A treatment for primary ciliary dyskinesia: efficacy of functional endoscopic sinus surgery.

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.