Immediate Management of Profuse Tracheostomy Bleeding in a Child with Treacher-Collins Syndrome
Insert a cuffed endotracheal tube through the tracheostomy stoma with the tip positioned distal to the bleeding source, or hyperinflate the existing tracheostomy cuff if present, to control profuse bleeding from a suspected tracheoinnominate fistula. 1, 2
Recognition of Life-Threatening Hemorrhage
- Profuse bleeding from a tracheostomy stoma should immediately raise suspicion for tracheoinnominate fistula, which is frequently fatal if not managed emergently 1, 2
- A sentinel bleed precedes catastrophic hemorrhage in approximately 50% of cases, making any significant bleeding a critical warning sign 1, 2
- Moderate bleeding from the stomal site or visible pulsation of the tracheostomy tube are specific indicators of tracheoinnominate fistula 1, 2
Immediate Airway Control and Hemorrhage Management
The priority is simultaneous airway control and direct compression of the bleeding source:
- If a cuffed tracheostomy tube is already in place, hyperinflate the cuff immediately to tamponade the fistula from within the trachea 1, 2
- If the current tube is uncuffed or inadequate, remove it and insert a cuffed endotracheal tube through the stoma, advancing the tip distal to the suspected fistula site (typically at the level of the innominate artery) 1, 2
- The cuffed tube provides direct tamponade of the bleeding vessel against the tracheal wall 1, 2
Alternative Compression Techniques
If immediate tube placement is not possible or unsuccessful:
- Apply digital pressure directly through the stoma to compress the innominate artery against the posterior sternum 1, 2
- This can be performed by inserting a gloved finger through the stoma and applying anterior pressure 1, 2
- Digital pressure to the innominate artery site externally may also be attempted 1, 2
Concurrent Resuscitation
- Initiate standard hemorrhagic shock resuscitation protocols simultaneously with airway management, including large-bore IV access, blood product administration, and hemodynamic support 1, 2
- Summon immediate surgical and anesthesia assistance for definitive operative management 1, 2
Definitive Management
- Emergency surgical exploration with division and ligation of both ends of the innominate artery is the definitive treatment and must be performed urgently once the airway is secured and bleeding is temporarily controlled 2
- This approach provides immediate hemorrhage control and eliminates rebleeding risk 2
Critical Pitfalls in Treacher-Collins Syndrome
Children with Treacher-Collins syndrome present unique airway challenges that complicate emergency management:
- These patients have severe craniofacial abnormalities including mandibular hypoplasia, making oral intubation extremely difficult or impossible 3, 4, 5
- Attempting oral intubation in this population during a bleeding emergency wastes critical time and is likely to fail 3, 4
- Treacher-Collins patients with tracheostomies are at risk for intratracheal granuloma formation from chronic cannulation, which can complicate stoma access 6
- The tracheostomy stoma is the only reliable airway access in these patients during emergencies 3, 4, 5
Why Other Options Are Incorrect
- Inserting a cuffed tracheostomy tube (option a) may be appropriate if immediately available, but a cuffed endotracheal tube is more universally accessible and achieves the same goal 1, 2
- Oral endotracheal intubation (option b) is contraindicated in Treacher-Collins syndrome due to severe mandibulofacial hypoplasia making laryngoscopy nearly impossible, and wastes critical time during life-threatening hemorrhage 3, 4, 5
- Applying a peristomal dressing (option c) is completely inadequate for profuse bleeding from tracheoinnominate fistula and will result in exsanguination 1, 2