The Grisel Syndrome: early interdisciplinary treatment needed to prevent severe upper cervical deformity - Experience from a high-volume spine center and systematic review of existing literature.

PURPOSE: Grisel Syndrome (GS) describes a rare, non-traumatic atlantoaxial rotatory subluxation in children typically associated with upper respiratory tract infection, or otorhinolaryngological surgery. The aim of this study was to systematically review the literature regarding interdisciplinary treatment approaches, consequences of delayed or misdiagnosed GS, and upper cervical deformities resulting from misdiagnosed GS. Additionally, we share our institutional experience with this pathology.

METHODS

We queried our institutional database for patients suffering from GS which were treated in our department between 2001 and 2024. A systematic literature search was conducted using Medline and the Cochrane Library, following PRISMA guidelines.

RESULTS

Our database revealed three patients with GS. One patient with misdiagnosed of GS presented with rigid occipital rotation, ultimately progressing to occipito-atlantoaxial rotatory dislocation (OAARD). Two patients were early diagnosed and successfully treated with halo vest immobilization. The systematic literature review identified 13 cases of OAARD across 12 reports. Etiologies included upper respiratory infections, otorhinolaryngological surgery, juvenile idiopathic arthritis, and minor trauma. The average time to diagnosis of OAARD was 5.9 months (0.5-18 months). The treatment involved occipito-atlantal fusion (43 %), atlantoaxial fusion with halo vest (29 %), or traction followed by halo vest (21 %).

CONCLUSION

GS and OAARD are underrecognized and often diagnosed late, causing irreversible deformity and permanent loss of upper cervical spine mobility. Painful torticollis following upper respiratory tract infection, otorhinolaryngological surgery, or minor trauma should be treated as GS until proven otherwise. Early recognition and interdisciplinary treatment are crucial to prevent progression and avoid invasive surgical interventions.