Obstructive sleep apnea in apert’s and pfeiffer’s syndromes: More than a craniofacial abnormality

Roger C. Mixter*, David J. David, William H. Perloff, Christopher G. Green, Richard M. Pauli, Peter M. Popic

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

69 Citations (Scopus)

Abstract

Nine acrocephalosyndactyly type I patients (Apert’s syndrome) and three acrocephalosyndactyly type V patients (Pfeiffer’s syndrome) were evaluated for the relative importance of upper and lower airway abnormalities in the generation of obstructive sleep apnea. All patients were found to have a combination of upper and lower abnormalities. The influence of lower pathology was greater in the infants, and the influence of upper airway, specifically pharyngeal, was greater in the adults. A com-parison between preoperative and postoperative poly-somnography revealed little improvement with standard craniofacial advancements. Furthermore, three patients are described who succumbed to pulmonary death despite tracheostomy. Conservative treatment with prone or lateral positioning and medical pulmonary regimens is advocated. Finally, the pathogenesis of this diffuse airway pathology is discussed.

Original languageEnglish
Pages (from-to)457-463
Number of pages7
JournalPlastic and Reconstructive Surgery
Volume86
Issue number3
Publication statusPublished - 1990
Externally publishedYes

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