What is atlantoaxial instability in Down syndrome?

What is atlantoaxial instability in Down syndrome?

Atlanto-axial instability (AAI) is a condition that affects the bones in the upper spine or neck under the base of the skull. The joint between the upper spine and base of the skull is called the atlanto-axial joint. In people with Down syndrome, the ligaments (connections between muscles) are “lax” or floppy.

What percentage of Down syndrome population has atlantoaxial instability?

Atlantoaxial instability (AAI) affects 10–20% of individuals with Down syndrome (DS). The condition is mostly asymptomatic and diagnosed on radiography by an enlarged anterior atlanto-odontoid distance.

What are the symptoms of atlantoaxial instability?

Neurologic manifestations include clumsiness, lack of coordination, abnormal gait, difficulty walking, easy fatigability, neck pain, limited next mobility, torticollis, sensory deficits, neurogenic bladder, upper motor neuron signs (spasticity, hyperreflexia, clonus, Babinski sign), paraplegia, hemiplegia, and …

How do you fix atlantoaxial instability?

To fix your Atlantoaxial instability, you need to fix your cervical ligaments. Surgery is often aimed at fixing the instability by fusing vertebral segments together. Adjacent segment degeneration after cervical fusion surgery was 32.8%

What type of joint is atlantoaxial?

It is classified as a pivot joint. The lateral atlantoaxial joints are bilateral joints formed between the lateral masses of the atlas and axis. These joints are classified as gliding, or plane joints….Atlantoaxial joint.

Type Atlantoaxial joint complex: Synovial joint; biaxial
Blood supply Deep cervical, occipital, vertebral arteries

What movement does the atlantoaxial joint allow?

The C1 C2 spinal motion segment, also called the atlantoaxial joint, is located in the upper portion of the cervical spine. It consists of the C1 and C2 vertebrae, and the anatomical structures connecting them. This segment provides rotational motion, supports the head, and protects the spinal cord and nerve pathways.

What prevents atlantoaxial instability?

Individuals with AAI are restricted from participation in certain activities that may result in cervical spine injury. These include gymnastics, diving, pentathlon, butterfly stroke, diving starts in swimming, high jump, soccer, and certain warmup exercises.

What causes atlantoaxial subluxation?

The causes of atlantoaxial subluxation may be multifactorial, including transverse ligament damage by a periodontoidal proliferative pannus or sequelae of ossification of the anterior and posterior longitudinal ligaments, associated inflammatory lesions (cervical spine osteoarthritis, atlantodental synovitis, erosions …

How is atlantoaxial subluxation treated?

Treatment of Atlantoaxial Subluxation Treatment includes symptomatic measures and cervical immobilization, usually beginning with a rigid cervical collar. Urgency of treatment is generally based on symptoms or presence of cord abnormalities on MRI in susceptible patients.

Which brace is used in atlantoaxial instability?

Initially, these patients are treated with a halo brace; however, if this treatment is unsuccessful because of a fixed deformity or recurrent deformity, posterior fusion of C1-2 is required. Posterior cervical spinal fusion can successfully treat symptomatic AAI in many cases.

What movement occurs at the atlantoaxial joint?

The primary movement of the atlantoaxial joint complex is rotation. In rotation the atlas together with the head, rotate around the dens of axis. This movement allows us to turn our head to look towards the left or towards the right.

Children with Down syndrome are at increased risk of developing compression of the spinal cord called atlantoaxial instability. This problem is caused by a combination of low tone, loose ligaments and bony changes.

What is the AAP’s position on atlantoaxial instability (AAI)?

In 1984, the AAP issued its first position statement on Atlantoaxial Instability (AAI) in children with Down Syndrome (DS): All children with DS who wish to participate in sports should have cervical spine X-rays.

What causes spinal cord instability in Down syndrome?

Atlantoaxial Instability in Children with Down Syndrome Children with Down syndrome are at increased risk of developing compression of the spinal cord called atlantoaxial instability. This problem is caused by a combination of low tone, loose ligaments and bony changes. The spinal cord can be pressed by the bones and cause nerve damage.

What is asymptomatic atlantoaxial dislocation?

Symptomatic AAI results from subluxation (excessive slippage) that is severe enough to injure the spinal cord, or from dislocation at the atlantoaxial joint. “Approximately 15% of youth with Down syndrome have AAI. Almost all are asymptomatic.

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