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Show ORIGINAL CONTRIBUTION Pituitary Apoplexy Causing Optic Neuropathy and Horner Syndrome Without Ophthalmoplegia Robert K. Shin, MD, Brett L. Cucchiara, MD, David S. Liebeskind, MD, Grant T. Liu, MD, and Laura J. Balcer, MD, MSCE Abstract: A 47- year- old woman presented with headache, acute monocular vision loss, and ipsilateral Horner syndrome. Apart from the optic neuropathy, all cranial nerve function was intact. Magnetic resonance imaging revealed an enlarged pituitary gland with compression of the orbital apex. The surgical specimen was consistent with pituitary apoplexy. The combination of headache, acute visual loss, and ipsilateral Horner syndrome without ophthalmoplegia, which may suggest carotid artery dissection, is evidently an unusual manifestation of pituitary apoplexy. ( JNeuro- Ophthalmol 2003; 23: 208- 210) Pituitary apoplexy, the sudden enlargement of a pituitary tumor, is a rare but potentially life- threatening condition. It is highly variable in presentation but is typically characterized by sudden headache, vision loss, and ophthal-moparesis and is generally associated with either tumor infarction or hemorrhage ( 1,2). The visual abnormalities, if monocular, may mimic retrobulbar optic neuritis ( 3). Oculomotor nerve palsies occur in more than 50% of patients with pituitary apoplexy, but trochlear and abducens palsies, or even complete ophthalmoplegia, can occur as well ( 4). These ocular motor findings may be unilateral or bilateral. We describe a patient with acute monocular vision loss and painful Horner syndrome, with no impairment of extraocular movements, as an unusual presentation of pituitary apoplexy. CASE REPORT A 47- year- old woman awoke with " blurry vision" in the OS and a headache over her left forehead and temple. She presented the following day to the ophthalmology clinic. Her past medical history was significant for hypertension, congestive heart failure, and a left ventricular Departments of Neurology and Ophthalmology, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania. Address correspondence to Laura J. Balcer, MD, MSCE, Department of Neurology, 3E Gates, 3400 Spruce Street, Philadelphia, PA 19104- 4283, USA; E- mail: lbalcer@ mail. med. upenn. edu apical thrombus. She had no prior ophthalmologic history. The patient's medications included lisinopril ( Zestril), fu-rosemide ( Lasix), digoxin, and warfarin ( Coumadin). She had a history of tobacco use and admitted to poor compliance with her medications. On examination, visual acuities were 20/ 15 OD and hand motions OS. The pupils were equal, measuring 4 mm in diameter in dim illumination. There was a brisk consensual response to direct light stimulation OD to 2 mm. A prominent ( 3+) relative afferent pupillary defect was present OS. The patient perceived 14/ 15 Ishihara color plates with the OD but could not see the control color plate with her left. Extraocular movements were full. Slit- lamp examination and ophthalmoscopy were normal. Confrontation visual field testing was normal in the OD but revealed dense central and inferior altitudinal defects in the OS. Computed tomography showed no evidence of intracranial hemorrhage, but the sella was noted to be " top normal" in size. She was found to be suboptimally anticoagu-lated ( INR = 1.2). Several hours later, the patient developed 2 mm of ptosis OS, 1 mm of lower lid elevation OS, and anisocoria ( pupillary diameters 6 mm OD, 3 mm OS in darkness). She continued to demonstrate a brisk consensual response to light stimulation OD. Pupillary dilation, however, was sluggish OS. There was no loss of facial sensation; corneal reflexes were intact bilaterally. Magnetic resonance angiography of the neck and head showed no evidence of carotid artery dissection, and echocardiography found no trace of her previously identified left ventricular thrombus. Magnetic resonance imaging revealed a 2 cm sellar mass consistent with a pituitary adenoma. No hemorrhage was identified, but there was peripheral enhancement and eccentric swelling to the left side, indenting the left optic nerve ( Fig. 1). Based on the acute clinical presentation and magnetic resonance imaging findings of a large sellar mass, the patient was diagnosed with pituitary apoplexy. She received high- dose corticosteroid treatment and underwent transsphenoidal resection of the tumor. Pathologic findings were consistent with bland infarction of a pituitary adenoma. Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. 208 J Neuro- Ophthalmol, Vol. 23, No. 3, 2003 PITUITAR Y APOPLEXY JNeuro- Ophthalmol, Vol. 23, No. 3, 2003 FIG. 1. Enhanced coronal T1- weighted magnetic resonance imaging scan shows asymmetric peripheral enhancement ( black arrow) of a 2 cm sellar mass. The left optic nerve ( white arrowhead) can be seen adjacent to the area of enhancement and slightly indented. Following surgical decompression of the tumor, the patient's headache resolved and visual acuity OS improved to 20/ 40 within 5 days. One year later, her visual acuity was 20/ 25 OS. Mild left optic nerve pallor and a small inferior nasal field defect to confrontation ( confirmed by automated perimetry) remained. There was no residual ptosis or anisocoria. DISCUSSION Our patient's unusual combination of acute optic neuropathy and painful Horner syndrome in the absence of ocular motility findings emphasizes the range of presentations of pituitary apoplexy. We can explain this combination of findings by considering the anatomy of the cavernous sinus and orbital apex. Postganglionic oculosympathetic fibers travel within the wall of the internal carotid artery into the cavernous sinus. Within the cavernous sinus, oculosympathetic fibers travel briefly with the abducens nerve ( VI), and ultimately join the ophthalmic nerve ( Vj) ( 5). Although the combination of Horner syndrome with pain in a trigeminal distribution might suggest a lesion in the cavernous sinus ( 6), such a localization would not account for the presence of visual loss. Moreover, because the ophthalmic nerve ( Vj) lies in the inferolateral wall of the cavernous sinus ( Fig. 2A), it would be unlikely for an expanding pituitary mass to affect it in the cavernous sinus while sparing the adjacent ocular motor nerves ( III, IV, and VI). More anteriorly, however, the ophthalmic nerve ( Vj) travels superiorly and medially as it approaches the orbital apex. The nasociliary branch of Vj enters the orbit through the medial aspect of the superior orbital fissure, carrying oculosympathetic fibers to the pupil dilator ( Fig. 2B). A pituitary mass could expand laterally and anteriorly to Chiaassmm_ ^) Internal Carotid Artery L Annulus of Zinn Pituitary Gland V1 V2 Superior Oblique m lllrd n. Medial IVth n. Rectus m. Nasociliary n B Superior Rectus m. QJ i~~~- Frontal n. Lacrimal n. IVth n. Lateral Rectus m. Inferior \ | Vlth n. Rectus m. \-* t Superior Orbital Fissure FIG. 2. A: Schematic coronal view of the left cavernous sinus. Cranial nerve V-, lies in the inferolateral wall. The dotted line demonstrates the location of our patient's pituitary mass with impingement on the left internal carotid artery wall and sympathetic fibers. B: Schematic coronal view of the left superior orbital fissure and annulus of Zinn. Note that the nasociliary branch of V-, travels medially, closest to the optic nerve. In our patient, the optic nerve and nasociliary branch of \ A| were likely compressed by tumor at the intracranial exit from the optic canal. Adapted with permission ( 13). Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. 209 JNeuro- Ophthalmol, Vol. 23, No. 3, 2003 Shin et al. compress both the optic nerve and the oculosympathetic fibers of the nasociliary nerve at the orbital apex, parsimoniously explaining the concurrence of optic neuropathy and oculosympathetic deficit in the absence of ocular motor palsies in our patient. Compression of the superior portion of the optic nerve against the dural shelf of the optic canal, which can occur when the optic nerve is elevated by tumor, may also provide an anatomic explanation for the inferior location of our patient's monocular visual field loss ( 7). As the combination of optic neuropathy and Horner syndrome has not, to our knowledge, been previously reported in the setting of pituitary apoplexy, it must be rare. As a cause of painful Horner syndrome, cervical or intracavernous carotid artery dissection is more common than pituitary apoplexy. In one report, painful Horner syndrome was present in 44% of patients with extracranial internal carotid artery dissection ( 8). In such cases, the oculosympathetic deficit originates at the site of dissection, where postganglionic fibers traveling within the wall of the carotid artery can be interrupted. Transient monocular vision loss or ischemic optic neuropathy may accompany the Horner syndrome in this setting ( 9). The vision loss results from embolism or reduced blood flow to the retina or optic nerve. Periocular, brow, and scalp hypesthesia and pain may occur in the setting of carotid dissection ( 10). Two mechanisms have been suggested to explain these manifestations ( 11). In the case of intracavernous carotid artery dissection, compression of Vj trigeminal fibers may occur; in the case of cervical carotid artery dissection, damage occurs to Y1 trigeminal afferent fibers due to mi-croembolization of nutrient arteries supplying the trigeminal nerve ( 12). Thus, the triad of optic neuropathy, trigeminal dysfunction, and Horner syndrome is hardly specific to orbital apex lesions. Carotid artery dissection must be ruled out in any patient, particularly if there are signs of inadequate optic nerve or retinal perfusion or retinal emboli on fundu-scopic examination. REFERENCES 1. McFadzean RM, Doyle D, Rampling R, et al. Pituitary apoplexy and its effect on vision. Neurosurgery 1991; 29: 669- 75. 2. Ostrov SG, Quencer RM, Hoffman JC, et al. Hemorrhage within pituitary adenomas: how often associated with pituitary apoplexy syndrome? AM 1989; 153: 153- 60. 3. Petersen P, Christiansen KH, Lindholm J. 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