Cerebrovascular lesions in sickle cell pediatric patients: Transcranial color duplex doppler sonography amd diffusion weighted MRI study with clinical correlation

ABSTRACT

Sickle cell disease [SCD] s a common inherited hemoglobin disorder. Cerebral infraction secondary to occlusive vasculopathy is a major complication in children with SCD, with a prevalence of 5.5% to 17%. Recent MRI studies have also reported a high incidence of silent infarcts [11-16%]. Trans cranial color duplex Doppler [TCD] study can reliably predict which children with SCD are at great risk for developing a stroke. Aim of the work Is to correlate the TCD and MRI findings with clinical and hematological criteria of children with SCD to identify patients at high risk for developing stroke. This study was conducted 30 children with SCD [16 SS and 14 S-beta T]. Their ages ranged from 2.5 to 19 years [mean age. 9.1 years +/- 4.3]. All were subjected to full history taking thorough clinical examination and laboratory investigations, complete blood picture, hemoglobin electrophoresis, reticulocytic count and liver function tests. The were all subjected to TCD mainly middle cerebral artery [MCA] on both sides and MRI using standard T-2 weighted images and diffusion-weighted imaging technique. Six children had a history of seizures and all were neurologically free except one with left sided hemiprses. The mean velocities [Vmean] of the right and left MCA were significantly higher in SS than S-BT. Four children [13.3%] had abnormal TCD findings with significantly high peak systolic velocities [PSV] of MCA. Three ofthem had abnormal MRI [one had old infract, another one had silent small recent infracts and one showed ischemic foci], the fourth showed no MRI abnormality. Regular TCD screening is useful in detecting the abnormal cerebral vessels and its recommended that cerebrovascular disease with TCD. Screening every 6 months for sikle cell disease pediatric patients may help to select high risk children for [DWI]. This will help determine candidates for regular transfusion therapy in an attempt to prevent the occurrence of stroke