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A6759 - Bilateral Vocal Cord Paralysis from Type 1 Chiari Malformation Masquerading as Chronic Obstructive Pulmonary Disease
Author Block: A. Kalansky1, A. M. Khan1, D. E. Murphy1, A. G. Peerless2; 1Internal Medicine, Jewish Hospital, Cincinnati, OH, United States, 2Otolaryngology, Jewish Hospital, Cincinnati, OH, United States.
Chronic obstructive pulmonary disease (COPD) is a group of progressive respiratory diseases and is the third most common cause of death in the United States. Chiari malformations are a group of neuroanatomical aberrancies characterized by the herniation of the cerebellar tonsils through the foramen magnum; symptomatic presentations in adulthood are rare.
We present a 44-year-old man with a history of dyspnea and wheezing beginning in childhood, smoking, and a diagnosis of COPD who presented with complaints of worsening symptoms for several weeks. Treatment for COPD exacerbation was initiated with steroids, nebulizers, and azithromycin, however, he failed to respond and required emergent endotracheal intubation for respiratory failure. After intubation, his wheezing completely subsided.
During his intensive care unit admission, the flow-volume loops on his ventilator were normal and thus raised suspicion for a diagnosis other than COPD. Bronchoscopy was negative for endobronchial obstruction or abnormality. Prior pulmonary function studies (PFTs) had been interpreted as consistent with COPD but when reviewed, showed a mixed restrictive and obstructive pattern of disease more consistent with a possible fixed extra-thoracic obstruction.
The patient was removed from mechanical ventilation on day 3, but quickly developed increased work of breathing and stridor despite having a clear lung exam prior to extubation. Due to suspicion for extra-thoracic pathology, bedside laryngoscopy was performed and revealed bilateral abductor paralysis of the vocal cords. The patient was quickly re-intubated and had a tracheostomy placed that same day, which resulted in the resolution of his presenting symptoms. Magnetic resonance imaging of his brain was performed and was significant for type 1 Chiari malformation with syringohydromyelia and downward displacement of the cerebellar tonsils of 9.1mm and 11mm.
The presentation of a 44-year-old man with worsening dyspnea due to a type 1 Chiari malformation causing bilateral vocal cord paralysis without other significant neurological symptoms is extremely rare. Our literature search revealed only five other reported cases in adults in which vocal cord palsy was the initial presentation for type 1 Chiari malformation.
Our patient had a smoking history and was diagnosed with COPD in his mid 30s. However, he had not responded appropriately to COPD therapy and in retrospect his PFTs were more consistent with an alternative diagnosis.
Chiari malformation is an unusual cause of bilateral vocal cord paralysis in the adult population. This case highlights the need to consider other rare causes of dyspnea, and wheezing in patients refractory to conventional treatment.