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2017 ; 33
(ä): 33-39
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Management of Life-Threatening Tracheal Stenosis and Tracheomalacia in Patients
with Mucopolysaccharidoses
#MMPMID27450368
Kampmann C
; Wiethoff CM
; Huth RG
; Staatz G
; Mengel E
; Beck M
; Gehring S
; Mewes T
; Abu-Tair T
JIMD Rep
2017[]; 33
(ä): 33-39
PMID27450368
show ga
Several different lysosomal storage diseases, mainly mucopolysaccharidosis (MPS)
type I, II, and VI, are complicated by severe obstruction of the upper airways,
tracheobronchial malacia, and/or stenosis of the lower airways. Although enzyme
replacement therapies (ERTs) are available, the impact of these on
tracheobronchial alterations has not been reported. By extending the life
expectancy of MPS patients with ERTs, airway problems may become more prevalent
at advanced ages. These airway abnormalities can result in severe, potentially
fatal, difficulties during anesthetic procedures. Usually, upper airway
obstruction is treated by tracheostomy. However, with lower airway malacia and/or
stenosis, there are no procedures available to date to address these
difficulties. We report the first cases using a new technique of tracheal
stenting in patients with MPS type VI (Maroteaux-Lamy syndrome) and type II
(Hunter syndrome) who had almost complete tracheal occlusion and total airway
collapse. An updated literature review is also reported.