Long-term survival after Cranio-cervical disjuction in a Ankylosing spondylitis patient: A case report and review of the literature

Author(s): Inverso M, Borruto MI, Zirio G, Genitiempo M, Velluto C, Mazzella GG, Ingusci D, Proietti L, Scaramuzzo L.

Introduction: Ankylosing spondylitis (AS) is a chronic inflammatory disease affecting the axial skeleton, leading to progressive ossification and spinal rigidity. These biomechanical alterations render patients highly susceptible to spinal fractures, even from low-energy trauma. Among these, cranio-cervical disjunction (CCD) is an extremely rare and often fatal injury. This case report describes the unprecedented survival of a 52-yearold male with AS who sustained multiple vertebral fractures, including a CCD, after a high-speed motorcycle accident. The case highlights the clinical complexity and importance of early multidisciplinary intervention.

Materials and Methods: We conducted a systematic review of Englishlanguage literature indexed in PubMed and Google Scholar using the search terms “ankylosing spondylitis” AND “spine fractures” OR “atlantooccipital dislocation.” Of 238 records, eight studies met inclusion criteria: case reports or series involving AS patients with CCD or multiple vertebral fractures. Data on demographics, neurological status, and survival were extracted and compared to our case.

Discussion: Our patient presented with life-threatening injuries, including CCD, bilateral pneumothorax, vertebral artery bleeding, and multiple spinal fractures. A comprehensive and aggressive management strategy was implemented: early airway control, targeted resuscitation, damagecontrol spinal fixation, vertebral artery embolization, and staged definitive surgery. Despite severe complications such as infections, pressure ulcers, thrombosis, and cranial nerve palsy, the patient survived for seven months—markedly longer than any other reported case of CCD in AS patients. Literature analysis revealed that survival beyond 7 days in such cases is exceedingly rare, with most patients succumbing within a week regardless of the number or location of fractures, or neurological status.

Conclusion: This case illustrates that prolonged survival after CCD in AS is possible through immediate, multidisciplinary intervention and sustained intensive care. It challenges existing notions of prognosis in AS-related spinal trauma and emphasizes the need for standardized protocols. Future research should explore whether aggressive, staged treatment strategies can systematically improve outcomes in this high-risk population.