Complications of tracheotomy in patients with mucopolysaccharidoses type II (Hunter syndrome)

Han-Sin Jeong1, Do-Yeon Cho, Kang Mo Ahn

  • 1Department of Otorhinolaryngology-Head and Neck Surgery, Sungkyunkwan University School of Medicine, Samsung Medical Center, 50 Irwon-dong, Gangnam-gu, Seoul 135-710, Republic of Korea. hansin.jeong@samsung.com

Abstract

Insights

Tracheotomy in patients with mucopolysaccharidosis (MPS) type II (Hunter syndrome) frequently leads to complications like airway stenosis. Careful consideration and discussion of risks with caregivers are essential before such procedures.

Area of Science:

  • Medical research
  • Surgical complications
  • Rare genetic disorders

Background:

  • Mucopolysaccharidosis type II (Hunter syndrome) is a rare genetic disorder affecting multiple organs.
  • Airway management is a critical concern in patients with MPS type II due to anatomical challenges.
  • Tracheotomy is sometimes necessary for airway management in these patients.

Purpose of the Study:

  • To evaluate the complication rate of tracheotomy in patients diagnosed with mucopolysaccharidosis type II (Hunter syndrome).

Main Methods:

  • Analysis of seven tracheotomy procedures performed between 2004 and 2005 in three MPS type II patients.
  • Assessment of specific complications including stomal narrowing, granulation, infrastomal tracheal stenosis, and wound infection.

Main Results:

  • All tracheotomies in MPS type II patients resulted in complications, despite securing a safe airway.
  • Infrastomal tracheal stenosis (85.7%) and stomal narrowing (71.4%) were the most common complications.
  • These complications complicated cannula care and frequently necessitated revision.

Conclusions:

  • Infrastomal tracheal stenosis and stomal narrowing are frequent complications following tracheotomy in MPS type II patients.
  • Tracheotomy should be approached with caution in MPS type II patients.
  • Preoperative discussion of potential tracheotomy complications with caregivers is crucial.

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