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Published on: October 23, 2018
Anaesthesia in patients with cystic fibrosis
1Istituto di Anestesiologia e Rianimazione, University of Rome 'La Sapienza', Rome, Italy. giorgio.dellarocca@uniroma1.it
Insights
Cystic fibrosis (CF) patients require specialized surgical care due to respiratory complications. Anesthesia is safe for minor surgeries in CF patients, with careful preoperative preparation crucial for minimizing risks.
Area of Science:
- Medical Genetics
- Pulmonology
- Anesthesiology
Background:
- Cystic fibrosis (CF) is an autosomal-recessive genetic disorder affecting multiple organs, primarily the lungs.
- CF affects approximately 1 in 2000 children, with a majority now surviving into adulthood.
- The increasing lifespan of CF patients leads to a higher prevalence of surgical interventions.
Purpose of the Study:
- To review the anesthetic considerations for patients with cystic fibrosis undergoing surgery.
- To highlight the importance of preoperative assessment and perioperative care in managing CF patients.
- To discuss the implications of advanced treatments like lung transplantation for CF anesthesia.
Main Methods:
- Review of preoperative assessment protocols for cystic fibrosis patients.
- Emphasis on strategies for optimal clearance of respiratory secretions.
- Discussion of pre-, intra-, and postoperative management plans, including physiotherapy and medical therapies.
Main Results:
- Anesthesia for minor surgical procedures in CF patients can be performed safely with a low incidence of postoperative respiratory complications.
- Comprehensive preoperative preparation, including physiotherapy and targeted medical treatments, is essential.
- Lung transplantation has significantly improved outcomes for end-stage CF patients, presenting unique anesthetic challenges.
Conclusions:
- Careful planning and management are critical for successful surgical outcomes in cystic fibrosis patients.
- Anesthesiologists play a vital role in optimizing care and minimizing risks for this patient population.
- Advances in treatment, including transplantation, offer improved prognoses but require specialized anesthetic approaches.
Abstract:
Cystic fibrosis is an autosomal-recessive disorder. In 1989 the gene mutation that causes cystic fibrosis was localized on the long arm of chromosome 7. Cystic fibrosis occurs in 1/2000 children and the majority now reach adulthood. In view of numerous clinical manifestations of cystic fibrosis, these patients frequently require surgery. Cystic fibrosis is therefore of increasing interest to anaesthesiologists. Preoperative assessment is reviewed. Pre-, intra- and postoperative care must be directed toward optimal clearance of viscous respiratory secretions, and should minimize the risk of postoperative respiratory complications. All procedures should be planned but it is very important to prepare patients for surgery, with daily physiotherapy, administration of therapeutic agents using aerosols, management of nutrition and pancreatic enzymes, and administration of vitamins and antibiotics if indicated. Currently, anaesthesia can safely be carried out in cystic fibrosis patients undergoing minor surgery, with very low incidence of postoperative respiratory complications. Finally, organ transplantation, and in particular lung transplantation, with all its attendant anaesthesiological implications, has improved the outcome for many patients with cystic fibrosis.
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