Arnold-Chiari malformations (ACM) and congenital syringomyelia/syrinx are rare neurological phenomenons that can present as acute respiratory failure and contribute to multiple extubation failures despite surgical intervention. A systematic review was conducted to scrutinize the current literature, screening 65 papers and including 12 papers (13 patients). Sixty-one percent of patients had type 1 ACM and 70% had a congenital syringomyelia. Neurosurgical intervention occurred in seven patients, five patients had at least one extubation failure which was due to apnea or reoccurrence of respiratory failure, and eight patients needed tracheostomies. The neurosurgical intervention aims to improve patient symptoms, but our data and current literature suggest that patients with these pathologies still undergo long ventilation weans and are not liberated from the ventilator due to ongoing respiratory failure.

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