Confirmation of Tolosa-Hunt Syndrome via Endoscopic Endonasal Biopsy of the Orbital Apex

Abstract

Objectives:

1) Review the criteria for diagnosing Tolosa-Hunt Syndrome (THS), a rare cause of painful ophthalmoplegia due to idiopathic granulomatous inflammation of the orbital apex or cavernous sinus. 2) Review the literature of THS and other surgical techniques used previously in the setting of THS.

Methods:

We present a literature review of THS, focusing on the surgical approaches to and findings in the cavernous sinus and orbital apex. We report a recent case of unilateral vision loss and painful ophthalmoplegia where endonasal endoscopic biopsy of the orbital apex was performed after initial evaluation failed to yield a diagnosis. We present the pre-operative imaging, intra-operative observations, and pathologic results of this patient with THS.

Results:

In our review of THS, there are no other documented reports of endonasal endoscopic biopsy of the orbital apex. There are few reports of variable neurosurgical approaches used previously in THS. In our case, endoscopic biopsy yielded pathologic evidence of granulomatous inflammation without evidence of infectious or neoplastic disease. The patient received intravenous steroids, and symptoms improved within 72 hours, confirming THS.

Conclusions:

THS is an extremely rare cause of painful ophthalmoplegia. Traditionally, THS is a diagnosis of exclusion without tissue confirmation, as biopsies are difficult to perform. This may delay diagnosis of a neoplasm. We present a single case of THS where endonasal endoscopic biopsy of the orbital apex confirmed granulomatous inflammation, suggesting that endoscopic techniques may be used as a safe, minimally invasive approach to the orbital apex in the setting of painful ophthalmoplegia.