Intracranial aneurysms in sickle-cell anemia: clinical features and pathogenesis

Abstract

Intracranial aneurysms are an unusual complication of sickle-cell anemia; only 15 patients have been described in the world literature. An additional 15 patients with sickle-cell anemia and subarachnoid hemorrhage (SAH) from ruptured intracranial aneurysms are presented. There was a high incidence of multiple aneurysms (60%); some of which were in unusual locations. The clinical and pathological features of this series of patients have provided a paradigm for acquired aneurysm formation that may be applicable to other intracranial aneurysms. Thirteen patients underwent craniotomy and clip ligation; the perioperative management of these patients is discussed. Of these 13, eight had a good recovery, three were left with moderate disability, one patient died of surgical complications, and one died of complications related to sickle-cell anemia. Two of the 15 patients died of SAH. The authors propose that endothelial injury from the abnormal adherence of sickle erythrocytes to the endothelium is the initiating event in arterial wall injury. Subsequently, there is fragmentation of the internal elastic lamina and degeneration of the smooth-muscle layer. Hemodynamic stress at these loci of arterial wall damage results in aneurysm formation. This hypothesis also explains other cerebrovascular manifestations of sickle-cell anemia, namely vaso-occlusive disease and hemorrhage without aneurysm formation. Pathological material from this series and data from the literature are presented to support this hypothesis.

How I treat and manage strokes in sickle cell disease

“…Among those with an etiology, subarachnoid hemorrhage is the most common and is often associated with intracranial aneurysm. 10,11 The presence of hemorrhagic stroke does not exclude the possibility of ischemic stroke, 12 and if a concomitant ischemic stroke is identified, there should be consideration for regular blood transfusion therapy. Other than the presence of aneurysms, risk factors for hemorrhagic stroke include low Hb, high steady-state leukocyte, 3 recent blood transfusion (in the last 14 days), treatment with corticosteroids, or ACS.…”

Elastic tissue abnormalities resembling pseudoxanthoma elasticum in β thalassemia and the sickling syndromes

“…81 Concurrently, in SCD, the reported cerebrovascular manifestations include not only vaso-occlusive events but hemorrhages as well; subarachnoid hemorrhage due to ruptured intracranial aneurysms is a recognized cause of morbidity and mortality in adults with SCD. 82,83 Interestingly, although investigators did not correlate these events to a generalized elastic tissue defect, pathology showed fragmentation of the internal elastic lamina of the arterial wall of the aneurysms, although aneurysms were multiple and characterized by atypical localization. 82,83 …”

“…82,83 Interestingly, although investigators did not correlate these events to a generalized elastic tissue defect, pathology showed fragmentation of the internal elastic lamina of the arterial wall of the aneurysms, although aneurysms were multiple and characterized by atypical localization. 82,83 …”

Comparative Study of Autopsy Findings in Sickle Cell Disease (SCD) At South Eastern Tertiary Hospital, Nigeria

“…Majority of death in SCD arises from intracranial (berry) anuersyms and subarachnoid haemorrhage 4 .The anuresyms here are unusual as a result of multiple fast and arises from unusual sites 2 . A 60% incidence of multiple aneurysms was seen in one series, with a patient having as many as five aneurysms and being found at ophthalamic and anterior choroidal arteries which are obviously unusual sites 6 . Meningitis specifically affects younger patients and is often bacterial in nature (streptococcus pneumonia) 3,7 .…”