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Vol. 35. Issue 4.
Pages 215-220 (July - August 2024)
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Vol. 35. Issue 4.
Pages 215-220 (July - August 2024)
Case Report
Lateral micro-orbitotomy as the technique of choice for orbital pseudotumor in IgG4-RD. Case report and review of the literature
Micro-orbitotomía lateral como técnica de elección para el pseudotumor orbitario en la ER-IgG4. Presentación de un caso y revisión de la literatura
Gema Bravo-Garridoa,
Corresponding author
, Jose María Narro-Donatea, Gabriel María Valdenebro-Cuadradob, José Masegosa-Gonzáleza
a Servicio de Neurocirugía, Hospital Universitario Torrecárdenas, Almería, Spain
b Servicio de Anatomía Patológica, Hospital Universitario Torrecárdenas, Almería, Spain
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Abstract

Immunoglobulin G4-related disease (IgG4-RD) is characterized by a systemic fibroinflammatory infiltrate that often involves the orbit in addition to other tissues. Thus it has to be considered in the differential diagnosis of orbital tumors. We report the clinical case of a 64-year-old woman who presented with right mydriasis, progressive proptosis and paralysis of the third cranial nerve of 1 year of evolution. Cranial MRI identified an intraconal lesion of the right orbit, located between the external and inferior rectus muscles and the optic nerve, and she was scheduled for surgery by transcranial approach with lateral micro-orbitomy. A satisfactory macroscopic excision was achieved with no remarkable complications and a definitive deferred histological result of pseudotumor by IgG4-RD. Follow-up for 24 months showed no tumor recurrence, and the patient clinically improved from ophthalmoplegia. This case highlights the efficacy of lateral orbitotomy in the etiologic diagnosis and successful therapeutic outcome of complex orbital lesions associated with IgG4-RD pseudotumor.

Keywords:
Lateral orbital approach
Orbital pseudotumor
Lateral orbitotomy
Endoscopic lateral orbitotomy
IgG4-related disease
Abbreviations:
IgG4-RD
MALT lymphoma
MRI
Resumen

La enfermedad relacionada con Inmunoglobulina G4 (ER-IgG4) se caracteriza por un infiltrado fibroinflamatorio sistémico que a menudo afecta a la órbita además de otros tejidos. Así pues se ha de considerar en el diagnóstico diferencial de los tumores orbitarios. Se expone el caso clínico de una mujer de 64 años que presentó midriasis derecha, proptosis progresiva y parálisis del III par craneal de 1 año de evolución. Mediante RM craneal se identificó una lesión intraconal de la órbita derecha, situada entre los músculos recto externo e inferior y el nervio óptico programándose para cirugía mediante abordaje transcraneal con micro-orbitomia lateral. Se consiguió una escisión macroscópica satisfactoria sin complicaciones reseñables y un resultado histológico diferido definitivo de pseudotumor por ER-IgG4. El seguimiento durante 24 meses no mostró recidiva tumoral, y la paciente mejoró clínicamente de la oftalmoplejía. Este caso subraya la eficacia de la orbitotomía lateral en el diagnóstico etiológico y los resultados terapéuticos satisfactorios de las lesiones orbitarias complejas asociadas al pseudotumor por ER-IgG4.

Palabras clave:
Abordaje lateral orbitario
Pseudotumor orbitario
Orbitotomía lateral
Orbitotomía lateral endoscópica
Enfermedad relacionada IgG4

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