Keywords
orbit - pseudotumor - proptosis - orbitotomy
Palavras-chave
órbita - pseudotumor - proptose - orbitotomia
Introduction
The idiopathic orbital inflammation, also known as orbital pseudotumor, was first described by Gleason in 1903.[1] A benign inflammatory process of the orbital tissues characterizes the condition. The pathophysiology is not completely known, but some conditions, such as rheumatologic and autoimmune diseases, as well as infections, can be related as triggers and predispose the illness. The disease does not present a predilection for gender or race and usually is unilateral.[2]
[3]
We herein present a case of a 31-year-old female patient who was refractory to the medication therapy and was referred to neurosurgery for decompression and pain relief.
Discussion
The clinical presentations are distinct, varying according to the chronicity, affected tissue, and mainly the anatomic location. Following the acute inflammation, the histopathologic presents with infiltrate and demonstrates the cardinal signs of the inflammation. As it becomes chronic, the fibrosis process takes place.[4]
This disease is self-limited in 28% of cases,[5] but the first-line therapy is corticosteroid use when needed. In this management, 78% presented positive results of the inflammation cardinal signs and other symptoms in 24 to 48 hours. However, only 37% were cured, and 52% presented with remission.[6]
[7] Furthermore, the second-line therapy is radiotherapy, indicated for patients with corticosteroid intolerance or resistance, being effective in 75% of the cases.[8]
[9]
Additionally, immunosuppressant therapy can be a possible treatment in patients with recurrence or persistent symptoms, but there is no actual consensus on the effectiveness, doses, and therapy time.[8] The surgical intervention takes the same place, and there are no studies on the correct indication and the best surgical approach. Several discussions suggest that worsening pain, visual disturbance, and cranial nerve palsy are the best indications for this condition.[10]
Conclusion
Surgical intervention for decompression can be necessary for some cases of idiopathic orbital inflammation. The lateral orbitotomy can be a “visual saving” surgery and should always be in the neurosurgeon armamentarium.
