Tracheostomy in children with mucopolysaccharidosis: A systematic review

Objectives

Children with mucopolysaccharidosis (MPS) with difficult airways may require tracheostomy, and surgery can be challenging. This review aims to study the indications, surgical aspects, postoperative complications and outcomes of tracheostomy in MPS children.

Methods

A systematic review following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines was conducted. Pubmed and Embase were searched for studies on tracheostomy in children with MPS. The Joanna Briggs Institute was used for quality appraisal.

Results

A total of 9 studies were included. Three were retrospectives, one was prospective, and five were case series. Overall, 34 subjects who underwent a tracheostomy were included. The mean age was 11 years and the male/female ratio was 1.7. MPS II was the most common type included (16/34). The percentage of tracheotomized MPS children was 10 % (ranging from 3.5 % to 15.5 %). Indications for tracheostomy were: progressive or urgent airway obstruction unresponsive to conservative treatments, before a planned surgical procedure, and in case of failed intubation/extubation. Characteristics clinical features make surgery difficult and predispose complications such as tracheal granulations, wound infection, stomal narrowing, persistent of abundant secretions and tracheitis. Considering tracheostomy outcome, two patients underwent decannulation, but one of them required reinsertion tracheostomy after one year. The duration of follow-up was 8.4 and 9.98 years.

Conclusion

Tracheostomy is an effective means of ensuring airway patency in children with MPS and is considered essentially permanent. Management can be challenging due to typical clinical features and progression of MPS. A planned multidisciplinary approach may prevent complications.