Chiari I-a 'not So' Congenital Malformation?

Overview

Journal Childs Nerv Syst

Specialty Pediatrics

Date 2019 Jul 12

PMID 31292759

Citations 13

Authors

Dominic N P Thompson

Affiliations

Soon will be listed here.

Abstract

The term Chiari I malformation (CIM) is imbedded in the paediatric neurosurgical lexicon; however, the diagnostic criteria for this entity are imprecise, its pathophysiology variable, and the treatment options diverse. Until recently, CIM has been considered to be a discrete congenital malformation requiring a uniform approach to treatment. Increasingly, it is recognised that this is an oversimplification and that a more critical, etiologically based approach to the evaluation of children with this diagnosis is essential, not only to select those children who might be suitable for surgical treatment (and, of course those who might be better served by conservative management) but also to determine the most appropriate surgical strategy. Whilst good outcomes can be anticipated in the majority of children with CIM following foramen magnum decompression, treatment failures and complication rates are not insignificant. Arguably, poor or suboptimal outcomes following treatment for CIM reflect, not only a failure of surgical technique, but incorrect patient selection and failure to acknowledge the diverse pathophysiology underlying the phenomenon of CIM. The investigation of the child with 'hindbrain herniation' should be aimed at better understanding the mechanisms underlying the herniation so that these may be addressed by an appropriate choice of treatment.

Citing Articles

Surgical modeling of Chiari-like malformation in rats: Insights from canine morphology.

Jung J, Cho C, Shin S, Chung E, Seo D, Kim W PLoS One. 2024; 19(9):e0310505.

PMID: 39298416 PMC: 11412529. DOI: 10.1371/journal.pone.0310505.

Regression of Chiari malformation type 2 following early postnatal meningomyelocele repair-a retrospective observation from an institutional series of patients.

Fric R, Beyer M, Due-Tonnessen B Childs Nerv Syst. 2024; 40(11):3641-3646.

PMID: 39207525 PMC: 11538272. DOI: 10.1007/s00381-024-06586-3.

Long-term outcome of operated Chiari I patients between 2005 and 2020 in Eastern Finland.

Moniruzzaman S, Kaipainen A, Tervonen J, Huttunen J, Jyrkkanen H, Huuskonen T Acta Neurochir (Wien). 2024; 166(1):115.

PMID: 38416251 PMC: 10902000. DOI: 10.1007/s00701-024-05999-y.

Preoperative estimation of intracranial compliance in symptomatic children with Chiari malformation type 1: impact on outcome and risk of complications.

Fric R, Bryne E, Warsza B, Due-Tonnessen B, Eide P Acta Neurochir (Wien). 2024; 166(1):22.

PMID: 38236374 PMC: 10796482. DOI: 10.1007/s00701-024-05897-3.

Research process, recap, and prediction of Chiari malformation based on bicentennial history of nomenclature and terms misuse.

He Y, Zhang M, Qin X, Huang C, Liu P, Tao Y Neurosurg Rev. 2023; 46(1):316.

PMID: 38030943 DOI: 10.1007/s10143-023-02207-w.

References

Grabb P, Mapstone T, Oakes W . Ventral brain stem compression in pediatric and young adult patients with Chiari I malformations. Neurosurgery. 1999; 44(3):520-7; discussion 527-8. DOI: 10.1097/00006123-199903000-00050. View

Milhorat T, Chou M, Trinidad E, Kula R, Mandell M, Wolpert C . Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery. 1999; 44(5):1005-17. DOI: 10.1097/00006123-199905000-00042. View

Rich P, Cox T, Hayward R . The jugular foramen in complex and syndromic craniosynostosis and its relationship to raised intracranial pressure. AJNR Am J Neuroradiol. 2003; 24(1):45-51. PMC: 8148961. View

Tubbs R, Wellons 3rd J, Blount J, Grabb P, Oakes W . Inclination of the odontoid process in the pediatric Chiari I malformation. J Neurosurg. 2003; 98(1 Suppl):43-9. DOI: 10.3171/spi.2003.98.1.0043. View

Tubbs R, Wellons 3rd J, Smyth M, Bartolucci A, Blount J, Oakes W . Children with growth hormone deficiency and Chiari I malformation: a morphometric analysis of the posterior cranial fossa. Pediatr Neurosurg. 2003; 38(6):324-8. DOI: 10.1159/000070416. View

Sacco D, Scott R . Reoperation for Chiari malformations. Pediatr Neurosurg. 2003; 39(4):171-8. DOI: 10.1159/000072467. View

Royo-Salvador M, Sole-Llenas J, Domenech J, Gonzalez-Adrio R . Results of the section of the filum terminale in 20 patients with syringomyelia, scoliosis and Chiari malformation. Acta Neurochir (Wien). 2005; 147(5):515-23. DOI: 10.1007/s00701-005-0482-y. View

Kim L, Rekate H, Klopfenstein J, Sonntag V . Treatment of basilar invagination associated with Chiari I malformations in the pediatric population: cervical reduction and posterior occipitocervical fusion. J Neurosurg. 2005; 101(2 Suppl):189-95. DOI: 10.3171/ped.2004.101.2.0189. View

Cinalli G, Spennato P, Sainte-Rose C, Arnaud E, Aliberti F, Brunelle F . Chiari malformation in craniosynostosis. Childs Nerv Syst. 2005; 21(10):889-901. DOI: 10.1007/s00381-004-1115-z. View

10.

Fagan L, Ferguson S, Yassari R, Frim D . The Chiari pseudotumor cerebri syndrome: symptom recurrence after decompressive surgery for Chiari malformation type I. Pediatr Neurosurg. 2005; 42(1):14-9. DOI: 10.1159/000089504. View

11.

Sgouros S, Kountouri M, Natarajan K . Posterior fossa volume in children with Chiari malformation Type I. J Neurosurg. 2006; 105(2 Suppl):101-6. DOI: 10.3171/ped.2006.105.2.101. View

12.

Pueyrredon F, Spaho N, Arroyave I, Vinters H, Lazareff J . Histological findings in cerebellar tonsils of patients with Chiari type I malformation. Childs Nerv Syst. 2006; 23(4):427-9. DOI: 10.1007/s00381-006-0252-y. View

13.

Sandberg D, Navarro R, Blanch J, Ragheb J . Anomalous venous drainage preventing safe posterior fossa decompression in patients with chiari malformation type I and multisutural craniosynostosis. Report of two cases and review of the literature. J Neurosurg. 2007; 106(6 Suppl):490-4. DOI: 10.3171/ped.2007.106.6.490. View

14.

Milhorat T, Bolognese P, Nishikawa M, McDonnell N, Francomano C . Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and chiari malformation type I in patients with hereditary disorders of connective tissue. J Neurosurg Spine. 2007; 7(6):601-9. DOI: 10.3171/SPI-07/12/601. View

15.

Tubbs R, Hill M, Loukas M, Shoja M, Oakes W . Volumetric analysis of the posterior cranial fossa in a family with four generations of the Chiari malformation Type I. J Neurosurg Pediatr. 2008; 1(1):21-4. DOI: 10.3171/PED-08/01/021. View

16.

Hayhurst C, Osman-Farah J, Das K, Mallucci C . Initial management of hydrocephalus associated with Chiari malformation Type I-syringomyelia complex via endoscopic third ventriculostomy: an outcome analysis. J Neurosurg. 2008; 108(6):1211-4. DOI: 10.3171/JNS/2008/108/6/1211. View

17.

Gupta A, Vitali A, Rothstein R, Cochrane D . Resolution of syringomyelia and Chiari malformation after growth hormone therapy. Childs Nerv Syst. 2008; 24(11):1345-8. DOI: 10.1007/s00381-008-0675-8. View

18.

Milhorat T, Bolognese P, Nishikawa M, Francomano C, McDonnell N, Roonprapunt C . Association of Chiari malformation type I and tethered cord syndrome: preliminary results of sectioning filum terminale. Surg Neurol. 2009; 72(1):20-35. PMC: 5999045. DOI: 10.1016/j.surneu.2009.03.008. View

19.

Aquilina K, Merchant T, Boop F, Sanford R . Chiari I malformation after cranial radiation therapy in childhood: a dynamic process associated with changes in clival growth. Childs Nerv Syst. 2009; 25(11):1429-36. DOI: 10.1007/s00381-009-0982-8. View

20.

Massimi L, Pravata E, Tamburrini G, Gaudino S, Pettorini B, Novegno F . Endoscopic third ventriculostomy for the management of Chiari I and related hydrocephalus: outcome and pathogenetic implications. Neurosurgery. 2011; 68(4):950-6. DOI: 10.1227/NEU.0b013e318208f1f3. View