Cerebral Anomalies and Chiari Type 1 Malformation

被引:22
作者
Galarza, Marcelo [1 ]
Martinez-Lage, Juan F. [1 ]
Ham, Steven [2 ]
Sood, Sandeep [2 ]
机构
[1] Virgen Arrixaca Univ Hosp, Reg Serv Neurosurg, Murcia, Spain
[2] Childrens Hosp Michigan, Dept Neurosurg, Detroit, MI 48201 USA
关键词
Cerebellar tonsillar ectopia; Chiari type 1 malformation; Cerebral dysgenesis; Hydrocephalus; Congenital central nervous system malformations; Craniovertebral junction; I MALFORMATION; ASSOCIATION; CRANIOSYNOSTOSIS; SEIZURES; DISORDER;
D O I
10.1159/000327220
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Objective: To analyze the association of diverse cerebral anomalies in a series of pediatric patients with cerebellar tonsillar ectopia. Methods: We reviewed the medical records of 60 children diagnosed with Chiari type 1 malformation (CM1), of these, 20 patients (11 boys and 9 girls; mean age 7.2 years, range 2-16 years) had an associated cerebral anomaly. Symptoms of tonsillar ectopia evolved over a mean of 12 months (range 3 months to 4 years). Syringomyelia was present in 5 cases. All patients underwent a posterior fossa decompression. Results: Disclosed anomalies included: congenital hydrocephalus (n = 11), cervicomedullary kinking (n = 5), focal cerebral heterotopia with epilepsy (n = 4), partial agenesis of the corpus callosum (n = 4), hypoplastic brain stem (n = 2), holoprosencephaly (n = 1), and subcortical dysplasia in the context of neurofibromatosis type 1 (n = 1). Other malformations included: subcortical hamartoma associated with neurofibromatosis type 1, craniofacial dysmorphism secondary to Noonan syndrome, congenital occipital plagiocephaly, os odontoideum, craniofacial cleft, juvenile rheumathoid arthritis with platybasia, and osteogenesis imperfecta with bathrocephaly and scoliosis. Conclusion: Craniocerebral anomalies in children treated for CM1 may be found consistently. The association of hydrocephalus, which was the most common anomaly in this cohort, with cerebellar tonsillar ectopia may contribute to a poor outcome in regard to tonsillar herniation symptoms. Copyright (C) 2011 S. Karger AG, Basel
引用
收藏
页码:442 / 449
页数:8
相关论文
共 23 条
[1]   Neurofibromatosis type 1 and type 1 Chiari malformation: An unusual association [J].
Battistella, PA ;
Perilongo, G ;
Carollo, C .
CHILDS NERVOUS SYSTEM, 1996, 12 (06) :336-338
[2]   Chiari I malformation: Association with seizures and developmental disabilities [J].
Brill, CB ;
Gutierrez, J ;
Mishkin, MM .
JOURNAL OF CHILD NEUROLOGY, 1997, 12 (02) :101-106
[3]  
Chiari H., 1891, Dtsch Med Wochenschr, V17, P1172, DOI [10.1055/s-0029-1206803, DOI 10.1055/S-0029-1206803]
[4]  
Colli R, 2001, Pediatr Med Chir, V23, P61
[5]  
GALARZA M, 2007, CHILD NERV SYST, V32, P121
[6]   Basilar invagination: a study based on 190 surgically treated patients [J].
Goel, A ;
Bhatjiwale, M ;
Desai, K .
JOURNAL OF NEUROSURGERY, 1998, 88 (06) :962-968
[7]   Association of Chiari I malformation, mental retardation, speech delay, and epilepsy: A specific disorder? [J].
Grosso, S ;
Scattolini, R ;
Paolo, G ;
Di Bartolo, RM ;
Morgese, G ;
Balestri, P .
NEUROSURGERY, 2001, 49 (05) :1099-1103
[8]   Type 1 Arnold-Chiari malformation and Noonan syndrome. A new diagnostic feature? [J].
Holder-Espinasse, M ;
Winter, RM .
CLINICAL DYSMORPHOLOGY, 2003, 12 (04) :275-275
[9]   Coexistence of oto-palato-digital syndrome type II and Arnold-Chiari I malformation in an infant [J].
Hung, PC ;
Wang, HS ;
Lui, TN .
BRAIN & DEVELOPMENT, 1999, 21 (07) :488-490
[10]   MRI DEMONSTRATION OF MIDBRAIN DEFORMITY IN ASSOCIATION WITH CHIARI MALFORMATION [J].
HUNTER, JV ;
YOUL, BD ;
MOSELEY, IF .
NEURORADIOLOGY, 1992, 34 (05) :399-401