Journal article
Chiari I malformation in the very young child: The spectrum of presentations and experience in 31 children under age 6 years
Pediatrics (Evanston), Vol.110(6), pp.1212-1219
2002
DOI: 10.1542/peds.110.6.1212
PMID: 12456921
Abstract
Introduction: The entity of hindbrain herniation without myelodysplasia in the very young child has been poorly described. A retrospective analysis of children diagnosed with Chiari I malformation (CM I) before their sixth birthday is presented.
Methods: Since 1985, 31 children with CM I (0.3-5.8) years of age have been diagnosed at University of Iowa Hospitals and Clinics. Their records were reviewed for presenting symptoms, signs, radiographic findings, treatment, complications, and outcome.
Results: The average age at diagnosis was 3.3 years. Sixteen patients were under age 3. Chief presenting complaints included impaired oropharyngeal function (35%), scoliosis (23%), headache or neck pain (23%), sensory disturbance (6%), weakness (3%), and other (10%). Sixty-nine percent of children under age 3 had abnormal oropharyngeal function. Three patients under age 3 (19%) had undergone fundoplication and/or gastrostomy before diagnosis of CM I. Common physical findings included abnormal tendon reflexes (68%), scoliosis (26%), abnormal gag reflex (13%), and normal examination (13%). Vocal cord dysfunction (26%, all under age 3) and syringohydromyelia (52%) were also seen. Twenty-five patients were treated surgically at our institution with posterior fossa decompression, duraplasty, and cerebellar tonsillar shrinkage. Three patients were lost to follow-up. Ninety-one percent of patients reported improved symptomatology at last follow-up (mean: 3.9 years). Three patients required reoperation for recurrence of symptoms. Syringomyelia improved in all patients. Scoliosis resolved in 2 of 8 patients, improved in 5, and stabilized in 1. There was no permanent morbidity from surgery.
Discussion: We show that children with Chiari I abnormality are very likely to present with oropharyngeal dysfunction if under age 3, and either scoliosis or headache or neck pain worsened by valsalva if age 3 to 5. These symptoms are very likely to improve after Chiari decompression, which can be done with low morbidity.
Conclusions: Very young children presenting with oropharyngeal dysfunction, pain worsened by valsalva, or scoliosis should prompt the clinician to consider CM I as a possible cause.
Details
- Title: Subtitle
- Chiari I malformation in the very young child: The spectrum of presentations and experience in 31 children under age 6 years
- Creators
- Jeremy D. W GREENLEE - Department of Neurosurgery, University of Iowa Hospitals and Clinics, Iowa City, Iowa, United StatesKathleen A DONOVAN - Department of Neurosurgery, University of Iowa Hospitals and Clinics, Iowa City, Iowa, United StatesDavid M HASAN - Department of Neurosurgery, University of Iowa Hospitals and Clinics, Iowa City, Iowa, United StatesArnold H MENEZES - Department of Neurosurgery, University of Iowa Hospitals and Clinics, Iowa City, Iowa, United States
- Resource Type
- Journal article
- Publication Details
- Pediatrics (Evanston), Vol.110(6), pp.1212-1219
- DOI
- 10.1542/peds.110.6.1212
- PMID
- 12456921
- NLM abbreviation
- Pediatrics
- ISSN
- 0031-4005
- eISSN
- 1098-4275
- Publisher
- American Academy of Pediatrics; Elk Grove Village, IL
- Language
- English
- Date published
- 2002
- Academic Unit
- Roy J. Carver Department of Biomedical Engineering; Stead Family Department of Pediatrics; Iowa Neuroscience Institute; Neurosurgery; Otolaryngology
- Record Identifier
- 9984040441702771
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