[Chiari type I malformation in children: presentation in 34 cases].
Incidental diagnoses of Chiari I malformation are more and more frequent in pauci or asymptomatic children. The value of neurophysiological investigations for surgical indications is discussed.


OBJECTIVE
To determine clinical presentations of Chiari I malformation and relative frequencies of severe and incidental forms, and to evaluate the usefulness of neurophysiological investigations for surgical indications.


METHODS
This retrospective study included 34 patients admitted from 1984 to 2000, with Chiari I malformation diagnosis in different pediatric (intensive care, neurology and neurosurgery) units of a university hospital.


RESULTS
The clinical presentation was severe (two children died) in four cases (12%). Signs of brain stem compression were found in 41% of cases, scoliosis in 21%, and incidental diagnosis in 26%. The polysomnography, performed in 12 cases, showed sleep-apneas in six cases. One child with an incidental diagnosis of Chiari I malformation had many sleep-apneas which required a surgical treatment. A surgical decompression was performed in 69% of cases, with clinical improvement in 77%.


CONCLUSIONS
The high incidence (12%) of severe forms revealing Chiari I malformations justifies systematic neurophysiological investigations for the initial evaluation and the follow-up of Chiari 1 malformations, including the less symptomatic forms.
Clinique de pédiatrie, hôpital Jeanne-de-Flandre, centre hospitalier et universitaire, 2, avenue Oscar-Lambret, 59037 Lille, France.
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Goran
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Leclerc
D000293:Adolescent; D001139:Arnold-Chiari Malformation / Q000175:diagnosis* / Q000473:pathology; D001933:Brain Stem / Q000473:pathology; D002648:Child; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D012600:Scoliosis / Q000209:etiology; D012720:Severity of Illness Index; D012891:Sleep Apnea Syndromes / Q000209:etiology