Skip to main content
Top
Published in: BMC Neurology 1/2022

Open Access 01-12-2022 | Stroke | Case report

Stroke in sickle cell disease in association with bilateral absence of the internal carotid arteries. Case report

Authors: Ivana Markovic, Zoran Milenkovic, Bosanka Jocic-Jakubi, Amna Al Futaisi, Kakaria Anupam Kakaria, Yasser Walli

Published in: BMC Neurology | Issue 1/2022

Login to get access

Abstract

Background

Congenital absence of the internal carotid artery (ICA) is a highly infrequent congenital incidence and occurs in less than 0.01% of the population; bilateral absence is exceedingly rare, diagnosed below 10% of the unilateral absence of the ICA. Sickle cell disease (SCD) is a serious disorder and carries a high risk of stroke.

Case presentation

We present a five-year-old child with SCD who experienced an ischemic stroke episode with epileptic seizures. Neuroimaging revealed the agenesis of both ICAs. The frequency, embryology, and collateral pathway of the vascular anomaly as the clinical presentation, of this rare hematologic disease, are discussed.

Conclusions

Sickle cell disease (SCD) carries a high risk of stroke. Congenital absence of ICA occurs in less than 0.01% of the population; bilateral absence is diagnosed below 10% of the unilateral absence of the ICA.
Literature
1.
go back to reference Platt OS. Preventing stroke in sickle cell anemia. N Engl J Med. 2005;353(26):2743–5.CrossRef Platt OS. Preventing stroke in sickle cell anemia. N Engl J Med. 2005;353(26):2743–5.CrossRef
2.
go back to reference Stephen HE. Sickle cell Anemia and associated Hemoglobinopathies. In: Goldman L, Bennett JC, editors. Cecil textbook of medicine. 21st ed. Philadelphia: Saunders; 2000. p. 893–905. Stephen HE. Sickle cell Anemia and associated Hemoglobinopathies. In: Goldman L, Bennett JC, editors. Cecil textbook of medicine. 21st ed. Philadelphia: Saunders; 2000. p. 893–905.
3.
go back to reference Balmaceda CM, Fetell MR. Hematologic and related diseases. In: Rowland LP, editor. Merritt’s textbook of neurology. 9th ed. Baltimore: Williams & Wilkins; 1995. p. 903–15. Balmaceda CM, Fetell MR. Hematologic and related diseases. In: Rowland LP, editor. Merritt’s textbook of neurology. 9th ed. Baltimore: Williams & Wilkins; 1995. p. 903–15.
5.
go back to reference Luk YS, Man EM, Sy AN. Bilateral congenital hypoplasia of the internal carotid arteries. Singapore Med J. 2010;51:163–5. Luk YS, Man EM, Sy AN. Bilateral congenital hypoplasia of the internal carotid arteries. Singapore Med J. 2010;51:163–5.
6.
go back to reference Nicoletti G, Sanguigni S, Bruno F, et al. Hypoplasia of the internal carotid artery: collateral circulation and ultrasonographic findings. A case report. Ultrasound. 2009;12:1–44.CrossRef Nicoletti G, Sanguigni S, Bruno F, et al. Hypoplasia of the internal carotid artery: collateral circulation and ultrasonographic findings. A case report. Ultrasound. 2009;12:1–44.CrossRef
7.
go back to reference Harrigan MR, Deveikis JP. Essential Neurovascular Anatomy. In: Harrigan MR, Deveikis JP, editors. Handbook of cerebrovascular disease and Neurointerventional technique series: contemporary medical imaging. New York: Humana Press; 2009. p. 1–87.CrossRef Harrigan MR, Deveikis JP. Essential Neurovascular Anatomy. In: Harrigan MR, Deveikis JP, editors. Handbook of cerebrovascular disease and Neurointerventional technique series: contemporary medical imaging. New York: Humana Press; 2009. p. 1–87.CrossRef
10.
go back to reference Beresini DC, Hieshima GB, Mehringer CM, Grinell VS. Bilateral absence of the internal carotid artery with sellar enlargement due to anomalous vascularity. Surg Neurol. 1981;16:9–16.CrossRef Beresini DC, Hieshima GB, Mehringer CM, Grinell VS. Bilateral absence of the internal carotid artery with sellar enlargement due to anomalous vascularity. Surg Neurol. 1981;16:9–16.CrossRef
11.
go back to reference Yokochi K, Iwase K. Bilateral internal carotid artery agenesis in a child with psychomotor developmental delay. Pediatr Neurol. 1996;15:76–8.CrossRef Yokochi K, Iwase K. Bilateral internal carotid artery agenesis in a child with psychomotor developmental delay. Pediatr Neurol. 1996;15:76–8.CrossRef
12.
go back to reference Quint DJ, Boulos RS, Spera TD. Congenital absence of the cervical and petrous internal carotid artery with intercavernous anastomosis. Am J Neuroradiol. 1989;10:435–9.PubMedPubMedCentral Quint DJ, Boulos RS, Spera TD. Congenital absence of the cervical and petrous internal carotid artery with intercavernous anastomosis. Am J Neuroradiol. 1989;10:435–9.PubMedPubMedCentral
13.
go back to reference Tasar M, Yetiser S, Tasar A, Uðurel S, Gönül E, Saðlam M. Congenital absence or hypoplasia of the carotid artery: Radioclinical issues. Am J Otolaryngol. 2004;25:339–49.CrossRef Tasar M, Yetiser S, Tasar A, Uðurel S, Gönül E, Saðlam M. Congenital absence or hypoplasia of the carotid artery: Radioclinical issues. Am J Otolaryngol. 2004;25:339–49.CrossRef
14.
go back to reference Lie TA. Congenital anomalies of the carotid arteries. Amsterdam: ExcerptaMedicaFondation; 1968. Lie TA. Congenital anomalies of the carotid arteries. Amsterdam: ExcerptaMedicaFondation; 1968.
16.
go back to reference Orakdöğen M, Berkman Z, Erşahin M, Biber N, Somay H. Agenesis of the left internal carotid artery associated with anterior communicating artery aneurysm: case report. Turk Neurosurg. 2007;17(4):273–6 PMid:18050072.PubMed Orakdöğen M, Berkman Z, Erşahin M, Biber N, Somay H. Agenesis of the left internal carotid artery associated with anterior communicating artery aneurysm: case report. Turk Neurosurg. 2007;17(4):273–6 PMid:18050072.PubMed
17.
go back to reference Dinç H, Alioglu Z, Erdöl H, Ahmetoglu A. Agenesis of the internal carotid artery associated with an aortic arch anomaly in a patient with congenital Horner.S syndrome. AJNR Am J Neuroradiol. 2002;23:929–31.PubMedPubMedCentral Dinç H, Alioglu Z, Erdöl H, Ahmetoglu A. Agenesis of the internal carotid artery associated with an aortic arch anomaly in a patient with congenital Horner.S syndrome. AJNR Am J Neuroradiol. 2002;23:929–31.PubMedPubMedCentral
19.
go back to reference Verduzco LA, Nathan DG. Sickle cell disease and stroke. Review article 2009. Blood. 2009;114:5117–25.CrossRef Verduzco LA, Nathan DG. Sickle cell disease and stroke. Review article 2009. Blood. 2009;114:5117–25.CrossRef
22.
go back to reference Ohene-Frempong K, Weiner SJ, Sleeper LA, Miller ST, Embury S, Moohr JW, et al. Cerebrovascular accidents in sickle cell disease: rates and risk factors. Blood. 1998;91:288–94.PubMed Ohene-Frempong K, Weiner SJ, Sleeper LA, Miller ST, Embury S, Moohr JW, et al. Cerebrovascular accidents in sickle cell disease: rates and risk factors. Blood. 1998;91:288–94.PubMed
23.
go back to reference Strouse JJ, Lanzkron S, Urrutia V. The epidemiology, evaluation, and treatment of stroke in adults with sickle cell disease. Expert Rev Hematol. 2011;4:597–606.CrossRef Strouse JJ, Lanzkron S, Urrutia V. The epidemiology, evaluation, and treatment of stroke in adults with sickle cell disease. Expert Rev Hematol. 2011;4:597–606.CrossRef
24.
go back to reference Broderick J, Talbot GT, Prenger E, Leach A, Brott T. Stroke in children within a major metropolitan area: the surprising importance of intracerebral hemorrhage. J Child Neurol. 1993;8(3):250–5.CrossRef Broderick J, Talbot GT, Prenger E, Leach A, Brott T. Stroke in children within a major metropolitan area: the surprising importance of intracerebral hemorrhage. J Child Neurol. 1993;8(3):250–5.CrossRef
25.
go back to reference Quinn CT, Rogers ZR, Buchanan GR. Survival of children with sickle cell disease. Blood. 2004;103:4023–7.CrossRef Quinn CT, Rogers ZR, Buchanan GR. Survival of children with sickle cell disease. Blood. 2004;103:4023–7.CrossRef
29.
go back to reference Switzer JA, Hess DC, Nichols FT, Adams RJ. Pathophysiology and treatment of stroke in sickle-cell disease: present and future. Lancet Neurol. 2006;5:501–12.CrossRef Switzer JA, Hess DC, Nichols FT, Adams RJ. Pathophysiology and treatment of stroke in sickle-cell disease: present and future. Lancet Neurol. 2006;5:501–12.CrossRef
31.
go back to reference Calvet D, Bernardin F, Gueguen A, et al. First ischemic stroke in sickle cell disease: are there any adult specificities? Stroke. 2015;46(8):2315–7.CrossRef Calvet D, Bernardin F, Gueguen A, et al. First ischemic stroke in sickle cell disease: are there any adult specificities? Stroke. 2015;46(8):2315–7.CrossRef
32.
go back to reference Hariman LM, Griffith ER, Hurtig AL, Keehn MT. Functional outcomes of children with sickle-cell disease affected by stroke. Arch Phys Med Rehabil. 1991;72(7):498–502.PubMed Hariman LM, Griffith ER, Hurtig AL, Keehn MT. Functional outcomes of children with sickle-cell disease affected by stroke. Arch Phys Med Rehabil. 1991;72(7):498–502.PubMed
35.
go back to reference King AA, Rodeghier MJ, Panepinto JA, Strouse JJ, Casella JF, Quinn CT, et al. Silent cerebral infarction, income, and grade retention among students with sickle cell anemia. Am J Hematol. 2014;89:E188–92.CrossRef King AA, Rodeghier MJ, Panepinto JA, Strouse JJ, Casella JF, Quinn CT, et al. Silent cerebral infarction, income, and grade retention among students with sickle cell anemia. Am J Hematol. 2014;89:E188–92.CrossRef
37.
go back to reference Fox CK, Glass HC, Sidney S, Lowenstein DH, Fullerton HJ. Acute seizures predict epilepsy after childhood stroke. Ann Neurol. 2013;74(2):249–56.PubMedPubMedCentral Fox CK, Glass HC, Sidney S, Lowenstein DH, Fullerton HJ. Acute seizures predict epilepsy after childhood stroke. Ann Neurol. 2013;74(2):249–56.PubMedPubMedCentral
41.
go back to reference Beverung LM, Strouse JJ, Hulbert ML, Neville K, Liem RI, Inusa B, et al. Health-related quality of life in children with sickle cell anemia: impact of blood transfusion therapy. Am J Hematol. 2015;90:139–43.CrossRef Beverung LM, Strouse JJ, Hulbert ML, Neville K, Liem RI, Inusa B, et al. Health-related quality of life in children with sickle cell anemia: impact of blood transfusion therapy. Am J Hematol. 2015;90:139–43.CrossRef
42.
go back to reference Ferriero DM, Fullerton HJ, Bernard TJ, Billinghurst L, et al. Management of stroke in neonates and children: a scientific statement from the American Heart Association/American Stroke Association. Stroke. 2019;50:e51–96.CrossRef Ferriero DM, Fullerton HJ, Bernard TJ, Billinghurst L, et al. Management of stroke in neonates and children: a scientific statement from the American Heart Association/American Stroke Association. Stroke. 2019;50:e51–96.CrossRef
45.
go back to reference Husson B, Lasjaunias P. Radiological approach to disorders of arterial brain vessels associated with childhood arterial stroke: a comparison between MRA and contrast angiography. Pediatr Radiol. 2004;34:10–5.CrossRef Husson B, Lasjaunias P. Radiological approach to disorders of arterial brain vessels associated with childhood arterial stroke: a comparison between MRA and contrast angiography. Pediatr Radiol. 2004;34:10–5.CrossRef
46.
go back to reference Roach ES, Golomb MR, Adams R, Biller J, et al. Management of Stroke in infants and children a scientific statement from a special writing Group of the American Heart Association Stroke Council and the council on cardiovascular disease in the young. Stroke. 2008;39:2644–91.CrossRef Roach ES, Golomb MR, Adams R, Biller J, et al. Management of Stroke in infants and children a scientific statement from a special writing Group of the American Heart Association Stroke Council and the council on cardiovascular disease in the young. Stroke. 2008;39:2644–91.CrossRef
Metadata
Title
Stroke in sickle cell disease in association with bilateral absence of the internal carotid arteries. Case report
Authors
Ivana Markovic
Zoran Milenkovic
Bosanka Jocic-Jakubi
Amna Al Futaisi
Kakaria Anupam Kakaria
Yasser Walli
Publication date
01-12-2022
Publisher
BioMed Central
Published in
BMC Neurology / Issue 1/2022
Electronic ISSN: 1471-2377
DOI
https://doi.org/10.1186/s12883-022-02702-5

Other articles of this Issue 1/2022

BMC Neurology 1/2022 Go to the issue