Surgery for craniocervical instability and spinal cord compression in children with Mucopolysaccharidosis type IV: A systematic review.

INTRODUCTION: Mucopolysaccharidosis type IV (MPS IV) often leads to instability and spinal cord compression at the craniocervical junction in children, confronting surgeons with technical challenges associated with young age. RESEARCH QUESTION: This systematic review examines the effects of age at surgery and type of fixation on achieving craniocervical bony fusion and the impact of decompression and solid fusion on neurological outcomes. MATERIAL AND METHODS: We systematically searched PubMed/MEDLINE, Embase, Web of Science, Scopus and Cochrane Library for cohorts reporting on 'Craniocervical instability', 'Spinal cord compression' and 'Mucopolysaccharidosis type IV' up till April 27, 2024. Inclusion criteria were MPS IV, craniocervical instability treatment and age ≤ 16 years. RESULTS: Eighteen studies were included, comprising 114 patients with a median age at surgery of 72.0 months (range: 17.0-191.0). A total of 88.6% (n = 101/114) of patients underwent craniocervical junction surgery, with rigid fixation used in 17.8% (n = 13/73) and decompression performed in 63.8% (n = 44/69) of cases. Craniocervical bony fusion was achieved in 93.8% (n = 91/97) of patients. Rigid fixation was less often used in children younger than three years (20.0%, n = 2/10), but fusion rate remained as high as 77.8% (n = 7/9). No statistically significant association between fixation type and bony fusion was detected (OR = 2.44, 95% CI 0.24-330.60, p = 0.51). There was a trend for neurological outcome to be better after performing decompression (OR = 4.48, 95% CI 0.87-23.26, p = 0.07). DISCUSSION AND CONCLUSION: Although rigid fixation was less often applied in children below the age of three, fusion and neurological outcomes were reasonably favorable. There was a trend toward better neurological outcome in patients undergoing decompression.