Skip to main content

Advertisement

Log in

Comparison of clinical and radiological manifestations and surgical outcomes of pediatric Chiari I malformations in different age groups

  • Original Paper
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Purpose

The aim of this study was to elucidate the differences in clinical and radiological manifestations and treatment outcomes of pediatric Chiari I malformation patients according to age.

Methods

We retrospectively reviewed the patients who received surgery in our institution for symptomatic Chiari I malformations between January 1991 and December 2012. Fifty-four patients were identified, and their medical records were reviewed for clinical presentation, radiological findings, surgical treatment, and outcomes including complications. We divided the patients into 3 groups: Group I (n = 4) younger than 3 years old; Group II (n = 9) between 3 and 5 years old; and Group III (n = 41) older than 5 years old. Surveyed data were compared among the groups. The mean follow-up period was 82.8 months.

Results

All of Group I patients presented with stem compression signs. Ventriculomegaly was common (3/4, 75 %), but no syrinx or scoliosis was observed. In Group II, scoliosis was the most common presentation (5/9, 56 %), and these patients rarely complained any other symptoms. Pain, such as headache (18/41, 44 %), was the predominant presentation in Group III. The incidences of syrinx and scoliosis were similar in Groups II and III. There were no differences in the treatment outcomes among the groups. The spinal curve did not improve in 4 of 5 Group II patients despite their early FMD surgeries. The incidence of complications related to cerebrospinal fluid leakage was higher in the young age group.

Conclusions

The clinical and radiological manifestations of pediatric Chiari I malformation appeared to be different according to age.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Aboulezz AO, Sartor K, Geyer CA, Gado MH (1985) Position of cerebellar tonsils in the normal population and in patients with Chiari malformation: a quantitative approach with MR imaging. J Comput Assist Tomogr 9:1033–1036

    Article  CAS  PubMed  Google Scholar 

  2. Badie B, Mendoza D, Batzdorf U (1995) Posterior fossa volume and response to suboccipital decompression in patients with Chiari I malformation. Neurosurgery 37:214–218

    Article  CAS  PubMed  Google Scholar 

  3. Di Rocco C, Frassanito P, Massimi L, Peraio S (2011) Hydrocephalus and Chiari type I malformation. Childs Nerv Syst 27:1653–1664. doi:10.1007/s00381-011-1545-3

    Article  PubMed  Google Scholar 

  4. Gardner WJ (1965) Hydrodynamic mechanism of syringomyelia: its relationship to myelocele. J Neurol Neurosurg Psychiatry 28:247–259

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  5. Greitz D (2006) Unraveling the riddle of syringomyelia. Neurosurg Rev 29:251–263. doi:10.1007/s10143-006-0029-5, discussion 264

    Article  PubMed  Google Scholar 

  6. Marin-Padilla M, Marin-Padilla TM (1981) Morphogenesis of experimentally induced Arnold–Chiari malformation. J Neurol Sci 50:29–55

    Article  CAS  PubMed  Google Scholar 

  7. Nishikawa M, Sakamoto H, Hakuba A, Nakanishi N, Inoue Y (1997) Pathogenesis of Chiari malformation: a morphometric study of the posterior cranial fossa. J Neurosurg 86:40–47. doi:10.3171/jns.1997.86.1.0040

    Article  CAS  PubMed  Google Scholar 

  8. Oldfield EH, Muraszko K, Shawker TH, Patronas NJ (1994) Pathophysiology of syringomyelia associated with Chiari I malformation of the cerebellar tonsils. Implications for diagnosis and treatment. J Neurosurg 80:3–15. doi:10.3171/jns.1994.80.1.0003

    Article  CAS  PubMed  Google Scholar 

  9. Gallo P, Szathmari A, De Biasi S, Mottolese C (2010) Endoscopic third ventriculostomy in obstructive infantile hydrocephalus: remarks about the so-called ‘unsuccessful cases’. Pediatr Neurosurg 46:435–441. doi:10.1159/000324913

    Article  PubMed  Google Scholar 

  10. Kim SK, Wang KC, Cho BK (2000) Surgical outcome of pediatric hydrocephalus treated by endoscopic III ventriculostomy: prognostic factors and interpretation of postoperative neuroimaging. Childs Nerv Syst 16:161–168, discussion 169

    Article  CAS  PubMed  Google Scholar 

  11. Koch D, Wagner W (2004) Endoscopic third ventriculostomy in infants of less than 1 year of age: which factors influence the outcome? Childs Nerv Syst 20:405–411. doi:10.1007/s00381-004-0958-7

    Article  PubMed  Google Scholar 

  12. Warf BC, Tracy S, Mugamba J (2012) Long-term outcome for endoscopic third ventriculostomy alone or in combination with choroid plexus cauterization for congenital aqueductal stenosis in African infants. J Neurosurg Pediatr 10:108–111. doi:10.3171/2012.4.PEDS1253

    Article  PubMed  Google Scholar 

  13. Mattei TA, Bond BJ, Sambhara D, Goulart CR, Lin JJ (2013) Benign extracerebral fluid collection in infancy as a risk factor for the development of de novo intracranial arachnoid cysts. J Neurosurg Pediatr 12:555–564. doi:10.3171/2013.8.PEDS1399

    Article  PubMed  Google Scholar 

  14. Albert GW, Menezes AH, Hansen DR, Greenlee JD, Weinstein SL (2010) Chiari malformation Type I in children younger than age 6 years: presentation and surgical outcome. J Neurosurg Pediatr 5:554–561. doi:10.3171/2010.3.PEDS09489

    Article  PubMed  Google Scholar 

  15. Greenlee JD, Donovan KA, Hasan DM, Menezes AH (2002) Chiari I malformation in the very young child: the spectrum of presentations and experience in 31 children under age 6 years. Pediatrics 110:1212–1219

    Article  PubMed  Google Scholar 

  16. Tubbs RS, Lyerly MJ, Loukas M, Shoja MM, Oakes WJ (2007) The pediatric Chiari I malformation: a review. Childs Nerv Syst 23:1239–1250. doi:10.1007/s00381-007-0428-0

    Article  PubMed  Google Scholar 

  17. Lee S, Wang KC, Cheon JE, Phi JH, Lee JY, Cho BK, Kim SK (2013) Surgical outcome of Chiari I malformation in children: clinico-radiological factors and technical aspects. Childs Nerv Syst 30:613–623. doi:10.1007/s00381-013-2263-9

    Article  Google Scholar 

  18. Bollo RJ, Riva-Cambrin J, Brockmeyer MM, Brockmeyer DL (2012) Complex Chiari malformations in children: an analysis of preoperative risk factors for occipitocervical fusion. J Neurosurg Pediatr 10:134–141. doi:10.3171/2012.3.PEDS11340

    Article  PubMed  Google Scholar 

  19. Smoker WR (1994) Craniovertebral junction: normal anatomy, craniometry, and congenital anomalies. Radiographics 14:255–277. doi:10.1148/radiographics.14.2.8190952

    Article  CAS  PubMed  Google Scholar 

  20. Eule JM, Erickson MA, O’Brien MF, Handler M (2002) 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) 27:1451–1455

    Article  Google Scholar 

  21. El-Ghandour NM (2012) Long-term outcome of surgical management of adult Chiari I malformation. Neurosurg Rev 35:537–546. doi:10.1007/s10143-012-0387-0, discussion 546-7

    Article  PubMed  Google Scholar 

  22. Attenello FJ, McGirt MJ, Gathinji M, Datoo G, Atiba A, Weingart J, Carson B, Jallo GI (2008) Outcome of Chiari-associated syringomyelia after hindbrain decompression in children: analysis of 49 consecutive cases. Neurosurgery 62:1307–1313. doi:10.1227/01.neu.0000333302.72307.3b, discussion 1313

    Article  PubMed  Google Scholar 

  23. Attenello FJ, McGirt MJ, Atiba A, Gathinji M, Datoo G, Weingart J, Carson B, Jallo GI (2008) Suboccipital decompression for Chiari malformation-associated scoliosis: risk factors and time course of deformity progression. J Neurosurg Pediatr 1:456–460. doi:10.3171/PED/2008/1/6/456

    Article  PubMed  Google Scholar 

  24. Flynn JM, Sodha S, Lou JE, Adams SB Jr, Whitfield B, Ecker ML, Sutton L, Dormans JP, Drummond DS (2004) Predictors of progression of scoliosis after decompression of an Arnold Chiari I malformation. Spine (Phila Pa 1976) 29:286–292

    Article  Google Scholar 

  25. Nagib MG (1994) An approach to symptomatic children (ages 4–14 years) with Chiari type I malformation. Pediatr Neurosurg 21:31–35

    Article  CAS  PubMed  Google Scholar 

  26. Massimi L, Pravata E, Tamburrini G, Gaudino S, Pettorini B, Novegno F, Colosimo C Jr, Di Rocco C (2011) Endoscopic third ventriculostomy for the management of Chiari I and related hydrocephalus: outcome and pathogenetic implications. Neurosurgery 68:950–956. doi:10.1227/NEU.0b013e318208f1f3

    PubMed  Google Scholar 

  27. Arai S, Ohtsuka Y, Moriya H, Kitahara H, Minami S (1993) Scoliosis associated with syringomyelia. Spine (Phila Pa 1976) 18:1591–1592

    Article  CAS  Google Scholar 

  28. Huebert HT, MacKinnon WB (1969) Syringomyelia and scoliosis. J Bone Joint Surg (Br) 51:338–343

    CAS  Google Scholar 

  29. Isu T, Iwasaki Y, Akino M, Abe H (1990) Hydrosyringomyelia associated with a Chiari I malformation in children and adolescents. Neurosurgery 26:591–596, discussion 596–597

    Article  CAS  PubMed  Google Scholar 

  30. Hayhurst C, Osman-Farah J, Das K, Mallucci C (2008) Initial management of hydrocephalus associated with Chiari malformation Type I-syringomyelia complex via endoscopic third ventriculostomy: an outcome analysis. J Neurosurg 108:1211–1214. doi:10.3171/JNS/2008/108/6/1211

    Article  PubMed  Google Scholar 

  31. Dyste GN, Menezes AH, VanGilder JC (1989) Symptomatic Chiari malformations. An analysis of presentation, management, and long-term outcome. J Neurosurg 71:159–168. doi:10.3171/jns.1989.71.2.0159

    Article  CAS  PubMed  Google Scholar 

  32. Tubbs RS, Beckman J, Naftel RP, Chern JJ, Wellons JC 3rd, Rozzelle CJ, Blount JP, Oakes WJ (2011) Institutional experience with 500 cases of surgically treated pediatric Chiari malformation Type I. J Neurosurg Pediatr 7:248–256. doi:10.3171/2010.12.PEDS10379

    Article  PubMed  Google Scholar 

  33. Isu T, Chono Y, Iwasaki Y, Koyanagi I, Akino M, Abe H, Abumi K, Kaneda K (1992) Scoliosis associated with syringomyelia presenting in children. Childs Nerv Syst 8:97–100

    Article  CAS  PubMed  Google Scholar 

  34. Bhangoo R, Sgouros S (2006) Scoliosis in children with Chiari I-related syringomyelia. Childs Nerv Syst 22:1154–1157. doi:10.1007/s00381-006-0090-y

    Article  PubMed  Google Scholar 

  35. Brockmeyer D, Gollogly S, Smith JT (2003) Scoliosis associated with Chiari 1 malformations: the effect of suboccipital decompression on scoliosis curve progression: a preliminary study. Spine (Phila Pa 1976) 28:2505–2509. doi:10.1097/01.BRS.0000092381.05229.87

    Article  Google Scholar 

  36. Krieger MD, Falkinstein Y, Bowen IE, Tolo VT, McComb JG (2011) Scoliosis and Chiari malformation Type I in children. J Neurosurg Pediatr 7:25–29. doi:10.3171/2010.10.PEDS10154

    Article  PubMed  Google Scholar 

  37. Oi S, Di Rocco C (2006) Proposal of “evolution theory in cerebrospinal fluid dynamics” and minor pathway hydrocephalus in developing immature brain. Childs Nerv Syst 22:662–669. doi:10.1007/s00381-005-0020-4

    Article  PubMed  Google Scholar 

  38. Symss NP, Oi S (2013) Theories of cerebrospinal fluid dynamics and hydrocephalus: historical trend. J Neurosurg Pediatr 11:170–177. doi:10.3171/2012.3.PEDS0934

    Article  PubMed  Google Scholar 

  39. Mattei TA, Sambhara D, Bond BJ, Lin J (2014) Clinical outcomes of temporary shunting for infants with cerebral pseudomeningocele. Childs Nerv Syst 30:283–291. doi:10.1007/s00381-013-2230-5

    Article  PubMed  Google Scholar 

  40. Williams B (1980) On the pathogenesis of syringomyelia: a review. J R Soc Med 73:798–806

    PubMed Central  CAS  PubMed  Google Scholar 

Download references

Acknowledgments

This research was supported by a grant of the Korea Health Technology R&D Project through the Korea Health Industry Development Institute (KHIDI), funded by the Ministry of Health & Welfare, Republic of Korea (grant number : HI12C0066).

Conflict of interest

The authors declare that they have no conflicts of interest.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Kyu-Chang Wang.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Lee, S., Kim, SK., Lee, J.Y. et al. Comparison of clinical and radiological manifestations and surgical outcomes of pediatric Chiari I malformations in different age groups. Childs Nerv Syst 31, 2091–2101 (2015). https://doi.org/10.1007/s00381-015-2849-5

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-015-2849-5

Keywords

Navigation