Strokes are considered to be among the main causes of children’s disability all over the world. This study is intending to describe types of strokes affecting Saudi children, in order to identify causes and risk factors of strokes, especially those related to congenital aspects. Results of this study are expected to establish the cornerstone of proper intervention plans to prevent disability due to stroke.

Dr. Abdel Galil M. Abdel Gader, Dr. Ahmed A. Al-Jarallah.

Strokes are considered to be among the main causes of children’s disability all over the world. Among those children who live after having a stroke, 75-100% suffer from some kind of disability such as motor disability, epilepsy, mental retardation and communication disorders.
Etiology and risk factors of strokes vary according to age and demographic class. Stroke studies, conducted on Saudis, were concentrated on adults and, so far, no comprehensive study addressing strokes in Saudi children has been conducted.
This study is intending to describe types of strokes affecting Saudi children, in a strategic way [During the past 5 years and for the next 2 years], in order to identify causes and risk factors of strokes, especially those related to congenital aspects. The study will assess the results of strokes along with identifying the factors that could produce positive results in Saudi children.
Results of this study are expected to establish the cornerstone of proper intervention plans to prevent disability
Recommendations:Because of the high prevalence and importance of multiple risk factors, a comprehensive investigation, including hematologic, neuroimaging and metabolic studies should be considered in every child who has presented with a stroke.  This can be achieved through the development of specialized stroke services, designing and implementing a childhood stroke registry via the Internet and the efficient use of Telemedicine to help in the initial management of patients at the various district and regional hospitals.  Inherited causes of stroke (e.g. SCD) can be prevented primarily through pre-marital genetic screening, while secondary cases are potentially preventable following informed genetic counseling after the identification of an index case. Conclusions:  1. This retrospective and prospective study, which spanned 10 years and 7 months, is one of the largest cohort of children with stroke at one medical centre worldwide. 2. Among the study group of 104 Saudi children, ischemic stroke accounted for the majority of cases (82%), whereas intracranial hemorrhage was less common (18%).  The relative paucity in the proportion of hemorrhagic stroke, compared to other international studies, may partly be accounted for by the fact that children with e.g. hereditary bleeding disorders succumb before they manage to reach the secondary or tertiary care hospitals. 3. The present study strongly highlighted the importance of prothrombotic disorders as a risk factor for stroke in Saudi children.  No previous study is available where the role of prothrombotic conditions in the pathogenesis of childhood stroke was explored, either in Saudi Arabia or the Middle Eastern Region. 4. Prothrombotic disorders were significantly detected more in the Prospective Study Group than in the Retrospective Study Group.  This emphasized the importance of availability of adequate coagulation laboratory service in the investigation of childhood stroke. 5. Large prospective follow-up studies are needed to evaluate all children with stroke, for prothrombotic conditions and investigate their families if hemostatic abnormalities were identified.  Such an approach will ensure that more children will benefit from the appropriate specific treatments designed to prevent stroke and its recurrence. 6. Contrary to previous similar studies from Saudi Arabia, sickle cell disease (SCD) accounted for greater than 10% of the ascertained risk factors and had severe manifestations; highlighting the severe phenotype of SCD which is also prevalent in Saudi Arabia in addition to the mild type. 7. More efforts should, therefore, be focused in elaborating sustained programs aimed at the primary prevention of SCD, through health education and pre-marital diagnosis. 8. Transcranial Dopller (TCD) screening made significant impact in identifying cerebrovascular disease associated with SCD.  This non-invasive and less technically-demanding technique needs to be made available at the health centres and hospitals, where patients with SCD are managed.  The availability of TCD potentially allows for the primary prevention of stroke through early intervention with therapies, such as regular transfusions and hydroxyurea. 9. Acute bacterial meningitis is an important potentially preventable cause of childhood stroke.  Immunogenic vaccines against the three commonest organisms (H. influenzae type b, N. meningitides and defined types of S. pneumoniae) are also needed to cover the young (<2 years) who are mostly affected. 10. The study described the first reported cases of stroke as a manifestation of neurobrucellosis, and suggested that investigation for neurobrucellosis should be part of the diagnostic work-up in Saudi Arabia and other similar countries of the Region. 11. Congenital toxoplasmosis and rubella syndrome were also identified risk factors for childhood stroke.  Both are potentially preventable through immunization and/or other maternity health programs. 12. Congenital and genetic cerebrovascular anomalies are also important risk factors.  Prompt diagnosis and neurosurgical intervention may be life-saving in these conditions. 13. Underlying cardiac anomalies should be excluded in cases of childhood stroke, since these conditions can be asymptomatic, as has been the case in one patient in this cohort. 14. Moyamoya syndrome is a significant underlying cause of stroke in Saudi children.  It was found to be associated with another disease in all identified cases.  The association of moyamoya syndrome and protein C deficiency was first reported in this cohort of patients, whereas the associations of the syndrome with wrinkly skin syndrome (OM1M 278250) and Adams-Oliver syndrome (OM1M 100300) have not, hitherto, been described. Revascularization surgery (encephaloduroarteriosynangiosis) is a safe intervention method even in patients with hypercoagulable disorders (protein C and S deficiencies) and in SCD.15. Inherited metabolic disorders (all due to underlying mitochondrial disorders in this cohort) caused stroke in 3.8%.  Specialized reference laboratories are required for the proper investigation of these cases, as a pre-requisite for genetic counseling aiming at the prevention of secondary cases. 16. Hypernatremic dehydration contributes to stroke in Saudi population.  The condition can be effectively controlled by health education to encourage breast feeding and the universal use of oral rehydration solution at the primary health care level. 17. Post-traumatic arterial dissection requires a high index of suspicion and adequate neuroimaging facilities for diagnosing the condition.  When identified, recurrences can be curbed by appropriate therapeutic interventions. 18. The outcome of stroke in Saudi children constituted a huge burden with high rate of motor, cognitive and language deficits.  Residual epilepsy affected 58%.  The socio-economic consequences for the victim, family and the community at large are quite challenging.  Programs aiming at the primary prevention of the causes of stroke remain the corner-stone in reducing the burden of childhood stroke on the patients, their families, the health service, and the society at large. 19. Because of the high prevalence and importance of multiple risk factors, complete investigation, including hematologic, neuroimaging and metabolic studies should be considered in every child with stroke.20. Based on the experience gained from this study and the international guidelines, a diagnostic algorithm outlining the approach to a child with a suspected stroke / cerebrovascular lesion, was formulated.  The algorithm might also be of use for managing other children from the Arabian Peninsula and Middle Eastern Region, with similar demographic, socio-economic and ethnic background. 21. Nationwide, handling childhood stroke can be achieved effectively by the development of specialized stroke care services, designing and implementing a childhood stroke registry via the Internet, and the efficient use of Telemedicine to help in the initial management of patients at various district and regional hospitals.

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King Saud University

The results of this project have been submitted to the Saudi Medical Journal to be printed as a stand alone Supplement to the Journal in 2006.

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