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

Surbhi Saini, Barbara Speller-Brown, Emily Wyse, Emily R. Meier, Jessica Carpenter, Ross M. Fasano, Monica S. Pearl

Research output: Contribution to journalArticle

8 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)531-539
Number of pages9
JournalNeurosurgery
Volume76
Issue number5
DOIs
StatePublished - May 21 2015

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Sickle Cell Anemia
Intracranial Aneurysm
Aneurysm
Magnetic Resonance Angiography
Magnetic Resonance Imaging
Brain
Population
Hemoglobinopathies
Subarachnoid Hemorrhage
Rupture
Hemoglobins
Genotype
Databases
Pediatrics

All Science Journal Classification (ASJC) codes

  • Surgery
  • Clinical Neurology

Cite this

Saini, S., Speller-Brown, B., Wyse, E., Meier, E. R., Carpenter, J., Fasano, R. M., & Pearl, M. S. (2015). Unruptured intracranial aneurysms in children with sickle cell disease: Analysis of 18 aneurysms in 5 patients. Neurosurgery, 76(5), 531-539. https://doi.org/10.1227/NEU.0000000000000670
Saini, Surbhi ; Speller-Brown, Barbara ; Wyse, Emily ; Meier, Emily R. ; Carpenter, Jessica ; Fasano, Ross M. ; Pearl, Monica S. / Unruptured intracranial aneurysms in children with sickle cell disease : Analysis of 18 aneurysms in 5 patients. In: Neurosurgery. 2015 ; Vol. 76, No. 5. pp. 531-539.
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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.",
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Saini, S, Speller-Brown, B, Wyse, E, Meier, ER, Carpenter, J, Fasano, RM & Pearl, MS 2015, 'Unruptured intracranial aneurysms in children with sickle cell disease: Analysis of 18 aneurysms in 5 patients', Neurosurgery, vol. 76, no. 5, pp. 531-539. https://doi.org/10.1227/NEU.0000000000000670

Unruptured intracranial aneurysms in children with sickle cell disease : Analysis of 18 aneurysms in 5 patients. / Saini, Surbhi; Speller-Brown, Barbara; Wyse, Emily; Meier, Emily R.; Carpenter, Jessica; Fasano, Ross M.; Pearl, Monica S.

In: Neurosurgery, Vol. 76, No. 5, 21.05.2015, p. 531-539.

Research output: Contribution to journalArticle

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T1 - Unruptured intracranial aneurysms in children with sickle cell disease

T2 - Analysis of 18 aneurysms in 5 patients

AU - Saini, Surbhi

AU - Speller-Brown, Barbara

AU - Wyse, Emily

AU - Meier, Emily R.

AU - Carpenter, Jessica

AU - Fasano, Ross M.

AU - Pearl, Monica S.

PY - 2015/5/21

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N2 - 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.

AB - 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.

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