Sleep-Related Hypoventilation in a Patient With Ehlers-Danlos Syndrome.

Although sleep apnea is frequently observed in patients with hypermobile Ehlers-Danlos syndrome (EDS), hypoventilation is uncommon. We present a case of a patient with hypermobile EDS without sleep apnea who was found to have isolated sleep-related hypoventilation. Hypoventilation is defined by elevated PaCO2. It is exacerbated during sleep when ventilatory control sensitivity is diminished. Hypoventilation is further worsened during rapid eye movement (REM) sleep when accessory respiratory muscles are atonic, and breathing is driven solely by the diaphragm. This report highlights the importance of considering hypoventilation in patients with EDS, especially those with relevant risk factors.