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 Pharyngeal Flap versus Sphincter Pharyngoplasty Surgery in Children with Velocardiofacial Syndrome (VCFS) Katherine Overton, B.S. & Abbie Olszewski, Ph.D., CCC-­‐SLP University of Nevada, Reno Introduc+on •  VCFS paIents typically have underlying velopharyngeal dysfuncIon (VPD) with atypical resonance and nasality. •  Surgical treatment may be needed if behavioral intervenIon is not successful. •  Pharyngeal Flap and Sphincter Pharyngoplasty are two common and effecIve surgical procedures. •  It remains highly important to find and understand the effects and potenIal consequences of both surgical procedures. PICO Ques+on (Gillam & Gillam, 2008) •  In paIents with velocardiofacial syndrome and associated velopharyngeal dysfuncIon (P), does a Pharyngeal Flap surgery (I) have befer results in speech characterisIcs (hypernasality, nasal air emission) (O) outcome ager surgery than a Sphincter Pharyngoplasty (C)? Case Scenario Results Authors Par+cipants Purpose Dependent Variables Brandao et al. (2011) Quasi-­‐Experimental N = 50 VCFS w/ VPD 25 syndromic 25 non-­‐syndromic The purpose of this study was to examine the Resonance effects of Pharyngeal Flap surgery on Nasality resonance, nasality and velopharyngeal VPF funcIon (VPF) for VCFS and non-­‐syndromic paIents. Pre/Post-­‐surgery: •  Resonance: Decreased hypernasality •  Nasality: StaIsIcal significance (SS) (p = .026) •  VPF: SS (p =.019 for VCFS) (p = .049 for non-­‐
syndromic) Post-­‐opera+ve Between Groups: •  Resonance: Not SS (p = .232) •  Nasality: Not SS (no p-­‐value recorded) •  VPF: Not SS (p = .131) Filip et al. (2013) RetrospecIve Blinded/ Randomized N = 12 VCFS w/VPD The purpose of this study was to evaluate Pharyngeal Flap surgery in regards to resonance, nasality and speech intelligibility for paIents with VCFS with associated VPD. Resonance: SS (p = .002) Nasality: SS (p = .033) Speech Intelligibility: SS (p = .021) Spruijt et al. (2012) SystemaIc Review N = 27 Studies 525 paIents total VCFS w/ VPD The purpose of this study was to evaluate Resonance mulIple surgical opIons in regards to Nasality resonance, nasality and speech intelligibility in Speech Intelligibility paIents with VCFS and associated VPD. Other surgeries included: fat injecIon, Furlow or Intravelar Veloplasty Pharyngeal Flap Pharyngoplasty, Honig Pharyngoplasty or Hynes Procedure. Resonance, Nasality, & Speech Intelligibility: No SS differences between surgeries (p = .08) Obstruc+ve Sleep Apnea (OSA): OSA were seen in Pharyngeal Flap and Sphincter Pharyngoplasty-­‐ not SS (p = .25) The purpose of this study was to evaluate effects of Sphincter Pharyngoplasty surgery in regards to resonance, nasality and speech intelligibility for paIents with VCFS and associated VPD. Resonance, Nasality, & Speech Intelligibility: 18/19 cases showed successful perceptual speech characteris.cs and confirmed Sphincter Pharyngoplasty a “reasonable alternaIve” to Pharyngeal Flap Resonance: Pharyngeal Flap: •  85% improvement •  Normal resonance to mild hypernasality Sphincter Pharyngoplasty: •  66% improvement •  Moderate to severe hypernasality WiP et al. (1999) RetrospecIve Ysunza et al. (2009) Quasi-­‐ Experimental N = 19 VCFS w/ VPD N = 29 VCFS w/ VPD Resonance Nasality Speech Intelligibility Resonance Nasality Speech Intelligibility The purpose of this study was to compare Resonance Pharyngeal Flap and Sphincter Pharyngoplasty surgery in regards to resonance for paIents with VCFS and associated VPD. •  Graduate Student at UNR •  Observing a VCFS paIent pre and post-­‐
surgery •  Client is a 7;0 female with VCFS and VPD and has resonance and nasality concerns. •  A Pharyngeal Flap surgery is when a “passive sog Issue obturator is placed in the middle of the velopharyngeal port” (Kummer, 2014). •  A Sphincter Pharyngoplasty surgery “[creates] a sphincter that encircles the velopharyngeal port” (Kummer, 2014). •  It is important to compare the effects of each surgery to see which would be most effecIve to improve resonance and nasality. Study Results Discussion External Evidence: The external evidence concluded that both surgeries are safe and effecIve for VCFS paIents with associated VPD; however, the research found that Pharyngeal Flap surgery has higher success rates with a lower risk of revision ager surgery. One side effect noted was obstrucIve sleep apnea, but overall it was not a staIsIcally significant factor as result of the surgery. Evidence Internal to Clinical Prac+ce: I feel comfortable discussing surgical opIons with my client and am able to refer to surgeons. However, I feel I need to learn more about the behavioral targets for therapy, but am excited to learn about them. My work seung is willing to work and collaborate with surgeons to create the best experience for the client. Evidence internal to Client: Due to the parents lack of knowledge with these surgeries, I would explain the surgeries and the common side-­‐effects. I will make sure my client is fully informed so I can determine the client’s preferences and values. Final Decision: Based on anIcipated client preferences and external evidence, I will recommend the Pharyngeal Flap surgery. This surgery would be the most effecIve surgery for my client due to the overall higher success rate in improving hypernasality and resonance and the lower rate of revisions post-­‐surgery in older children. I will also collaborate with other clinicians who have experience with resonance and nasality intervenIon strategies to provide post-­‐surgery behavioral therapy with my client. Methods •  Search Terms: Velocardiofacial syndrome, velopharyngeal dysfunc.on, Pharyngeal Flap, and Sphincter Pharyngoplasty •  Databases: PubMed, EBSCO •  Appraisal: 10 arIcle were appraised resulIng in ≥ 90% Inter-­‐rater reliability •  5 arIcles supported the PICO quesIon References Brandao, G., Freitas, J., Genaro, K., Yamashita, R., Fukushiro, A., & Lauris, J. (2011). Speech outcomes and velopharyngeal func.on a<er surgical treatment of velopharyngeal insufficiency in individuals with signs of velocardiofacial syndrome. JOURNAL OF CRANIOFACIAL SURGERY, 22(5), 1736-­‐1742. doi:10.1097/SCS.0b013e31822e624f Filip, C., Matzen, M., Aukner, R., Moe, M., Hogevold, H., Abyholm, F., Tonseth, K. (2013). Superiorly based pharyngeal flap for treatment of velopharyngeal insufficiency in pa.ents with 22q11.2 dele.on syndrome. JOURNAL OF CRANIOFACIAL SURGERY, 24(2), 501-­‐504. doi:10.1097/SCS.0b013e31827c84f2 Gillam, S., & Gillam, R. (2008). Teaching graduate students to make evidence-­‐based intervenIon decisions -­‐ applicaIon of a seven-­‐step process within an authenIc learning context. TOPICS IN LANGUAGE DISORDERS, 28(3), 212-­‐228. doi:10.1097/01.TLD.0000333597.45715.57 Kummer, A. W. (2014). Cle< Palate and CraniofacialAnomalies: Effects on Speech and Resonance Third Edi.on. Cligon Park, NY: Cengage Learning . Spruijt, N., ReijmanHinze, J., Hens, G., Vander Poorten, V., & van der Molen, A. (2012). In search of the op.mal surgical treatment for velopharyngeal dysfunc.on in 22q11.2 dele.on syndrome: A systema.c review. PLOS ONE, 7(3), e34332. doi:10.1371/journal.pone.0034332 Wif, P., Cohen, D., Grames, L. M., & Marsh, J. (1999). Sphincter pharyngoplasty for the surgical management of speech dysfunc.on associated with velocardiofacial syndrome. BriIsh Journal of PlasIc Surgery, 52(8), 613-­‐618. doi:10.1054/bjps.1999.3168 Ysunza, A., Pamplona, M. C., Molina, F., & Hernández, A. (2009). Surgical planning for restoring velopharyngeal func.on in velocardiofacial syndrome. InternaIonal Journal of Pediatric Otorhinolaryngology, 73(11), 1572-­‐1575. doi:10.1016/j.ijporl.2009.08.007