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Show ' 011 mal of Cllllical NCllro- vl'hthallllology 8( 1): 13- 17, 1988, Abducens Nerve Palsy and Horner's Syndrome Revisited Gerald G. Striph, M. D., and Ronald M. Burde, M. D. ".' 19HH Raven Press, Ltd" New York Sympathetic fibers destined for the eve join the abducens nerve for a short distance within the cavernous sinus; thus, a unilateral sixth nerve palsy with an ipsilateral Horner's syndrome is of significant localizing value. We report two cases of cavernous sinus lesions producing an ipsilateral abducens palsy and Horner's syndrome: one case due to an intrinsic and the other to an extrinsic lesion of the sinus region. Key Words: Abducens nerve palsv- Cavernous sinus - Horner's syndrome- Ipsilateral sixth nerve palsv. From the Departments of Ophthalmology ( G. G. S, R. M. B) and Neurology and Neurological Surgery ( R. M. B). Washington University School of Medicine, St. Louis, Missouri, U. S. A. Address correspondence and reprint requests to Dr. Ronald M. Burde, Department of Ophthalmology, Box 8096, 660 South Euclid Avenue, St. Louis, MO 63110, U. S. A. 13 In 1979, Parkinson ( 1) postulated that lesions within the cavernous sinus could produce an ipsilateral sixth nerve palsy and Horner's syndrome. He based his hypothesis on the anatomical finding that pericarotid third- order sympathetic neurons destined for the orbit join the sixth nerve for a short distance before transferring to the ophthalmic division of the fifth nerve to enter the orbit. Subsequently, there has been a smattering of such case reports. Clinically, this syndrome was recognized by Abad and associates ( 2) in 1981, and more recently, was reemphasized by Gutman and colleagues ( 3). Others have noted this association ( 4- 8). We present two additional examples of this association: one with an intracavernous and the other with an extracavernous lesion. CASE REPORTS Case 1 A 74- year- old white woman was referred because of binocular diplopia that was only partially corrected by spectacles with incorporated prism. Her best- corrected visual acuity was 20/ 25 in the right eye and 20/ 30 in the left. External examination showed no ptosis or proptosis. Pupils were -! mm and 3 mm, right and left eyes, respectively; both were reactive to light and without relative atferent pupillary detect. Corneal sensation was relatively decreased bilaterally. She exhibited 10 diopters of right esotropia in primary position, which increased to 25 diopters in left gaze. She demonstrated secondary deviation when fixating with the left eye. She could not abduct the left eye beyond the midline. Slit lamp and fundus examinations were unremarkable. Kinetic perimetry was normal. A high- resolution computed tomography ( CT) scan with the intravenous injection of con- / 4 G. G. STRIPH AND R. M. BURDE trast material showed a 3 x 3 cm enhancing mass near the sella turcica, which was interpreted as representing an internal carotid artery aneurysm in the cavernous sinus. Chest x- ray, including apical lordotic views, was normal. Arteriography confirmed the CT findings ( Fig. 1). She elected not to have surgical intervention and has been followed for 3 years. Three months after the initial evaluation, she developed 1 mm of ptosis in the left eye. Over the next 2 years, she developed numbness in the distribution of the first division of the left trigeminal nerve. The left abducens nerve palsy became slightly worse. The relative miosis and ptosis on the left side increased during the follow- up period. Cocaine testing failed to either dilate the pupil or elevate the lid on the involved side. Case 2 A previously healthy 30- year- old white man presented with a month- long illness that began with a 6- h period of nausea and vomiting associated with a severe headache. The headache resolved over several days. Ten days prior to examination, he noted the onset of binocular horizontal diplopia that was associated with his right eye turning in. He was seen by a physician who noted a right abducens nerve palsy and bilateral papilledema. He was referred for evaluation. His best- corrected visual acuity was 20/ 20 in both eyes. The pupils were 4 mm and 4.5 mm, right and left eyes, respectively, with brisk reaction to light and no relative afferent pupillary defect. He had a marked abduction deficit in the FIG. 1. Anteroposterior view of arteriogram of case 1 demonstrating large left Internal carotid aneurysm in the cavernous sinus. ABDUCENS NERVE PALSY AND HORNER'S SYNDROME / 5 right eye. His external examination revealed acromegalic features. Further questioning revealed that his ring size increased from 9.5 to 17.5 in the previous 3 years. The patient had 2 mm of ptosis, but no proptosis on the right. Color vision testing was within normal limits. Corneal sensation was intact. Kinetic perimetry demonstrated an enlarged blind spot in both eyes. Slit lamp examination was normal. Fundus examination revealed bilateral disc edema, but was otherwise normal. Cocaine testing failed to dilate the right pupil. A high- resolution CT scan showed a lesion compatible with a pituitary tumor that eroded the floor of the sella turcica and extended into the sphenoid sinus and suprasellar cistern. The erosion of the lateral wall of the sella displaced, but did not invade, the right cavernous sinus. Figure 2 is a T 1weighted magnetic resonance imaging ( MRI) scan showing the pituitary lesion involving the right cavernous sinus. Figure 3 is a Tz- weighted image of the same lesion. The brightness of the lesion in both T1- and Tz- weighted images suggests a hem- FIG. 2. MRI ( TR = 0.5; TE = 17) scan ( coronal) of the pituitary lesion in case 2. The left side of the image corresponds to the patient's right side. orrhagic content, although this could represent cholesterol or a mucocele. He was admitted to the hospital and underwent transsphenoidal resection of the pituitary adenoma; the adenoma had a hemorrhagic core. In the immediate postoperative period, he had partial resolution of the disc edema and Horner's syndrome; the left abduction deficit remained. DISCUSSION The abducens nerve pierces the dura of the clivus and passes around the petroclinoid ligament to enter the cavernous sinus. It travels within the sinus itself, rather than in the lateral wall. The nerve exits the sinus by way of the superior orbital fissure and innervates the lateral rectus muscle by multiple, dichotomously branching twigs ( 8). Postganglionic sympathetic nerve fibers enter the cavernous sinus with the internal carotid artery. Those fibers bound for the eye split off and join the sixth nerve within the cavernous sinus. Before entering the superior orbital fissure, the sympathetic fibers join the ophthalmic branch of the trigeminal nerve. Ultimately, these fibers join the nasociliary nerve, pass through the ciliary ganglion, and reach the eye with the long and short ciliary nerves ( 8- 11). The cavernous sinuses are symmetric structures paired on either side of the pituitary fossa. Dura forming the lateral wall contains the oculomotor, trochlear, ophthalmic, and maxillary nerves. The abducens and sympathetic nerves travel within the sinus, as does the internal carotid artery. The optic nerves and chiasm are above, and the sphenoid sinuses are anterior and inferior. Cavernous sinus lesions are characterized by multiple cranial nerve palsies and are frequently related to anomalous arteriovenous conditions, pituitary tumors, and sinus or orbital disease ( 9,12). Case 1 is interesting in that the Horner's syndrome was not appreciated initially. The initial anisocoria was unassociated with ptosis, and the Horner's subsequently became manifest. This apparently also occurred in the first case reported by Gutman and associates ( 1). The patient's trigeminal distribution numbness also progressed, initially involving the ophthalmic and then the maxillary distribution, presumably due to expansion of the intracavernous aneurysm. In cases of isolated sixth nerve palsy, the development of a Horner's syndrome, as well as involvement of other nerves I Clill Nelm1- ophthalmol, Vol. 8, No. L 1988 16 G. G. STRIPH AND R. M. BURDE FIG. 3. MRI ( TR = 1.5; TE = 120) scan ( midsagittal) of the pituitary lesion in case 2. traversing the cavernous sinus, should be recognized as having significant localizing value. Case 2 is of interest not only because of the association of the sixth nerve palsy and Horner's syndrome, but also because of the presence of disc edema in a patient with acromegaly. The sixth nerve paresis and its accompanying sympathetic fibers are best explained on the basis of increased pressure within the cavernous sinus secondary to expanding pituitary tumor pressing against the wall of the sinus. It is of note that the other cranial nerves within the sinus that travel in the lateral wall of the sinus remained unaffected. We are unaware of other patients in whom this combination of sixth nerve deficit and Horner's syndrome was caused by a lesion outside the cavernous sinus. Bilateral disc edema in association with acromegaly and a pituitary tumor was described previously by Mueller and colleagues ( 13). Disc edema is not commonly associated with growth hormone- secreting tumors or other pituitary tumors ( 14,15). Although disc edema in association with pituitary tumors is not common, it is belipved to nccur with massive tumors extending su-o 0 ".,"". 11 Il':', illll, producing an / eli" Nl'liro- ophthalmol, Vol. S, No. 1, 1988 obstructive hydrocephalus. Our second patient did have some minimal tumor extension into the suprasellar cistern; however, it was believed to be insufficient to cause bilateral papilledema. The etiology of the disc edema is unclear, although various theories have been proposed, including a local increase in intracranial pressure compressing the optic nerves, decreased venous outflow from the area of the chiasm, and elevated growth hormone causing an entrapment syndrome of the optic nerves, such as that seen in acromegalic patients with the carpal tunnel syndrome ( 13). Acknowledgment: This investigation was supported in part by an unrestricted grant from Research to Prevent Blindness, Inc., New York, NY ( Department of Ophthalmology). REFERENCES 1. Parkinson D. Bernard, MitchelL Horner syndrome and others? SlIrg NCllroI1979; 1l: 221- 3. 2. Abad ] M, Alvarez F. Blazquez MG. An unrecognized neurological syndrome: sixth- nerve palsy and Horner's syndrome due to traumatic intracavernous carotid aneurysm. SUyg NClly,, 1 1981; 16: 140- 4. 3. Gutman I, Levartovski S, Goldhammer Y, Tadmor R, ABDUCENS NERVE PALSY AND HORNER'S SYNDROME / 7 Findler G. Sixth nerve palsy and unilateral Horner's syndrome. Ophthaimoiosy 1986; 93: 913- 6. 4. Thompson HS, Mensher JH. Adrenergic mydriasis in Horner's syndrome. Am J Ophlhnil/ IOI 1971; 72: 472- 80. 5. Parkinson D. Traumatic aneurysms. SlIrg Nellrol 1985; 23: 253- 4. 6. Wemple JB, Smith GW. Extracranial carotid aneurysm. Report of four cases. I NellroslIrS 1966; 24: 667- 71. 7. Gelber BR, Sundt TM Jr. Treatment of intraca\' ernous and giant carotid aneurysms by combined internal carotid ligation and extra- to intracranial bypass. J Nell/"( ISlIrX l': lXO; SJ' 1- 10. 8. Miller NR. Walsh tlIld Hoyt's Clil/ ical Nellr< l' 0l'iltlltllllloh, SY, <' 01 2 ( ed 4). Baltimore: Williams & Wilkins, 1985; 424- 7, 5X3- 7, 703- 4. 9. Harris FS, Rhoton AL Jr. Anatomv of the cavernllUS sinw'. A microsurgical study. I NellroslIr,~ 1976; 45: 169- 80. 10. Johnston JA, Parkinson D. Intracranial sympathetic pathways assllciated with the sixth cranial nerve. J NellJ" llslIrS 1974; 39: 236- 43 11. Parkinslln D, Johnston], Chaudhuri A. Sympathetic connections to the fifth and sixth cranial nerves. AI/ at Rec 1978; 191: 221- 6. 12. Boxley TM, Schatz J. Clinical diagnosis of cavernous sinus svndromes. Nellr( ll Clil/ 1983; 1: 929- 53. 13. MUL'ller GL. McKenna T], Kelly G, L'l al. Papilledema in two patients with acromegaly and intrasellar pituitary tumors. Arch II/ Il'm lvled 1981; J41: 149J - 5. 14 Landolt AM, Wilson CB. Tumors of the sella and parasellar area in adults. In: Youmans JR ( ed), Ncrlrll/ o" ical SlIr" er\ f, 1' 015 ( ed 2). Philadelphia: W. B. Saunders, 1982: 3130. ' . J5. Hollenhorst RW, Yllunge BR. Ocular manifestations produced by adenomas of the pituitary gland: analysis of 1,000 cases. In: Kohler PO, Ross GT ( eds), DiaSI/' JSis al/ d TreatlIlel/ t "{ Pitllitllrtl TIII/ IOrs. Amsterdam: Excerpta Medica, 1973: 53- 68 I Ctil/ N" lln1-" l'llthalmt1l. 1\' 1. 8. N", 1. 1988 |
