Case report of Os odontoideum causing Ondine's curse

Abstract

Ondine's curse or central hypoventilation syndrome is most common congenital disorder which is diagnosed in infancy. In the majority of cases, no structural abnormality is identified. We describe the case of an 18-year-old patient who presented with Ondine's curse secondary to an os odontoideum.

Os Odontoideum: A Comprehensive Clinical and Surgical Review

“…Although the etiology, natural history, and need for surgery remain controversial, clinical presentation among patients with OO can be classified into four main categories: incidental finding in asymptomatic patients, local symptoms, cervical myelopathic symptoms and signs, and symptoms related to vertebrobasilar ischemia [ 28 ]. This wide range of manifestations can be attributed to multiple factors, such as the slow progression of atlanto-axial instability and irritation caused by an OO [ 16 ], the anatomical type of OO (dystopic vs. orthotopic) [ 29 ], and the radiographic morphology of the atlanto-axial joint, the round type being associated with more severe myelopathic manifestations than either the cone or blunt-tooth types [ 30 ].…”

“…Patients with myelopathy can present with transient or progressive symptoms related to the site and degree of compression [ 25 , 32 ]. The literature documents a wide range of such symptoms, including weakness, numbness, and paresthesias [ 2 ], the central cord syndrome, the Brown-Séquard syndrome [ 34 ], Lhermitte’s phenomenon [ 35 ], the central hypoventilation syndrome (Ondine’s curse) [ 29 ], and cardiorespiratory arrest and sudden death [ 36 ]. One case report described the association of OO with the ossification of the posterior atlanto-axial membrane in an adult patient with severe myelopathy and assumed that the ossification, which is a rare cause of myelopathy, was a consequence of chronic mechanical stress on the ligament [ 37 ].…”

Treatment of os odontoideum in a patient with spastic quadriplegic cerebral palsy

“…This also seems unlikely as apnea only occurring during sleep. Another case report of OO [24] reported similar loss of respiratory drive. In this report, myelopathy and shortness of breath were the presenting symptoms.…”

Os odontoideum

“…[ 6 , 7 ] Increased motion at the C1 to C2 level combined with chronic mechanical stress on the craniocervical ligament can further induce symptoms of central cord syndrome, hypoventilation syndrome (Ondine's curse), Brown-Sequard syndrome, Lhermitte's phenomenon, and cardiorespiratory arrest. [ 14 , 69 , 70 , 71 , 72 ] In addition to the myelopathic sensory motor and cardiorespiratory disturbances resulting from impingement of the bulbospinal junction, other symptoms of OO associated with CVJ instability can include sleep apnea, lower cranial nerve dysfunctions, hyperesthesia, bowel and bladder dysfunction, hypoanesthesia, allodynia, and hyperalgesia. [ 17 , 73 ]…”