Airway Management of the Deformed Trachea Using T-Tube Stents in Patients with Mucopolysaccharidosis Type IVA

Lee, Yi-Hao MD
Hsieh, Li-Chun MD, PhD
Su, Chin-Hui MD, PhD
Lin, Hsiang-Yu MD, PhD
Lin, Shuan-Pei MD, PhD
Lee, Kuo-Sheng MD

¹Department of Otorhinolaryngology—Head and Neck Surgery, MacKay Memorial Hospital, Taipei
²Department of Audiology and Speech-Language Pathology, MacKay Medical College, New Taipei City
³School of Medicine, Taipei Medical University, Taipei
⁴Department of Medicine, MacKay Medical College, New Taipei City
⁵Department of Pediatrics, MacKay Memorial Hospital, Taipei
⁶MacKay Junior College of Medicine, Nursing and Management, Taipei
⁷Department of Infant and Child Care, National Taipei University of Nursing and Health Sciences, Taipei

Kuo-Sheng Lee, MD, Department of Otorhinolaryngology—Head and Neck Surgery, MacKay Memorial Hospital, No. 92, Sec. 2, Zhongshan N. Road, Taipei 10449. Email: [email protected]

Annals of Otology, Rhinology & Laryngology 131(5):p 562-566, May 2022. | DOI: 10.1177/00034894211032778

Introduction:

Mucopolysaccharidosis (MPS) type IVA usually results in airway obstruction due to thoracic cage deformity and crowding of intrathoracic structures, causing tracheal compression by the tortuous innominate artery.

Objectives:

To offer an alternative and effective method in dealing with the challenged deformity of the airway in patients with MPS type IVA.

Methods:

We present 3 patients with MPS type IVA who underwent airway stenting using Montgomery^® T-tube stents. Three-dimensional reconstructed computed tomography was essential to design the T-tube and evaluate the anatomical relationship between the innominate artery and the trachea. The Y-shaped Montgomery^® Pediatric Safe-T-Tube™ is more suitable for MPS type IVA. Regular follow-ups using fiberoptic bronchoscopy are necessary to evaluate the complications.

Results:

All 3 patients had good outcomes during the follow-ups until present, despite the complication of granulation formation, which was resolved by revising the limbs of the T-tube.

Conclusions:

T-tube stents placed below the vocal cord may restore airway patency and preserve laryngeal function, including respiration, phonation, and swallowing, in patients with MPS type IVA.