Case of the Month 12: Answer

Congrats to Sasi for winning this month’s CotM! Mona had the correct diagnosis in her differential but Sasi’s thorough explanation highlighted a lot of the important considerations we have to have when dealing with pediatric torticollis.

In summary: 7 y/o boy with acquired torticollis x 2 weeks. Preceded by a URI. No reported trauma. Had radiculopathy-like symptoms last week.
Decreased ROM on PE with muscle spasm on L, head tilt to the R, and some cervical LAD. No headaches, fevers, vomiting, or other complaints.

What is your differential dx for this patient?

Potential causes of acquired torticollis in this 7 year old with symptoms for 2 weeks should include traumatic, infectious, and neoplastic etiologies. There is no h/o trauma however ligamentous injury, spinal epidural hematoma, and atlantoaxial rotatory subluxation should be considered as seemingly minor trauma may cause these injuries to occur. The patient reports having URI symptoms and URIs can cause it on their own, but RPA, Lemierre syndrome and cervical pyogenic spondylitis/osteomyelitis are rare but deadly disease processes that need to be always considered. Posterior fossa and cervical soft tissue tumors can present as torticollis, although fossa tumors usually present with some sign of elevated ICP, which this patient lacks. Finally, it could just be simple dystonia or muscle spasm, but the length of the patient’s symptoms is concerning for a more insidious diagnosis.

What should your workup entail?

Labwork is likely to not be helpful in this patient, unless you suspect the patient has a severe infection causing SIRS with evidence on a CBC. NSAIDS and/or muscle relaxants are warranted for any patient with pain or spasm. If their is any suspicion for fracture or subluxation a soft cervical collar at least should be placed. Lateral soft tissue neck and cervical spine plain films will help identify RPA or obvious cervical spine fractures. However, to r/o many infectious or neoplastic etiologies a CT scan needs to be performed. MRI brain to r/o posterior fossa tumor could be considered if all other studies provide no answer and patient is not improving with medical therapy.

So what does he have?

It is difficult to be certain on one diagnosis in this boy from just HPI and PE, but most of the above diagnoses in the differential will not cause radiculopathy unless there is involvement of the nerve roots (and likely the cervical spine). His preceding URI makes infectious etiologies a concern, however he clinically looks well and exam didn’t reveal anything overt. Can we combine our concerns for infection and cervical spine injury into one diagnosis? Yes we can, because this boy has Grisel’s Syndrome, otherwise known as nontraumatic atlantoaxial rotatory subluxation (AARS).

Believe it or not, pharyngeal surgery, inflammation of the neck, pharyngitis, or even a URI have been shown to cause C1-C2 subluxation. It occurs almost exclusively in the pediatric population and largely in school-age children. The current predominating theory is that inflammation or infection in the neck can travel via the vascular system of the ENT area through anastamoses with vessels surrounding the ligaments of C1 and C2. This inflammatory process causes hyperemia and a weakening of the stabilizing ligaments, allowing subluxation to occur. However, most clinicians and researchers will agree that we really don’t know how it occurs, only that these processes are somehow linked to ligamentous instability.

Another clue that we could probably never hang our hat on is which SCM has the muscle spasm. In contrast to muscular torticollis or dystonia, the spasmed SCM is opposite to the head tilt (the chin points toward the tight SCM). Think of it as the opposing SCM is trying to pull the tilted head back into position. CT of the neck will reveal the subluxation.

What is the management?

AARS less than 7 days can be managed with a soft collar, analgesia and rest. The hope is that as inflammation reduces, the ligaments will retighten and spontaneous reduction will occur. However this patient has 2 weeks of symptoms, which more often requires orthopedic consultation for cervical traction in order to allow reduction to occur. If this fails, immobilization with a halo or surgical intervention may need to occur.

References:
Uptodate
Emedicine
Bocciolini, C., et al. Grisel’s syndrome: a rare complication following adenoidectomy. Acta Otorhinolaryngol Ital. 2005 Aug; 25(4): 245–249.
Herman, MJ. Torticollis in infants and children: common and unusual causes. Instr Course Lect. 2006;55:647-53.