ABSTRACT
Background: Sickle cell disease (SCD) is autosomal recessive hemolytic anemia due to hemoglobinopathy commonly in Sub-Saharan Africa, and particularly in Sudan. The disease induces a pro-inflammatory cascade in the intimal layer that leads to hyperplasia and progressive stenosis in the major vessel of the circle of Willis. This is associated with the development of Moyamoya collaterals. The aim of this study is to highlight the frequency of Moyamoya syndrome in Sudanese pediatric patients with sickle cell disease presenting with stroke. Materials and methods: A descriptive cross-sectional hospital-based study was conducted in the Department of Hematology at Gaafar Ibnauf Pediatric Tertiary Hospital in Khartoum state in Sudan, in the period between March 2021 and August 2021. Secondary data has been collected from the medical record after ethical approval and informed consent. Results: A total of 104 patients were included in the study. The males were the majority, about 60 (57.7) compared to 44 (42.3%) females. More than half of our study population was in the school-age 54 compared to 42 adolescents and only 8 patients of preschool age. Only 50 out of 104 patients had diagnostic MRA which revealed features of Moyamoya syndrome in 48 (96%) patients. Motor weakness (100%), aphasia (52.9%), and facial palsy (35%) were the major stroke presentations. The left anterior circulation territory was the most common site of moyamoya features involvement in 31 patients out of 48. Conclusion: Stroke is a common problem in Sudanese pediatric patients with sickle cell disease, with a very high frequency of Moyamoya vasculopathy among patients with stroke presentation. The burden of Moyamoya syndrome in Sudanese pediatric patients with sickle cell disease is underestimated due to the cost of the available screening and diagnostic tools.
ABSTRACT
INTRODUCTION: and importance:Bilateral extradural hematomas account for less than 5% of all entities of extradural hematomas. The condition is generally caused by traumatic brain injury, which can form separate hematomas contralaterally or joint bilateral extradural hematomas commonly owing to superior sagittal sinus injury. In light of the above, this is the first case of such a condition to be reported from Sudan in the literature. CASE PRESENTATION: A 31-years-old male presented with headache, confusion, and 4 episodes of non-projectile vomiting with a GCS score of 14, after being assaulted by direct blunt head trauma. CT brain showed acute extradural hematoma on the right frontotemporal part compared to a parietal extradural hematoma on the contralateral side. The patient underwent bilateral craniotomy with a wide question mark-like skin flap on the right temporoparietal side followed by 5 burr holes. On the left side, parietal craniotomy was made with an inverted U-shaped skin flap and 4 burr holes on the left parietal side, after that two surgical drains were inserted bilaterally. The patient was discharged on the third postoperative day with a GCS of 15. DISCUSSION: Although bilateral extradural hematomas are rare and grave conditions, prompt surgical intervention has shown low morbidity and mortality rate with markedly favorable postoperative outcomes. CONCLUSION: Bilateral extradural hematomas can be attributed to extended linear fracture caused by superior sagittal sinus injury. CT scan is the gold standard imaging. However, MRI and MRV can be used to demonstrate injury or occlusion of the Superior sagittal sinus.
