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Cogan-Reese syndrome
ICD-10 Codes
Related ICD-10:
Description
Cogan-Reese Syndrome: A Rare Eye Condition
Cogan-Reese syndrome (CRS) is a rare and complex eye condition that affects the iris, cornea, and endothelium. It is a variant of iridocorneal endothelial (ICE) syndrome, which is characterized by variable iris atrophy, pigmented and pedunculated nodules on the iris, and other symptoms.
Key Characteristics:
- Variable Iris Atrophy: The iris may become thin and develop holes or gaps, leading to vision problems.
- Pigmented and Pedunculated Nodules: Small colored lumps may form on the surface of the iris, which can be pigmented (colored) or non-pigmented.
- Corneal Endothelial "Beaten Metal" Appearance: The cornea's inner layer may develop a characteristic "beaten metal" appearance due to endothelial cell damage.
- Corneal Edema: Swelling of the cornea can occur, leading to vision problems.
Symptoms:
- Unilateral reduced vision
- Unilateral pain
- Diffuse or sectoral color changes of the iris
- Glaucomatous visual field defects in late stages
Causes and Diagnosis: CRS is a rare condition with unknown etiology, although viral etiology has been suggested. Diagnosis is typically made through clinical examination and imaging studies.
References:
- [1] Cogan-Reese syndrome is a clinical variant of iridocorneal endothelial (ICE) syndrome characterized by variable iris atrophy, pigmented and pedunculated nodules. ([5])
- Characteristics unique to CRS include corneal endothelial “beaten metal” appearance, corneal edema, and other symptoms. ([6])
- Symptoms may include a matted or smudged appearance to the surface of the iris; the development of small colored lumps on the iris (nodular iris nevi); the development of glaucomatous visual field defects in late stages. ([1], [8])
Additional Characteristics
- Variable Iris Atrophy
- Pigmented and Pedunculated Nodules
- Corneal Endothelial "Beaten Metal" Appearance
- Corneal Edema
Signs and Symptoms
Cogan-Reese syndrome is a rare eye disorder characterized by several distinct signs and symptoms.
Key Features:
- Iris Atrophy: One of the primary features of Cogan-Reese syndrome is the atrophy of the iris, which can lead to a matted or smudged appearance on the surface of the eye [6][11].
- Pigmented Pedunculated Nodules: The presence of pigmented pedunculated nodules on
Additional Symptoms
- Iris Atrophy
- Pigmented Pedunculated Nodules
- Vitreous Detachment
Diagnostic Tests
Based on the provided context, it appears that there are limited resources available for diagnostic tests specifically related to Cogan-Reese syndrome.
However, I can provide some general information on diagnostic tests that may be relevant to this condition. According to search result [9], corneal and anterior segment structural changes in patients with Cogan-Reese syndrome have been described using three different methods. This suggests that various diagnostic
Additional Diagnostic Tests
- Confocal microscopy
- Corneal and anterior segment structural examination
- Slit-lamp biomicroscopy
Treatment
Treatment Overview
Cogan-Reese syndrome, also known as non-necrotizing interstitial keratitis (NNIK), is a rare eye disorder that can lead to vision loss if left untreated. The primary focus of treatment for this condition is on managing glaucoma and addressing the underlying inflammation.
- Glaucoma Management: Treatment approaches for Cogan-Reese syndrome often involve managing glaucoma, which can be achieved through various medications and surgical procedures [1].
- Topical Medications: Topical nonsteroidal anti-inflammatory drugs (NSAIDs) have been successfully used to treat Cogan-Reese syndrome in some cases [2][3]. However, the duration of treatment and recurrence rates may vary.
- Steroids and Other Medications: In addition to NSAIDs, other topical medications such as corticosteroids, cycloplegics, and disease-modifying anti-rheumatic drugs (DMARDS) may be used to manage symptoms [4][6].
Treatment Approaches
While there is no consensus on the most effective treatment approach for Cogan-Reese syndrome, a combination of glaucoma management and topical medications may provide relief from symptoms. It's essential to consult with an eye care professional for personalized guidance.
- Antiglaucoma Medications: Antiglaucoma medications, including beta blockers, alpha agonists, and carbonic anhydrase inhibitors, are considered first-line therapies [7].
- Surgical Procedures: In some cases, surgical procedures may be necessary to manage glaucoma or address other complications associated with Cogan-Reese syndrome.
References
[1] Sep 12, 2024 — Treatment focuses mainly on glaucoma management and may include eye drops and surgical procedures. [2] Jan 26, 2022 — The CME was successfully treated with topical nonsteroidal anti-inflammatory drugs (NSAID). [3] by H Bouvarel · 2022 · Cited by 2 — The CME was successfully treated with topical nonsteroidal anti-inflammatory drugs (NSAID). [4] Jun 25, 2024 — Cogan syndrome is typically treated with steroids. [5] Treatment Approaches. Antiglaucoma medications, including beta blockers, alpha agonists, and carbonic anhydrase inhibitors, are considered first-line therapies. [6] by N Madhan — Medical therapy was initiated with oral and topical aqueous suppressants, topical steroid drops, and a cycloplegic agent.
Recommended Medications
- Beta blockers
- Carbonic anhydrase inhibitors
- Topical nonsteroidal anti-inflammatory drugs (NSAIDs)
- Cortico-steroids
- Cycloplegics
- Disease-modifying anti-rheumatic drugs (DMARDS)
- Alpha agonists
💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.
Differential Diagnosis
Differential Diagnosis of Cogan-Reese Syndrome
Cogan-Reese syndrome, a rare iridocorneal endothelial syndrome, requires careful consideration of differential diagnoses to ensure accurate diagnosis and treatment. The following conditions are often considered in the differential diagnosis of Cogan-Reese syndrome:
- Essential Iris Atrophy: A condition characterized by progressive thinning and atrophy of the iris, which can lead to glaucoma and vision loss.
- Chandler Syndrome: A rare disorder that affects the cornea and iris, causing edema, decompensation, and secondary glaucoma.
- Iris Melanoma: A type of cancer that affects the iris, which can cause changes in iris color, vision loss, and glaucoma.
- **Microcystic Degenerative Mac
Additional Differential Diagnoses
- Essential Iris Atrophy
- Iris Melanoma
- Microcystic Degenerative Maculopathy
- Chandler syndrome
Additional Information
- relatedICD
- http://example.org/icd10/H47.023
- oboInOwl#hasOBONamespace
- disease_ontology
- oboInOwl#creation_date
- 2014-09-23T12:31:25Z
- oboInOwl#id
- DOID:0060217
- oboInOwl#created_by
- emitraka
- oboInOwl#hasDbXref
- UMLS_CUI:C1168173
- IAO_0000115
- An eye disease characterized by variable iris atrophy, pigmented and pedunculated nodules located_in iris and attachment of the iris to the cornea (peripheral anterior synechiae) and characterized_by glaucoma.
- oboInOwl#inSubset
- http://purl.obolibrary.org/obo/doid#NCIthesaurus
- rdf-schema#domain
- https://w3id.org/def/predibionto#has_symptom_1243
- owl#annotatedSource
- t336686
- core#notation
- DOID:0060217
- rdf-schema#label
- Cogan-Reese syndrome
- rdf-schema#subClassOf
- http://purl.obolibrary.org/obo/DOID_5614
- 22-rdf-syntax-ns#type
- http://www.w3.org/2002/07/owl#Class
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