1. Is surgical treatment of hypernasal speech in VCFS special? Sherard A. Tatum, MD, FAAP, FACS Associate Professor of Otolaryngology Associate Professor of Pediatrics Upstate Medical University Syracuse, NY, USA Sherard A. Tatum, MD, FAAP, FACS Associate Professor of Otolaryngology Associate Professor of Pediatrics Upstate Medical University Syracuse, NY, USA

2. Velopharyngeal insufficiency (VPI) Failure of the velar and pharyngeal musculature to close the portion of the throat that separates the oral cavity from the nasal cavity during speech.

3. Treatment goal Block sound and air from coming out of the nose without causing respiratory problems, sleep apnea, and excessive stuffiness.

4. Surgical Options There are many available surgical procedures available to treat VPI, and all of them will work in some cases. However, VCFS is a special case. Many strategies that work in other patients have consistently poor outcomes in VCFS.

5. Special Factors in VCFS Hypotonia of the palate and pharynx A very deep, large pharynx Structural and functional asymmetry of the palate and pharynx Abnormal placement of the internal carotid arteries Abnormal articulation patterns Higher frequency of airway obstruction Speech and language delay

6. Hypotonia

8. Deep, large pharynx Posterior rotation of the skull base is a common VCFS feature As the skull base rotates back, the pharynx moves back increasing pharyngeal depth

9. Internal carotid arteries

10. Asymmetry

12. Causes of airway/apnea problems Research has shown the following factors contribute to airway/apnea problems: Tonsils Narrowing of the pharynx caused by side-to- side closure of the flap donor site Flaps that are too low, increasing negative pressure in the hypopharynx Research has shown the following factors contribute to airway/apnea problems: Tonsils Narrowing of the pharynx caused by side-to- side closure of the flap donor site Flaps that are too low, increasing negative pressure in the hypopharynx Solution

13. Upstate Protocol Modified superiorly based pharyngeal flap Adenotonsillectomy before flap Adenoidectomy allows the nasopharyngeal mucosa to be available for high flap Tonsillectomy to prevent lateral port and oropharyngeal obstruction Flap raised at or above above the velum to make it as high as possible Donor site closed by elevation of posterior pharyngeal wall rather than side-to-side Modified superiorly based pharyngeal flap Adenotonsillectomy before flap Adenoidectomy allows the nasopharyngeal mucosa to be available for high flap Tonsillectomy to prevent lateral port and oropharyngeal obstruction Flap raised at or above above the velum to make it as high as possible Donor site closed by elevation of posterior pharyngeal wall rather than side-to-side

14. Short, High, Wide Flap velum flap velum Lateral ports

15. VPI Rating Scale International Working Group, 1990 (Golding-Kushner et al., 1990, CPJ, 20:337-347 ) uBased on: videofluoroscopy and nasopharyngoscopy uPalate and pharyngeal wall motion rated using a ratio scale uStudies done immediately before surgery uBased on: videofluoroscopy and nasopharyngoscopy uPalate and pharyngeal wall motion rated using a ratio scale uStudies done immediately before surgery

16. Multiview Videofluoroscopy

17. 0.50.0 0.3 MVF Frontal View Rest: 0.0 Side wall function: 0.0 - 1.0 Typical: 0.3 - 0.5

18. REST SPEECH 0.0 1.0 MVF Lateral View

19. 0.0 1.0 MVF Base View Side Wall Movement: 0.0 - 1.0 typical: 0.3 - 0.5 Palate Movement: 0.0 - 1.0 typical: 0.5 - 1.0 Posterior Wall Movement: 0.0 - 1.0 typical: 0.0 - 0.5 0.4

20. Nasopharyngoscopy What We SeeWhat Patient Sees

21. Nasopharyngoscopy Rating scale 0.0 - 1.0 Palate Posterior wall Lateral walls (ML 0.5) Tonsils and adenoids SMCP Rating scale 0.0 - 1.0 Palate Posterior wall Lateral walls (ML 0.5) Tonsils and adenoids SMCP

22. 0.5 0.01.0 0.0 1.0 At Rest

23. 0.3 0.2 1.0 0.0 1.0 0.0 Partial Closure

24. Complete Closure LW 0.5

25. Surgical Technique

26. Technique Soft Palate Donor Site Short FlapConventional Flap

27. DONORSITECLOSUREDONORSITECLOSURE Traditional Modified

28. Donor Site Closure ModifiedTraditional

29. Closure of Donor Site Lateral Closure Vertical Closure Lateral pharyngeal wall

30. Measures and Follow-up Immediately post-op Cardiac/apnea monitors Continuous oximetry Follow up at 1 week, 3 - 6 months, annually Clinical screening for OSA Polysomnogram if symptoms and signs of obstruction Nasopharyngoscopy Speech assessment Immediately post-op Cardiac/apnea monitors Continuous oximetry Follow up at 1 week, 3 - 6 months, annually Clinical screening for OSA Polysomnogram if symptoms and signs of obstruction Nasopharyngoscopy Speech assessment

31. Obstructive Symptoms Snoring Restlessness Nasal dyspnea Chronic rhinorrhea Mouth breathing Sleep disordered breathing Snoring Restlessness Nasal dyspnea Chronic rhinorrhea Mouth breathing Sleep disordered breathing Exercise intolerance Sinusitis Otitis media Denasality Exercise intolerance Sinusitis Otitis media Denasality

32. Results 94 pharyngeal flaps 12 had previous operations elsewhere 9 had 1 previous operation 5 Sphincter pharyngoplasties 2 previous secondary palatoplasties, one combined with a sphincter pharyngoplasty 2 pharyngeal flaps 3 had multiple operations 1 had sphincter pharyngoplasty with 2 revisions 1 had 2 palatoplasties and fat injections 1 had 5 previous palatoplasties 94 pharyngeal flaps 12 had previous operations elsewhere 9 had 1 previous operation 5 Sphincter pharyngoplasties 2 previous secondary palatoplasties, one combined with a sphincter pharyngoplasty 2 pharyngeal flaps 3 had multiple operations 1 had sphincter pharyngoplasty with 2 revisions 1 had 2 palatoplasties and fat injections 1 had 5 previous palatoplasties

33. Flap design based on diagnostic information 94 pharyngeal flaps 71 very wide 14 moderately wide 1 narrow 8 skewed to one side 94 pharyngeal flaps 71 very wide 14 moderately wide 1 narrow 8 skewed to one side

34. Complications 94 pharyngeal flaps 3 returns to OR for bleeding 3 surgical revisions for partial dehiscence 1 port dilation 5 with moderate hyponasality 2 with persistent obstructive symptoms negative PSGs - RDI < 5 94 pharyngeal flaps 3 returns to OR for bleeding 3 surgical revisions for partial dehiscence 1 port dilation 5 with moderate hyponasality 2 with persistent obstructive symptoms negative PSGs - RDI < 5

35. Outcomes 94 pharyngeal flaps 88/94 with elimination of hypernasality (93.6%) 5 with hypernasality, 3 revised, 2 would benefit from additional treatment and are pending treatment depending on outcome of speech therapy 94 pharyngeal flaps 88/94 with elimination of hypernasality (93.6%) 5 with hypernasality, 3 revised, 2 would benefit from additional treatment and are pending treatment depending on outcome of speech therapy

36. Summary Small gap VPI can be managed successfully multiple ways, but such cases are rare with VCFS Large gaps, asymmetric gaps are common in VCFS and are best managed with wide pharyngeal flaps Preoperative adenotonsillectomy and short flaps with vertical donor site closure reduce the obstructive symptoms associated with wide flaps Small gap VPI can be managed successfully multiple ways, but such cases are rare with VCFS Large gaps, asymmetric gaps are common in VCFS and are best managed with wide pharyngeal flaps Preoperative adenotonsillectomy and short flaps with vertical donor site closure reduce the obstructive symptoms associated with wide flaps

37. Example: before and after

38. Example: speech before and after

39. Thank You