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A treatment for primary ciliary dyskinesia: Efficacy of functional endoscopic sinus surgery
Author(s) -
Parsons David S.,
Greene Bradley A.
Publication year - 1993
Publication title -
the laryngoscope
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.181
H-Index - 148
eISSN - 1531-4995
pISSN - 0023-852X
DOI - 10.1288/00005537-199311000-00010
Subject(s) - medicine , primary ciliary dyskinesia , surgery , functional endoscopic sinus surgery , sinusitis , edema , respiratory tract , incidence (geometry) , pediatrics , anesthesia , respiratory system , lung , bronchiectasis , physics , optics
Primary ciliary dyskinesia (PCD) is an inherited disorder manifested in children as chronic otomastoiditis, recurrent pneumonia, and chronic sinusitis. The failure of the ciliary beat pattern to effectively function in the respiratory tract produces stasis of secretions with secondary inflammation, edema, and infection. The authors report three young children with PCD who presented with variable severities of symptoms. Each had the aforementioned respiratory tract problems. The child with the most severe symptomatology was treated with a variety of medical options, including long‐term gamma globulin injections, but hospitalizations persisted twice per month because of severe sinopulmonary illness. All three of the children underwent functional endoscopic sinus surgery (FESS). In addition, two children received pressure equalization (PE) tubes. One child required a revision procedure. Their surgical outcomes are discussed. Follow‐up of FESS in three children with this disorder shows a marked improvement in symptomatology with a decreased incidence of hospitalization and a somewhat decreased need for medical therapy.