Scoliosis is frequently a presenting sign of Chiari malformation type I (CM-I) with syrinx. The authors’ goal was to define scoliosis in this population and describe how radiological characteristics of CM-I and syrinx relate to the presence and severity of scoliosis.
A large multicenter retrospective and prospective registry of pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and syrinx (≥ 3 mm in axial width) was reviewed for clinical and radiological characteristics of CM-I, syrinx, and scoliosis (coronal curve ≥ 10°).
Based on available imaging of patients with CM-I and syrinx, 260 of 825 patients (31%) had a clear diagnosis of scoliosis based on radiographs or coronal MRI. Forty-nine patients (5.9%) did not have scoliosis, and in 516 (63%) patients, a clear determination of the presence or absence of scoliosis could not be made. Comparison of patients with and those without a definite scoliosis diagnosis indicated that scoliosis was associated with wider syrinxes (8.7 vs 6.3 mm, OR 1.25, p < 0.001), longer syrinxes (10.3 vs 6.2 levels, OR 1.18, p < 0.001), syrinxes with their rostral extent located in the cervical spine (94% vs 80%, OR 3.91, p = 0.001), and holocord syrinxes (50% vs 16%, OR 5.61, p < 0.001). Multivariable regression analysis revealed syrinx length and the presence of holocord syrinx to be independent predictors of scoliosis in this patient cohort. Scoliosis was not associated with sex, age at CM-I diagnosis, tonsil position, pB–C2 distance (measured perpendicular distance from the ventral dura to a line drawn from the basion to the posterior-inferior aspect of C2), clivoaxial angle, or frontal-occipital horn ratio. Average curve magnitude was 29.9°, and 37.7% of patients had a left thoracic curve. Older age at CM-I or syrinx diagnosis (p < 0.0001) was associated with greater curve magnitude whereas there was no association between syrinx dimensions and curve magnitude.
Syrinx characteristics, but not tonsil position, were related to the presence of scoliosis in patients with CM-I, and there was an independent association of syrinx length and holocord syrinx with scoliosis. Further study is needed to evaluate the nature of the relationship between syrinx and scoliosis in patients with CM-I.
ABBREVIATIONSAIS = adolescent idiopathic scoliosis; CM-1 = Chiari malformation type I; CXA = clivoaxial angle; pB–C2 = measured perpendicular distance from the ventral dura to a line drawn from the basion to the posterior-inferior aspect of C2; PRSRC = Park-Reeves Syringomyelia Research Consortium.
Correspondence Jennifer M. Strahle: Washington University School of Medicine, St. Louis Children’s Hospital, St. Louis, MO. firstname.lastname@example.org.
INCLUDE WHEN CITING Published online August 16, 2019; DOI: 10.3171/2019.5.PEDS18527.
Disclosures Dr. Alden reports being a consultant for Biomarin Pharmaceutical and Shire Pharmaceutical. Dr. Limbrick reports receiving support of non–study-related clinical or research effort overseen by author from Microbot Medical, Inc.
AttenelloFJMcGirtMJAtibaAGathinjiMDatooGWeingartJ: Suboccipital decompression for Chiari malformation-associated scoliosis: risk factors and time course of deformity progression. J Neurosurg Pediatr1:456–4602008
EuleJMEricksonMAO’BrienMFHandlerM: Chiari I malformation associated with syringomyelia and scoliosis: a twenty-year review of surgical and nonsurgical treatment in a pediatric population. Spine (Phila Pa 1976)27:1451–14552002
EuleJM, EricksonMA, O’BrienMF, HandlerM: Chiari I malformation associated with syringomyelia and scoliosis: a twenty-year review of surgical and nonsurgical treatment in a pediatric population. 27:1451–1455, 200210.1097/00007632-200207010-00015)| false
GodzikJ, DardasA, KellyMP, HolekampTF, LenkeLG, SmythMD, : Comparison of spinal deformity in children with Chiari I malformation with and without syringomyelia: matched cohort study. 25:619–626, 20162598120610.1007/s00586-015-4011-1)| false
GodzikJKellyMPRadmaneshAKimDHolekampTFSmythMD: Relationship of syrinx size and tonsillar descent to spinal deformity in Chiari malformation Type I with associated syringomyelia. J Neurosurg Pediatr13:368–3742014
GodzikJ, KellyMP, RadmaneshA, KimD, HolekampTF, SmythMD, : Relationship of syrinx size and tonsillar descent to spinal deformity in Chiari malformation Type I with associated syringomyelia. 13:368–374, 201410.3171/2014.1.PEDS1310524527859)| false
HwangSW, SamdaniAF, JeaA, RavalA, GaughanJP, BetzRR, : Outcomes of Chiari I-associated scoliosis after intervention: a meta-analysis of the pediatric literature. 28:1213–1219, 201210.1007/s00381-012-1739-322526438)| false
OldfieldEHMuraszkoKShawkerTHPatronasNJ: Pathophysiology of syringomyelia associated with Chiari I malformation of the cerebellar tonsils. Implications for diagnosis and treatment. J Neurosurg80:3–151994
OldfieldEH, MuraszkoK, ShawkerTH, PatronasNJ: Pathophysiology of syringomyelia associated with Chiari I malformation of the cerebellar tonsils. Implications for diagnosis and treatment. 80:3–15, 1994827101810.3171/jns.1994.80.1.0003)| false
RavindraVMOnwuzulikeKHellerRSQuigleyRSmithJDaileyAT: Chiari-related scoliosis: a single-center experience with long-term radiographic follow-up and relationship to deformity correction. J Neurosurg Pediatr21:185–1892018
StrahleJ, MuraszkoKM, KapurchJ, BapurajJR, GartonHJ, MaherCO: Chiari malformation Type I and syrinx in children undergoing magnetic resonance imaging. 8:205–213, 201110.3171/2011.5.PEDS112121806364)| false
StrahleJ, SmithBW, MartinezM, BapurajJR, MuraszkoKM, GartonHJ, : The association between Chiari malformation Type I, spinal syrinx, and scoliosis. 15:607–611, 201510.3171/2014.11.PEDS1413526030330)| false
TubbsRS, DoyleS, ConklinM, OakesWJ: Scoliosis in a child with Chiari I malformation and the absence of syringomyelia: case report and a review of the literature. 22:1351–1354, 200610.1007/s00381-006-0079-6)| false
ZhuZ, ShaS, ChuWC, YanH, XieD, LiuZ, : Comparison of the scoliosis curve patterns and MRI syrinx cord characteristics of idiopathic syringomyelia versus Chiari I malformation. 25:517–525, 201610.1007/s00586-015-4108-626162920)| false