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Published in: Child's Nervous System 11/2015

01-11-2015 | Original Paper

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

Authors: Sungjoon Lee, Seung-Ki Kim, Ji Yeoun Lee, Ji Hoon Phi, Jung Eun Cheon, In One Kim, Byung-Kyu Cho, Kyu-Chang Wang

Published in: Child's Nervous System | Issue 11/2015

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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.
Literature
1.
go back to reference 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 9:1033–1036CrossRefPubMed 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 9:1033–1036CrossRefPubMed
2.
go back to reference Badie B, Mendoza D, Batzdorf U (1995) Posterior fossa volume and response to suboccipital decompression in patients with Chiari I malformation. Neurosurgery 37:214–218CrossRefPubMed Badie B, Mendoza D, Batzdorf U (1995) Posterior fossa volume and response to suboccipital decompression in patients with Chiari I malformation. Neurosurgery 37:214–218CrossRefPubMed
6.
go back to reference Marin-Padilla M, Marin-Padilla TM (1981) Morphogenesis of experimentally induced Arnold–Chiari malformation. J Neurol Sci 50:29–55CrossRefPubMed Marin-Padilla M, Marin-Padilla TM (1981) Morphogenesis of experimentally induced Arnold–Chiari malformation. J Neurol Sci 50:29–55CrossRefPubMed
9.
go back to reference 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 CrossRefPubMed 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 CrossRefPubMed
10.
go back to reference 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 CrossRefPubMed 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 CrossRefPubMed
12.
go back to reference 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 CrossRefPubMed 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 CrossRefPubMed
13.
go back to reference 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 CrossRefPubMed 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 CrossRefPubMed
15.
go back to reference 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 110:1212–1219CrossRefPubMed 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 110:1212–1219CrossRefPubMed
17.
go back to reference 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 CrossRef 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 CrossRef
18.
20.
go back to reference 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) 27:1451–1455CrossRef 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) 27:1451–1455CrossRef
22.
23.
go back to reference 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 CrossRefPubMed 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 CrossRefPubMed
24.
go back to reference 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) 29:286–292CrossRef 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) 29:286–292CrossRef
25.
go back to reference Nagib MG (1994) An approach to symptomatic children (ages 4–14 years) with Chiari type I malformation. Pediatr Neurosurg 21:31–35CrossRefPubMed Nagib MG (1994) An approach to symptomatic children (ages 4–14 years) with Chiari type I malformation. Pediatr Neurosurg 21:31–35CrossRefPubMed
26.
go back to reference 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 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
27.
go back to reference Arai S, Ohtsuka Y, Moriya H, Kitahara H, Minami S (1993) Scoliosis associated with syringomyelia. Spine (Phila Pa 1976) 18:1591–1592CrossRef Arai S, Ohtsuka Y, Moriya H, Kitahara H, Minami S (1993) Scoliosis associated with syringomyelia. Spine (Phila Pa 1976) 18:1591–1592CrossRef
28.
go back to reference Huebert HT, MacKinnon WB (1969) Syringomyelia and scoliosis. J Bone Joint Surg (Br) 51:338–343 Huebert HT, MacKinnon WB (1969) Syringomyelia and scoliosis. J Bone Joint Surg (Br) 51:338–343
29.
go back to reference 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 CrossRefPubMed 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 CrossRefPubMed
30.
go back to reference 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 CrossRefPubMed 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 CrossRefPubMed
32.
go back to reference 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 CrossRefPubMed 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 CrossRefPubMed
33.
go back to reference 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 8:97–100CrossRefPubMed 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 8:97–100CrossRefPubMed
Metadata
Title
Comparison of clinical and radiological manifestations and surgical outcomes of pediatric Chiari I malformations in different age groups
Authors
Sungjoon Lee
Seung-Ki Kim
Ji Yeoun Lee
Ji Hoon Phi
Jung Eun Cheon
In One Kim
Byung-Kyu Cho
Kyu-Chang Wang
Publication date
01-11-2015
Publisher
Springer Berlin Heidelberg
Published in
Child's Nervous System / Issue 11/2015
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-015-2849-5

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