2002 OITE Question 175

Question

A 16-year-old girl was treated as an infant for torticollis. She has not been seen for follow-up since age 2 years. She is now completely asymptomatic but has returned for evaluation because here family recalls being told that she might need cervical fusion when she becomes a teenager. Figures 48a and 48b show cervical flexion-extension lateral radiographs. What is the most likely diagnosis?


1- Normal cervical spine


2- Occiptoatlantal instability


3- Atlantoaxial instability


4- Klippel-Feil syndrome but no instability


5- Subaxial instability

Figures

Answer (click for answer)

Answer – 4


The differential diagnosis of torticollis is extensive and can be broken down into congenital and acquired etiologies. Congenital torticollis may be further subdivided into occipitocervical anomalies and soft tissue conditions. This patient has Klippel-Feil syndrome with the not infrequent pattern of occipitalization of the atlas in conjunction with a C2 to C3 fusion. These patients are at a high risk for instability at the C1-C2 level and need to be followed carefully. Although these radiographs are difficult to interpret, there is no evidence of instability. The occiput and atlas are fused, so there can be no instability. The atlanto-dens interval (ADI) is maintained in both flexion and extension, so that articulation is stable. The spinolaminar line as well as the alignment of the anterior borders of the vertebral bodies is well maintained, so there is no subaxial instability.

Reference: OKU – Spine