Coronavirus disease 2019-associated persistent cough and Chiari malformation type I resulting in acute respiratory...

Chiari malformation type I (CM-I) is the herniation of cerebellar tonsils through the foramen magnum, potentially resulting in the obstruction of cerebrospinal fluid flow and brainstem compression. Sleep-disordered breathing (SDB) is common in patients with CM-I, and symptomatic exacerbations have been described after Valsalva-inducing stressors. Acute decompensation in the setting of coronavirus disease 2019 (COVID-19) has not been described.

After violent coughing episodes associated with COVID-19 infection, a 44-year-old female developed several months of Valsalva-induced occipital headaches, episodic bulbar symptoms, and worsening SDB, which led to acute respiratory failure requiring mechanical ventilation. Imaging demonstrated 12 mm of cerebellar tonsillar descent below the foramen magnum, dorsal brainstem compression, and syringobulbia within the dorsal medulla. She underwent posterior fossa and intradural decompression with near-complete resolution of her symptoms 6 months postoperatively.

Although CM-I can remain asymptomatic, Valsalva-inducing stressors, including COVID-19 infection, can initiate or acutely exacerbate symptoms, placing patients at risk for CM-I-associated brainstem dysfunction and, in rare cases, acute respiratory failure. Worsening Valsalva maneuvers can contribute to further cerebellar tonsil impaction, brainstem compression, syringomyelia/syringobulbia, and worsening CM-I intradural pathology. Ventilator support and timely decompressive surgery are paramount, as brainstem compression can reduce central respiratory drive, placing patients at risk for coma, neurological deficits, and/or death.