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Minerva Pediatrica 2020 Jul 29

DOI: 10.23736/S0026-4946.20.05802-8

Copyright © 2020 EDIZIONI MINERVA MEDICA

language: English

Pediatric unilobar resection in primary ciliary dyskinesia

Carlos S. SENDON 1, Robert A. COWLES 2, David J. WORHUNSKY 2, Daniel HODSON 1, Raffaella MOROTTI 3, Alia BAZZY-ASAAD 1, Americo E. ESQUIBIES 4

1 Section of Pulmonary, Allergy and Immunology, and Sleep Medicine, Department of Pediatrics, Yale University, New Haven, CT, USA; 2 Section of Pediatric Surgery, Department of Surgery, Yale University, New Haven, CT, USA; 3 Section of Pediatric Pathology, Department of Pathology, Yale University, New Haven, CT, USA; 4 Division of Pediatric Pulmonology, Department of Pediatrics, SUNY Downstate Medical Center, Brooklyn, NY, USA


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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 is 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.


KEY WORDS: Ciliary motility disorders; Bronchiectasis; Spirometry

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