Subarachnoid hemorrhage in sickle-cell disease

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✓ The neurological complications of sickle-cell disease include cerebral infarction and intracerebral hemorrhage; subarachnoid hemorrhage (SAH) has been infrequently reported. Among 325 patients with sickle-cell disease followed at the University of Illinois between 1975 and 1989, 11 cases of SAH were identified. Aneurysms were found in 10 of these patients, three of whom had multiple aneurysms. All of the patients had some degree of anemia and nine underwent craniotomy without hematological or neurological complications. From this review it appears that SAH is not uncommon in sickle-cell disease patients and tends to occur at a younger age and with smaller aneurysm size than in the general population. With proper perioperative management, including exchange transfusions to reduce the proportion of hemoglobin S to less than 30%, these patients can undergo angiography and craniotomy without an increased incidence of complications. The techniques used in managing sickle-cell disease patients with SAH are discussed.

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Address reprint requests to: John A. Anson, M.D., Department of Neurosurgery, Barrow Neurological Institute, 350 West Thomas Road, Phoenix, Arizona 85013-4496.

© AANS, except where prohibited by US copyright law.

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Figures

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    Location of 16 aneurysms in 11 sickle-cell patients with subarachnoid hemorrhage.

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    Photomicrographs of a section of middle cerebral artery lesion in a patient with sickle-cell disease. A: The internal elastic lamina is broken and irregular. There is proliferation of the intima, but the media is normal without primary disease of the vasa vasorum. H & E, × 12. B: Higher-power view showing sickled red blood cells incorporated into the proliferated intima. Note that cells often do not appear sickled when in tissue section. H & E, × 50.

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    Case 11. Left: Preoperative cerebral angiogram demonstrating a 15 × 10-mm aneurysm of the left carotid artery, presumably at the origin of the ophthalmic artery. Right: Postoperative cerebral angiogram showing successful clipping of the carotid aneurysm with no residual aneurysm or vasospasm.

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