Unruptured intracranial aneurysms in children with sickle cell disease: analysis of 18 aneurysms in 5 patients

Neurosurgery. 2015 May;76(5):531-8; discission 538-9; quiz 539. doi: 10.1227/NEU.0000000000000670.

Abstract

Background: Intracranial aneurysms (IAs) are rare in the general pediatric population and account for <2% of all cerebral aneurysms. Only 7 children with sickle hemoglobinopathy and IAs have been reported, the majority of which were discovered after rupture.

Objective: To report the prevalence of unruptured IAs in a selected population of children with sickle cell disease (SCD) and to describe the aneurysm morphology, hematologic characteristics, and management in this patient population.

Methods: A retrospective review of the electronic database for all children with SCD who underwent brain magnetic resonance imaging or angiography from January 2002 to August 2013 at a single institution was performed. Records were reviewed for IA, age, sex, sickle cell genotype, neurological symptoms, hematologic indexes, transcranial Doppler findings, and management.

Results: Five of 179 children (2.8%) with SCD imaged by brain magnetic resonance imaging or angiography were diagnosed with IAs. None presented with subarachnoid hemorrhage. Four patients (80%) had HbSS disease, and 1 patient had hemoglobin sickle cell HbSC disease. A total of 18 aneurysms were detected; the majority of patients had multiple aneurysms (80%) and bilateral involvement (60%).

Conclusion: Children with SCD are at risk for developing multiple intracranial aneurysms, and a high index of suspicion must be maintained during the interpretation of routine magnetic resonance imaging or angiography of the brain.

MeSH terms

  • Adolescent
  • Anemia, Sickle Cell / complications*
  • Cerebral Angiography
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Infant
  • Intracranial Aneurysm / complications
  • Intracranial Aneurysm / epidemiology*
  • Intracranial Aneurysm / pathology*
  • Magnetic Resonance Imaging
  • Male
  • Prevalence
  • Retrospective Studies