Craniovertebral junction reducible and irreducible compressive pathologies: free hands or free tools? Light and shadows
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EDITORIAL
Craniovertebral junction reducible and irreducible compressive pathologies: free hands or free tools? Light and shadows in paediatric practice Massimiliano Visocchi 1
# Springer-Verlag GmbH Germany, part of Springer Nature 2020
In this paper titled Atlantoaxial instability treated with freehand C1–2 fusion in a 3-year old boy with Morquio syndrome, the authors, Eun Ji Moon, MD, Sang Joon Chong, MD, PhD and Jin Hoon Park, MD, PhD describe the case of a just 3-year Morquio old boy presenting with a 5-month history of progressive quadriparesis and severe neck pain [1]. The preoperative cervical imaging showed a compressive myelopathy at C1–2 and atlantoaxial subluxation. Atlantoaxial fixation and decompression were performed successfully with posterior C1–2 fusion using a free-hand technique. After the operation, the patient had improved neck pain and quadriparesis, and finally, he had been able to walk independently after postoperative 8 months. The authors conclude that this is the first report dealing with a paediatric Morquio patient treated just with C1–2 screwing and fusion with a free-hand technique. As far as I realize, the most important topic of this paper is the biochemical considerations on Morquio syndrome, the odontoid malformations, the surgical options and the intraoperative assistance.
Biochemical considerations In this special case, the clinical issue is carried by Morquio syndrome, also known as Mucopolysaccharidosis type IV (MPS IV); as well known, this is a rare metabolic disorder in which the body cannot process certain types of sugar molecules called glycosaminoglycans (AKA GAGs or mucopolysaccharides). In Morquio patients, the specific GAG which builds up in the body is called keratan sulfate. This birth * Massimiliano Visocchi [email protected] 1
Craniovertebral Junction Operative Unit, Fondazione Policlinico Universitario A. Gemelli, Institute of Neurosurgery, Catholic University of Rome, Rome, Italy
defect, which is autosomal recessive, is a type of lysosomal storage disorder. The build-up of GAGs in different parts of the body, along with developmental anomalies, causes symptoms in many different organ systems. In the USA, the incidence rate for Morquio is estimated at between 1 in 200,000 and 1 in 300,000 live births [2, 3].
Odontoid malformations A whole body skeletal survey in Morquio syndrome generally shows skeletal dysplasia with hypoplasia, thoracolumbar kyphosis and dysmorphic, flattened vertebral bodies with short stature, odontoid hypoplasia and atlanto-axial instability are pathognomonic features of Morquio syndrome. Historically, atlantoaxial instability with skeletal dysplasia has been treated by occipitocervical fusion with external orthosis [2, 3]. Considering these patients’ bone size, quality and age in these genetic disorders of skeletal dysplasia, C1–2 fusion is hard to establish stability. For sure, the experience of the Korean neurosurgeons is innovative and represents a new paradigm in this surgery. In Morquio syndrome os odontoideum, not always os
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