Description

Ahn Yuen and Rubin described an algorithm for the management of patients with idiopathic orbital inflammation (orbital pseudotumor). An orbital pseudotumors usually responds to systemic steroids. The authors are from the Massachusetts Eye and Ear Infirmary in Boston.


 

Idiopathic orbital inflammation = benign (nonneoplastic), noninfective inflammatory condition without identifiable, local or systemic causes.

 

Initial patient evaluated is directed to exclude infection, trauma, thyroid orbitopathy, sarcoidosis, Wegener's granulomatosis, other autoimmune diseases, neoplasm and other causes. If a complete diagnostic workup has been unable to identify a cause, then initiate therapy for presumed idiopathic orbital inflammation.

 

Initial therapy for mild disease: nonsteroidal anti-inflammatory agents (NSAIDS) to help control pain.

 

Initial therapy for moderate or severe disease: high dose oral corticosteroids

Dose: prednisone 1.0 - 1.5 mg per kg per day

Duration: 1-2 weeks with taper over the next 5-8 weeks.

 

Retreatment if condition relapses on tapering steroids or recurs:

Dose: prednisone 1.5 mg per kg per day or higher

Duration: 1-2 weeks with taper over the next 8-10 weeks (slower taper)

 

Biopsy is performed if:

(1) the disease course is atypical

(2) disease is refractory to systemic steroids or rebounds during steroid taper

 

Radiation therapy is used if the patient:

(1) does not respond to systemic steroid therapy or

(2) is intolerant to steroid therapy

 

Dose of radiation: 15-20 Gy fractioned over 10 days

 

If the patient is refractory to systemic steroids and radiation, then:

(1) reconsider diagnosis and look for other causes

(2) consider chemotherapy

(3) consider surgical debulking if focus can be easily resected or if disease progressive

Clinical Outcome

Interpretation

complete relief of symptoms

therapeutic success

partial or no relief of symptoms

therapeutic failure

 


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