Introduction Cerebral infarction secondary to occlusive vasculopathy is a major complication of patients with sickle cell dis- ease (hemoglobin SS) with a prevalence ranging from 5.5 % [1] to 17 % [2]. The stenotic lesions involve large vessels in the intracranial internal, middle, and anterior cerebral artery circulation and progress for months and years before symptoms develop. Prevention of stroke symptoms by hypertransfusion therapy is theoretically Joanna J. Seibert Charles M. Glasier Russell S. Kirby Janice W.Allison Charles A. James David L. Becton David L. Kinder Katie S. Cox Ellie L. Flick Fabian Lairry Jacquelyn Ford Jackson Rochelle A. Graves Transcranial Doppler, MRA, and MRI as a screening examination for cerebrovascular disease in patients with sickle cell anemia: an 8-year study Received: 17 April 1997 Accepted: 7 July 1997 Presented at the Third Conjoint Meeting, International Pediatric Radiology, Boston, Massachusetts, 25±30 May 1996 J. J. Seibert ( ) ) × C. M. Glasier × J. W. Allison × C. A. James × D. L. Kinder × K. S. Cox × F.Lairry × R. A. Graves Department of Radiology/Slot 105, Arkansas Children's Hospital, 800 Marshall St., Little Rock, AR 72202-3591, USA R. S. Kirby × E. L. Flick The Center for Ambulatory Research, The University of Arkansas for Medical Sciences and Arkansas Children's Hospital, Little Rock, Arkansas, USA D. L. Becton × J. F. Jackson Department of Hematology, The University of Arkansas for Medical Sciences and Arkansas Children's Hospital, Little Rock, Arkansas, USA Abstract Objective. The authors previously reported five transcranial Doppler ultrasonography (TCD) findings as significant in detecting clinical cerebrovascular disease in a 4-year study in patients with sickle cell disease. This is a follow-up to evaluate the validity of the original findings over another 4-year period during which the study population doubled. A clinical follow-up of the original asymptomatic sickle cell patients with positive TCD, MRA, and MRI was also made. Materials and methods. Over an 8- year period TCD, MRI, and MRA were prospectively performed in 90 sickle cell patients who were clinical- ly asymptomatic for stroke and in 27 sickle cell patients with clinical stroke. Results. Of the 4 out of original 46 control patients in 1992 who had pos- itive MRA and TCD, 3 have subse- quently had clinical stroke. None of the 9 original patients with positive TCD and positive MRI but negative MRA have developed stroke. All five original TCD indicators of dis- ease were still significant (P < 0.05) for detecting clinical disease: maxi- mum velocity in ophthalmic artery (OA) > 35 cm/s, mean velocity in middle cerebral artery (MCA) > 170 cm/s, resistive index (RI) in OA < 50, velocity in OA greater than in MCA, and velocity in posterior ce- rebral (PCA), vertebral, or basilar arteries greater than in MCA. Four additional factors were also signifi- cant: turbulence, PCA or ACA with- out MCA, RI < 30, and maximum velocity in MCA > 200 cm/s. Conclusion. Positive MRA with a positive TCD in an asymptomatic patient in long-term follow-up sug- gests a trend for developing clinical stroke. A 4- to 8-year follow-up of nine patients with positive TCD, positive MRI, but not positive MRA did not show development of clinical stroke. Nine Doppler findings are significant in screening for clinically symptomatic vascular disease in sickle cell patients. It is recommend- ed that children with sickle cell dis- ease be screened for cerebrovascular disease with TCD. If one or two indi- cators of abnormality are present, MRA is recommended. If the MRA is positive, the patient may be con- sidered for transfusion therapy or other treatment for prevention of stroke. Pediatr Radiol (1998) 28: 138±142 Ó Springer-Verlag 1998