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Case 012: Infant with Stridor and Feeding Difficulty

Presentation

A 4-month-old female is referred for persistent stridor and feeding difficulties. Parents report noisy breathing since birth that worsens with feeds and when lying flat. She has had two episodes of "choking" during bottle feeds.

Birth History: Term, uncomplicated delivery, no NICU stay

Vital Signs: - Heart rate: 130 bpm - Respiratory rate: 40 - SpO2: 97% on room air

Exam: - Inspiratory stridor, worse when supine - No intercostal retractions - Clear lungs - No murmur - Normal perfusion

Initial Workup

Chest X-ray: - Right-sided aortic arch - Normal heart size - No infiltrates

Barium swallow: - Posterior indentation of esophagus

Clinical Reasoning

Question 1: What is the significance of right aortic arch on CXR?

Answer **Right aortic arch suggests vascular ring** Normal: Left aortic arch Right arch associations: - Vascular ring (double aortic arch, right arch with aberrant left subclavian) - TOF (right arch in ~25%) - Truncus arteriosus - May be isolated In symptomatic infant with stridor + posterior esophageal compression + right arch → **suspect vascular ring**

Question 2: What is the differential for vascular rings?

Answer **Complete vascular rings (encircle trachea/esophagus):** 1. **Double aortic arch** (most common complete ring) 2. **Right aortic arch with aberrant left subclavian and left ligamentum** **Incomplete rings (partial compression):** 1. **Innominate artery compression** (most common cause of anterior tracheal compression) 2. **Pulmonary artery sling** (left PA from right PA - compresses right mainstem) 3. **Aberrant right subclavian** (from left arch - posterior compression only, usually asymptomatic)

Question 3: What imaging confirms the diagnosis?

Answer **Gold standard: CT angiography or MR angiography** CT advantages: - Faster (no sedation as long) - Excellent spatial resolution - Shows airway compression MRA advantages: - No radiation - Excellent for soft tissue **Both show:** - Arch anatomy - Relationship to trachea/esophagus - Associated anomalies **Bronchoscopy:** - Can visualize tracheal compression - Pulsatile compression = vascular

Imaging Results

CT Angiography findings: - Double aortic arch with dominant right arch - Left arch smaller but patent - Tracheal compression at level of arches - Esophageal compression between arches - No intracardiac anomaly

Question 4: What is the treatment?

Answer **Surgical division of vascular ring** For double aortic arch: - Divide the smaller (usually left) arch - Divide ligamentum arteriosum - Relieves compression **Approach:** Left thoracotomy or VATS (video-assisted thoracoscopic surgery) **Timing:** - Symptomatic patients: Repair promptly - Severe symptoms (apnea, significant feeding difficulty): Urgent repair - This patient: Semi-urgent given significant symptoms **Cardiopulmonary bypass usually NOT required** (unless associated intracardiac defect)

Question 5: What is the post-operative course?

Answer **Expected recovery:** **Immediate:** - Stridor may persist initially (tracheomalacia from chronic compression) - Usually improves over weeks to months as trachea remodels **Complications to watch for:** - Residual tracheomalacia (may need observation, rarely tracheostomy) - Recurrent laryngeal nerve injury (hoarseness) - Chylothorax (thoracic duct injury) **Long-term:** - Generally excellent outcomes - Stridor typically resolves by 6-12 months post-op - Most have no long-term restrictions

Teaching Points

  1. Right aortic arch on CXR + stridor = suspect vascular ring
  2. Double aortic arch is most common complete ring
  3. CT or MR angiography is diagnostic
  4. Surgical division is curative
  5. Tracheomalacia may cause persistent symptoms initially post-op
  6. Does not require cardiopulmonary bypass in most cases