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| Research article summary (published 29 Apr 2009): |
Pfeiffer syndrome: a treatment evaluation.
Full Abstract
BACKGROUND: Pfeiffer syndrome is rarely encountered, even at major craniofacial centers. Published reports indicate high mortality rates (25 to 85 percent) for severely affected subtypes. The authors reviewed their surgically treated patients to improve outcomes. METHODS: The authors conducted a 17-year, single-center, retrospective outcome assessment of all children treated for Pfeiffer syndrome, with data summarized using descriptive statistics. RESULTS: Of 802 patients treated for craniosynostosis, 28 were identified with Pfeiffer syndrome: 17 were classified as type I (61 percent), seven were classified as type II (25 percent), and four were classified as type III (14 percent). The mean age was 10 years (range, 12 months to 39 years), with an average of 9.3 operations per child (2.5 cranial vaults, 1.1 Le Fort III procedures). Fifty-nine percent had external auditory canal atresia (100 percent of type III patients), and 29 percent had some visual disturbance. Tracheostomies were recommended in 100 percent of type II and III patients, and two type II patients required tracheal stenosis repairs. Eighty-four percent had acquired Chiari malformations (100 percent of type II and III patients), and 61 percent required treatment for hydrocephalus. Fifty percent of shunted patients (mean age, 7 years) have required Chiari decompressions, but no patients undergoing endoscopic third ventriculostomies (mean age, <3 years) have required treatment. The mortality rate was 7 percent, with both deaths occurring at home without proximity to surgery. CONCLUSIONS: The authors' mortality rates for type II and III Pfeiffer syndrome are lower than those previously published. The authors believe a preemptory tarsorrhaphy strategy can prevent visual loss and that further reductions in mortality rates are possible with aggressive airway management (early tracheostomies) and more frequent screening (e.g., magnetic resonance imaging, sleep studies) for Chiari malformations.
Author information
Author/s: Fearon, Jeffrey A (JA); Rhodes, Jennifer (J);
Affiliation: Craniofacial Center, Medical City Children's Hospital, Dallas, Texas, USA. cranio700(-atsign-)aol.com
Journal and publication information
Publication Type: Journal Article
Journal: Plastic and reconstructive surgery (Plast Reconstr Surg), published in United States. (Language: eng)
Reference: 2009-May; vol 123 (issue 5) : pp 1560-9
Dates: Created 2009/05/01; Completed 2009/06/09;
PMID: 19407629, status: MEDLINE (last retrieval date: 6/9/2009, IMS Date: 09 Jun 2009 00:00:00)
Sourced from the National Library of Medicine. Abstract text and other information may be subject to copyright.
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