Robert S. Glade, MD, FAAP Co-Director, VPI Multidisciplinary Clinic of Oklahoma Pediatric ENT of Oklahoma Velopharyngeal dysfunction Velopharyngeal Velopharyngeal Velopharyngeal mislearning incompetance insufficiency (pharyngeal sound (neurolophysiologic (structural or substitution for oral dysfunction causing anatomic deficiency) sound) poor movement) Velopharyngeal Mislearning Speech Therapy Velopharyngeal Incompetence Ideal Patient Pharyngeal Flap-Surgery Incompetent palate, surgical candidate Pharyngeal Bulb Poor surgical candidate, short palate Pharyngeal Lift Poor surgical candidate, long palate Velopharyngeal Insufficiency - Surgery Ideal patient Posterior wall augmentation Small central gap, post adenoidectomy VPI Furlow palatoplasty Submucous , occult submucous cleft palate, and secondary cleft palate repair with small gap (less than 5mm-1cm) Sphincter pharyngoplasty Coronal or bowtie closure pattern with lateral gaps Pharyngeal flap Sagittal or central closure pattern with large, central gap, inadequate palatal length, palatal hypotonia • Muscles of VP closure – Levator veli palatini • Principle elevator (most important for VP closure) – Tensor veli palatini • Opens eustachian tube • ? Tension to velum – Musculus uvulae • Only intrinsic velar muscle • Adds bulk to dorsal uvula – Superior constrictor • Produces inward movement of lateral pharyngeal walls • Passavants ridge – Not universal Passavant’s Ridge Velopharyngeal Dysfunction Robert Glade, MD FAAP After repair – 20-50% develop VPD •Levator orientation •Scar tissue •Palatal length • Bifid uvula • Zona pellucida (muscular diastasis with intact mucosa over soft palate) • Palpable notch in posterior border of hard palate • Unrepaired, approximately 50% develop VPD Absence or deficiency of muscularis uvulae Diastasis of levator levi palatini NO BIFID UVULA Diagnosed with flexible nasoendoscopy Posterior soft palate V- notch (deficiency of uvula) • Transient VPD following adenoidectomy – Typically resolves spontaneously – Persistent VPD • 1:5000 – 1:10,000 • Identify high risk patients • Always look for bifid uvula and palpate soft palate • Trisomy 21 – Hypotonia • Velocardiofacial syndrome (VCFS) – Pharyngeal hypotonia – Cleft palate • Kabuki syndrome – Pharyngeal hypotonia – Cleft palate • Acquired – Stroke, head injury – Neurologic diseases • MS, ALS, Parkinson • Idiopathic • Hypernasal speech – Nasal air emission – Nasal resonance • Compensatory articulation errors • Glottal stops (Kitty Cat = i-e- at) • Poor speech intelligibility • Decreased speech volume • Hoarseness – Vocal strain to overcome nasal escape – Increased incidence of nodules Articulation assessment Trained speech therapist!!! Palatogram/Videofluoroscopy Fiberoptic nasoendoscopy Nasometry Pressure-flows Palatogram/Videofluoroscopy Allows examiner to see through tissues AP/Lateral views allow visualization of both AP and lateral wall movement Difficult to see small gap Radiation! Typically need to check more than once. Nasoendoscopy Flexible laryngoscope passed through the nose to visualize palatal closure from above Pass through middle meatus if possible in order to look down at velum during speech My preference!!! Pattern of VPD dependent of dysfunctional muscle A) Coronal Anterior closure B) Sagittal closure Lateral wall closure C) Circular closure Lateral and anterior closure D) Bowtie Treatment options Speech therapy Velopharyngeal mislearning Compensatory misarticulations Continued after surgical treatment Prosthetic appliances Palatopharyngeal obturators Palatal lifts Pharyngeal bulbs Not well tolerated children Surgical management Augmentation of posterior pharyngeal wall (injection pharyngoplasty) Palatal lengthening (Furlow palatoplasty) Pharyngeal flap Sphincter pharyngoplasty Combination procedures Lifts soft palate into contact with posterior pharyngeal wall Palatal hypotonia Adequate palatal length Stroke, muscular degenerative DO Obturates defects within the palate Large palatal fistula Hypernasal speech Regurgitation of liquids • Used for deficient palatal length •Often used for cancer patients after surgical resection Velopharyngeal Dysfunction Robert Glade, MD FAAP Ideal patient Small, central gap, post adenoidectomy patient Injection pharyngoplasty Teflon, fat, hydroxyapatite Figure out where the gap is present Nasal endoscopy/video fluoroscopy Pick the surgery that fills that gap with the least morbidity Inject absorbable material into the posterior pharyngeal wall (“immobile passavants”) Transoral view of the nasopharynx (120 degree scope) First review nasoendoscopy to ascertain level of closure Set up like tonsillectomy Check posterior pharynx for pulsations The 3 yellow dots indicate the area of the posterior pharyngeal wall to be injected Injection Pharyngoplasty With Calcium Hydroxyapatite for Treatment of Velopalatal Insufficiency. Sipp, J; Ashland, Jean; PhD, SLP; Hartnick, Christopher; MD, MS Archives of Otolaryngology -- Head & Neck Surgery. 134(3):268-271, March 2008. 2 Injection of calcium hydroxylapatite with 25-guage needle 1-4 mL hydroxyapatite injected into retropharyngeal soft tissue Injection Pharyngoplasty With Calcium Hydroxyapatite for Treatment of Velopalatal Insufficiency. Sipp, J; Ashland, Jean; PhD, SLP; Hartnick, Christopher; MD, MS Archives of Otolaryngology -- Head & Neck Surgery. 134(3):268-271, March 2008. Post injection view of nasopharynx showing soft tissue mound raised by calcium hydroxyapatite. Injection Pharyngoplasty With Calcium Hydroxyapatite for Treatment of Velopalatal Insufficiency. Sipp, J; Ashland, Jean; PhD, SLP; Hartnick, Christopher; MD, MS Archives of Otolaryngology -- Head & Neck Surgery. 134(3):268-271, March 2008. 8/12 patients - successful speech at 3 months 4 followed 24 months – all sustained benefit 7/8 successes had no craniofacial anomaly Table. Characteristics of Children Undergoing Injection ¾ failures had Pharyngoplasty craniofacial Injection Pharyngoplasty With Calcium Hydroxylapatite for Velopharyngeal Insufficiency: Patient Selection and Technique. anomaly Brigger, Matthew; MD, MPH; Ashland, Jean; Hartnick, Christopher; MD, MEpi Archives of Otolaryngology -- Head & Neck Surgery. 136(7):666-670, July 2010. 4 • Ideal patient • Only addresses the soft palate! • Incomplete secondary cleft of soft palate • SMC, and Occult SMC palate • Secondary repair of cleft palate after straight line closure • Levator muscles located anteriorly/ do not form sling • Reorients levator in correct position • Lengthens palate Efficacy as secondary procedure after intravelar veloplasty (Chen et al.) Retrospective review 18 patients underwent furlow after primary palatoplasty Complete velopharyngeal closure in 16/18 (89%) Nasoendoscopy Most successful procedures had a preoperative gap <5mm 2 failures had preoperative gap >10mm Conclusion: Most important predictive factor for success is preoperative gap size Efficacy as treatment for submucous cleft palate (Chen et al.) 30 patients (ages 3-26 years) Gap <5mm 29/30 (96%) achieved competent velopharyngeal function after Furlow Judged by perceptual speech evaluation Conclusion: Furlow palatoplasty can correct VPI in patients with SMCP and small gap (Perkins et al.) ? Correlation between postoperative efficacy and preoperative gap size 148 patients underwent Furlow Secondary procedure for repaired cleft palate and primary SMCP Classified preoperative closure on nasoendoscopy Small gap – VP closure 80-100% Moderate gap – VP closure 50-80% Large gap – VP closure <50% Indications VPI after primary repair cleft palate Submucous cleft palate with large gap Inadequate palatal length Palatal hypotonia Ideal patient Sagittal closure pattern Good lateral wall movement Poor AP movement Bardach et al, 1999 Bardach et al, 1999 Sullivan et al. (Boston) 79 patients non-syndromic patients who required pharyngeal flap for VPI after cleft palate repair 65/79 (85%) normal speech 10/79 (13%) borderline insufficiency 2/79 (2%) persistent VPI 2/79 (2%) developed OSA Chegar et al. (Syracuse) 54 patients underwent staged adenotonsillectomy before pharyngeal flap 51/54 (94%) complete resolution of VPI Complications Nasal obstruction 8/54 (15%) Hyponasal speech 4/54 (7%) Primary snoring 4/54 7% All negative for OSA on sleep study Cole et al. (Baylor) Post operative complications 150 patients 12/150 (8%) required post operative O2 3/150 (2%) developed post operative bleed 14/150 (9%) developed primary snoring 5/150 (3%) OSA Speech benefits of pharyngeal flap persist after flap division Agarwal et al. 12 patients who underwent flap division for OSA 12/12 – resolution or dramatic improvement of OSA 12/12 immediate transient hypernasality of speech 11/12 velopharyngeal function returned to pre-division state – two months Hypothesis Persistent posterior pharyngeal wall bulk aids in closure Learned speech mechanisms learned with flap up persist after taken down Indications VPI after cleft palate repair Primary VPI Ideal patient Coronal closure pattern Poor lateral wall motion Adequate AP closure Superior based flaps are marked to include the palatopharyngeus Flaps connected by a vertical incision at the level where soft palate closure occurs Preoperative nasoendoscopy Witt D, Craniofacial, Palatoplasty Speech Dysfunction, Aug. 2008, http://emedicine.medscape.com/article/1281032-overview Musculomucosal flaps are dissected and overlapped across the vertical incision Witt D, Craniofacial,