Journal of Neuro-Ophthalmology, June 1988, Volume 8, Issue 2

Occult Pituitary Apoplexy Associated with Craniopharyngioma

Craniopharyngiomas generally are not considered in the differential diagnosis of pituitary apoplexy. A case of craniopharyngioma is presented, which emphasizes the need for magnetic resonance imaging in the setting of overt or subclinical pituitary apoplexy.

Journal of Clinical Neuro-ophthalmology 8(2): 99-104, 1988. Occult Pituitary Apoplexy Associated with Craniopharyngioma Rodney I. Kellen, M.B., B.C.H., MMed, F.R.C.S., Ronald M. Burde, M.D., and Fred J. Hodges, III, M.D. © 1988 Raven Press, Ltd., New York Craniopharyngiomas generally are not considered in the differential diagnosis of pituitary apoplexy. A case of craniopharyngioma is presented, which emphasizes the need for magnetic resonance imaging in the setting of overt or subclinical pituitary apoplexy. Key Words: Craniopharyngiomas-Pituitary apoplexy. From the Departments of Ophthalmology (R.I.K., R.M.B.), Neurology and Neurological Surgery (R.M.B.), and the Mal­hnkcrodt Institute of Radiology (F.}.H.), Washington Univer­Sity School of Medicine, St. Louis, Missouri. Address correspondence and reprint requests to Ronald M. Burde, M.D., Department of Ophthalmology, Montefiore Med­Ical Center, 111 East 210 Street, Bronx, NY 10467, U.S.A. 99 In nonobstetric cases, pituitary apoplexy implies sudden hemorrhagic necrosis of the pituitary gland associated with pituitary tumor. David and associates (1) regard pituitary apoplexy as a syn­drome produced by any sudden enlargement of a preexisting pituitary adenoma. The onset of this event is usually heralded by the classic triad of headache, oculomotor or afferent visual system dysfunction, and evidence of subarachnoid hem­orrhage. In the past, this condition was always considered to be an emergency, requiring urgent surgical decompression to prevent a fatal outcome or at least to minimize resultant morbidity (2-4). More recently, lesser forms of pituitary apoplexy have been described that were successfully man­aged without surgical intervention (5-8). Thus, it is appropriate to consider pituitary apoplexy as a state with a spectrum of severity ranging from subclinical to rapidly fatal (7). We recently saw a patient who presented with an occult hemorrhage in a preexisting craniopharyngioma following minor head trauma. CASE REPORT A 37-year-old white man was diagnosed as having a craniopharyngioma at age 15. He pre­sented at that time with a history of diplopia and was found to have external ophthalmoplegia and hydrocephalus. Radiographic studies demon­strated intracranial parasellar calcification. Despite a lack of tissue diagnosis, the sellar region was ir­radiated (dose unknown). Until October of 1986, the patient did well with stable visual acuities of 20/15 in both eyes and normal visual fields and ex­traocular motility. He did, however, exhibit endo­crine dysfunction, i.e., he was impotent with low serum testosterone, and he also required mainte- OCCULT PITUITARY APOPLEXY 101 FIG. 1. Midsagittal T,-weighted image showing high (white), intermediate (gray), and low (black) signal intensity making up the intra- and suprasellar mass. The high signal below the mass posteriorly is due to the fatty bone marrow of the clivus, while the absent signal beneath the sella represents air in the sphenoid sinus (T, = TR 500, TE 15). tuitary apoplexy using CT scans and believed they could identify blood within an adenomatous pitu­itary gland. They were further able to discriminate between acute and chronic hemorrhage. In unen­hanced scans they noted soft-tissue masses with acute hemorrhage or areas of low density alone in­dicating chronic hemorrhage or necrosis. With contrast there was no enhancement, partial en­hancement, uniform enhancement, or ring-like enhancement. Post et al. did, however, recognize that some cases of both acute and chronic hemor­rhage could be overlooked. Fujimoto and asso­ciates (15) described a density level of fluid blood within a ring-enhancing lesion, which they be­lieved was almost certain evidence of pituitary apoplexy within a pituitary adenoma. In the pres­ence of a prominent episode of pituitary apoplexy the mere presence of a large sellar mass was thought to be adequate to confirm the diagnosis (16). In all these cases, angiography was under­taken to exclude a suprasellar aneurysm. The advent of MR imaging has greatly enhanced the physician's ability to detect intracranial pa­thology, even where CT has failed (17,18). MR imaging is particularly useful in detecting the presence of subacute hemorrhage. Several tissues are known or thought to cause elevated signal in TI-weighted, Tz-weighted, and proton density se­quences, including subacute or chronic hemor­rhage, cholesteatoma, and mucocele. Mixed signals could, of course, represent a variety of normal and pathologic tissues, but the lucent ap­pearance on CT, in this case, tends to exclude such entities as recent blood clot, intrasellar calci­fication, and solid pituitary adenoma or menin­gioma. Cerebrospinal fluid as in empty sella and arachnoid cyst; cystic craniopharyngioma or cystic adenoma; epidermoid, dermoid or teratomatous cyst; inflammatory exudate (abscess); mucocele; and fatty tissue are considerations. Craniopharyngioma may contain cholesterol, keratin nodules, epithelial nests or cords, hemor­rhagic products, and necrosis (19). By MR imaging it is frequently cystic appearing (20). High protein J Clill Neuro-ophthaltllol, Vol. 8, No.2, 1988 OCCULT PITUITARY APOPLEXY 103 .. _l> FIG. 3. Photomicrograph of cholesterol clefts (small arrow) with associated degenerating red blood cells (large arrow). Cystic spaces lined by palisading epithelial cells are typical of craniopharyngioma (asterisks) ( x 90). and suprasellar lesion could easily represent hem­orrhage 1 or more weeks old. The clinical presentation of our patient with a history of recent minor head trauma associated with decreased vision, abnormal visual fields, and a past history of a para sellar mass suggests sudden expansion of the parasellar mass lesion. Surgical and histopathological confirmation of hemorrhage into the craniopharyngioma is, we feel, supported by the MR findings, despite the lack of absolute precision in defining tissue type by MR imaging. Although craniopharyngioma previously was not thought to be associated with hemorrhagic infarction of the pituitary gland (22), it should be considered in the differential diag­nosis of pituitary apoplexy. When acute hemor­rhage is clinically suspected in the setting of a known parasellar tumor, CT is more diagnostic in the first week. 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