Atlantoaxial rotatory fixation.

OBJECTIVE: Atlantoaxial rotatory fixation (AARF) remains a recondite entity. Our normative study using CT motion analysis shows that there is a high degree of concordance for rotational behavior of C1 and C2 in children 0 to 18 years. C1 always crosses C2 at or near 0 degree. The predictable relationship between C1 and C2 is depicted by 3 distinct regions on the motion curve: when C1 rotates from 0 to 23 degrees, it moves alone while C2 remains stationary at 0 (the single-motion phase). When C1 rotates from 24 to 65 degrees, C1 and C2 move together (the double-motion phase), but C1 always moves faster as C2 is being pulled by yoking ligaments. From 65 degrees onward, C1 and C2 move in unison (the unison-motion phase) with a fixed, maximal separation angle of approximately 43 degrees, the head rotation being carried exclusively by the subaxial segments. Because of this high concordance among patients and a relatively narrow variance from the mean, the physiological composite motion curve can be used as a normal template for the diagnosis and classification of AARF.
METHODS: Using a 3-position CT protocol to obtain the diagnostic motion curve, we identified 3 distinct types of AARF. Type I AARF patients show essentially unaltered ("locked") C1-C2 coupling regardless of corrective counterrotation, with curves that are horizontal lines in the upper 2 quadrants of the template. Type II AARF patients show reduction of the C1-C2 separation angle with forced correction, but C1 cannot be made to cross C2. Their curves slope downward from the right to left upper quadrants but never traverse the x axis. Type III AARF patients show C1-C2 crossover but only when the head is cranked far to the opposite side. Their motion curves traverse the x axis far left of 0 degree (C1 < -20). Thus, type I, II, and III AARF are in descending degrees of pathological stickiness. A fourth group of patients showing motion curve features between normal and type III AARF are designated as belonging to a diagnostic gray zone (DGZ). The AARF patients are further classified as acute if treatment is started less than 1 month from the onset of symptoms, as subacute if the delay in treatment is 1 to 3 months, and chronic if treatment delay exceeds 3 months. The treatment protocol for AARF consists of reduction using either halter or caliper traction and then immobilization with brace or halo, depending on the AARF type and chronicity. Recurrent slippage and irreducibility are treated with C1-C2 fusion.
RESULTS: The treatment course and outcome of AARF are analyzed according to the AARF type and chronicity. The difficulty and duration of treatment, the number of recurrent slippage, the rate of irreducibility, the need for halo and fusion, and the percentage ultimately losing normal C1-C2 rotation are significantly greater in type I patients than type III patients, with type II patients somewhere in between. Likewise, all parameters are much worse in patients with any type of chronic AARF than acute AARF. The worse subgroup is chronic type I versus the best subgroup of acute type III. Recurrent AARF patients do much worse than nonrecurrent AARF patients. Recurrence is, in turn, adversely influenced by both the severity (type) and chronicity of AARF. The symptoms of most DGZ patients will resolve with analgesics, but a few remain symptomatic or deteriorate to true AARF requiring the full treatment.
CONCLUSION: Thus, children with painful torticollis should undergo the 3-position CT protocol not only to confirm the diagnosis of AARF but also to grade its severity. Closed reduction with traction should be instituted immediately to avoid the serious consequences of chronicity. Proper typing and reckoning of the pretreatment delay are requisites for selecting treatment modalities. Recurrent dislocation and incomplete reduction should be treated with posterior C1-C2 fusion in the best achievable alignment.