Constriction band at the craniocervical junction in Maroteaux-Lamy syndrome

Abstract

A 13-year-old girl with a history of mucopolysaccharidosis (MPS) type VI (Maroteaux-Lamy syndrome) presented to our clinic with a progressive history of spastic quadriparesis after a suboccipital craniectomy and C1 laminectomy for bony decompression of the craniocervical junction and concurrent occipitocervical fusion. Magnetic resonance imaging (MRI) workup is shown in Fig. 1. She underwent a re-do surgery, including intradural exploration to lyse a meningeal constriction band around the cervicomedullary junction (Fig. 2). Spinal cord compression is one of the major clinical complications of Maroteaux-Lamy syndrome [1–3]. Abnormal storage of glycoaminoglycans occurs in the dura mater, meninges, and supporting ligaments. In cases such as the present report, we recommend decompression (both extradural and intradural) and concurrent occipitocervical fusion (or, in cases of unfavorable bony cervical anatomy, occipitocervicothoracic fusion) with spinal instrumentation.