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- Incompetent , 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 VPI Furlow 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 ) • 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  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 • – 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  Check posterior 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 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, Palatoplasty Speech Dysfunction, Aug. 2008, http://emedicine.medscape.com/article/1281032-overview  Flaps overlapped and inset  Leaves a small central gap  Gap size variable based on nasoendoscopy  Lateral defects are closed

Witt D, Craniofacial, Palatoplasty Speech Dysfunction, Aug. 2008, http://emedicine.medscape.com/article/1281032-overview

 Losken, et al.  250 patients  63 - Primary VPI  127 – VPI secondary to CP  32 – VCFS  15 – Submucous cleft palate  13- other  Outcome measure– revision rate secondary to persistent VPI  32/250 (12.8%) required revision  Highest in patents with VCFS (21.8%)  Lowest in patients with primary VPI (6.3%)  30/32 – normal speech after 1 revision  87% primary success rate, 99% after 1 revision  Witt et al.  58 patients  Outcome: perioperative and postoperative OSA  8/58 (14%) developed perioperative OSA  5 – Pierre Robin  3 – Perinatal respiratory difficulties  6/8 resolved by POD 3  2/58 (3%) persistent OSA  Managed with CPAP  VPI  Palatal anomalies common (75%)  80% occult, 20% overt  Palatal/pharyngeal hypotonia  Increased pharyngeal depth  Basicranial angulation  Basicranial angulation  Leads to long face, puffy eyelids, retruded mandible, increased pharyngeal depth • Facial features – Small ears – Long face with malar flattening – Broad nasal root – Hooded eyelids – Cleft and/or palate – Small mouth and chin – Diagnosed with FISH – 22q deletion syndromes  Treatment options  Speech therapy  Low IQ  Furlow palatoplasty  Sphincter pharyngoplasty  Pharyngeal flap  Medialized carotids  50%  ? Preoperative MRA  Can usually detect on nasoendoscopy  VPD common after, but not isolated to patients with cleft palate  All VPD patients require speech therapy  Surgical options need to be specifically tailored to each individual based on patient’s VP closure pattern and size of velopharyngeal gap