AIR-11
Uncommon Comorbid Congenital Airway and Pulmonary Anomalies
Jahangiri M, Willer B, Titler S
University of Iowa, Iowa City, Iowa, United states
1.8 kg female with known left sided congenital diaphragmatic hernia (CDH) at 32 6/7 weeks gestation was apneic and bradycardic upon delivery. Immediate intubation and ventilation attempts were unsuccessful. Bag-mask ventilation improved oxyhemoglobin saturations to 90% and heart rate to >100 bpm. Patient was transferred to the operating room for direct laryngoscopy and rigid bronchoscopy. A Miller 0 laryngoscope used by Otolaryngology revealed widely splayed arytenoids and complete opening of the larynx posteriorly into the esophagus. A 3.0 mm rigid bronchoscope advanced into the trachea confirmed contiguous communication between trachea and esophagus distal to the carina. While chest compressions were initiated for bradycardia, the scope was exchanged for a 2.5 mm rigid bronchoscope and advanced into the right bronchus. An attempt to ventilate the right lung returned minimal CO2. Given the extent of multiple severe disease processes in a premature neonate, the decision was made to stop resuscitation. The patient was pronounced dead approximately three hours after delivery. Autopsy revealed a type IV laryngotracheoesophageal cleft (LTEC), left-sided congenital diaphragmatic hernia and severe pulmonary hypoplasia.
Congenital diaphragmatic hernia is the result of disordered embryogenesis and failure of fusion of elements that give rise to the diaphragm. Despite recent surgical and ventilator related advances, the mortality rate for CDH remains approximately 50%; mostly due to co-morbid cardiac, renal, or bowel anomalies (1). To our knowledge, only one prior case report of a patient with CDH having a co-morbid high grade LTEC exists (2).
Laryngotracheoesophageal clefts are congenital midline defects of the posterior larynx and trachea, resulting in an abnormal communication between the larynx and hypopharynx (3). The clinical features of patients with LTECs vary based on the severity. Shorter clefts present with cough, stridor, hoarse cry, and feeding difficulties, while longer clefts result in frequent and severe aspirations, infections, and respiratory failure.
Securing an airway and establishing ventilation in patients with LTECs is challenging. Their existence or extent is often unknown at birth, leaving the anesthesiologist little time to gather required equipment and develop a plan to secure the airway. Additionally, the endotracheal tube can be dislodged into the esophagus resulting in inadequate ventilation. Finally, ventilation can be complicated by aspiration pneumonia, need for one-lung ventilation, or ventilation via custom made bifurcated endotracheal tubes. Therefore, it is important for pediatric anesthesiologists to be familiar with challenges patients with uncommon comorbid airway and pulmonary anomalies may present.
1. McHoney M. Congenital diaphragmatic hernia, management in the newborn. Pediatr Surg Int. 2015; 31:1005-13.
2. Ryan A, et al. Congenital diaphragmatic hernia: associated malformations – cystic adenomatoid malformation, extralobular sequestration, and laryngotracheoesophageal cleft: two case reports.
3. Dwarakanath S, et al. Unexpected presentation of a type IV laryngotrachoesophageal cleft: anesthetic implications of a rare case. Indian J Anaesth 2014;58:746-8.
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