Clinical Characteristics, Imaging Findings and Surgical Outcomes of Chiari Malformation Type I in Pediatric and Adult Patients

Abstract

A growing number of children and adolescents are being diagnosed as Chiari malformation type I (CM-I) for behavioral disorders, developmental delay, seizures, or abnormal orpharyngeal function. The aim of this study was to compare the clinical characteristics, imaging findings and surgical outcomes of CM-I in pediatric and adult patients. Between January 2014 and June 2017, 84 patients with CM-I underwent surgical treatment in our department. We divided the patients into two groups: pediatric group (n=11, age <18 years) and adult group (n=73, age ≥18 years). Data on clinical characteristics, imaging findings, surgical outcomes, and prognosis were retrospectively reviewed and compared between these two groups. For clinical presentation, scoliosis (36.4%) and developmental delay (36.4%) were more common in pediatric patients, whereas, sensory disturbance (58.9%) and motor weakness (41.1%) were more common in adult patients. Imaging findings showed that the incidence of hydrocephalus and craniovertebral junctional abnormalities was significantly higher in pediatric group than in adult group (P<0.05). Compared to adult group, pediatric group showed a better improvement or resolution of syrinx and tonsillar herniation after surgical treatments (P<0.05). The total Chicago Chiari Outcome Scale (CCOS) score in pediatric patients at the last followup was significantly higher than that in adult patients (P=0.002). In conclusion, the clinical characteristics and imaging findings appeared to be different in pediatric and adult patients with CM-I. The surgical outcomes of pediatric patients were shown to be significantly better than those of adult patients.

Keywords

Chiari malformation type I / adult patients / pediatric patients / clinical manifestations / surgical outcome

References

[1] SchijmanE. History, anatomic forms, and pathogenesis of Chiari I malformations. Childs Nerv Syst, 2004, 20(5): 323-328

[2] DengX, YangC, GanJ, et al.. Long-Term Outcomes After Small-Bone-Window Posterior Fossa Decompression and Duraplasty in Adults with Chiari Malformation Type I. World Neurosurg, 2015, 84(4): 998-1004

[3] PillayPK, AwadIA, LittleJR, et al.. Symptomatic Chiari malformation in adults: A new classification. Neurologia, 1994, 6(5): 1110-1113

[4] AitkenLA, LindanCE, SidneyS, et al.. Chiari type I malformation in a pediatric population. Pediatr Neurol, 2009, 40(6): 449-454

[5] HidaK, IwasakiY, AbeH. Pediatric syringomyelia with chiari malformation: its clinical characteristics and surgical outcomes. Surg Neurol, 1999, 51(4): 383-390

[6] DobersteinCA, TorabiR, KlingePM. Current Concepts in the Pathogenesis, Diagnosis, and Management of Type I Chiari Malformations. R I Med J, 2017, 100(6): 47-49

[7] ArnautovicA, SplavskiB, BoopFA, et al.. Pediatric and adult Chiari malformation type I surgical series 1965-2013: a review of demographics, operative treatment, and outcomes. J Neurosurg Pediatr, 2015, 15(2): 161-177

[8] MeadowsJ, GuarnieriM, BrautM, et al.. Type I Chiari Malformation: A Review of the Literature. Neurosurg Q, 2001, 11(3): 220-229

[9] PomeraniecIJ, KsendzovskyA, AwadAJ, et al.. Natural and surgical history of Chiari malformation type I in the pediatric population. J Neurosurg Pediatr, 2016, 17(3): 343-352

[10] PorettiA, AshmawyR, GarzonmuvdiT, et al.. Chiari Type 1 Deformity in Children: Pathogenetic, Clinical, Neuroimaging, and Management Aspects. Neuropediatrics, 2016, 47(5): 293-307

[11] GreenbergJK, EricM, YarbroughCK, et al.. Outcome methods used in clinical studies of Chiari malformation type I: a systematic review. J Neurosurg, 2015, 122(2): 262-272

[12] YarbroughCK, GreenbergJK, SmythMD, et al.. External validation of the Chicago Chiari Outcome Scale. J Neurosurg Pediatr, 2014, 13(6): 679-684

[13] HayhurstC, OsmanfarahJ, DasK, et al.. Initial management of hydrocephalus associated with Chiari malformation type I-syringomyelia complex via endoscopic third ventriculostomy: an outcome analysis. J Neurosurg, 2008, 108(6): 1211-1214

[14] MassimiL, CaldarelliM, FrassanitoP, et al.. Natural history of Chiari type I malformation in children. Neurol Sci, 2011, 32(3): S275-277

[15] LeeS, KimSK, JiYL, et al.. Comparison of clinical and radiological manifestations and surgical outcomes of pediatric Chiari I malformations in different age groups. Childs Nerv Syst, 2015, 31(11): 2091-2101

[16] TubbsRS, BeckmanJ, NaftelRP, et al.. Institutional experience with 500 cases of surgically treated pediatric Chiari malformation type I. J Neurosurg Pediatr, 2011, 7(3): 248-256

[17] GilmerHS, XiM, YoungSH. Surgical decompression for Chiari malformation type I: An age-based outcomes study based on the Chicago Chiari Outcome Scale. World Neurosurg, 2017, 107(8): 285-290

[18] MilhoratTH, ChouMW, TrinidadEM, et al.. Chiari I Malformation: Description of Syndrome, Clinical Manifestation, and Inheritance Patterns in 364 Symptomatic Patients. Neurosurgery, 1998, 43(3): 1005-1017

[19] AlfieriA, PinnaG. Long-term results after posterior fossa decompression in syringomyelia with adult Chiari Type I malformation. J Neurosurg Spine, 2012, 17(5): 381-387

[20] BerettaE, VetranoIG, CuroneM, et al.. Chiari malformation-related headache: outcome after surgical treatment. Neurol Sci, 2017, 38(1): 95-98

[21] VictorioMC, KhouryCK. Headache and Chiari I Malformation in Children and Adolescents. Semin Pediatr Neurol, 2016, 23(1): 35-39

[22] GadKA, YousemDM. Syringohydromyelia in Patients with Chiari I Malformation: A Retrospective Analysis. AJNR Am J Neuroradiol, 2017, 38(9): 1833-1838

[23] GreenleeJD, DonovanKA, HasanDM, et al.. Chiari I malformation in the very young child: the spectrum of presentations and experience in 31 children under age 6 years. Pediatrics, 2002, 110(6): 1212-1219

[24] HaoX, ChuLY, RuiH, et al.. Posterior fossa decompression with and without duraplasty for the treatment of Chiari malformation type I—a systematic review and meta-analysis. Neurosurg Rev, 2017, 40(2): 1-9

[25] MassimiL, PravatàE, TamburriniG, et al.. Endoscopic third ventriculostomy for the management of Chiari I and related hydrocephalus: outcome and pathogenetic implications. Neurosurgery, 2011, 68(4): 950-956