Tracheobronchial stents in mucopolysaccharidosis.
Int J Pediatr Otorhinolaryngol
; 83: 187-92, 2016 Apr.
Article
en En
| MEDLINE
| ID: mdl-26968075
ABSTRACT
INTRODUCTION:
The mucopolysaccharidoses are a group of hereditary disorders pathologically characterized by tissue accumulation of glycosaminoglycans due to deficient lysosomal metabolism which often leads to progressive airway stenosis and respiratory insufficiency. Relentless and treatment-refractory narrowing of the lower airways with ensuing severe limitation of quality of life is a challenging problem in mucopolysaccharidoses. CASE REPORTS We report 2 cases of MPS (Hunter's and Maroteaux-Lamy's syndrome resp.) in whom tracheal stents were placed to relieve severe tracheal obstruction. The first patient could be weaned from mechanical ventilation after stent placement but showed significant long-term stent-related morbidity. The second patient suffered a severe procedure-related complication due to positioning problems typical for MPS.CONCLUSIONS:
Very good short-term success can be achieved with airway stent placement in patients with MPS and severe lower airway stenosis but a high risk of severe complications and important long-term morbidity have to be weighed against potential individual benefit.Palabras clave
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Estenosis Traqueal
/
Traqueostomía
/
Stents
/
Mucopolisacaridosis II
/
Mucopolisacaridosis VI
/
Obstrucción de las Vías Aéreas
Tipo de estudio:
Etiology_studies
Aspecto:
Patient_preference
Límite:
Adolescent
/
Child
/
Humans
/
Male
Idioma:
En
Revista:
Int J Pediatr Otorhinolaryngol
Año:
2016
Tipo del documento:
Article