What are the surgical causes of respiratory distress in newborns?

Surgical Causes of Respiratory Distress in Newborns

The primary surgical causes of neonatal respiratory distress include congenital diaphragmatic hernia, congenital cystic adenomatoid malformation, congenital lobar emphysema, esophageal atresia with or without tracheoesophageal fistula, and airway obstructive lesions such as bilateral choanal atresia. 1

Mechanism-Based Classification

Surgical causes of respiratory distress operate through three main mechanisms: airway obstruction, pulmonary collapse or displacement, and parenchymal disease or insufficiency. 1

Airway Obstructive Lesions

Bilateral choanal atresia represents a neonatal emergency requiring immediate surgical intervention. 2

• Bilateral choanal atresia occurs in 1 in 7,000-8,000 live births with female predominance (2:1) and presents as 40% bilateral, 60% unilateral cases. 2
• Newborns are obligate nasal breathers during the first 3-4 weeks of life, making bilateral atresia life-threatening immediately after birth. 2
• These infants present with periodic respiratory distress and cyanosis that is relieved by crying, along with severe feeding problems and aspiration. 2
• Immediate airway management requires oropharyngeal airway intubation within the first hours of life before planning surgical correction within the first days of life. 2
• Diagnosis is established by inability to pass a catheter through either nostril into the pharynx, confirmed by endoscopic examination and high-resolution CT scan. 2
• The atretic plate is either purely bony (30%) or mixed bony/membranous (70%), with 50% having associated congenital abnormalities (up to 75% in bilateral cases). 2
• CHARGE malformation is the most frequent syndromal association (20% of cases), requiring two of the following: Coloboma, Heart defects, Choanal Atresia, Retardation, Genito-urinary abnormalities, and Ear defects. 2

Other airway obstructive lesions include macroglossia, Pierre-Robin syndrome, lymphangioma, teratoma, mediastinal masses or cysts, subglottic stenosis, and laryngotracheomalacia. 1

Pulmonary Collapse or Displacement

Congenital diaphragmatic hernia (CDH) is a critical surgical emergency that should not be treated with surfactant, as it worsens outcomes. 2

• CDH causes respiratory distress through pulmonary hypoplasia and displacement of abdominal contents into the thoracic cavity. 1, 3
• Surfactant treatment in CDH patients did not improve outcomes and actually increased the need for ECMO, incidence of chronic lung disease, and mortality. 2
• Management requires immediate surgical intervention with multidisciplinary critical care support. 3
• CDH patients are susceptible to stress-induced persistent pulmonary hypertension (PPHN), particularly after surgical intervention. 4

Esophageal atresia with or without tracheoesophageal fistula represents another non-pulmonary cause requiring urgent surgical management. 1, 3

Parenchymal Disease or Insufficiency

Congenital cystic adenomatoid malformation (CCAM) requires prompt lobectomy, as mechanical ventilation causes clinical deterioration due to air trapping within cystic spaces. 1, 4

• CCAM may be diagnosed on fetal ultrasound and presents with respiratory distress at birth. 4
• Intubation and mechanical ventilation worsen the condition by causing air trapping, necessitating immediate surgical intervention. 4
• Patients with CCAM can develop PPHN postoperatively (4 hours to 5 days after lobectomy), requiring ECMO support in severe cases. 4
• The clinical course mirrors CDH patients, where surgical stress combined with hypoxia and hypercarbia stimulates hyperactive pulmonary vasculature leading to PPHN. 4

Congenital lobar emphysema also causes respiratory distress through parenchymal insufficiency and requires surgical management. 1

Clinical Recognition and Imaging

• Imaging plays a major role in preoperative diagnosis of surgical causes of respiratory distress. 1
• Chest radiography is the initial diagnostic tool, with specific findings directing further evaluation. 1
• Proper preoperative resuscitation dramatically improves surgical outcomes. 1

Critical Pitfalls to Avoid

• Do not administer surfactant to infants with congenital diaphragmatic hernia, as this increases mortality, ECMO requirements, and chronic lung disease. 2
• Do not delay surgical intervention in bilateral choanal atresia—these infants require immediate airway management within hours of birth. 2
• Do not mechanically ventilate CCAM patients without planning immediate surgery, as ventilation worsens air trapping and clinical status. 4
• Recognize that surgical causes may develop PPHN postoperatively, requiring readiness for ECMO support. 4