Cerebral oximetry in patients with sickle cell disease.

Background: There is limited information concerning the brain's oxygen supply and demand in patients with sickle cell disease.

Design: We measured near-infrared spectroscopy of brain oxygenation in 27 patients with sickle cell disease regardless of vaso-occlusive crisis, 14 normal healthy controls, and five anaemic patients without sickle cell disease. We also measured pre- and post-transfusion cerebral oximetry in 14 additional sickle cell disease patients who were on transfusion programmes.

Results: The mean cerebral oxygen saturation in the combined steady-state and vaso-occlusive crisis population was found to be significantly lower than that in the controls and in anaemic patients without sickle cell disease (47.7% vs. 61.3%, 59.8%, P < 0.0001). Cerebral oxygen saturation failed to correlate with the haemoglobin concentration (r = 0.51, P > 0.5). However, cerebral oxygen saturation increased from 40.4% to 49.6% (P = 0.01) and correlated significantly with the haemoglobin level (r = 0.553, P = 0.003) in 14 subjects studied before and after transfusions. In seven subjects who received simple transfusions, cerebral oxygen saturation correlated strongly and positively with the haemoglobin level (r = 0.811, P = 0.001) and with percent normal haemoglobin (r = 0.786, P = 0.002), and negatively with abnormal sickle haemoglobin (r = -0.775, P = 0.003). None of these correlations was found to be statistically significant in the seven subjects given exchange transfusions. Cerebral oxygen saturation measured in the sickle cell disease subjects after transfusions was still significantly lower than in the anaemic subjects without sickle cell disease and in the normal controls (49.6% vs. 59.8% and 61.3%, P = 0.001).

Conclusions: We found that patients with sickle cell disease have subnormal values of cerebral oxygen saturation. Red cell transfusions significantly increased the brain oxygenation in these patients. Cerebral oximetry may be a useful, noninvasive method for assessing the effect of circulating normal red cells in sickle cell patients after transfusions.