Journal List > J Rhinol > v.23(2) > 1044358

Moon, Yang, Hong, and Lee: A Case of Tolosa-Hunt Syndrome Presented with Headache, Ptosis and Vision Loss

Abstract

Cavernous sinus syndrome (CSS) is defined as the involvement of two or more of the third, fourth, fifth (V1, V2) or sixth cranial nerves or involvement of only one of them in combination with a neuroimaging-confirmed lesion in the cavernous sinus. Some cases of CSS are attributed to Tolosa-Hunt syndrome (THS), an idiopathic inflammatory disease of the cavernous sinus. THS is characterized by painful ophthalmoplegia due to granulomatous inflammation in the cavernous sinus. THS is a diagnosis of exclusion that requires a vigorous series of differential diagnoses, and corticosteroid therapy is known to dramatically resolve clinical findings of THS. We report a case of a patient with painful ophthalmoplegia associated with vision loss, which was suspected to be THS. This patient followed a relatively typical clinical course of THS on steroid pulse therapy. We emphasize the differential diagnosis of THS, its presentation, and treatment.

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Fig. 1.
On pre-op evaluation, ptosis of the left eye, left-superior fixation of the eye ball on resting state, and limitation of the left eye on left gaze was observed, indicating weakness of the left 3rd and 6th cranial nerves.
jr-23-134f1.tif
Fig. 2.
On pre-op nasal endoscopic examination, the left maxillary osteomeatal unit was intact, and no lesions were observable in the ethmoid complex.
jr-23-134f2.tif
Fig. 3.
On pre-op computer tomo-grphy, soft tissue density indicating mucosal thickening of the left sphenoid sinus was observed (A, white arrow), soft tissue was not enhanced on contrast (B, white arrow).
jr-23-134f3.tif
Fig. 4.
Fat-saturated T2 weighted view on MRI shows infiltrative mass in the left orbital apex (A, white arrow), including the inferior orbital fissure, extending to the left cavernous sinus (B, white arrow).
jr-23-134f4.tif
Fig. 5.
Gadolinium enhanced T1 weighted MR images show intense but inhomogeneous enhancement in the left orbital apex (A, white arrow), and cavernous sinus (B, white arrow).
jr-23-134f5.tif
Fig. 6.
Intraoperative endoscopy showed no lesions of sphenoid sinus, other than mild mucosal thickening (NS: nasal septum, SS: sphenoid sinus, MT: middle turbinate).
jr-23-134f6.tif
Fig. 7.
On physical examination of eye movements 1 week after endoscopic drainage and biopsy, and 10 days of pulse steroid therapy, no ptosis or left eye movement limitation was observed.
jr-23-134f7.tif
Fig. 8.
Fat-saturated T2 weighted view on MRI, done on day 40 of steroid pulse therapy, shows reduced extent of infiltrative mass in the left orbital apex (A, white arrow), and improved state of lesion of the inferior orbital fissure and left cavernous sinus (B, white arrow).
jr-23-134f8.tif
Fig. 9.
Gadolinium enhanced T1 weighted MR images done on day 40 of pulse therapy, show improvement of inhomogeneous enhancement in the left orbital apex (A, white arrow), and cavernous sinus (B, white arrow).
jr-23-134f9.tif
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