What are the surgical causes of respiratory distress in newborns, including additional causes under each category?

Surgical Causes of Respiratory Distress in Newborns: Expanded Categories

Surgical causes of neonatal respiratory distress are organized by underlying mechanism: airway obstruction, pulmonary collapse/displacement, and parenchymal disease, with each category containing multiple specific conditions requiring distinct surgical approaches. 1, 2

Airway Obstructive Lesions

Nasal and Nasopharyngeal Obstruction

Bilateral Choanal Atresia

• Occurs in 1 in 7,000-8,000 live births with 2:1 female predominance, presenting as 40% bilateral and 60% unilateral cases 1
• Life-threatening immediately after birth because newborns are obligate nasal breathers during the first 3-4 weeks of life 1
• Presents with periodic respiratory distress and cyanosis that is relieved by crying (pathognomonic sign), along with severe feeding problems and aspiration 1
• 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) 1
• 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 1
• Diagnosis is established by inability to pass a catheter through either nostril into the pharynx, confirmed by endoscopic examination and high-resolution CT scan 1
• Immediate airway management requires oropharyngeal airway or intubation within the first hours of life before planning surgical correction within the first days of life 1

Congenital Nasal Pyriform Aperture Stenosis

• A rare cause of upper airway obstruction that can be symptomatic in variable degrees depending on the severity of obstruction 3
• Potential syndromic character with association of other malformations, such as single median incisor or intracranial midline anomalies 3
• Systematically requires cerebral MRI when diagnosed to evaluate for associated intracranial midline anomalies 3
• Treatment ranges from conservative management to surgical intervention depending on clinical response 3

Oropharyngeal and Tongue Obstruction

Macroglossia

• Enlarged tongue causing mechanical airway obstruction, commonly seen in Beckwith-Wiedemann syndrome, Down syndrome, or isolated congenital macroglossia 2
• Causes positional airway obstruction that worsens in supine position 2

Pierre-Robin Sequence

• Triad of micrognathia (small mandible), glossoptosis (posterior displacement of tongue), and cleft palate 2
• The posteriorly displaced tongue falls back and obstructs the pharyngeal airway 2
• Requires prone positioning initially; severe cases may need nasopharyngeal airway, tongue-lip adhesion, or mandibular distraction osteogenesis 2

Neck Masses and Mediastinal Lesions

Lymphangioma (Cystic Hygroma)

• Congenital malformation of lymphatic vessels causing compressive masses in the neck or mediastinum 2
• Can cause acute airway compromise through direct compression or hemorrhage into the lesion 2

Teratoma

• Germ cell tumors that can arise in the neck, mediastinum, or oropharynx 2
• Cervical teratomas can cause severe airway obstruction requiring ex utero intrapartum treatment (EXIT) procedure at delivery 2

Other Mediastinal Masses and Cysts

• Include bronchogenic cysts, enteric duplication cysts, and thymic masses 2
• Cause respiratory distress through tracheal or bronchial compression 2

Laryngeal and Tracheal Lesions

Subglottic Stenosis

• Narrowing of the airway below the vocal cords, either congenital or acquired 2
• Presents with biphasic stridor and respiratory distress that worsens with agitation 2

Laryngotracheomalacia

• Abnormal collapse of laryngeal or tracheal structures during respiration due to cartilage weakness 2
• Most common cause of stridor in infants; majority self-resolve by 18-24 months 4
• 90% of patients with tracheomalacia, bronchomalacia, or tracheobronchomalacia improve with time alone 4
• Severe cases may require aortopexy or direct tracheobronchopexy 4
• β-agonists may adversely affect airway dynamics in these children and should be avoided 4

Vascular Rings and Slings

• Congenital vascular anomalies that encircle and compress the trachea and esophagus 4
• Include double aortic arch, right aortic arch with aberrant left subclavian artery, and pulmonary artery sling 4
• Unlike tracheomalacia, these are unlikely to self-resolve and require surgical correction for symptomatic patients 4
• 88-100% of patients improve with surgery, with complete resolution in more than 50% 4
• Recurrent laryngeal nerve injury is the most common surgical complication, occurring in less than 10% of patients 4

Pulmonary Collapse or Displacement

Congenital Diaphragmatic Hernia (CDH)

• Herniation of abdominal contents into the thoracic cavity through a diaphragmatic defect, causing pulmonary hypoplasia and compression 2
• Left-sided defects (Bochdalek hernia) are most common (85-90% of cases) 2
• Presents with severe respiratory distress immediately after birth, scaphoid abdomen, and displaced heart sounds 2
• CRITICAL PITFALL: Do not administer surfactant to infants with CDH, as this increases mortality, ECMO requirements, and chronic lung disease 1, 4
• Measurements of disaturated phosphatidylcholine from lungs of infants with CDH show altered pool sizes and kinetics despite normal synthetic rates 4
• Requires immediate intubation, nasogastric decompression, and surgical repair after stabilization 2

Pneumothorax and Air Leak Syndromes

• Accumulation of air in the pleural space causing lung collapse 5, 6
• Can occur spontaneously (1-2% of term newborns) or secondary to mechanical ventilation 5
• Presents with sudden deterioration, decreased breath sounds, and shift of cardiac impulse 6
• Requires needle decompression followed by chest tube placement in symptomatic cases 6

Parenchymal Disease or Insufficiency

Congenital Cystic Adenomatoid Malformation (CCAM)

• Developmental abnormality resulting in cystic and adenomatoid lung tissue that does not communicate with the bronchial tree 2
• Can cause respiratory distress through mass effect, mediastinal shift, and compression of normal lung tissue 2
• Classified into five types based on cyst size and histology 2
• May present with recurrent infections or pneumothorax 2
• Requires surgical resection of affected lobe 2

Congenital Lobar Emphysema

• Overdistension of a pulmonary lobe due to bronchial obstruction or cartilage deficiency 2
• Most commonly affects the left upper lobe (42%), followed by right middle lobe (35%) 2
• Causes progressive respiratory distress as the affected lobe hyperinflates and compresses adjacent lung tissue 2
• Radiographically shows hyperlucent, hyperexpanded lobe with mediastinal shift 2
• Requires lobectomy in symptomatic cases 2

Pulmonary Sequestration

• Non-functioning lung tissue with anomalous systemic arterial blood supply that does not communicate with the tracheobronchial tree 2
• Intralobar sequestration (75%) shares pleural covering with normal lung; extralobar sequestration (25%) has separate pleural covering 2
• Can present with recurrent infections, heart failure from left-to-right shunting, or respiratory distress from mass effect 2

Associated Conditions Requiring Surgical Consideration

Esophageal Atresia with or without Tracheoesophageal Fistula (TEF)

• Congenital interruption of esophageal continuity, with 85% having distal TEF 2
• Presents with excessive oral secretions, choking with first feeding, and inability to pass nasogastric tube 2
• When TEF is present, gastric distension from air entering through the fistula can cause respiratory compromise 2
• Requires immediate recognition, head-up positioning, continuous suction of upper pouch, and surgical repair 2

Diagnostic Approach

Imaging plays a major role in pre-operative diagnosis of surgical causes of respiratory distress 2:

• Chest radiography is the initial imaging modality for all newborns with respiratory distress 6
• High-resolution CT scan is essential for defining anatomic details of airway obstruction, vascular anomalies, and parenchymal lesions 1, 2
• Flexible fiberoptic bronchoscopy with airway survey identifies anatomic abnormalities in approximately 33% of patients with respiratory symptoms 4
• Bronchoscopy is particularly valuable when respiratory distress persists despite treatment with bronchodilators, inhaled corticosteroids, or systemic corticosteroids 4

Critical Management Principles

• Proper pre-operative resuscitation dramatically improves surgical results 2
• Immediate airway management takes priority in obstructive lesions, particularly bilateral choanal atresia requiring intervention within hours of birth 1
• Distinguish between self-limited conditions (tracheomalacia) and those requiring surgical intervention (vascular rings) to avoid unnecessary treatments 4
• Approximately 30% of patients with respiratory symptoms benefit from airway survey through either direct surgical intervention or avoiding unnecessary tests for benign, self-limited conditions 4