Pierre Robin Sequence : Incidence of Speech-Correcting Surgeries and Fistula Formation

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Gustafsson , C , Vuola , P , Leikola , J & Heliövaara , A 2020 , ' Pierre Robin Sequence : Incidence of Speech-Correcting Surgeries and Fistula Formation ' , Cleft Palate - Craniofacial Journal , vol. 57 , no. 3 , pp. 344-351 . https://doi.org/10.1177/1055665619874991

Title: Pierre Robin Sequence : Incidence of Speech-Correcting Surgeries and Fistula Formation
Author: Gustafsson, Charlotta; Vuola, Pia; Leikola, Junnu; Heliövaara, Arja
Contributor: University of Helsinki, HUS Musculoskeletal and Plastic Surgery
University of Helsinki, HUS Musculoskeletal and Plastic Surgery
University of Helsinki, HUS Musculoskeletal and Plastic Surgery
University of Helsinki, HUS Musculoskeletal and Plastic Surgery
Date: 2020-03
Language: eng
Number of pages: 8
Belongs to series: Cleft Palate - Craniofacial Journal
ISSN: 1055-6656
URI: http://hdl.handle.net/10138/327049
Abstract: Objective: Children with Pierre Robin sequence (PRS) and cleft palate have a high rate of velopharyngeal insufficiency (VPI) following primary palatoplasty. Our purpose was to determine the long-term incidence of speech-correcting surgeries (SCSs) and fistula rates in PRS after primary palatoplasty and the influence of possible causal factors. Design: A retrospective single-center, observational chart review study. Participants: After exclusion, the study cohort comprised 78 nonsyndromic PRS children (48 females) born between 1990 and 2009 and treated at the Cleft Palate and Craniofacial Center of Helsinki University Hospital, Finland. Causal factors included gender, surgeon, age at primary palatoplasty, surgical technique, airway obstruction in infancy, and cleft severity. We analyzed the outcome at age 8 years and at data retrieval, with a median follow-up of 14 years (range: 8-27 years). Results: Thirty-four (43.6%) children received SCS by age 8 years, and of the 19 (24.4%) postoperative fistulas, 6 (7.7%) underwent closure. At data retrieval, 37 (47.4%) children had undergone SCS and 8 (10.3%) had a fistula closure. Median age at SCS was 6 years. The results showed no significant association for gender, surgeon, age at primary palatoplasty, surgical technique, cleft severity, or airway obstruction in infancy regarding incidence of SCS, fistulas, or repaired fistulas. Conclusion: Pierre Robin sequence in children is associated with a high incidence of SCS and fistula formation, which necessitates accurate clinical follow-up and observation of speech development. The development of VPI in PRS is complex and most likely involving multiple factors.
Subject: Pierre Robin sequence
cleft palate
velopharyngeal function
pharyngoplasty
fistula
VELOPHARYNGEAL INSUFFICIENCY
PHARYNGEAL FLAP
PALATE
MANAGEMENT
OUTCOMES
CHILDREN
INFANTS
REPAIR
DIAGNOSIS
DENMARK
313 Dentistry
3126 Surgery, anesthesiology, intensive care, radiology
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