| Pfeiffer syndrome: a treatment evaluation. | |
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MedLine Citation:
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PMID: 19407629 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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Jeffrey A Fearon; Jennifer Rhodes |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Plastic and reconstructive surgery Volume: 123 ISSN: 1529-4242 ISO Abbreviation: Plast. Reconstr. Surg. Publication Date: 2009 May |
Date Detail:
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Created Date: 2009-05-01 Completed Date: 2009-06-09 Revised Date: 2011-02-16 |
Medline Journal Info:
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Nlm Unique ID: 1306050 Medline TA: Plast Reconstr Surg Country: United States |
Other Details:
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Languages: eng Pagination: 1560-9 Citation Subset: AIM; IM |
Affiliation:
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Craniofacial Center, Medical City Children's Hospital, Dallas, Texas, USA. cranio700@aol.com |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Acrocephalosyndactylia
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mortality*,
therapy* Adolescent Adult Child Child, Preschool Humans Infant Retrospective Studies Young Adult |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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