Journal of Neuro-Ophthalmology, March 1984, Volume 4, Issue 1

Pituitary apoplexy presenting with epistaxis.

A 42-year-old woman developed headache and epistaxis followed by fever, stiff neck, and loss of vision of the right eye. The diagnosis of simple epistaxis was changed to mucormycosis, then to bacterial meningitis and then to sphenoid sinusitis, before the correct diagnosis of pituitary apoplexy was established by CT scan. Epistaxis is yet another confusing symptom of pituitary apoplexy.

J. Clin. Neuro-ophthalmol. 4: 7-8, 1984. Pituitary Apoplexy Presenting with Epistaxis JAMES R. KEANE, M.D. Abstract A 42-year-old woman developed headache and ep­istaxis followed by fever, stiff neck, and loss of vision of the right eye. The diagnosis of simple epistaxis was changed to mucormycosis, then to bacterial meningitis and then to sphenoid sinusitis, before the correct diagnosis of pituitary apoplexy was established by CT scan. Epistaxis is yet an­other confusing symptom of pituitary apoplexy. Epistaxis is rarely the herald of neurologic dis­orders. Minor nosebleeds occasionally precede cranial nerve involvement by tumors of the na­sopharynx or by mucormycosis. Severe epistaxis may be the first sign of a rare post-traumatic carotid artery aneurysm in the cavernous sinus. l In the following patient, some of the more com­mon manifestations of hemorrhage into a pitui­tary tumor were preceded by epistaxis. Case Reports A 42-year-old woman came to the emergency room following a nosebleed ("one cup of blood") accompanied by severe headache. She had prominent eyes and coarse facial fea­tures/ and flecks of blood (but no necrosis) were seen in the nose and nasopharynx. When a screening blood glucose showed a value of 544 mg/dl, she was admitted to the diabetes service with a diagnosis of possible muconnycosis. The following day her temperature rose to 1020 and she experienced neck stiffness, face pain, vomiting, and confusion. After a lumbar puncture showed 1,800 RBCs, 72 WBC (4% PMNs), a protein of 252 mgjdl, and a glucose of 117 mgj dl, she was transferred to the infectious disease service with the diagnosis of bacterial meningitis. Nasal sinus x-rays were read as nonnal, but sinus tomograms and a limited CT scan showed definite sphenoid sinus opacification. On the fourth hospital day, a loss of vision was From the Department of Neurology, Los Angeles County­University of Southern California Medical Center, Los Ange­les, California. March 1984 noted (light perception only in the right eye, nonnal acuity and visual fields in the left) and preparations were made for drainage of presumed bacterial sinusitis. However, a repeat CT scan demonstrated a suprasellar and intrasellar tumor that extended into the sphenoid sinus (Fig. 1). She was begun on steroid therapy and during the next week the headaches eased and her vision returned to nonnal. Endocrine studies docu­mented high levels of growth honnone. Subse­quent transphenoidal removal of a necrotic pitui­tary adenoma was uneventful. Discussion The manifestations of sudden enlargement of a pituitary adenoma due to hemorrhage or in­farction have been widely reported and well­publicized,~- 4 but the diagnosis continues to be a difficult one because of the wide variety of symp­toms and signs. Lateral extension of the tumor will compress the ocular motor nerves in the cavernous sinus, while suprasellar swelling and hemorrhage produce sterile hemorrhagic menin­gitis and chiasmal compression. An acute oculomotor nerve palsy accompanied by headache and stiff neck suggests a ruptured cerebral aneurysm. The presence of stupor or coma will interfere with the precise elucidation of eye signs and a diagnosis of brain stem stroke may seem warranted. Fever, obtundation, and stiff neck all point to the diagnosis of meningitis; when lumbar puncture seemingly confinns this diagnosis, the possibility of a noninfectious cause often is overlooked. In the presence of fever, stiff neck, and rapid immobility of one or both eyes, a diagnosis of septic cavernous sinusitis may be made. Complete ophthalmoplegia may suggest botulism, acute ophthalmoplegic polyneuropa­thy/ or the Wernicke-Korsakoff syndrome (Table 1). Recognizing pituitary apoplexy in all of its manifestations is a test of diagnostic acumen. Skull x-rays may not show obvious sellar changes, and a high-quality CT scan is essential for confirmation of the diagnosis. 3 Hemorrhage and necrosis occur in up to 10% of pituitary tumors. ~ Dramatic tumor bleeding and swelling, true pituitary apoplexy, is a much 7 Epistaxis TABLE 1. Pituitary Apoplexy-Differential Diagnosis Diagnosis Distinguishing Features 1. Bacterial meningitis CSF glucose low/chiasmaI signs rare 2. Ruptured intracranial More CSF blood and fewer aneurysm WBCs acutely 3. Sphenoid sinusitis Possible history of sinusitis 4. Muconnycosis Preexisting disease (diabetes)/ necrosis 5. Cavernous sinusitis Proptosis and conjunctival edema 6. Brain stem stroke Long tract signs/chiasm spared 7. Acute cranial neuro- Absent reflexes/facial weak-pathy (Guillain- ness/vision spared Barre syndrome) 8. Wernicke-Korsakoff Ataxia/malnutrition/nonnal syndrome CSF-pupils-vision less common condition found in 1% or less of • • ") 4: pItUitary tumors.-· The present case adds epistaxis to the many symptoms of pituitary apoplexy. It is surprising that nosebleeds are not more common since larger pituitary adenomas frequently extend into the sphenoid sinus. Indeed, spontaneous or post­treatment involution of such tumors is one of the causes of nontraumatic cerebrospinal fluid rhi­norrhea." Figure 1. Saggital reconstruction through the sella turcica (in plane illustrated in the [ower, horizontal CT section) shows tumor in the sphenoid sinus with disrup­tion of the sellar floor. References 1. Keane, J.R., and Talalla, A: Post-traumatic intra­cavernous aneurysm: Epistaxis with monocular blindness preceded by chromatopsia. Arch. Ophthalmol. 87: 701-705,1972. 2. Mohr, G., and Hardy, J.: Hemorrhage, necrosis, and apoplexy in pituitary adenomas. Surg. Neural. 18: 181-190, 1982. 3. Post, M.}.D., and David, N.}.: Pituitary apoplexy: A radiographic-clinical correlation. In Neuro-oph­thalmology Focus, }.L. Smith, Ed. Masson Publish­ing USA, New York, 1982, pp. 395-401. 4. Ebersold, M.J., Laws, E.R., Jr., Scheithauer, B.W., and Randall, R.Y.: Pituitary apoplexy treated by transsphenoidal surgery: A clinicopathological and immunocytochemical study. f. Neurosurg. 58: 315­320, 1983. 5. Landolt, AM.: Cerebrospinal fluid rhinorrhea: A complication of therapy for invasive prolactino­mas. Neurosurgery 11: 395-401, 1982. Acknowledgments The author thanks Murray Thale, M.D., who pro­vided neurosurgical details; and Jamshid Ahmadi, M.D., who assisted with radiographic interpretations. Write for reprints to: James R. Keane, M.D., 1200 North State Street, Los Angeles, California 90033. Journal of Clinical Neuro-ophthalmology