sirolimus and Airway-Obstruction

Head and neck lymphatic malformations can create airway management challenges requiring tracheotomy. Sirolimus, an inhibitor of mammalian target of rapamycin (mTOR), may inhibit growth of lymphatic malformations. We describe two patients born with large lymphatic malformations with improved airway symptoms following sirolimus therapy. Patient #1 underwent tracheotomy and multi-modal therapy including sirolimus with reduction in airway involvement but regrowth after discontinuation of sirolimus. Patient #2 also experienced a significant response to sirolimus allowing for extubation and discharge without tracheotomy. Early initiation of sirolimus therapy should be considered as a means to avoid tracheotomy in complex head and neck lymphatic malformations.

Topics: Airway Obstruction; Combined Modality Therapy; Female; Head; Humans; Immunosuppressive Agents; Infant; Infant, Newborn; Lymphatic Abnormalities; Male; Neck; Recurrence; Sclerotherapy; Sirolimus; Tracheotomy

The aim of this study was to evaluate the use of balloon-expandable metallic stents in the treatment of children with congenital tracheal stenosis in whom conventional therapy has failed. From 2010 to 2011, balloon-expandable metallic stents were implanted into the trachea of eight infants aged 2-20 months in the paediatric intensive care unit. The infants had severe airway obstruction caused by congenital tracheal stenosis. Tracheal stents were placed after intraluminal balloon dilatation of the tracheal stenosis, inserted with balloon catheters and implanted into the desired position bronchoscopically. The stents were 12 to 29 mm long and 4 mm in diameter. Seven children were relieved of airway obstruction after this procedure. However, a child died due to severe sepsis after the placement of bronchial stents. No granulation tissue developed over the stents in any of the children. Stents have been in place for 1-6 months after insertion without any other complication. Balloon-expandable metallic stents are effective in relieving airway obstruction by congenital tracheal stenosis in children. This technique may provide an important remedy for congenital tracheal stenosis in children.

Topics: Airway Obstruction; Bronchoscopy; Catheterization; China; Drug-Eluting Stents; Humans; Infant; Intensive Care Units, Pediatric; Metals; Prosthesis Design; Sirolimus; Time Factors; Tracheal Stenosis; Treatment Outcome

Topics: Airway Obstruction; C-Reactive Protein; Calcineurin Inhibitors; Dose-Response Relationship, Drug; Female; Humans; Hypoproteinemia; Immunosuppressive Agents; Lung Transplantation; Middle Aged; Pleural Effusion; Sirolimus; Tomography, X-Ray Computed; Treatment Outcome

Topics: Airway Obstruction; Animals; Bronchiolitis Obliterans; Cricetinae; Cyclosporine; Immunosuppressive Agents; Isoxazoles; Leflunomide; Mesocricetus; Polyenes; Rats; Rats, Inbred Lew; Sirolimus; Trachea; Transplantation, Heterologous