The Chiari Malformation, Type 1, in Early Childhood

Abstract

Neurosurgery| February 01 2003 The Chiari Malformation, Type 1, in Early Childhood AAP Grand Rounds (2003) 9 (2): 18–19. https://doi.org/10.1542/gr.9-2-18 Views Icon Views Article contents Figures & tables Video Audio Supplementary Data Peer Review Share Icon Share Twitter LinkedIn Tools Icon Tools Get Permissions Cite Icon Cite Search Site Citation The Chiari Malformation, Type 1, in Early Childhood. AAP Grand Rounds February 2003; 9 (2): 18–19. https://doi.org/10.1542/gr.9-2-18 Download citation file: Ris (Zotero) Reference Manager EasyBib Bookends Mendeley Papers EndNote RefWorks BibTex toolbar search toolbar search search input Search input auto suggest filter your search All PublicationsAll JournalsAAP Grand RoundsPediatricsHospital PediatricsPediatrics In ReviewNeoReviewsAAP NewsAll AAP Sites Search Advanced Search Topics: arnold-chiari malformation Source: Greenlee JDW, Donovan KA, Hasan DM, 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;110:1212–1219. The Chiari malformation type 1 (CM1) is defined as abnormal protrusion of the tonsils of the cerebellum through the foramen magnum down into the cervical spinal canal. The quantitative threshold for diagnosis of CM1 is somewhat arbitrary, but the most widely accepted definition is descent of the tonsils >5 mm below the plane of the foramen.1 The developmental lesion responsible for CM1 is believed, in most instances, to be insufficient growth of the osseous boundaries of the posterior cranial fossa: there is simply not enough room within the posterior fossa to accommodate the neural structures that belong there.2,3 Symptoms and neurological signs may develop either on the basis of mechanical compression of the brainstem at the foramen magnum or on the basis of disturbance of cerebrospinal fluid (CSF) dynamics, leading to syringomyelia.3,4 The potential clinical manifestations of CM1 are legion. In the current era of widely available magnetic resonance imaging (MRI), CM1 has become a common incidental finding on brain or cervical spine studies obtained for minor complaints.1 The natural history and potential clinical significance of asymptomatic CM1 remains largely unknown. The not inconsequential prevalence of asymptomatic CM1 in the general population and the polymorphous clinical presentations of this condition can make for occasional difficult questions about indications for surgical treatment. The authors from the University of Iowa have performed a retrospective review of a single surgeon’s experience with CM1 in children under the age of 6 during the era of MRI. There were 31 patients with CM1; 16 were under 3 years of age (mean age at diagnosis, 3.3 years). Impaired oropharyngeal function (35% for total group) was the predominant presentation among the infants (69%), and scoliosis (26% for total group) was the predominant presentation in the preschool age group (75%). Headache and neck pain (23%), sensory disturbance (6%), and weakness (3%) were also presenting complaints. Only 1 patient was judged to be asymptomatic. Only 4 patients had normal neurological examinations. Sixteen patients (52%) had syringomyelia, and all 8 patients with scoliosis had syringomyelia. Other physical findings included abnormal tendon reflexes (68%), abnormal gag reflex (13%), and vocal cord dysfunction (26%). Twenty-five patients underwent surgical treatment. Ninety-two percent of patients enjoyed resolution or improvement of preoperative symptoms, and no patient was made worse by surgery. The response of oropharyngeal dysfunction to surgical treatment is not detailed in the report, except to note that the 3 children who had undergone gastrostomy before treatment of their CM1 remained gastrostomy-dependent. Scoliosis improved in every case. This report is a useful description of the phenomenology of CM1 in early childhood from the practice of the foremost neurosurgical authority on the pediatric craniocervical junction in North America. Several comments may place this report in a helpful perspective: first, the eminence of... You do not currently have access to this content.