Distinctive Spine Deformities in Patients with Hurler (IH) and Hurler-Scheie (I-H/S) Syndrome
Journal Title: Journal of Orthopaedic Science and Research - Year 2021, Vol 2, Issue 3
Abstract
Purpose: Progressive kyphoscoliosis is not of uncommon occurrence in patients with MPSs. Cranio-cervical junction in patients with MPSs are under the threat of three life threatening elements, namely GAGs accumulation, C1-2 instability, and progressive cervical vascular abnormalities. Material and Methods: Seven patients’ two girls and five boys with age range from 3 to 9 years presented with progressive kyphoscoliosis and atlanto-axial instability. Phenotype/genotype confirmed the diagnosis of Hurler syndrome and Hurler-Scheie syndrome. Though, spine deformities were to a certain extent similar in both types but with different age of onset. Results: Children with kyphoscoliosis of apical Cobb’s angle ranging between 60/65° were corrected up to 5° with normal sagittal spine balance. All showed an improvement in the neurological and functional status of Frankel motor scale (PreOp - C / PostOp - D) and Nurick scale (PreOp - 2-3 / PostOp - 2-3). The severity of myelopathy on the mJOA scale decreased (PreOp - 12 / PostOp - 10). Three children were excluded from surgical interventions because their contrast- enhanced computed tomography angiography of the cervical and cerebral arteries showed three hazardous abnormalities. Two children showed variable coiling and kinking of the vertebral and the basilar arteries resulting in an exaggerated redundancy which is compatible with the diagnosis of dolichoarteriopathy. Third child showed progressive narrowing of the left subclavian artery. Conclusion: The method of spine operations in children with Hurler and Hurler-Scheie syndromes depend on the age of the child, the site and type of spine malformation and the proper assessment of any associated cervical/cerebral malformation via contrast- enhanced computed tomography angiography. Patients were operated on, via the correction of kyphoscoliosis with the 5.5 trans pedicular system. Patients with atlanto-axial instability underwent decompression at the C0-C2 or C0-C3 level and occipito spondylodesis by costal autograft accordingly.
Authors and Affiliations
Sergey Ryabykh, Polina Ochirova, Mohammad Shboul, Alexander Gubin, Alexander Burtsev, Marat Saifutdinov, Susanne Gerit Kircher, Ali Al Kaissi
Keywords
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- EP ID EP698827
- DOI http://dx.doi.org/10.46889/JOSR.2021.2304
- Views 78
- Downloads 0
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