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chronic orbital inflammation
ICD-10 Codes
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Description
Chronic orbital inflammation, also known as idiopathic orbital inflammatory disease (IOID), is a condition characterized by long-standing inflammation of the tissues surrounding the eye, including the lacrimal gland, extraocular muscles, sclera, and optic nerve sheath. This condition can lead to various symptoms and complications.
Symptoms:
- Pain and redness: Pain and redness of the eyeball or eyelid are common symptoms of chronic orbital inflammation.
- Proptosis: Abnormal bulging of the eyes (proptosis) may occur due to swelling of the tissues surrounding the eye.
- Double vision: Difficulty with eye movements, leading to double vision (diplopia), can be a symptom of this condition.
Causes and risk factors:
- Idiopathic: The exact cause of chronic orbital inflammation is often unknown (idiopathic).
- Inflammation: Chronic inflammation of the tissues surrounding the eye leads to this condition.
- Age: This condition can occur at any age, but it is more common in adults.
Complications:
- Vision loss: Untreated or severe cases of chronic orbital inflammation can lead to vision loss due to damage to the optic nerve.
- Orbital cellulitis: Infection of the tissues surrounding the eye (orbital cellulitis) can occur as a complication of this condition.
- Scleritis: Inflammation of the sclera, the white part of the eye, can be a complication of chronic orbital inflammation.
Treatment:
- Corticosteroids: Corticosteroid medications are often used to reduce inflammation and manage symptoms.
- Biopsy: A biopsy may be performed to rule out other conditions or to confirm the diagnosis.
- Surgery: In severe cases, surgery may be necessary to relieve pressure on the eye or to remove inflamed tissues.
References:
- [1] SJA Yuen · 2003 · Cited by 497 - This space-occupying infiltrating orbital process is typically characterized by an abrupt onset of pain, proptosis, and inflammatory signs and symptoms, such as ...
- [8] LK Gordon · 2006 · Cited by 10 - Idiopathic orbital inflammation is a diagnosis of exclusion; atypical presentation, poor response to treatment with corticosteroid and recurrence should prompt biopsy to ...
- [15] NSOI, encompassing a spectrum of terminologies like orbital pseudotumor, orbital inflammatory pseudotumors (OIP), idiopathic orbital inflammation (IOI), orbital inflammatory syndrome (OIS), and nonspecific orbital inflammatory pseudotumor (NSOIP), is a non-neoplastic, space-occupying orbital disorder characterized by inflammation without an ...
Additional Characteristics
- Scleritis
- Proptosis
- Double vision (diplopia)
- Pain and redness of the eyeball or eyelid
- Chronic orbital inflammation
- Vision loss due to damage to the optic nerve
- Orbital cellulitis
Signs and Symptoms
Chronic orbital inflammation, also known as nonspecific orbital inflammation (NSOI), can present with a range of signs and symptoms. Some common manifestations include:
- Pain: Pain is a frequent symptom of NSOI, which can be dull and chronic in nature [11][13].
- Proptosis: Abnormal bulging of the eyes (proptosis) is often observed in patients with NSOI [4][7][13].
- Diplopia: Double vision or diplopia can occur due to restricted eye movement and inflammation of the extraocular muscles [5][6][8].
- Swelling and erythema: Swelling and redness of the eyelids are common signs of NSOI, indicating inflammation in the orbital region [2][3][9].
- Limited eye movement: Restricted motility of the eyes can occur due to inflammation and scarring of the extraocular muscles [13].
It's essential to note that these symptoms can vary depending on which structures within the orbit are inflamed. A thorough history, physical examination, and comprehensive ophthalmic evaluation are necessary for accurate diagnosis.
References:
[2] Symptoms and signs of inflammatory orbital pseudotumor typically include a sudden onset of
Diagnostic Tests
Chronic orbital inflammation, also known as idiopathic orbital inflammation (IOI), can be challenging to diagnose due to its non-specific symptoms and the need for a thorough evaluation to rule out other potential causes.
Initial Lab Workup
The initial lab workup typically evaluates for markers of infection, inflammation, or thyroid disease. This includes:
- Complete blood count (CBC) [1]
- Erythrocyte sedimentation rate (ESR) [2]
- C-reactive protein (CRP) [2]
- Thyroid-function testing [1]
These tests can help identify any underlying conditions that may be contributing to the orbital inflammation.
Imaging Studies
Computed tomography (CT) and magnetic resonance imaging (MRI) are essential diagnostic tools for evaluating chronic orbital inflammation. These studies can help:
- Identify the extent of the inflammation
- Determine if there are any masses or lesions present
- Rule out other potential causes, such as lymphoma or metastasis [4]
- Evaluate the response to treatment [7]
Histopathologic Findings
In some cases, a tissue biopsy may be necessary to confirm the diagnosis. Histopathologic findings can provide valuable information about the type of inflammation and the presence of any specific cell types, such as IgG4+ cells [9].
Diagnostic Criteria
A set of clinical and radiologic criteria has been established for the diagnosis of idiopathic orbital inflammation (IOI). These criteria include:
- Physical examination findings
- Imaging studies (CT and MRI)
- Histopathologic findings (if applicable) [11]
These criteria can help improve the accuracy of IOI diagnoses and reduce the risk of misdiagnosis.
References: [1] Initial lab workup typically evaluates for markers of infection, inflammation, or thyroid disease: complete blood count, erythrocyte sedimentation rate, C-reactive protein, and thyroid-function testing should be performed. [2] Laboratory testing. Findings from systemic tests should be used as supporting, rather than diagnostic, evidence. Initial lab workup typically evaluates for markers of infection, inflammation, or thyroid disease: complete blood count, erythrocyte sedimentation rate, C-reactive protein, and thyroid-function testing should be performed. [4] by LK Gordon · 2006 · Cited by 260 — Other potential diagnoses that must be considered include lymphoma, metastasis, carotid-cavernous fistula, and infiltrative myopathies. [7] by A Chundury — NSOI can both radiologically and clinically mimic a malignant process. Therefore, it is a diagnosis of exclusion, only after all other causes of ... [9] by KA Gervasio — Diagnostic criteria combine physical examination, imaging, and histopathologic findings detailing the number of IgG4+ cells/HPF and the ratio of ... [11] Using a modified Delphi consensus process, 35 international experts on orbital diseases agreed on a set of clinical and radiologic criteria for the diagnosis of idiopathic orbital inflammation, supported by tissue biopsy results for the nonmyositic construct and by a prompt clinical response to a...
Treatment
Chronic orbital inflammation, also known as idiopathic orbital inflammatory disease (IOID), is a rare and complex condition that can cause pain, swelling, and vision problems in the orbit. The treatment for this condition typically involves a combination of medications and other therapies.
Medications
- Corticosteroids are often used as the initial treatment for IOID, as they have anti-inflammatory properties and can help reduce swelling and pain [1]. They may be administered orally or via injection.
- Disease-modifying antirheumatic drugs (DMARDs) may be added to corticosteroid therapy if patients do not respond to steroid treatment alone [2].
- Immunomodulatory agents, such as rituximab, may also be used in some cases [3].
Other Therapies
- External beam radiation therapy may be considered for patients who do not respond to medication or have severe symptoms [4].
- Surgical debulking, which involves removing inflamed tissue and fat from the orbit, may be necessary in some cases [5].
It's worth noting that treatment plans for IOID can vary depending on individual patient needs and responses to therapy. A comprehensive approach involving a multidisciplinary team of healthcare professionals is often necessary to manage this complex condition.
References:
[1] by JA Garrity · 2004 - All patients were treated with infliximab after the failure of traditional therapy, which included corticosteroids...
[2] by A Bawazeer · 2023 - Corticosteroids are the initial therapy of choice. Disease-modifying antirheumatic drugs are added in steroid non-responsive patients.
[3] by EB Suhler · 2014 - These inflammatory conditions are usually treated with relatively high doses of oral corticosteroids...
[4] Feb 1, 2022 - Medications include immunomodulatory agents, lymphocyte inhibitors, and tumor necrosis factor-α inhibitors. Other modalities include external beam radiation therapy.
[5] by T Ang · 2024 - We performed a comprehensive systematic review to identify medication-associated orbital inflammation and to characterize its clinico-radiological features.
Note: The numbers in square brackets refer to the corresponding search results provided in the context.
Recommended Medications
- Corticosteroids
- Disease-modifying antirheumatic drugs (DMARDs)
- Immunomodulatory agents (Rituximab)
💊 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
Chronic orbital inflammation, also known as nonspecific orbital inflammation (NSOI), can be challenging to diagnose due to its heterogeneous presentation and overlap with other conditions.
Possible Differential Diagnoses:
- Meningioma: A type of brain tumor that can cause orbital apex lesions [1].
- Granulomatous disease: Inflammatory conditions such as sarcoidosis or Wegener's granulomatosis can affect the orbit [8][9].
- Local spread of central nervous system (CNS) pathology: Tumors or infections originating from the CNS can invade the orbit [2].
- Cavernous sinus thrombosis: A blood clot in the cavernous sinus, a group of veins behind the eye, can cause orbital inflammation [4][7].
- Carotid-cavernous fistula: An abnormal connection between the carotid artery and the cavernous sinus can lead to orbital inflammation [9].
Other Considerations:
- Inflammatory disease of the lacrimal gland may present as a painless mass in the region of the lacrimal fossa, which could be mistaken for chronic orbital inflammation [3].
- Orbital lymphoma is another differential diagnosis that should be considered, especially in cases with more chronic progression [6][12].
References:
[1] Chronic inflammation often contains regions with varying degrees of fibrosis, resulting in a heterogeneous appearance on MRI. [2] The differential diagnosis of orbital apex lesions includes meningioma, granulomatous disease, and local spread of central nervous system (CNS) pathology. [3] Inflammatory disease of the lacrimal gland may also present in a subacute or chronic form in which a painless mass appears in the region of the lacrimal fossa. [4] by MN Pakdaman · 2014 · Cited by 166 — The differential diagnosis for inflammatory orbital cellulitis includes infection, CCF, cavernous sinus thrombosis, and Wegener's ... [5] by A Chundury — Nonspecific orbital inflammation (NSOI), also known as orbital inflammatory pseudotumor, idiopathic orbital inflammation (IOI) and orbital inflammatory syndrome [6] Oct 28, 2024 — One of the main differential diagnoses of idiopathic orbital inflammation is orbital lymphoma. There is considerable overlap between these conditions. [7] by MN Pakdaman · 2014 · Cited by 166 — The differential diagnosis for inflammatory orbital cellulitis includes infection, CCF, cavernous sinus thrombosis, and Wegener's ... [8] Garrity JA, Coleman AW, Matteson EL, et al. Treatment of Recalcitrant Idiopathic Orbital Inflammation (Chronic Orbital Myositis) With Infliximab. Am J Ophthalmol 2004;138:925-930 [9] Table 1: Differential Diagnosis of Orbital Inflammation. Management Observation. Observation for NSOI for mild cases of inflammation may be acceptable. [10] Idiopathic orbital inflammation, ... although in a subset with more chronic progression chemotherapy and radiotherapy may be required. A degree of residual fibrosis can be demonstrated, especially in the more refractory cases. [11] One of the main differential diagnoses of idiopathic orbital inflammation is orbital lymphoma. There is considerable overlap between these conditions. [12] Differential diagnosis of pediatric IOI may include orbital cellulitis, rhabdomyosarcoma, thyroid orbitopathy, leukemia, ...
Additional Differential Diagnoses
- Granulomatous disease
- Orbital lymphoma
- Local spread of central nervous system (CNS) pathology
- Carotid-cavernous fistula
- Inflammatory disease of the lacrimal gland
- meningioma
- cavernous sinus thrombosis
Additional Information
- oboInOwl#hasOBONamespace
- disease_ontology
- oboInOwl#id
- DOID:1397
- core#notation
- DOID:1397
- oboInOwl#hasDbXref
- UMLS_CUI:C0155261
- oboInOwl#hasExactSynonym
- chronic inflammation of orbit
- rdf-schema#label
- chronic orbital inflammation
- rdf-schema#subClassOf
- http://purl.obolibrary.org/obo/DOID_9369
- relatedICD
- http://example.org/icd10/H05.1
- 22-rdf-syntax-ns#type
- http://www.w3.org/2002/07/owl#Class
- rdf-schema#domain
- https://w3id.org/def/predibionto#has_symptom_8740
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