Case of simultaneous Chiari 1.5 malformation and syringobulbia
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CASE REPORT
Case of simultaneous Chiari 1.5 malformation and syringobulbia Katie Carsky 1 & Joe Iwanaga 1,2
&
Aaron S. Dumont 1 & R. Shane Tubbs 1,2,3,4,5
Received: 19 July 2020 / Accepted: 5 October 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
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. Keywords Chiari 1.5 . Syringobulbia . Cerebellar tonsils . Brain stem . Chiari malformation
Introduction In normal anatomy, the only major central nervous system structure to pass through the foramen magnum is the spinal cord. Chiari I malformation (CIM) is characterized by displacement of the cerebellar tonsils below the level of the foramen magnum. Chiari 1.5 malformation (C1.5) is CIM with the addition of caudal descent of the brain stem and is sometimes considered a more severe variant of CIM. Although C1.5 presents with brain stem herniation and a more severe degree of tonsillar herniation, it is comparable in clinical significance and surgical outcome to C1M [1].
Syringobulbia (SB) is a CSF-filled cavity of the brain stem. It is related to syringomyelia (SM), which is a CSF-filled cavity of the spinal cord. SB is much rarer than SM, though the two conditions are closely linked. The cavity in the brain stem that occurs in SB can lead to symptoms such as cranial nerve deficits including facial palsies, vertigo, and nystagmus, among others [2, 3]. We encountered a case of simultaneous C1.5 and SB. While there are reports of coexisting C1.5 and SM and rare cases of CIM and SB, this case appears to be the first reported instance of these two rare conditions, C1.5 and SB, occurring concurrently.
Case report * Joe Iwanaga [email protected] 1
Department of Neurosurgery, Tulane Center for Clinical Neurosciences, Tulane University School of Medicine, 131 S. Robertson St. Suite 1300, New Orleans, LA 70112, USA
2
Department of Neurology, Tulane University School of Medicine, New Orleans, LA, USA
3
Department of Anatomical Sciences, St. George’s University, St. George’s, Grenada
4
Department of Structural & Cellular Biology, Tulane University School of Medicine, New Orleans, LA, USA
5
Department of Neurosurgery and Ochsner Neuroscience Institute, Ochsner Health System, New Orleans, LA, USA
A 14-year-old female presented with Valsalva-induced headach
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