Is surgical treatment of hypernasal speech in VCFS special?

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

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

7. 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

11. 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 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

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

15. VPI Rating ScaleInternational Working Group, 1990(Golding-Kushner et al., 1990, CPJ, 20:337-347) • Based on: videofluoroscopy and nasopharyngoscopy • Palate and pharyngeal wall motion rated using a ratio scale • Studies done immediately before surgery

16. Multiview Videofluoroscopy

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

18. MVF Lateral View REST SPEECH 1.0 0.0

19. MVF Base View 1.0 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 0.0 0.4 0.4

20. Nasopharyngoscopy What We See What Patient Sees

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

22. At Rest 1.0 0.5 0.0 1.0 0.0

23. Partial Closure 1.0 0.2 0.0 1.0 0.3 0.0

24. LW 0.5 LW 0.5 Complete Closure

25. Surgical Technique

26. Technique Conventional Flap Short Flap Soft Palate Soft Palate Donor Site

27. D O N O R S I T E C L O S U R E Traditional Modified

28. Donor Site Closure Modified Traditional

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

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

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

33. Flap design based on diagnostic information • 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

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

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

37. Example: before and after

38. Example: speech before and after

39. Thank You