Foramen magnum stenosis in patients with mucopolysaccharidoses: diagnosis and surgical treatment. Review of literature

Abstract

Cervico-medullary compression and atlantoaxial dislocation syndromes are the dominant clinical manifestations of mucopolysaccharidoses at the craniovertebral level. The review provides an analysis of international literary sources concerning modern aspects of diagnosis and neurosurgical correction of the foramen magnum stenosis in patients with different types of mucopolysaccharidoses. The existing surgical approaches to determining the indications and choosing the method of surgical treatment and some aspects of the use of enzyme replacement therapy and hematopoietic stem cell transplantation are presented. A variety of options for surgical correction of pathology at the craniovertebral level have been demonstrated in patients with different types of mucopolysaccharidosis, however the described recommendations can be considered from the point of view of traditions of the hospitals or personal experience of surgeons, but not as recognized standards of treatment this pathology. Further accumulation of individual observations or clinical series is required to conduct a comparative analysis of the effectiveness of various approaches on a sufficiently large and homogeneous material to determine standards for the diagnosis and treatment of craniovertebral junction pathology in patients with mucopolysaccharidoses.