What is the treatment approach for trachea (windpipe) deformity in newborns?

Management of Tracheal Deformity in Newborns

The management of tracheal deformity in newborns requires a multidisciplinary approach with diagnosis via flexible bronchoscopy followed by targeted interventions based on the specific type and severity of the deformity, with tracheostomy being necessary for severe cases that cannot be managed with less invasive approaches. 1

Types and Diagnosis

• Tracheal deformities in newborns commonly present as tracheomalacia (excessive collapse of the trachea due to structural anomalies of tracheal cartilage or posterior membrane) or tracheomegaly (abnormal widening of the trachea) 1
• Flexible bronchoscopy is the gold standard for diagnosis, allowing direct visualization of the airway and assessment of the severity of collapse or deformity 1
• Tracheomalacia was found in 45% and bronchomalacia in 34% of infants with chronic lung disease undergoing flexible bronchoscopy 1
• Acquired tracheomegaly has been documented in very preterm neonates (birth weight less than 1,000g) following mechanical ventilatory support 2

Clinical Presentation

• Infants with tracheal deformities may be asymptomatic at rest or present with:
  • Homophonous wheezing often unresponsive to bronchodilators 1
  • Increased wheezing with expiratory effort 1
  • Cyanotic spells ("BPD spells") 1
  • Stridor 1
  • Recurrent respiratory infections 1
  • Difficulty with secretion clearance 1

Treatment Approach

Non-surgical Management

• Oxygen supplementation should be provided to maintain oxygen saturations above 92% while asleep for infants with tracheal deformities causing hypoxemia 1
• Careful monitoring during sleep is essential as episodes of desaturation are more common during REM sleep 1
• Positive end-expiratory pressure (PEEP) should be used for ventilated infants to assist in establishing functional residual capacity 1
• Avoid high peak pressures during mechanical ventilation as these can worsen tracheal deformation; strategies aimed at limiting peak pressures or minimizing mean airway pressures are recommended 1
• β-adrenergic agents such as racemic epinephrine may provide temporary relief for patients with mild to moderate subglottic stenosis who experience acute symptom exacerbation with upper respiratory infections 1

Surgical Interventions

• Tracheostomy is indicated when other means of correcting the obstruction have been ruled out 1

  • In neonates, a vertical tracheotomy is preferred rather than a cartilage window to avoid creating stenosis 1, 3
  • Maturation sutures should be used to accelerate stoma healing 1, 3
  • Stay sutures should be placed on either side of the vertical tracheostomy to aid opening of the lumen in an emergency 1

• For severe tracheomalacia:

  • Tracheostomy tube placement alone or with continuous positive airway pressure may be effective 1
  • Elongated tracheostomy tubes can be used to stent the distal trachea 1
  • Aortopexy (surgical lifting of the aorta and suturing it to the posterior surface of the sternum) may be considered for vascular compression of the trachea 4
  • External tracheal splints or expandable intraluminal metallic stents have been used for distal tracheomalacia and bronchomalacia 1

• For subglottic stenosis:

  • An anterior cricoid split may allow widening of the subglottic space without tracheostomy 1
  • If anterior cricoid split fails, tracheostomy is necessary followed by gradual surgical correction through techniques like laryngotracheal reconstruction 1

• For fixed airway obstruction:

  • Open tube resection of granulation tissue 1
  • Balloon dilation under direct visualization or fluoroscopic guidance 1
  • Electroresection for distal tracheal and bronchial stenoses 1

Special Considerations for Neonatal Tracheostomy

• Tracheostomy tubes in neonates are generally uncuffed and do not have an inner tube to avoid reducing the internal diameter of the tracheostomy tube lumen 1
• Neonatal tracheostomy tubes are shorter in length than pediatric tubes 1
• Cuffed tubes are occasionally required if high ventilation pressures are needed or if there is high risk of aspiration 1
• The procedure should be performed as a planned operation rather than an emergency whenever possible 1
• Silicone rubber tracheostomy tubes have been shown to reduce problems with stomal granulation 5

Monitoring and Follow-up

• Continuous monitoring of heart rate, oxygen saturation, blood pressure, and temperature is essential during any airway procedure 1
• Regular assessment of the airway via flexible bronchoscopy may be necessary to evaluate treatment effectiveness and disease progression 1
• Families should be counseled that respiratory infections often result in the need for increased respiratory support 1
• Speech development may be delayed in children requiring tracheostomy, necessitating specialized care and monitoring 1

Complications and Pitfalls

• Pressure-induced deformation can occur at pressures commonly used in supporting infants with respiratory insufficiency 1
• The magnitude of pressure-induced deformation is directly related to the compliance of the airway and inversely related to age 1
• Tracheostomy in neonates carries risks of:
  • Pirulent tracheitis 5
  • Interstitial thyroid hemorrhage 5
  • Pneumothorax 5
  • Subcutaneous emphysema 5
  • Postoperative bleeding 5
• Avoid passage of suction catheters beyond the distal tip of artificial airways to prevent mucosal damage 1
• Use negative pressures below 50-80 cm H₂O during suctioning to prevent mucosal damage 1