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Updated: Dec 13, 2025
High-speed Video Microscopy Analysis for First-line Diagnosis of Primary Ciliary Dyskinesia
Published on: January 19, 2022
Pediatric unilobar resection in primary ciliary dyskinesia
Carlos S Sendon1, Robert A Cowles2, David J Worhunsky2
1Section of Pulmonary, Allergy, Immunology, and Sleep Medicine, Department of Pediatrics, Yale University, New Haven, CT, USA.
Insights
Surgical lung resection can improve quality of life for children with Primary Ciliary Dyskinesia (PCD) and bronchiectasis when medical treatments fail. This approach helps improve growth, nutrition, and lung function in select cases.
Area of Science:
- Pulmonology
- Pediatric Surgery
- Genetics
Background:
- Primary ciliary dyskinesia (PCD) leads to chronic lung infections and progressive bronchiectasis.
- Current PCD management focuses on symptom control, with no cure or regenerative options.
- Surgical lung resection (lobectomy) is cautiously recommended only for rare, severe, localized bronchiectasis cases.
Purpose of the Study:
- To evaluate the efficacy of selective surgical lung resection in children with PCD-associated bronchiectasis unresponsive to medical therapy.
- To demonstrate potential improvements in quality of life, growth, nutritional status, and pulmonary function.
Main Methods:
- Retrospective analysis of children with PCD and localized, medically refractory bronchiectasis.
- Inclusion of selective surgical removal of severely defective lung segments.
- Administration of prophylactic antibiotics alongside surgical intervention.
Main Results:
- Selective lung resection in selected cases of unilobar PCD-associated bronchiectasis improved quality of life.
- Patients experienced enhanced growth and nutritional status post-surgery.
- Pulmonary function showed improvement or maintenance after surgical intervention.
Conclusions:
- Surgical lung resection can be a viable option for select children with PCD and localized bronchiectasis when other therapies are insufficient.
- This intervention may lead to significant improvements in overall health outcomes for affected children.
- Careful patient selection and discussion are crucial before considering lung resection for PCD.
Abstract:
Primary ciliary dyskinesia (PCD) causes chronic infections and progressive bronchiectasis that can lead to severe lung disease. Because there are no cures or regenerative therapy options for PCD, treatment of severe lung disease in PCD is focused on managing symptoms, including aggressive administration of antibiotics and diligent airway clearance. The Genetic Disorders of Mucociliary Clearance Consortium (GDMCC) does not recommend routine lobectomy, reserving its use for "rare cases of PCD with severe, localized bronchiectasis" and warns that a lobectomy should be treated with caution. However, if aggressive medical management fails, selective surgical removal of severely defective lung may result in maintenance or improvement of pulmonary function. Certainly, the decision to recommend lung resection in the face of chronic bronchiectasis from PCD requires an extensive discussion before it is considered as an alternative treatment. The purpose of this manuscript was to demonstrate that in selected cases of unilobar disease with bronchiectasis that are not responsive to other therapies (antibiotics and airway clearance), removal of localized necrotic areas of the lung along with prophylactic antibiotics can improve the quality of life of children with PCD associated bronchiectasis and improve growth and nutritional status, and pulmonary function.
