4
views
0
recommends
+1 Recommend
0 collections
    0
    shares
      • Record: found
      • Abstract: found
      • Article: found
      Is Open Access

      Long-term Speech Outcomes of Cleft Palate Repair in Robin Sequence versus Isolated Cleft Palate

      research-article

      Read this article at

      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          Supplemental Digital Content is available in the text.

          Background:

          Whether treatment of cleft palate (CP) associated with Robin sequence (RS) should attain outcomes similar to those of isolated cleft palate (ICP) remains unknown. This study compares treatment and outcomes in both conditions and delineates predictors of long-term outcome.

          Methods:

          This retrospective case series of consecutive syndromic and isolated RS- and ICP-patients (1990–2016) includes indications and outcomes of straight-line repair with intravelar veloplasty (SLIV) or Furlow repair depending on cleft and airway characteristics.

          Results:

          Seventy-five RS and 83 ICP patients underwent CP repair. Velopharyngeal insufficiency (VPI) occurred in 41% of RS versus 17% of ICP patients ( P = 0.012), and in 60% of patients with syndromic RS versus 16% with isolated RS ( P = 0.005). In multivariable logistic regression analysis, wider and more severe CP anatomy was the only factor independently associated with VPI ( P = 0.028), in contrast to age at repair, syndromic RS compared with isolated RS, and isolated RS compared with ICP and initial tongue-lip adhesion. Secondary Furlow after primary SLIV was used to treat VPI in all groups, and more frequently in syndromic versus isolated RS patients ( P = 0.025).

          Conclusions:

          Variability of RS anatomy and airway compromise necessitates individualized treatment protocols. Despite differing CP etiology and other variables, our findings demonstrate cleft anatomy as the only independent variable predictive of VPI comparing RS and ICP patients. Patients with isolated RS should ultimately attain similar VPI outcomes compared with ICP patients. Obstructive speech operations in RS patients can be avoided without compromising speech outcome by reserving the prsocedure for secondary cases.

          Related collections

          Most cited references41

          • Record: found
          • Abstract: found
          • Article: not found

          Robin sequence: from diagnosis to development of an effective management plan.

          The triad of micrognathia, glossoptosis, and resultant airway obstruction is known as Robin sequence (RS). Although RS is a well-recognized clinical entity, there is wide variability in the diagnosis and care of children born with RS. Systematic evaluations of treatments and clinical outcomes for children with RS are lacking despite the advances in clinical care over the past 20 years. We explore the pathogenesis, developmental and genetic models, morphology, and syndromes and malformations associated with RS. Current classification systems for RS do not account for the heterogeneity among infants with RS, and they do not allow for prediction of the optimal management course for an individual child. Although upper airway obstruction for some infants with RS can be treated adequately with positioning, other children may require a tracheostomy. Care must be customized for each patient with RS, and health care providers must understand the anatomy and mechanism of airway obstruction to develop an individualized treatment plan to improve breathing and achieve optimal growth and development. In this article we provide a comprehensive overview of evaluation strategies and therapeutic options for children born with RS. We also propose a conceptual treatment protocol to guide the provider who is caring for a child with RS.
            Bookmark
            • Record: found
            • Abstract: found
            • Article: found
            Is Open Access

            Epidemiology, Etiology, and Treatment of Isolated Cleft Palate

            Isolated cleft palate (CPO) is the rarest form of oral clefting. The incidence of CPO varies substantially by geography from 1.3 to 25.3 per 10,000 live births, with the highest rates in British Columbia, Canada and the lowest rates in Nigeria, Africa. Stratified by ethnicity/race, the highest rates of CPO are observed in non-Hispanic Whites and the lowest in Africans; nevertheless, rates of CPO are consistently higher in females compared to males. Approximately fifty percent of cases born with cleft palate occur as part of a known genetic syndrome or with another malformation (e.g., congenital heart defects) and the other half occur as solitary defects, referred to often as non-syndromic clefts. The etiology of CPO is multifactorial involving genetic and environmental risk factors. Several animal models have yielded insight into the molecular pathways responsible for proper closure of the palate, including the BMP, TGF-β, and SHH signaling pathways. In terms of environmental exposures, only maternal tobacco smoke has been found to be strongly associated with CPO. Some studies have suggested that maternal glucocorticoid exposure may also be important. Clearly, there is a need for larger epidemiologic studies to further investigate both genetic and environmental risk factors and gene-environment interactions. In terms of treatment, there is a need for long-term comprehensive care including surgical, dental and speech pathology. Overall, five main themes emerge as critical in advancing research: (1) monitoring of the occurrence of CPO (capacity building); (2) detailed phenotyping of the severity (biology); (3) understanding of the genetic and environmental risk factors (primary prevention); (4) access to early detection and multidisciplinary treatment (clinical services); and (5) understanding predictors of recurrence and possible interventions among families with a child with CPO (secondary prevention).
              Bookmark
              • Record: found
              • Abstract: found
              • Article: not found

              Best Practices for the Diagnosis and Evaluation of Infants With Robin Sequence: A Clinical Consensus Report.

              Robin sequence (RS) is a congenital condition characterized by micrognathia, glossoptosis, and upper airway obstruction. Currently, no consensus exists regarding the diagnosis and evaluation of children with RS. An international, multidisciplinary consensus group was formed to begin to overcome this limitation.
                Bookmark

                Author and article information

                Journal
                Plast Reconstr Surg Glob Open
                Plast Reconstr Surg Glob Open
                GOX
                Plastic and Reconstructive Surgery Global Open
                Lippincott Williams & Wilkins (Hagerstown, MD )
                2169-7574
                21 January 2021
                January 2021
                : 9
                : 1
                : e3351
                Affiliations
                From the [* ]Department of Surgery, Division of Plastic and Reconstructive Surgery, University of California San Francisco, San Francisco, Calif.
                []Craniofacial Center, University of California San Francisco, San Francisco, Calif.
                []Department of Pediatrics, Division of Medical Genetics and Genomics, University of California San Francisco, San Francisco, Calif.
                [§ ]Department of Surgery, Division of Plastic and Reconstructive Surgery, University Medical Center Amsterdam, Emma Children’s Hospital, Amsterdam, the Netherlands.
                Author notes
                Jason H. Pomerantz, MD, Division of Plastic and Reconstructive Surgery, Department of Surgery, University of California San Francisco, 505 Parnassus Ave, San Francisco, CA 94143, E-mail: Jason.Pomerantz@ 123456ucsf.edu
                Article
                00024
                10.1097/GOX.0000000000003351
                7859383
                33564582
                bc506420-42d9-4859-991c-39aa8e7ccbf1
                Copyright © 2021 The Authors. Published by Wolters Kluwer Health, Inc. on behalf of The American Society of Plastic Surgeons.

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.

                History
                : 3 November 2020
                : 16 November 2020
                Categories
                Pediatric/Craniofacial
                Original Article
                Custom metadata
                T
                TRUE
                UNITED STATES

                Comments

                Comment on this article