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Show Journal of Neuro- Ophthalmology 20( 1): 14- 16, 2000. © 2000 Lippincott Williams & Wilkins, Inc., Philadelphia Hemangioma of the Mandibular Branch of the Trigeminal Nerve in the Meckel Cave Presenting With Facial Pain and Sixth Nerve Palsy Paul W. Brazis, MD, Robert E. Wharen, MD, Leo F. Czervionke, MD, Robert J. Witte, MD, and Arthur D. Jones, MD In a 25- year- old woman with episodic periorbital- temporal pain who eventually developed a sixth nerve palsy, magnetic resonance imaging revealed a lesion predominantly in the Meckel cave that was found to be a capillary hemangioma arising from the mandibular division of the trigeminal nerve. Hemangiomas of the Meckel cave must be considered in cases of facial pain with a sixth nerve palsy, even if there are no clinical findings of trigeminal neuropathy. Key Words: Capillary hemangioma- Cavernous hemangioma- Facial pain- Meckel cave- Sixth nerve palsy. Hemangiomas consist of vascular channels lined by a single layer of endothelial cells, with the vascular spaces consisting of capillaries ( capillary hemangiomas) or of more dilated vessels ( cavernous hemangiomas). Intracranial extracerebral hemangiomas are uncommon and tend to involve especially the cavernous sinus ( 1- 7). Cavernous hemangiomas in the Meckel cave are rare and typically present with trigeminal neuropathy ( 8,9). Capillary hemangiomas in this region are even less common. We present a case of capillary hemangioma of the mandibular branch of the trigeminal nerve in the Meckel cave that presented with facial pain and a sixth nerve palsy. CASE REPORT A 25- year- old woman experienced diplopia and facial pain. In April 1997, she noted the onset of severe pain in the left periorbital- temporal region that was continuous and worsened over a period of 2 weeks. The pain was " gnawing" and was occasionally associated with super- Manuscript received March 18, 1999; accepted October 4, 1999. From the Departments of Neurology ( PWB), Ophthalmology ( PWB), Neurosurgery ( REW), Neuro- Radiology ( LFC, RJW), and Pathology ( ADJ), Mayo Clinic Jacksonville, Jacksonville, Florida. Address correspondence and reprint requests to Paul W. Brazis, MD, c/ o Mayo Clinic Jacksonville, 4500 San Pablo Road, Jacksonville, FL 32224. imposed spontaneous " jabs" of pain. The pain gradually subsided and eventually ceased completely over a period of several weeks, only to recur again, rather suddenly, in another month. The pain then cleared again over several weeks, but two further episodes, lasting weeks, occurred in December 1997 and January 1998. No further attacks of pain had been reported since January 1998; however, earlier in this same month, she noted the onset of horizontal diplopia, worse at distance than near and worse on left gaze. Although the diplopia seemed to improve in February 1998, later in February it was again prominent and persistent. The patient did not report any facial numbness or paresthesia, visual loss, or other neurologic problems. The patient was initially seen at the Mayo Clinic Jacksonville on March 13, 1998. Examination of the afferent visual system was normal. The pupils were 6 mm bilaterally, reacted well to light and near, and no relative afferent pupillary defect was present. There was an esotropia of 4 prism diopters at distance in primary position that went to 12 prism diopters on left gaze and was nil on right gaze. Abduction was limited to 75% of normal in the left eye. Facial sensation, corneal reflexes, masseter function, and facial strength were normal. Results of slit lamp and funduscopic examinations were normal. No other abnormalities were noted following a general neurologic examination. Magnetic resonance imaging revealed an ovoid lesion in the left Meckel cave, with a high signal intensity on axial T2- weighted Fast Spin Echo image that enhanced with gadolinium on axial Tl- weighted images ( Fig. 1). This mass was isointense to brain tissue on unenhanced Tl- weighted images and extended into the left cavernous sinus and foramen ovale ( Fig. 2). A combined subtemporal- intradural and extradural approach was used to expose the lesion, which arose from the mandibular branch of the trigeminal nerve in the Meckel cave. This approach best exposes the origin of the trigeminal nerve ( 10). The lesion was completely excised. 14 HEMANGIOMA OF THE TRIGEMINAL NERVE 15 FIG. 1. Ovoid lesion ( long arrow) in left Meckel cave enhances on gadolinium- enhanced axial T1- weighted image. Normal Meckel cave ( short arrow) on right contains cerebrospinal fluid, which has high intensity. Pathologic examination revealed the lesion to consist of a proliferation of capillary- sized vessels lined by flattened endothelial cells ( Fig. 3). Cluster of differentiation- 34 immunoperoxidase stain showed positive staining for endothelial cells composing this benign lesion. The pathologic changes were consistent with a capillary hemangioma. By April 7, 1998, the patient's diplopia had resolved completely. DISCUSSION The patient's episodic left periorbital- temporal pain was likely because of irritation of the trigeminal nerve FIG. 2. Coronal T1- weighted gadolinium- enhanced image. The mass ( arrows) extends into the left cavernous sinus and enhances with gadolinium paramagnetic contrast. FIG. 3. Low- powered photomicrographs of the capillary hemangioma showing proliferation of capillary- sized vessels lined by flattened endothelial cells. CD34 immunoperoxidase stain ( not shown) showed positive staining for the endothelial cells. branches or ganglion by the hemangioma. The reason for the remissions and exacerbations of the patient's pain is unknown but could have been because of episodic hemorrhage or vascular engorgement within the hemangioma. The left sixth nerve palsy resulted from abducens nerve involvement near or in the cavernous sinus. Hemangiomas only rarely arise in the cavernous sinus and may cause retrobulbar pain, facial numbness, ophthalmoplegia ( especially sixth nerve palsies), or a combination of these findings ( 1- 7). For example, Lee et al. ( 7) described a woman with an isolated left sixth nerve palsy who was found to have a cavernous sinus hem angioma. Hemangiomas arising in the Meckel cave are even more uncommon than lesions that arise within the cavernous sinus. For example, Fehlings et al. ( 8) described a patient presenting with the subacute onset of facial pain, hypesthesia in all three divisions of the trigeminal nerve, and temporalis/ masseter muscle wasting who was found to have a cavernous hemangioma within the Meckel cave, involving the gasserian ganglion. Kuntzer et al. ( 9) also described a patient with numbness of the right jaw that slowly extended to involve the right cheek and face. Examination revealed sensory loss in the right ophthalmic and maxillary distribution of the trigeminal nerve, an abolished right corneal reflex, and masseter/ temporalis muscle wasting. The patient eventually developed a right sixth nerve palsy and was found to have a cavernous hemangioma of the Meckel cave, causing direct gasserian ganglion compression. Our patient was unique in that she presented with episodes of periorbital- temporal pain, without evidence of trigeminal- distribution sensory loss; she also presented with sixth nerve palsy from a capillary hemangioma of the mandibular branch of the trigeminal nerve within the Meckel cave that also compressed other branches of the trigeminal nerve and eventually compressed the abducens nerve. It is not rare for masses to cause subjective trigeminal symptoms ( e. g., pain or paresthesias) without significant trigeminal distribution sensory loss. J Neuro- Ophthalmol, Vol. 20, No. 1, 2000 16 P. W. BRAZIS ET AL. The major benign lesions of the Meckel cave or the REFERENCES cavernous sinus that may resemble hemangiomas include ,. Rigamonti D, Pappas TE, Spetzier RF, Johnson PC. Extracerebral meningiomas and schwannomas. These latter lesions cavernous angiomas of the middle fossa. 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