SURGICAL TREATMENT OF CRANIOVERTEBRAL STENOSIS IN TWO SIBLINGS WITH TYPE VI MUCOPOLYSACCHARIDOSIS

TRATAMENTO CIRÚRGICO DE ESTENOSE CRANEO-VERTEBRAL EM DOIS IRMÃOS COM MUCOPOLISACAREDOSE TIPO VI

TRATAMIENTO QUIRÚRGICO DE ESTENOSIS CRANEO-VERTEBRAL EN DOS HERMANOS CON MUCOPOLISACARIDOSIS TIPO VI

ABSTRACT

We describe two cases of surgical treatment of craniovertebral stenosis in preschool-aged brothers with Maroteaux-Lamy (MPS type VI) syndrome. The older brother was diagnosed with MPS during her second pregnancy. Literature describing familial cases of the disease and the treatment strategy in young children with MPS type VI and spinal canal stenosis is scarce. Based on the presented observations, indications, surgical treatment approaches, and perioperative management of patients with mucopolysac-charidosis are suggested. MPS type VI may have familial forms of the disease and the course of craniovertebral stenosis is similar in siblings. Surgical treatment of craniovertebral stenosis in these patients should be performed timely. We adhere to the point of view of early treatment of craniovertebral stenosis in patients with MPS before irreversible spinal cord dysfunction develops. Level of Evidence IV; Prognostic Studies - Investigating the Effect of a Patient Characteristic on the Outcome of Disease and Case series.

Keywords:
Vertebrobasilar Insufficiency; Mucopolysaccharidosis VI; Perioperative Care; Surgical Procedure