Surgical considerations in cystic fibrosis: what every general surgeon needs to know
John D Chetwood1,2, Anastasia Volovets1,2, Sheila Sivam3
1AW Morrow Gastroenterology and Liver Centre, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia.
Insights
Cystic fibrosis (CF) patients are living longer and facing new surgical challenges. This review updates general surgeons on managing CF-related gastrointestinal conditions in these complex patients.
Area of Science:
- Gastroenterology
- Surgical Oncology
- Pulmonology
Background:
- Cystic fibrosis (CF) is a multisystem disorder impacting the gastrointestinal tract.
- Improved therapies increase CF patient lifespan to ~50 years.
- Longer lifespans lead to increased surgical comorbidities in CF patients.
Purpose of the Study:
- To provide general surgeons with an update on surgical issues in cystic fibrosis patients.
- To highlight the unique challenges in managing CF patients with surgical conditions.
- To prepare surgeons for multidisciplinary care of complex CF patients.
Main Methods:
- This is a review article.
- It synthesizes current knowledge on CF-related surgical conditions.
- Focuses on gastrointestinal manifestations and management.
Main Results:
- CF patients present with surgical issues like distal intestinal obstruction syndrome and colorectal cancer.
- Management requires understanding CF-specific challenges: immunosuppression, respiratory compromise, multi-organ dysfunction.
- Surgical care for CF patients is evolving.
Conclusions:
- General surgeons will increasingly manage CF patients with surgical conditions.
- A thorough understanding of CF pathophysiology is crucial for effective surgical care.
- Multidisciplinary team involvement is essential for optimal outcomes in CF surgical patients.
Abstract:
Cystic fibrosis (CF) is a complex multiorgan disease, which often affects the gastrointestinal tract. With improved CF specific therapies and multidisciplinary management, patients with CF are now living longer with a median life expectancy of around 50 years. This increased life expectancy has resulted in corresponding increase in presentations of the CF patient with comorbid surgical conditions that were never important considerations. Investigations and management of these conditions, such as distal intestinal obstruction syndrome and colorectal cancer warrant good clinical understanding of the unique challenges that CF patients present including chronic immunosuppression, impaired respiratory function and their multi-organ dysfunction. The purpose of this review is to provide general surgeons with a contemporary update on the CF related surgical issues as they are likely to become increasingly involved in the care of these complex patients and form an integral part of the multidisciplinary team.
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