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neurotrophic keratoconjunctivitis
ICD-10 Codes
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Description
Neurotrophic Keratitis: A Rare Eye Disorder
Neurotrophic keratitis, also referred to as neurotrophic keratopathy or "NK," is a rare eye disorder that affects the cornea. The cornea is the clear outer layer at the front of the eye.
- Causes and Symptoms: Neurotrophic keratitis is caused by damage to the corneal nerves, leading to decreased corneal sensitivity and poor corneal healing [5][6]. Early signs can include red-eye, blurred vision, and decreased visual acuity. As the disease progresses, epithelial defects and stromal ulceration may occur [8].
- Stages of Neurotrophic Keratitis: The severity of neurotrophic keratitis can be divided into three stages (Mackie classification system):
- Stage 1: Mild, nonspecific signs and symptoms, including rose bengal staining of the inferior palpebral conjunctiva [9].
- Stage 2: Large persistent epithelial defect characterized by smooth, rolled edges.
- Stage 3: Deep corneal ulcer, stromal melting, and sterile hypopyon [3].
- Risk Factors: Common causes of NK include long-term use of contact lenses, history of corneal surgery, chemical and physical burns, herpes keratitis, and underlying conditions such as diabetes or neurosurgical procedures [12][15].
Neurotrophic keratitis is a degenerative disorder resulting from loss of corneal sensation, breakdown of the epithelial lining with decreased wound healing. It is essential to recognize this condition early to allow appropriate treatment that will promote corneal healing and prevent progression to more extensive, permanent corneal destruction [11].
References:
[1] Context result 4 [2] Context result 5 [3] Context result 3 [4] Context result 10 [5] Context result 6 [6] Context result 7 [7] Context result 8 [8] Context result 12 [9] Context result 13 [10] Context result 14 [11] Context result 11 [12] Context result 12 [13] Context result 15
Additional Characteristics
- Neurotrophic keratitis
- Neurotrophic keratopathy
- Nonspecific signs and symptoms
- Large persistent epithelial defect
- Deep corneal ulcer
- Stromal melting
- Sterile hypopyon
- Long-term use of contact lenses
- History of corneal surgery
- Chemical and physical burns
- Herpes keratitis
- Diabetes or neurosurgical procedures
Signs and Symptoms
Early Signs and Symptoms
Neurotrophic keratoconjunctivitis (NK) can present with seemingly innocuous symptoms, making it challenging to diagnose in the initial stages. Some early signs and symptoms include:
- Blurred vision
- Mild eye irritation or stinging sensation
- Decreased or fluctuating vision
- Sensitivity to light
- Redness of the eyes
These symptoms may be mild and can progress to more severe forms if left untreated.
Common Symptoms
As NK progresses, patients may experience a range of symptoms, including:
- Stinging, burning, or irritation in the eyes
- Blurry or fluctuating vision
- Eye discharge (mucus)
- Sensitivity to light
- Redness and inflammation of the eye
In some cases, patients may not experience any symptoms at all, especially if the condition is mild.
Severe Symptoms
If left untreated, NK can lead to severe complications, including:
- Devastating vision loss
- Risk of losing the eye due to corneal thinning or infection
- Corneal inflammation and slow healing
It's essential to seek medical attention promptly if you experience any symptoms or suspect that you may have NK.
References:
- [2] It may present with seemingly innocuous symptoms, like blurred vision, or lack symptoms altogether in the initial stages of neurotrophic keratitis.
- [3] Keratoconjunctivitis sicca: This condition is also known as dry eye syndrome, and it stems from having a lack of tears or tears of a poor quality. The symptoms are similar to those of NK.
- [9] If symptoms are present, patients will feel irritation, burning, stinging, fluctuating vision, light sensitivity, redness, foreign body sensation, and contact ...
- [10] Symptoms of Neurotrophic Keratitis. Early signs and symptoms include: Stinging, burning, or irritation in your eyes; Blurry or fluctuating vision; Eye discharge (mucus) Sensitivity to ...
- [13] Symptoms of Neurotrophic Keratitis. If you have neurotrophic keratitis, because of your reduced corneal sensitivity due to the damage to your corneal nerve endings you may have mild, or no symptoms at all. Alternatively, you can experience any of the following: Stinging, burning or irritation of your eyes; Decreased, blurry or fluctuating vision
Additional Symptoms
- Redness of the eyes
- Mild eye irritation or stinging sensation
- Decreased or fluctuating vision
- Stinging, burning, or irritation in the eyes
- Blurry or fluctuating vision
- Eye discharge (mucus)
- Risk of losing the eye due to corneal thinning or infection
- Corneal inflammation and slow healing
- loss of vision
- blurred vision
- light sensitivity
Diagnostic Tests
Diagnostic Tests for Neurotrophic Keratoconjunctivitis
Neurotrophic keratoconjunctivitis (NKC) is a degenerative disease characterized by corneal sensitivity reduction, spontaneous epithelium breakdown, and impairment of corneal healing. Accurate diagnosis of NKC requires a combination of clinical history, complete eye examination, and assessment of corneal sensitivity.
Diagnostic Procedures
- Corneal Sensitivity Testing: Decreased corneal sensitivity is the hallmark sign of NKC. Corneal sensitivity testing should be performed as soon as it is suspected [6].
- Cochet-Bonnet Eesthesiometer: This instrument can better identify "relative" or more mildly decreased corneal sensitivity (hypoesthesia) [13].
- Lacrimal Function Tests: These tests are used to check how well the eyes make tears, which is essential for diagnosing and managing NKC [14].
- Complete Eye Examination: A thorough eye examination is necessary to identify any underlying conditions that may be contributing to NKC.
- Instrumental and Laboratory Tests: Several instrumental and laboratory tests may aid in the diagnosis and management of NKC. Some of these tests are mandatory for a prompt diagnosis (level 1), while others help in assessing the severity of the disease [2].
Clinical Findings
NKC can be divided into three stages based on the Mackie classification:
- Stage 1: Characterized by decreased corneal sensitivity and spontaneous epithelium breakdown.
- Stage 2: Marked by increased epithelial defect size and decreased corneal sensation.
- Stage 3: The most severe stage, characterized by large epithelial defects, corneal ulceration, and melting [3].
References
[1] Clinical Findings. Neurotrophic keratitis can be divided into three stages based on the Mackie classification.
[2] Several instrumental and laboratory tests may aid the diagnosis and management of NK.
[3] Stage 3 Neurotrophic Keratitis (Courtesy of Stephen Orlin, MD).
[6] Decreased corneal sensitivity is the hallmark sign of neurotrophic keratitis (NK), corneal sensitivity testing should be performed as soon as it is suspected.
[13] It is critical to diagnose the underlying condition, whether you are testing with a Cochet-Bonnet esthesiometer, which can better identify “relative” or more mildly decreased corneal sensitivity (hypoesthesia).
Note: The references provided are based on the information available in the search results and may not be an exhaustive list of all relevant studies and articles.
Additional Diagnostic Tests
- Complete Eye Examination
- Corneal Sensitivity Testing
- Cochet-Bonnet Eesthesiometer
- Lacrimal Function Tests
- Instrumental and Laboratory Tests
Treatment
Treatment Options for Neurotrophic Keratoconjunctivitis
Neurotrophic keratoconjunctivitis (NKC) is a rare and degenerative eye condition characterized by impaired corneal sensation, leading to epithelial breakdown and potential complications. While there are
Recommended Medications
- Restasis
- Lacrisert
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Differential Diagnosis
Differential Diagnoses for Neurotrophic Keratitis
Neurotrophic keratitis, a degenerative corneal disease caused by impairment of corneal sensory innervation, can be challenging to diagnose due to its similarity with other ocular surface and corneal diseases. The following conditions should be considered in the differential diagnosis:
- Bacterial Keratitis: A bacterial infection that can cause corneal ulcers and inflammation.
- Corneal Mucous Plaques: A condition characterized by the presence of mucous plaques on the cornea, which can be caused by various factors including dry eye disease.
- Dry Eye Disease (Keratoconjunctivitis Sicca): A chronic condition that affects the tear film and can cause symptoms such as dryness, irritation, and blurred vision.
- Herpes Simplex Virus (HSV) Keratitis: An infection caused by the herpes simplex virus that can lead to corneal inflammation and scarring.
These conditions should be ruled out through a thorough ophthalmological examination and clinical history before diagnosing neurotrophic keratitis. The presence of severe corneal staining or epithelial defect in absence of ocular discomfort symptoms (due to reduced corneal sensitivity) is pathognomonic of NK, but it's essential to consider these differential diagnoses.
References:
- [1] Context 4
- [2] Context 3
- [7] Context 9
- [12] Context 5
Additional Differential Diagnoses
- Bacterial Keratitis
- Dry Eye Disease (Keratoconjunctivitis Sicca)
- Corneal Mucous Plaques
- herpes simplex virus keratitis
Additional Information
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