Radiation plays a well-defined role in managing a variety of benign conditions. Its mechanism of action and dose prescription vary widely depending on the disease process. Terms such as pseudotumor orbitae, orbital pseudotumor, non-specific orbital inflammation (NSOI), and idiopathic orbital inflammatory syndrome are used interchangeably in the literature. These describe a spectrum of non-specific, non-neoplastic inflammatory conditions, constituting the third most common orbital pathologies encountered after Graves’ orbitopathy and orbital lymphoproliferative disorders. Acute pain, erythema, chemosis, swelling, and restricted eye motility are the most common symptoms at presentation, with diagnosis established through an MRI of the brain and orbits. A biopsy is not routinely indicated unless the condition is determined to be refractory to first-line management. Corticosteroids at doses of 50–100 mg per day for 1–2 weeks, administered as an extended taper, are the gold standard treatment of choice. Steroid refractoriness, intolerance, or contraindications to steroid use are indications for a patient to be considered for anti-inflammatory radiation with a dose of 20–30 Gy. Radiation has been shown to provide immediate symptomatic relief and reasonable long-term control, with negligible acute and long-term toxicities. Techniques like IMRT and VMAT efficiently spare the organs at risk, significantly reducing the risk of developing cataracts. Surgery, chemotherapy, and targeted agents have a limited role in managing orbital pseudotumor, reserved only for cases that do not respond to any other treatment modality.

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Pseudotumor Orbitae

  • Sobhana Vallabhaneni

摘要

Radiation plays a well-defined role in managing a variety of benign conditions. Its mechanism of action and dose prescription vary widely depending on the disease process. Terms such as pseudotumor orbitae, orbital pseudotumor, non-specific orbital inflammation (NSOI), and idiopathic orbital inflammatory syndrome are used interchangeably in the literature. These describe a spectrum of non-specific, non-neoplastic inflammatory conditions, constituting the third most common orbital pathologies encountered after Graves’ orbitopathy and orbital lymphoproliferative disorders. Acute pain, erythema, chemosis, swelling, and restricted eye motility are the most common symptoms at presentation, with diagnosis established through an MRI of the brain and orbits. A biopsy is not routinely indicated unless the condition is determined to be refractory to first-line management. Corticosteroids at doses of 50–100 mg per day for 1–2 weeks, administered as an extended taper, are the gold standard treatment of choice. Steroid refractoriness, intolerance, or contraindications to steroid use are indications for a patient to be considered for anti-inflammatory radiation with a dose of 20–30 Gy. Radiation has been shown to provide immediate symptomatic relief and reasonable long-term control, with negligible acute and long-term toxicities. Techniques like IMRT and VMAT efficiently spare the organs at risk, significantly reducing the risk of developing cataracts. Surgery, chemotherapy, and targeted agents have a limited role in managing orbital pseudotumor, reserved only for cases that do not respond to any other treatment modality.