Postobstructive pulmonary oedema during anaesthesia in children with mucopolysaccharidoses

R W M Walker1, V Colovic, D N Robinson

  • 1Department of Anaesthetisia, Royal Manchester Children's Hospital, Pendlebury, Manchester, UK.

Insights

Patients with severe mucopolysaccharidoses have a high risk of postobstructive pulmonary edema during anesthesia. Anesthesia management requires careful consideration due to this predisposition.

Area of Science:

  • Anesthesiology
  • Medical Genetics
  • Pulmonology

Background:

  • Mucopolysaccharidoses are rare genetic disorders affecting multiple organ systems.
  • Patients with mucopolysaccharidoses often present complex anesthetic challenges.
  • Pulmonary complications are a significant concern in these patients.

Observation:

  • Five cases of severe mucopolysaccharidoses patients developing postobstructive pulmonary edema during anesthesia are reported.
  • These patients exhibited unique physiological characteristics complicating airway management.
  • The onset of pulmonary edema was observed post-anesthetic airway manipulation.

Findings:

  • Severe mucopolysaccharidoses predispose patients to postobstructive pulmonary edema.
  • Anesthetic management in these patients carries a heightened risk of airway obstruction.
  • Prompt recognition and management of airway obstruction are crucial to prevent pulmonary complications.

Implications:

  • Anesthesiologists must be aware of the increased risk of pulmonary edema in mucopolysaccharidoses patients.
  • Specialized anesthetic techniques and vigilant monitoring are necessary for safe airway management.
  • Further research into anesthetic protocols for mucopolysaccharidoses is warranted to improve patient outcomes.

Related Concept Videos

Breathing01:05

Breathing

The process of breathing, inhaling and exhaling, involves the coordinated movement of the chest wall, the lungs, and the muscles that move them. Two muscle groups with important roles in breathing are the diaphragm, located directly below the lungs, and the intercostal muscles, which lie between the ribs. When the diaphragm contracts, it moves downward, increasing the volume of the thoracic cavity and creating more room for the lungs to expand. When the intercostal muscles contract, the ribs...
Upper Respiratory Drugs: Antitussives, Expectorants, and Mucolytics01:23

Upper Respiratory Drugs: Antitussives, Expectorants, and Mucolytics

Respiratory symptoms, such as congestion and cough, commonly accompany respiratory tract conditions. Various medications, such as antitussives, expectorants, and mucolytics, play crucial roles in providing relief.
Antitussives include codeine, dextromethorphan (Robitussin), and benzonatate (Tessalon). Codeine and dextromethorphan exert their effects centrally by suppressing the cough reflex center in the medulla.  Benzonatate operates peripherally within the respiratory tract by anesthetizing...
Chronic Obstructive Pulmonary Disease II: Emphysema01:23

Chronic Obstructive Pulmonary Disease II: Emphysema

Emphysema, a major phenotype of chronic obstructive pulmonary disease (COPD), is characterized by irreversible destruction of alveolar walls and permanent enlargement of distal airspaces. Unlike chronic bronchitis, which primarily affects the airways, emphysema predominantly involves the lung parenchyma, where structural damage leads to airflow limitation.PathophysiologyIt most commonly results from prolonged exposure to cigarette smoke and other toxic gases, particularly cigarette smoke.
Pulmonary Edema II: Pathophysiology01:18

Pulmonary Edema II: Pathophysiology

Pulmonary edema is the accumulation of fluid in the interstitial and alveolar spaces of the lungs, impairing gas exchange and oxygen delivery. It may be cardiogenic or noncardiogenic, but both reduce oxygenation and lung compliance.Cardiogenic Pulmonary EdemaCardiogenic edema results from increased hydrostatic pressure in pulmonary capillaries, usually due to left ventricular dysfunction from myocardial infarction, heart failure, or valvular disease. Ineffective cardiac pumping causes blood to...