Atlantoaxial dislocation due to Os odontoideum in down syndrome: Literature review and case reports.

Autor: Nguyen LH; Viet Duc University Hospital, Ha Noi, Viet Nam; University of Medicine and Pharmacy, Vietnam National University, Ha Noi, Viet Nam., Nguyen KM; Viet Duc University Hospital, Ha Noi, Viet Nam; University of Medicine and Pharmacy, Vietnam National University, Ha Noi, Viet Nam., Nguyen TLB; Viet Duc University Hospital, Ha Noi, Viet Nam; University of Medicine and Pharmacy, Vietnam National University, Ha Noi, Viet Nam., Do HM; Viet Duc University Hospital, Ha Noi, Viet Nam., Bui HM; Viet Duc University Hospital, Ha Noi, Viet Nam; University of Medicine and Pharmacy, Vietnam National University, Ha Noi, Viet Nam. Electronic address: buiminhhoang.dhydtn@gmail.com., Nguyen HV; Thai Nguyen University of Medicine and Pharmacy, Thai Nguyen, Viet Nam.
Jazyk: angličtina
Zdroj: International journal of surgery case reports [Int J Surg Case Rep] 2024 Jul; Vol. 120, pp. 109888. Date of Electronic Publication: 2024 Jun 08.
DOI: 10.1016/j.ijscr.2024.109888
Abstrakt: Introduction and Importance: Os odontoideum is a rare condition commonly associated with atlantoaxial instability (AAI) and leading to atlantoaxial dislocation. The incidence of Os odontoideum is higher in patients with Down syndrome. Similar to odontoid fractures, atlantoaxial dislocation in patients with Os odontoideum can result in neurological deficits, disability, and even mortality.
Case Presentation: We present two cases of Os odontoideum accompanied by Down syndrome. Both patients were hospitalized due to progressive tetraparesis after falls several months prior. Upon examination, the patients exhibited myelopathy and were unable to walk or stand. MRI revealed spinal stenosis at the C1-C2 level due to atlantoaxial dislocation. C1-C2 fixation using Harms' technique was performed in both cases. One case experienced a complication involving instrument failure, necessitating revision surgery.
Clinical Discussion: Due to the characteristics of transverse ligament laxity, low muscle tone, excessive joint flexibility, and cognitive impairment, children with both Down syndrome and Os odontoideum are at a high risk of disability and even mortality from spinal cord injury. Most authors recommend surgical management when patients exhibit atlantoaxial instability. Additional factors such as low bone density, cognitive impairment, and a high head-to-body ratio may increase the risk of surgical instrument failure and nonunion postoperatively in patients with Down syndrome.
Conclusion: Os odontoideum is a cause of AAI in patients with DS. Indication of surgery in the presence of AAI helps to resolve neurological injury and prevent further deterioration. The use of a cervical collar is considered to prevent instrument failure postoperatively.
Competing Interests: Declaration of competing interest No potential conflict of interest relevant to this article was reported.
(Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)
Databáze: MEDLINE