NEUROLOGIC SEQUELAE SECONDARY TO ATLANTOAXIAL INSTABILITY IN DOWN-SYNDROME - IMPLICATIONS IN OTOLARYNGOLOGIC SURGERY

Objective: A comprehensive overview of atlantoaxial instability in Dow n syndrome as it relates to head and neck surgery and recommendations as derived from a review of the literature. Data Sources: English-lang uage literature: otolaryngologic problems in Down syndrome and atlanto axial biomechanics. Data Synthesis: Children affected with Down syndro me frequently require otolaryngologic procedures such as myringotomy w ith ventilation tubes or adenotonsillar surgery. Atlantoaxial instabil ity occurs in 10% to 20% of patients with Down syndrome who are at ris k for atlantoaxial subluxation and subsequent complications during ane sthetic induction and during positioning and manipulation associated w ith surgery. To identify patients who are at risk for atlantoaxial sub luxation, guidelines have been adapted from the recommendations of the American Academy of Pediatrics and the Special Olympics Inc, which in clude preoperative neurologic assessments and cervical roentgenograms in the neutral, flexion, and extension positions. Children with an atl antodental interval of greater than 4.5 mm or with peripheral neurolog ic findings should have further evaluation. Conclusions: A small perce ntage of patients with Down syndrome are at risk for atlantoaxial inst ability and subluxation. All patients with Down syndrome should have a preoperative neurologic assessment screening by the operating surgeon and/or a cervical roentgenogram in the lateral, extension, and flexio n positions. Any abnormality should be investigated before surgery.