| Identifier |
Clivus_Chordoma |
| Title |
Clivus Chordoma |
| Creator |
Shirley H. Wray, MD, PhD, FRCP |
| Affiliation |
(SHW) Professor of Neurology, Harvard Medical School; Director, Unit for Neurovisual Disorders, Massachusetts General Hospital, Boston, Massachusetts |
| Subject |
Esotropia; Abduction Weakness; Sixth Nerve Palsy; Clivus Chordoma; Chordoma |
| History |
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. |
| Anatomy |
The abducens nucleus of the sixth nerve lies in the floor of the fourth ventricle, at the level of the lower pons, and contains three groups of neurons: 1. Abducens motoneurons which innervate the ipsilateral lateral rectus muscle. 2. Abducens internuclear neurons, which project to the contralateral medial rectus subnucleus of the oculomotor nucleus via the medial longitudinal fasciculus 3. Neurons that project to the cerebellar flocculus The genu of the facial nerve curves over the dorsal and lateral surfaces of the nucleus, while the medial longitudinal fasciculus lies medial to each nucleus. The abducens nerve fascicle join its passage in the pons, lies adjacent to the motor nucleus and fascicle of the facial nerve, the motor nucleus of the trigeminal nerve, the spinal tract of the trigeminal nerve, the superior olivary nucleus, the central tegmental tract, and the corticospinal tract. The sixth nerve emerges from the brainstem between the pons and medulla, lateral to the pyramidal prominence. It then runs upwards along the ventral surface of the pons, lateral to the basilar artery, and passes between the pons and the anterior inferior cerebellar artery to ascend through the subarachnoid space along the clivus. It then pierces the dura mata, crosses around and through the inferior petrosal sinus, and passes under the petroclinoid (Gruber's ligament) in Dorello's canal to enter the cavernous sinus. In the cavernous sinus, the nerve bends laterally around the intracavernous segment of the internal carotid artery (ICA) and runs medial and parallel to the ophthalmic division (V1) of the trigeminal nerve. The ocular sympathetic fibers leave the ICA and join briefly with the abducens nerve before joining the ophthalmic division (V1) of the trigeminal nerve. Unlike the oculomotor (third nerve) and trochlear (fourth nerve) nerves, the abducens nerve does not lie within the lateral wall of the sinus, but rather it runs within the body of the sinus. The sixth nerve enters the orbit through the superior orbital fissure, passes through the annulus of Zinn, and innervates the lateral rectus muscle. See Ref (8) and (12) for full discussion. |
| Disease/Diagnosis |
Clivus Chordoma |
| Clinical |
This 46 year old patient with an inoperable clivus chordoma has in her childhood photographs At age 6 exotropia of the left eye (OS) At age 7 exotropia appears less prominent The assumption was an exotropia due to a non-paralytic strabismus. At age 46 she became symptomatic with horizontal diplopia on looking left and OS deviated in. She had: • Esotropia OS • Paresis of full abduction • Paresis of abduction with OS fixing alone confirmed a paralytic sixth nerve palsy • An alternating exophoria at distance |
| Presenting Symptom |
Difficulty focusing |
| Ocular Movements |
Esotropia; Abduction Weakness; Sixth Nerve Palsy |
| Neuroimaging |
No neuroimaging studies are available in this patient. |
| Treatment |
Proton beam therapy to the mass and skull base. |
| Etiology |
Review ref (8) |
| References |
1. Chen KS, Hung IJ, Lin KL. Isolated abducens nerve palsy: an unusual presentation of leukemia. J Child Neurol 2002;17:850-851. http://www.ncbi.nlm.nih.gov/pubmed/12585727 2. Currie JN, Lubin JH, Lessell S. Chronic isolated abducens paresis from tumors at the base of the brain. Arch Neurol 1983;40:226-229. http://www.ncbi.nlm.nih.gov/pubmed/6830471 3. Harada T, Ohashi T, Ohki K et al. Clival chordoma presenting as acute esotropia due to bilateral abducens palsy. Ophthalmologica 1997;21:109-111. http://www.ncbi.nlm.nih.gov/pubmed/9097318 4. Harbison JW, Lessell S, Selhorst JB. Neuro-ophthalmology of sphenoid sinus carcinoma. Brain 1984;108:855-870. http://www.ncbi.nlm.nih.gov/pubmed/6478180 5. Ikezaki K, Toda K, Abe M, Tabuchi K. Intracavernous epidermoid tumor presenting with abducens nerve paresis - case report. Neurologia Medico-Chirurgica 1992;32:360-364. http://www.ncbi.nlm.nih.gov/pubmed/1381064 6. Ilhan O, Sener EC, Ozyar E. Outcome of abducens nerve paralysis in patients with nasopharyngeal carcinoma. Eur J Ophthalmol 2002;12:55-59. http://www.ncbi.nlm.nih.gov/pubmed/11936446 7. Keane JR. Bilateral sixth nerve palsy. Analysis of 125 cases. Arch Neurol 1976;33:681-683. http://www.ncbi.nlm.nih.gov/pubmed/184766 8. Leigh RJ, Zee DS. Diagnosis of Peripheral Ocular Motor Palsies and Strabismus. Ch 9:385-474. In: The Neurology of Eye Movements, Fourth Edition. Oxford University Press, NY 2006. 9. Lopez R, David NJ, Gargano F, Post JD. Bilateral sixth nerve palsies in a patient with massive pituitary adenoma. Neurology 1981;31:1137-1138. http://www.ncbi.nlm.nih.gov/pubmed/7196535 10. Morioka T, Matsushima T, Yokoyama N. Muratami H, Fujii K, Fukui M. Isolated bilateral abducens nerve palsies caused by rupture of a vertebral artery aneurysm. J Clin Neuroopthalmol 1992;12:263-267. http://www.ncbi.nlm.nih.gov/pubmed/1287052 11. Volpe NJ, Liebach NJ, Munzenrider JE, Lessell S. Neuro-ophthalmological findings in chordoma and chondrosarcoma of the skull base. Am J Ophthalmol 1993;115:97-104. http://www.ncbi.nlm.nih.gov/pubmed/8420385 12. Wong AM. Nuclear and Infranuclear Ocular Motor Disorders Chp 12:191-242. In: Eye Movement Disorders. Oxford University Press, 2008. |
| Language |
eng |
| Format |
application/pdf |
| Format Creation |
Microsoft PowerPoint |
| Type |
Text |
| Relation is Part of |
938-3 |
| Collection |
Neuro-Ophthalmology Virtual Education Library: Shirley H. Wray Collection: https://novel.utah.edu/Wray/ |
| Publisher |
North American Neuro-Ophthalmology Society |
| Holding Institution |
Spencer S. Eccles Health Sciences Library, University of Utah |
| Rights Management |
Copyright 2002. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s66yggjq |
| Setname |
ehsl_novel_shw |
| ID |
2174191 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s66yggjq |
