Orbital Pseudotumor: Neurosurgical Perspective

Abstract

Idiopathic orbital inflammation is the third most common orbital affection, characterized by a benign inflammatory process, which is neither infectious nor neoplastic and can affect any orbital tissue. A 31-year-old female patient was referred to neurosurgical evaluation due to left orbital pseudotumor, which was nonresponsive to high doses of corticosteroid therapy. During the neurological examination, we identified reduced visual acuity, complete external ophthalmoplegia, ocular pain, and severe proptosis. A neurosurgical intervention with lateral orbitotomy was performed, with great pain relief. In the present case report, we discuss the disease and its natural course and call the attention of neurosurgeons to prompt intervention in selected cases.

Resumo

A inflamação orbital idiopática é a terceira causa mais comum de afecção orbital, caracterizada por processo inflamatório benigno, não infeccioso nem neoplásico, o qual pode acometer qualquer tecido orbital. Uma paciente de 31 anos, foi encaminhada para neurocirurgia em decorrência de um pseudotumor orbital à esquerda, o qual não respondeu ao tratamento com altas doses de corticosteroides. No exame neurológico, foram identificadas redução da acuidade visual, oftalmoplegia externa, dor ocular e ptose severa. Neste relato de caso, discutimos o curso natural da doença e chamamos a atenção dos neurocirurgiões para a intervenção de urgência em casos selecionados.

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.

Case Report

A 31-year-old female patient was diagnosed with left orbital pseudotumor. The patient was evaluated by an ophthalmologist that prescribed high doses of corticosteroids. Regarding increased doses, the symptoms became worse, and she was referred to neurosurgery for evaluation. An orbital magnetic resonance imaging (MRI) scan demonstrated ocular muscle infiltration and proptosis ([Fig. 1]). Due to reduced visual acuity, complete external ophthalmoplegia, ocular pain, and severe proptosis, a lateral decompressive orbitotomy was proposed. With a small skin incision, the decompression was made without difficulties ([Fig. 2]). A postoperative computed tomography demonstrated the lateral orbitotomy ([Fig. 3]). Visual acuity improved on the first postoperative day. The patient is under rheumatologist follow-up using azathioprine.

Fig. 1

Fig. 2

Fig. 3

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.

Conflict of Interests

The authors have no conflict of interests to declare.

• References

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• 3 Espinoza GM. Orbital inflammatory pseudotumors: etiology, differential diagnosis, and management. Curr Rheumatol Rep 2010; 12 (06) 443-447
• 4 Weber AL, Romo LV, Sabates NR. Pseudotumor of the orbit. Clinical, pathologic, and radiologic evaluation. Radiol Clin North Am 1999; 37 (01) 151-168 , xi
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• 6 Mombaerts I, Schlingemann RO, Goldschmeding R, Koornneef L. Are systemic corticosteroids useful in the management of orbital pseudotumors?. Ophthalmology 1996; 103 (03) 521-528
• 7 Chiu CS, Rubin PA. Pharmacotherapies and nonpharmacotherapies for orbital inflammatory diseases. Int Ophthalmol Clin 2004; 44 (03) 165-185
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• 10 Yeşiltaş YS, Gündüz AK. Idiopathic Orbital Inflammation: Review of Literature and New Advances. Middle East Afr J Ophthalmol 2018; 25 (02) 71-80

Publication History

Received: 30 May 2023

Accepted: 23 October 2024

Article published online:
27 March 2025