Clivus_Chordoma

This 46 year old patient had at age 6, a tendency for the left eye to wander out. Her face photograph at that age shows an exotropia and at age 7, a year later, the exotropia was not quite as prominent. It was assumed that the exotropia was due to a non-paralytic strabismus. Past History: At age 1 fracture of the skull Since childhood the left eye wandered out She was never told she had a strabismus and never wore prism glasses. In Sept 1994 at age 46, she experienced intermittent double vision, seeing the image split apart horizontally, and persisting for approximately 2 minutes. By blinking her eyes she was able to refocus a single image. In December 1994 she had transient "kaleidoscopic" vision, by which she meant horizontal and oblique diplopia when she woke up. She noted this looking at the television set, the window frame or her bedside clock. By mid-December she had persistent horizontal double vision in primary gaze worse on looking to the left and was no longer able to refocus a single image but saw only one image if she closed one eye. She was referred to the Neurovisual Clinic at the Massachusetts General Hospital and admitted. Symptomatic Inquiry: No headaches, vertigo, ptosis, or unsteadiness Family History: Positive for diabetes and hypertension No history of neurological disease. Neuro-ophthalmological examination: Visual acuity 20/20 OD, 20/30 reads J2 OS Visual fields, pupils and fundus examination normal. Ocular Motility OS: Esotropia Weakness of full abduction Cranial nerves 3 and 4 normal Convergence normal Alternating exophoria at distance Vertical gaze normal Lower cranial nerves normal Neurological examination: Normal Diagnosis: Left sixth nerve palsy superimposed on a childhood non-paralytic esotropia Brain CT showed: 1.A lytic destructive lesion in the clivus with bony particles within the lesion. 2.The mass extended from the sella to the foramen magnum with extension laterally to the petrous apices and carotid canals. 3.There was a suggestion of erosion of the petrous apex on the left. 4.Partial erosion of the posterior wall of the sella 5.The mass abutted the posterior part of the left and right cavernous sinus Brain MRI showed: 1.A clival mass with extension into Meckel's cave and cavernous sinus on the left and circumferential encasement of the proximal left cavernous internal carotid artery (ICA). 2.Partial encasement of the right proximal cavernous ICA. Diagnosis: Clivus Chordoma Differential Diagnosis: Chondrosarcoma Neurosurgery Consult: The important features that established the tumor as inoperable, were the imaging studies showing infiltration and destruction of bone along the midline of the clivus stretching from the pituitary down to the lower clivus in addition to extension of the tumor into the left cavernous sinus encasing the left ICA. The patient was advised that the tumor was inoperable and biopsy was recommended. A transsphenoidal biopsy was performed. Pathology: Chordoma Treatment: Proton beam radiation to the mass and skull base. See also: http://content.lib.utah.edu/cdm/ref/collection/ehsl-shw/id/35