Diffuse idiopathic skeletal hyperostosis: A functional enemy of vertebral stability - Case series and surgical consideration of craniovertebral junction involvement

Giuseppe R Giammalva; Rosario Maugeri; Luigi M Cusimano; Andrea S Sciortino; Lapo Bonosi; Lara Brunasso; Roberta Costanzo; Francesco Signorelli; Silvana Tumbiolo; Domenico G Iacopino; Massimiliano Visocchi

doi:10.4103/jcvjs.jcvjs_72_23

Diffuse idiopathic skeletal hyperostosis: A functional enemy of vertebral stability - Case series and surgical consideration of craniovertebral junction involvement

J Craniovertebr Junction Spine. 2023 Jul-Sep;14(3):274-280. doi: 10.4103/jcvjs.jcvjs_72_23. Epub 2023 Sep 18.

Affiliations

¹ Department of Biomedicine Neurosciences and Advanced Diagnostics, Unit of Neurosurgery, University of Palermo, Palermo, Italy.
² Department of Neurosurgery, Fondazione Policlinico Universitario "A. Gemelli" IRCCS, Catholic University of the Sacred Heart, Rome, Italy.
³ Department of Neurosurgery, Villa Sofia Hospital, Palermo, Unit of Neurosurgery, Hospital "Villa Sofia", Palermo, Italy.

Abstract

Context: Diffuse idiopathic skeletal hyperostosis (DISH) or Forestier's syndrome may reduce vertebral mobility, thus affecting the stability of adjacent vertebral segments and promoting spinal stenosis, vertebral dislocation, and unstable fracture secondary to low-energy trauma.

Aims: This study aimed to contribute with a case series of three patients affected by DISH undergone surgery with occipitocervical fixation for craniovertebral junction (CVJ) instability since the poor literature about CVJ instability and surgery in patients affected by DISH.

Settings and design: This was a multicentric case series.

Subjects and methods: Literature about CVJ instability and surgery in patients affected by DISH is poor. Thus, we present a case series of three patients affected by DISH, who underwent surgery with occipitocervical fixation with different clinical and radiological patterns.

Results: CVJ represents one of the most mobile joints of the spine and is at greater risk for instability. Moreover, instability itself may act as primum movens for several degenerative conditions such as cervical spondylosis, ossification of the posterior longitudinal ligament, and cervical deformities. On the contrary, DISH itself may worsen CVJ instability because of subaxial spine stiffness. In case of DISH, the rigid unit formed by several ossified vertebral bodies acts as a long lever arm, increasing the forces applied to the hypermobile CVJ and reducing the dynamic buffer capability of ossified spine. On the other hand, vertebral instability increases the odds of fractures. In such cases, CVJ posterior instrumentation and fusion is an effective and feasible surgical technique, aimed to restore vertebral stability and to halt the progression of spinal stenosis.

Conclusions: Due to the altered dynamics cervical spine along with the possible comorbidities, treatment indication and surgery for patients affected by DISH must be tailored case by case.

Keywords: Craniovertebral junction; diffuse idiopathic skeletal hyperostosis; instability; occipitocervical fixation.