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Respiratory obstruction as a sign of brainstem dysfunction in infants with Chiari malformations
F Portier1, R Marianowski, M P Morisseau-Durand
1Department of ENT, Hôpital Lariboisière, Paris, France.
Insights
Chiari malformations can cause laryngeal respiratory obstruction in children, often with brainstem dysfunction. Surgical decompression for Chiari malformations led to full recovery in all treated patients.
Area of Science:
- Pediatric Neurology
- Otolaryngology
- Neurosurgery
Background:
- Laryngeal respiratory obstruction linked to Chiari malformations was first documented in 1932.
- This condition can manifest with various signs of brainstem dysfunction in children.
Purpose of the Study:
- To investigate the association between Chiari malformations and laryngeal respiratory obstruction in pediatric patients.
- To evaluate the effectiveness of surgical intervention for Chiari malformations in resolving these obstructions.
Main Methods:
- Case study of six children with laryngeal respiratory obstruction and signs of brainstem dysfunction.
- Diagnostic imaging using T1- and T2-weighted MRI to identify Chiari malformations.
- Assessment of surgical outcomes following decompressive surgery for Chiari malformations.
Main Results:
- Observed a variable nature of laryngeal obstruction (e.g., vocal cord paralysis, laryngomalacia) and associated brainstem dysfunction.
- T1- and T2-weighted MRI proved effective in diagnosing Chiari malformations.
- Decompressive surgery for Chiari malformations resulted in full functional recovery for five out of six patients.
Conclusions:
- Chiari malformations should be considered in children with laryngeal respiratory obstruction, particularly with co-occurring brainstem dysfunction.
- Symptomatic treatment of brainstem signs is recommended prior to surgical correction of Chiari malformations.
- Decompressive surgery is a highly effective treatment for Chiari malformation-associated laryngeal obstruction.
Abstract:
Laryngeal respiratory obstruction associated with Chiari malformations was first described in 1932. We studied this type of obstruction in six children with one or several disorders pointing to brainstem dysfunction (failure to thrive, velopharyngeal incompetence, gastroesophageal reflux, or vagal hypertonia). The nature of the laryngeal obstruction was highly variable (vocal cord paralysis, paradoxical vocal cord motion, laryngomalacia) as were the frequency and severity of associated disorders. Chiari malformations should be routinely sought in a child with laryngeal respiratory obstruction occurring at birth or later, whatever the endoscopic diagnosis, especially when signs of brainstem dysfunction are present. The best tool for diagnosing the Chiari malformation is T1- and T2-weighted MRI. Signs of brainstem dysfunction must be treated symptomatically, before treating Chiari malformations by decompressive surgery. This latter approach led to full functional recovery in all five children who underwent the procedure. Palliative surgical treatment should be reserved for patients in whom this procedure is unsuccessful.