Emergency diagnosis of pituitary apoplexy

Brief Reports

Emergency Diagnosis of Pituitary Apoplexy DENISE M. WILSON, DO,* ROBERT F. SAUL, MDt

A 6&yearokl man, chronically anticoagulatedfor a prosthetic heart valve, sufferedvisual loss from hemorrhageinto an unsuspectedpiiuitaty tumor. The diagnosisof pituitaryapoplexywas made in the emergencydeparhnent. The pabnt’s coagulopathywas reversed, and he undetwentan uneventful transsphenoidalhypophysectomy.Marked recovery of vision occurred over the next six months.The clinicalfeaturesof this uncommonlydiagnosedsyndrome are reviewed, and the impotiance of recognizingthis entity with the adventof computedtomographicscanningis emphasized.(Am J EmergMed 1987;5:375378)

The diagnosis of a pituitary adenoma is infrequently made in the emergency department. Patients usually complain of a gradual decline in vision or symptoms of endocrine dysfunction and most commonly are seen as outpatients. Patients with pituitary tumors who require emergency management are uncommon and usually have developed sudden hemorrhage or ischemic infarction of the mass, a condition known as pituitary apoplexy. It is rarely recognized as the primary diagnosis on initial examination. ’ We present a case of a patient who was transferred to our institution with the diagnosis of possible intracranial hemorrhage, only to discover in the emergency department that he had bled into an unsuspected pituitary tumor. It is important for the emergency physician to recognize this entity early to facilitate appropriate surgical and medical management. CASE REPORT A 60-year-oldman awakened from sleep with a severe, occipital headache. Over the next 24 hours, he developed nausea, vomiting, From the Departments of *Emergency Medicine and tNeurology and Ophthalmology, Geisinger Medical Center, Danville, Pennsylvania. Manuscript received February 16, 1986; revision accepted January 21, 1987. Address reprint requests to Dr. Saul: Departments of Neurology and Ophthalmology, Geisinger Medical Center, Danville, PA 17822. Key Words: Acute chiasmal syndrome, pituitary apoplexy, sudden blindness. 0735-6757187 $0.00 + .25

blurred vision, and increasing lethargy. He was seen at a local hospital emergency department and was transferred to our institution for evaluation of a suspected intracranial hemorrhage. Eight years earlier he had undergone aortic valve replacement for aortic stenosis. He required chronic anticoagulation. He had noted a decrease in visual acuity of each eye for the last year, unimproved with corrective lenses. He had no sexual drive and was unable to perform intercourse for the last three years. There was no history of hypertension, diabetes mellitus, recent trauma, or infection. In the emergency department, the patient was somnolent, but arousable and conversant. Blood pressure was 156/66 mm Hg; heart rate, 88 bpm; respirations, 16 breaths/min; and temperature, 37.2”C orally. The neck was supple, and there was a left hemotympanum. His visual acuity was 20170 in the right eye and 20/800 in the left eye. The right pupil was 0.5 mm larger than the left, sluggishly reactive, with an afferent defect. Fhnduscopic examination showed mild optic nerve pallor bilaterally. Confrontation visual fields showed a bitemporal defect. There was no ocular nerve palsy. The remainder of his neurologic examination was normal. His cardiac examination was consistent with a functioning aortic valve prosthesis. Pertinent laboratory values included a leukocyte count of 10,400 cells/mm3 with a normal differential; hemoglobin, 9.3 g/dl; hematocrit, 27%; and platelet count, 257,000 cells/mm3. The urinalysis, blood urea nitrogen, and electrolytes were normal. The prothrombin time was 27 seconds with a control of 11 seconds. The partial thromboplastin time was 52 seconds with a control of 31 seconds. Chest radiographs showed the prosthetic valve in proper position, and there was left ventricular hypertrophy on the electrocardiogram. Computed tomography (CT) of the brain showed an enhancing intrasellar mass lesion with suprasellar extension (Figs. 1 and 2). One area of high density absorption value was seen in the superior aspect of the dome of the mass before contrast (Fig. 3). The radiographic features were thought to be most compatible with hemorrhage into a pituitary tumor. The patient was admitted and given 10 mg of dexamethasone sodium phosphate and 0.2 mg of levothyroxine sodium parenterally. Therapy with phytonadione and fresh frozen plasma reversed his coagulopathy. Cerebral angiography excluded a cerebral aneurysm. The patient underwent uneventful transsphenoidal surgery with removal of a large hemorrhagic pituitary adenoma. Over the ensuing six months, his visual acuity returned to 20120 in each eye, and his visual fields showed minimal bitemporal defects.

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Coronal contrast enhanced

FIGURE 2 (bork~~ kf). FIGURE 3

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(CT) scan of the brain showing intrasellar

CT scan of same mass filling suprasellar

mass with suprasellar

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(bottomright). Unenhanced axial view showing high-density areas in superior aspect of the dome of mass compatible with fresh hemorrh lage

WILSON AND SAUL H EMERGENCY DIAGNOSIS OF PITUITARY APOPLEXY

DISCUSSION Pituitary apoplexy is the syndrome used to encompass the clinical picture of massive sudden hemorrhage or infarction of a pituitary tumor. This condition is uncommon and frequently misdiagnosed initially.’ It may vary in its clinical presentation and may mimic intracranial or subarachnoid hemorrhage, bacterial meningitis, sudden expansion of an intracavemous aneurysm, or cavernous sinus thrombosis. We reviewed the most common presenting signs and symptoms in 28 cases from several recent studies. The earliest symptoms, in order of frequency, included severe headache, visual loss, diplopia, nausea, vomiting, lethargy, and stiff neck.2-4 Neuro-ophthalmic impairment depends on the direction the expanding mass takes. Upward extension of the tumor into the optic chiasm produces bitemporal visual field defects. Lateral expansion causes encroachment of invasion of the cavernous sinus, resulting in various combinations of bilateral ocular nerve palsies. Occasionally, an isolated oculomotor palsy may herald the onset of the apoplectic event .5 Along with the neuro-ophthalmic signs and symptoms, there may be clues of preexisting endocrine dysfunction. Hair loss, lethargy, and sexual impotence should arouse suspicion of anterior pituitary involvement. Symptoms of polydipsia and polyuria point to less common neurohypophyseal encroachment. Severe, acute panhypopituitarism can lead to death in a short time, necessitating rapid replacement of cortisol and possibly thyroid hormone. A number of predisposing conditions have been associated with pituitary apoplexy. These include trauma, radiation, estrogens, sudden changes in blood pressure, diabetic ketoacidosis, bromocriptine, and anticoagulation.“’ Our patient had many of the salient features of pituitary apoplexy, specifically, sudden onset of severe headache, visual loss, altered mentation, and evidence of preexisting endocrine dysfunction. We believe that the use of anticoagulants probably predisposed this patient to hemorrhage . With the easy accessibility of CT, the diagnosis of pituitary apoplexy can be made earlier and must be recognized by the acute care physician as a neurosurgical emergency. The typical CT findings include a contrast-enhancing intrasellar mass that may extend into the suprasellar area and/or the cavernous sinus.* Fresh hemorrhage may be suspected when there are areas of high density on the uncontrasted CT scan. A low-density area that does not enhance is compatible with ischemic infarction. If CT scanning is not readily available, a plain roentgenogram of the skull will show an eroded, enlarged sella turcici (Fig. 4). After confirming a clinical suspicion of a primary intrasellar mass that has acutely enlarged, prompt referral to a center with a neurosurgeon is necessary.

FIGURE 4. Lateral skull roentgenogram turcica with a double floor (arrow).

showing enlarged eroded sella

At this juncture, the condition of the patient determines further evaluation and treatment. Progressive visual loss, ophthalmoplegia, or mental obtundation dictates immediate cerebral angiography or magnetic resonance imaging to exclude a giant internal carotid artery aneurysm, which may present in exactly the same manner. Once accomplished, intravenous administration of corticosteroids to prevent acute adrenal failure followed by transsphenoidal hypophysectomy is recommended. Without significant neuro-ophthalmic compromise, the patient who is awake and alert probably can be watched, evaluated for appropriate hormonal replacement, and observed for any evidence of developing diabetes insipidis. Once stabilized, the patient can undergo operation electively. In conclusion, the combination of sudden headache, visual loss, and/or ophthalmoplegia should lead the clinician to suspect the possibility of pituitary apoplexy. Historical evidence of pituitary dysfunction adds further credence to the diagnosis and should prompt immediate neuro-ophthalmic and neurosurgical consultation. CT scanning is the most rapid means of radiographically confirming one’s clinical suspicion. Rapid administration of intravenous corticosteroids may be lifesaving. REFERENCES 1. David NJ, Gargano FP, Glaser JS: Pituitary apoplexy in clinical perspective. In Glaser JS, Smith JL (eds): Neuro-Ophthalmology, vol. 8. St. Louis, CV Mosby, 1975, pp 140-165

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2. Post MJD, David NJ: Pituitary apoplexy: Radiographic-clinical correlation. In Smith JL (ed): Neuro-Ophthalmology Focus, 1982. New York, Masson, 1982, pp 177-221 3. Conomy JC, Ferguson JH, Brodkey JS: Spontaneous infarction in pituitary tumors: Neurologic and therapeutic aspects. Neurology 1975;25:580-587 4. Saul RF, Hilliker JK: Third nerve palsy: The presenting sign of a pituitary adenoma in five patients and the only neurological sign in four patients. J Clin Neuro-Ophthalmol 1985;5:185-193 5. Cardoso ER, Peterson EW: Pituitary apoplexy: A review. Neurosurgery 1984;14:363-373

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6. Rovit RL, Fein JM: Pituitary apoplexy: A review and reappraisal. J Neurosurg 1972;37:280-282 7. Weisberg LA: Pituitary apoplexy: Association of degenerative change in pituitary adenoma with radiotherapy and detection by cerebral computerized tomography. Am J Med 1977; 63:109-l 15 8. Rengachaty SS, Tomita T, Jeffreys BF, et al: Structural changes in human pituitary tumors after bromocriptine therapy. Neurosurgery 1982;10:242-251 9. Nourizadeh AR, Pitts FW: Hemorrhage into pituitary adenoma during anticoagulant therapy. JAMA 1965;193:623*25