IgG4-Related Disease Involving Orbital Apex and Skull Base
Learn about a case of IgG4-related disease presenting with orbital apex and skull base involvement in a 58-year-old female. Find out about the clinical presentation, imaging findings on MRI, elevated serum IgG4 levels, and management with systemic glucocorticoids leading to clinical improvement.
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IgG4-related disease (IgG4-RD) Orbital apex and skull base involvement By: Prof.Dr. Sherif Shama Consultant Radiologist, Sheikh Jaber Al-Ahmad Al-Sabah Hospital, Kuwait Professor Of Radio-diagnosis, Head and Neck imaging Unit, Department of Radio- diagnosis, Faculty Of medicine, University Of Alexandria, Egypt.
58 years female patient presented with gradual long-standing left sided propotosis with visual disturbance and trigeminal neuralgia -Her MRI revealed markedly enhancing space occupying lesion targeting the left orbital apex surrounding the optic nerve. -The lesion showed T2 low signal. -Extension into the left Meckel s cave and left cavernous sinus. -Peri-neural spread along mandibular division of the trigeminal nerve (V3) reaching the masticator space. -Extension to the left pterygo-palatine fossa and along the left infra-orbital nerve. -The extra-ocular muscles were thickened and hyper-enhancing . -Serum IgG4 level was elevated (>135 mg/dL). -Due to high risk biopsy, systemic glucocorticoids were started and the patient improved clinically and on follow-up laboratory and radiological investigations.
B A C -Markedly enhancing SOL targeting the left orbital apex, surrounding the optic nerve (arrow in A, axial T1 fat sat +C). D -The lesion is showing T2 low signal (arrow in B, axial T2 WI) -It is reaching the left pterygo-palatine fossa (arrow in C, axial T1 fat sat +C). -The extra-ocular muscles are thickened and hyper- enhancing (arrow in D axial T1 fat sat +C).
E F G The lesion is implicating the left Meckel s cave ( arrow in E, Coronal T2WI). -Peri-neural Extension along the left V3 down to masticator space (arrow in F, coronal T1 fat sat +C). G, coronal T1 fat sat +C showed hyper-enhancement of the extra-ocular muscle and enhancing left infra-orbital nerve (arrow).
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