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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
While the white blood cell count, erythrocyte sedimentation rate, and C-reactive protein tests may be abnormal and there may be abnormally high levels of platelets in the blood or too few red blood cells in the blood, none of these findings is a reliable indicator of the disease. A slit-lamp examination is essential. Recent work has suggested that high-resolution MRI and antibodies to inner ear antigens may be helpful. Cogan syndrome can occur in children, and is particularly difficult
to recognize in that situation.
The cornerstone of diagnosis is an accurate history, and a good clinical examination of the eye, to eliminate traumatic uveitis. Ultrasonography is a useful tool, as it can detect a thickened iris, but only in the hands of an expert.
Due to the different underlying causes, proper diagnosis, treatment, and prognosis can only be determined by an eye care professional. Punctate epithelial erosions may be treated with artificial tears. In some disorders, topical antibiotic is added to the treatment. Patients should discontinue contact lens wear until recovery.
According to Mackie's classification, neurotrophic keratitis can be divided into three stages based on severity:
1. "Stage I:" characterized by alterations of the corneal epithelium, which is dry and opaque, with superficial punctate keratopathy and corneal oedema. Long-lasting neurotrophic keratitis may also cause hyperplasia of the epithelium, stromal scarring and neovascularization of the cornea.
2. "Stage II:" characterized by development of epithelial defects, often in the area near the centre of the cornea.
3. "Stage III:" characterized by ulcers of the cornea accompanied by stromal oedema and/or melting that may result in corneal perforation.
To detect "Acanthamoeba" on a contact lens in a laboratory, the contact lens is placed on a non-nutrient agar saline plate seeded with a gram-negative bacteria such as "E. coli". If "Acanthamoeba" are present, they will reproduce readily and become visible on the plate under 10-20X objective on an inverted microscope. Polymerase chain reaction can also be used to confirm a diagnosis of "Acanthamoeba" keratitis, especially when contact lenses are not involved. "Acanthameoba" is also characterized by a brawny edema and hazy view into the anterior chamber. Late stages of the disease also produces a ring shaped corneal ulcer. Signs and symptoms include severe pain, severe keratitis (similar to stromal herpetic disease), corneal perineuritis, and ring ulcer (late in the disease process).
A physician often can diagnose ichthyosis by looking at the skin. A family history is very useful. In some cases, a skin biopsy is done to help to confirm the diagnosis. In some instances, genetic testing may be helpful in making a diagnosis. Diabetes has not been definitively linked to acquired ichthyosis or ichthyosis vulgaris; however, there are case reports associating new onset ichthyosis with diabetes.
Ichthyosis has been found to be more common in Native American, Asian, Mongolian groups. There is no way to prevent ichthyosis.
Ichthyosis is a genetically and phenotypically heterogeneous disease that can be isolated and restricted to the skin manifestations or associated with extracutaneous symptoms. One of which is limb reduction defect known as CHILD syndrome; a rare inborn error of metabolism of cholesterol biosynthesis that is usually restricted to one side of the body. A research done in Egypt proved that it is not a child syndrome and discussed all the case report.
A study in Austria found over the course of the testing, a total of 154 cases of "Acanthamoeba" keratitis. The age of the positive tests ranged from 8 to 82 years old and 58% of the people were female. The data showed that 89% of the infected patients were contact lens wearers and 19% required a corneal transplant.
A specific clinical diagnosis of HSV as the cause of dendritic keratitis can usually be made by ophthalmologists and optometrists based on the presence of characteristic clinical features. Diagnostic testing is seldom needed because of its classic clinical features and is not useful in stromal keratitis as there is usually no live virus. Laboratory tests are indicated in complicated cases when the clinical diagnosis is uncertain and in all cases of suspected neonatal herpes infection:
- Corneal smears or impression cytology specimens can be analyzed by culture, antigen detection, or fluorescent antibody testing. Tzanck smear, i.e.Papanicolaou staining of corneal smears, show multinucleated giant cells and intranuclear inclusion bodies, however, the test is low in sensitivity and specificity.
- DNA testing is rapid, sensitive and specific. However, its high cost limits its use to research centers.
- Demonstration of HSV is possible with viral culture.
- Serologic tests may show a rising antibody titer during primary infection but are of no diagnostic assistance during recurrent episodes.
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.
Treatment of herpes of the eye is different based on its presentation: epithelial keratitis is caused by live virus while stromal disease is an immune response and metaherpetic ulcer results from inability of the corneal epithelium to heal:
Horses that suffer from this disease can never be considered cured, although they can be managed by careful use of the therapy described above, and fast detection of new flare-ups. If the disease is not properly treated, it will eventually lead to blindness.
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)
Keratitis–ichthyosis–deafness syndrome (also known as "Erythrokeratodermia progressiva Burns," "Ichthyosiform erythroderma, corneal involvement, and deafness," and "KID syndrome,") presents at birth/infancy and is characterized by pregressive corneal opacification, either mild generalized hyperkeratosis or discrete erythematous plaques, and neurosensory deafness.
It is caused by a mutation in connexin 26.
Diagnosis is done by direct observation under magnified view of slit lamp revealing the ulcer on the cornea. The use of fluorescein stain, which is taken up by exposed corneal stroma and appears green, helps in defining the margins of the corneal ulcer, and can reveal additional details of the surrounding epithelium. Herpes simplex ulcers show a typical dendritic pattern of staining. Rose-Bengal dye is also used for supra-vital staining purposes, but it may be very irritating to the eyes. In descemetoceles, the Descemet's membrane will bulge forward and after staining will appear as a dark circle with a green boundary, because it does not absorb the stain. Doing a corneal scraping and examining under the microscope with stains like Gram's and KOH preparation may reveal the bacteria and fungi respectively. Microbiological culture tests may be necessary to isolate the causative organisms for some cases. Other tests that may be necessary include a Schirmer's test for keratoconjunctivitis sicca and an analysis of facial nerve function for facial nerve paralysis.
For more severe disease, oral corticosteroids may be necessary to reduce the inflammatory response. When large amounts of steroids are required or if the disease is severe and is not responding to steroid therapy, other immunosuppressive medications often are recommended. These immunosuppressive drugs include methotrexate, cyclophosphamide, cyclosporine or azathioprine. In some cases, combinations of these medicines are prescribed. Occasionally, if the disease has damaged blood vessels, cochlear implantation may
need to be done to correct the problem.
Cinnarizine is mainly used to treat nausea and vomiting associated with motion sickness, vertigo, Ménière's disease, or Cogan's syndrome. Studies have shown it to produce significant improvement in hearing loss in some patients.
Although corneal abrasions may be seen with ophthalmoscopes, slit lamp microscopes provide higher magnification which allow for a more thorough evaluation. To aid in viewing, a fluorescein stain that fills in the corneal defect and glows with a cobalt blue-light is generally instilled first.
A careful search should be made for any foreign body, in particular looking under the eyelids. Injury following use of hammers or power-tools should always raise the possibility of a penetrating foreign body into the eye, for which urgent ophthalmology opinion should be sought.
A positive VDRL of Treponema pallidum immobilization test confirms diagnosis of luetic(syphilitic) interstitial keratitis
Scleritis is best detected by examining the sclera in daylight; retracting the lids helps determine the extent of involvement. Other aspects of the eye exam (i.e. visual acuity testing, slit lamp examination, etc.) may be normal. Scleritis may be differentiated from episcleritis by using phenylephrine or neosynephrine eye drops, which causes blanching of the blood vessels in episcleritis, but not in scleritis.
Ancillary tests CT scans, MRIs, and ultrasonographies can be helpful, but do not replace the physical examination.
The diagnosis is made by an ophthalmologist/optometrist correlating typical history, symptoms and signs. Many times it may be missed and misdiagnosed as bacterial ulcer. A definitive diagnosis is established only after a positive culture report (lactophenol cotton blue, calcoflour medium), typically taking a week, from the corneal scraping. Recent advances have been made in PCR ref 3./immunologic tests which can give a much quicker result.
In advanced stages, corneal neovascularization can threaten eyesight, which is why routine (annual) eye exams are recommended for contact lens patients.
Despite the temporary nature of the vision loss, those experiencing amaurosis fugax are usually advised to consult a physician immediately as it is a symptom that may herald serious vascular events, including stroke. Restated, “because of the brief interval between the transient event and a stroke or blindness from temporal arteritis, the workup for transient monocular blindness should be undertaken without delay.” If the patient has no history of giant cell arteritis, the probability of vision preservation is high; however, the chance of a stroke reaches that for a hemispheric TIA. Therefore, investigation of cardiac disease is justified.
A diagnostic evaluation should begin with the patient's history, followed by a physical exam, with particular importance being paid to the ophthalmic examination with regards to signs of ocular ischemia. When investigating amaurosis fugax, an ophthalmologic consult is absolutely warranted if available. Several concomitant laboratory tests should also be ordered to investigate some of the more common, systemic causes listed above, including a complete blood count, erythrocyte sedimentation rate, lipid panel, and blood glucose level. If a particular cause is suspected based on the history and physical, additional relevant labs should be ordered.
If laboratory tests are abnormal, a systemic disease process is likely, and, if the ophthalmologic examination is abnormal, ocular disease is likely. However, in the event that both of these routes of investigation yield normal findings or an inadequate explanation, noninvasive duplex ultrasound studies are recommended to identify carotid artery disease. Most episodes of amaurosis fugax are the result of stenosis of the ipsilateral carotid artery. With that being the case, researchers investigated how best to evaluate these episodes of vision loss, and concluded that for patients ranging from 36–74 years old, "...carotid artery duplex scanning should be performed...as this investigation is more likely to provide useful information than an extensive cardiac screening (ECG, Holter 24-hour monitoring, and precordial echocardiography)." Additionally, concomitant head CT or MRI imaging is also recommended to investigate the presence of a “clinically silent cerebral embolism.”
If the results of the ultrasound and intracranial imaging are normal, “renewed diagnostic efforts may be made,” during which fluorescein angiography is an appropriate consideration. However, carotid angiography is not advisable in the presence of a normal ultrasound and CT.
Dyschromatosis symmetrica hereditaria (also known as "reticulate acropigmentation of Dohi", and "symmetrical dyschromatosis of the extremities") is a rare autosomally inherited dermatosis. It is characterized by progressively pigmented and depigmented macules, often mixed in a reticulate pattern, concentrated on the dorsal extremities. It presents primarily in the Japanese, but has also been found to affect individuals from Europe, India and the Caribbean.
Depending on severity, therapies may range from topical or oral anti-inflammatories to irrigation and surgical repair.
Punctate epithelial erosions may be seen with different disorders:
- Rosacea
- Dry-eye syndrome
- Blepharitis
- Acute bacterial conjunctivitis
- Trauma
- Exposure keratopathy from poor eyelide closure
- Ultraviolet or chemical burn
- Contact lens-related disorder such as toxicity or tight lens syndrome
- Trichiasis
- Entropion or ectropion
- Floppy eyelid syndrome
- Chemotherapy i.e. cytosine arabinoside
- Thygeson's Superficial Punctate Keratopathy