International Journal of Pediatric Otorhinolaryngology
Case reportMontgomery© T-tubes in the management of multilevel airway obstruction in mucopolysaccharidosis
Introduction
The mucopolysaccharidoses (MPS) represent a heterogeneous group of inherited metabolic disorders. The underlying abnormality is a deficiency of one of the hydrolytic enzymes present in the lysosomes, which normally degrade glycosaminoglycans (GAGs) in a stepwise fashion. Deficiency of these enzymes leads to accumulation of partially degraded GAGs within the cells, as they cannot be broken down by alternative methods. The accumulated GAGs affect cell/tissue function and the clinical phenotype is dependent upon the enzyme deficiency involved and the cell type this substrate is most prevalent in [1], [2]. The MPS subtypes are inherited in an autosomal recessive fashion except for MPS II, which is X-linked. Each subtype displays a wide variety of signs and symptoms as well as differing levels of severity [3].
Inherent to a number of the MPS disorders is airway compromise, particularly MPS I (Hurler/Hurler Scheie), MPS II (Hunter), MPS IVA (Morquio), MPS VI (Maroteaux–Lamy) and MPS VII (Sly) [4]. Airway obstruction may be due to adenotonsillar hypertrophy, macroglossia, reduced mouth opening, laryngopharyngeal GAG deposits and tracheobronchomalacia [4]. The airway obstruction is often multilevel and multifactorial, with the accumulation of GAG deposits within the airway leading to progressive airway obstruction [5]. Airway obstruction in MPS is considered by many to represent the most challenging paediatric airway, encompassing the attempt to ameliorate airway obstruction and to safely secure the airway during general anaesthesia [6].
We present the first case series detailing the use of tracheal T-tubes to assist in the management of airway obstruction in children and young people with MPS. Our management of progressive airway obstruction in MPS will be discussed along with the treatment algorithm developed in our tertiary referral centre.
Section snippets
Methods
We undertook a retrospective case note review of all MPS patients requiring a T-tube to assist in the management of airway obstruction at our tertiary Centre. The primary outcome measure for this study was the effectiveness of the T-tube insertion on airway patency and the secondary outcome measures were complications directly attributable to the T-tube and the requirement for further airway surgery.
Discussion
The Montgomery© T-tube was first described in 1964 by the American surgeon Dr William Montgomery. The original T-tube consisted of two pieces of acrylic used to prevent post-operative tracheal stenosis following tracheal surgery [7]. The tube was initially too rigid and evolved into a one-piece silicone tube with three limbs. The tube serves both as a stent and as a tracheostomy tube. The vertical limb resides within the lumen of the trachea and typically extends a variable distance from below
Conclusion
Tracheal T-tubes are a safe, viable, well-tolerated alternative to tracheostomy tubes in MPS patients who require definitive management of multilevel airway obstruction and the tracheostomy tube has failed. Tracheal T-tubes provide several advantages when compared to conventional tracheostomies, including easy phonation, utilisation of the normal heat and moisture exchange mechanism, stenting of the distal airways and improvement in tracheomalacia, with a resultant improvement in quality of
Competing interests
All authors declare no competing interests.
Acknowledgement
This study is dedicated to the memory of Professor Ed Wraith, Consultant in Paediatric Inherited Metabolic Disease, who died suddenly in 2013. Ed was arguably the world’s leading expert in the field of MPS and his death is a tragic loss to this group of patients.
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