Abstract
Chiari 1.5 malformation (C1.5) is a rare condition characterized by caudal descent of the brain stem, together with descent of the cerebellar tonsils characteristic of Chiari I malformation (CIM). Syringobulbia (SB) is rarely associated with CIM and to date has not been reported in a patient with C1.5. An adolescent female presented with Valsalva-induced headaches and a left abducens nerve palsy. Imaging revealed C1.5 and a huge syringomyelia extending cranially into the herniated medulla oblongata as syringobulbia. Simultaneous cases of C1.5 and syringomyelia (SM) have been described, but, to our knowledge, this is the first reported case of simultaneous C1.5 and SB. As SB can have life altering consequences, the clinician should keep this possibility in their differential diagnosis.
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Liu W, Wu H, Aikebaier Y, Wulabieke M, Paerhati R, Yang X (2017) No significant difference between Chiari malformation type 1.5 and type I. Clin Neurol Neurosurg 157:34–39
Valentini MC, Bracchi M, Gaidolfi E, Savoiardo M (1986) Radiologic demonstration of syringobulbia: Report of 8 cases. Acta Radiologica 369:245–247
Sahoo S, Pearl PL (2003) Syringobulbia. In: NORD guide to rare disorders. Lippincott Williams & Wilkins. Philadelphia, pp 590-591
Tubbs RS, Iskandar BJ, Bartolucci AA, Oakes WJ (2004) A critical analysis of the Chiari 1.5 malformation. J Neurosurg (Pediatrics 2) 101:179–183
Shen J, Shen J, Huang K, Wu Y, Pan J, Zhan R (2019) Syringobulbia in patients with Chiari malformation type I: a systematic review. BioMed Res Int 4:1–8
Tubbs RS, Bailey M, Barrow WC, Loukas M, Shoja MM, Oakes WJ (2009) Morphometric analysis of the craniocervical juncture in children with Chiari I malformation and concomitant syringobulbia. Childs Nerv Syst 25:689–692
Menezes AH, Greenlee JDW, Dlouhy BJ (2018) Syringobulbia in pediatric patients with Chiari malformation type I. J Neurosurg Pediatr 22:52–60
Azahraa Haddad F, Qaisi I, Joudeh N, Dajani H, Jumah F, Elmashala A, Adeeb N, Chern JJ, Tubbs RS (2018) The newer classifications of the Chiari malformations with clarifications: an anatomical review. Clin Anat 31:314–322
Shoja MM, Johal J, Oakes WJ, Tubbs RS (2018) Embryology and pathophysiology of the Chiari I and II malformations: a comprehensive review. Clin Anat 31:202–215
Shoja MM, Ramdhan R, Jensen CJ, Chern JJ, Oakes WJ, Tubbs RS (2018) Embryology of the craniocervical junction and posterior cranial fossa, part II: Embryogenesis of the hindbrain. Clin Anat 31:488–500
Shoja MM, Ramdhan R, Jensen CJ, Chern JJ, Oakes WJ, Tubbs RS (2018) Embryology of the craniocervical junction and posterior cranial fossa, part I: Development of the upper vertebrae and skull. Clin Anat 31:466–487
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Carsky, K., Iwanaga, J., Dumont, A.S. et al. Case of simultaneous Chiari 1.5 malformation and syringobulbia. Childs Nerv Syst 37, 2409–2411 (2021). https://doi.org/10.1007/s00381-020-04924-9
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DOI: https://doi.org/10.1007/s00381-020-04924-9