Case report of endoprosthesis -Y implantation in severe respiratory failure in the MPSII patient; comparison with literature data

Springer Science and Business Media LLC - Tập 20 Số 1 - 2020
Wojciech Gocyk1, Janusz Warmus1, H Olechnowicz1, Mirosław Bik-Multanowski2, Łukasz Pawliński3, Beata Kieć‐Wilk3
1Department of Thoracic Surgery, John Paul II Hospital Krakow, Krakow, Poland
2Clinical Department of Medical Genetics, Children University Hospital, Krakow, Poland
3Clinical Department of Metabolic Diseases, University Hospital, Krakow, Poland

Tóm tắt

Abstract Background The tracheobronchomalacia is a life-threatening complication of mucopolysaccharidosis (MPS) without known effective, optimal treatment. The severe expiratory collapse of the trachea and bronchi is one of causes of the high rate of deaths in the course of airway impairment in MPSII patients. Case presentation Due to the adynamic tracheobronchomalacia despite of enzymatic treatment (ERT) in our MPSII patient, a life-saving tracheal bifurcated type-Y endoprosthesis (a self-expanding, metal stent for the prosthesis of tracheal and bronchial stenosis) was implanted. In the followed months, the breathing efficiency improved, but then gradual worsening, progression of bronchi occlusion at the stent border resulted in patient’s death. Conclusion The Y-stent implantation appears to be a short-term, life-saving solution without satisfactory long-term effects due to the progress of peripheral bronchomalacia and increased tissue proliferation and granulation, that arises during the illness’ course.

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