone. Steroidal drops should be used with caution and the eye pressure should be regularly checked during treatment.
- Soft contact lenses.
- Ciclosporin is an experimental treatment for TSPK. It is usually used during transplants as it reduces the immune system response.
- Tacrolimus (Protopic 0.03% ointment) is also an experimental treatment.
- Laser eye treatment.
- Amniotic membrane (Case Study)