Voice Disorders in Medically Complex Children

Transcription

1 Voice Disorders in Medically Complex Children Roger C. Nuss, MD, FACS Geralyn Harvey Woodnorth, M.A., CCC-SLP Department of Otolaryngology and Communication Enhancement Children s Hospital Boston Harvard Medical School

2 The Medically Complex Child Can at first be a bit overwhelming Underlying medical problems may have similar effects on larynx and ability to phonate

3 Themes in medically complex child Laryngeal / vocal fold scarring Vocal fold immobility

4 Themes in medically complex child Compensatory techniques, vocal hyperfunction are common Overall weakness / debilitation may limit respiratory support Compromise of airway may limit ultimate vocal outcomes

5 Case Example Teenager with: Dev. Delay Hypotonia Subglottic stenosis Prior LTR Replacement of trach Cricoarytenoid fixation

6 What range of medical issues are seen in the complex patient? Usually combination of problems related to: Prematurity Complex congenital heart disease Genetic disorder Autoimmune disease Iatrogenic

7 Congenital Laryngeal Disease Laryngeal web Laryngeal stenosis Laryngeal cleft

8 Complex Congenital Heart Disease Tetralogy of Fallot Right sided arch Double arch Single ventricle

9 Pulmonary Disease Bronchopulmonary dysplasia Chronic lung disease of prematurity Congenital lobar emphysema Restrictive lung disease Asthma

10 Case Example School age boy with : Asthma Obesity Severe OSA GERD Polypoid corditis

11 Cystic fibrosis Pulmozyme related vocal fold polyposis Poor pulmonary reserve, breath support Hemoptysis

12 Vocal Fold Palsy / Paresis Idiopathic - congenital Neurologic Chiari malformation Hydrocephalus CNS injury Stroke

13 Vocal Fold Immobility - Iatrogenic Cardiac surgery PDA ligation TOF repair VSD repair Tracheo-esophageal fistula repair Vagal nerve stimulator

14 Cricoarytenoid ankylosis Prolonged intubation Prolonged immobility fixation

15 Autoimmune related disease Crohn s disease Juvenile rheumatoid arthritis Wegener s disease QuickTime and a decompressor are needed to see this picture.

16 Case Example: Lipoid Proteinosis Teenager with longstanding coarse low pitched voice quality Poor intelligibility on telephone No OSA QuickTime and a decompressor are needed to see this picture.

17 How do Complex Medical Problems affect the Voice? Pulmonary reserve Breath support Incompetent glottis Inability to adduct vocal folds Decreased phonation time Supraglottic compensation / hyperfunction

18 How do Complex Medical Problems affect the Voice? Inflammatory changes of glottis GERD / LPR

19 How do Complex Medical Problems affect the Voice? Scarring of glottis Prior surgery Recurrent procedures for laryngeal papillomas

20 What are reasonable surgical interventions? Improve the Airway Subglottal stenosis repair Repair glottic web Management of RRP Hemangioma management Vascular malformations of larynx

21 Surgical Interventions Improve glottal closure Reduce risk of aspiration Improve subglottal pressure Improve vocal fold mucosal wave entrapment Techniques Laryngeal framework surgery Injection medialization

22 Injection Medialization Laryngoplasty

23 Surgical Interventions Improve vocal fold surfaces Prevention Treatment of scarring Vibratory characteristics

24 Medical Interventions Control of asthma Recognition + treatment of GERD Allergy management Autoimmune diseases

25 Why does surgery alone not correct the voice disturbance A child s internal set point for their vocal output may not be the desired goal.? Children with congenital laryngeal pathology may never had produced a normal voice Vocal compensatory techniques may need to be unlearned

26 Effective voice treatment with medically complex children requires a team approach SLP ORL Related disciplines: Pulmonary GI Cardiology Etc.

27 Voice Evaluation History Hearing Medical evaluation Laryngeal structure and function Instrumental measurements Acoustic Aerodynamic Videostroboscopy Speech mechanism Respiration Phonation Resonance Articulation Perceptual evaluation CAPE-V Quality of life index Parent/child perspective

28 CAPE V MI MO SE SCORE Overall C I /100 Severity Roughness C I /100 Breathiness C I /100 Strain C I /100 Pitch C I /100 Loudness C I /100

29 Recurring findings around voice disorders in medically complex children

30 Primary findings. Vocal hyperfunction Incomplete glottic closure Dyscoordination respiration/phonation

31 Post Surgery Surgery by itself is not a fix for longstanding voice difficulties

32 Post Surgery Residual limitations Improved capacity yet insignificant adaptation - Learned behavior is retained, muscle memory

33 Behavioral Voice Treatment Education and support Eliminate maladaptive behaviors Coordination respiration/phonation Vocal improvement Environmental enhancements

34 Compensatory Behaviors Eliminate maladaptive behaviors Buccal speech Phonation on inspiration Ventricular phonation Non-compulsory vs necessary compensation

35 Respiration Coordination respiration / phonation Phonation on exhalation Replenishing breaths Phrasing

36 Voice Enhancement Improved quality Reduce vocal hyperfunction as possible Optimum, most efficient vocal function Increased vocal range and flexibility

37 Voice Therapy Techniques Chant talk, singing Chewing Forward focus/resonant voice therapy Lip, tongue trills Humming, nasal consonants Kazoo-like productions Phonation on inhalation Vocal function exercises Sustained, smooth production of vowels Pitch glides Yawn-Sigh

38 Comfortable Therapy Environment Exploration Try new things Follow the leader Encouragement / Support Gradually shape improved production

39 Feedback Specific feedback Describe what you feel / hear Visual and/or auditory feedback

40 Goal Directed Voice Therapy Identify / define target voice Establish goals across task hierarchy Train self-evaluation Plan for generalization Goals for increased vocal range and flexibility

41 Consideration: Principles of Motor Learning High number responses Intense repetitive practice Move from blocked training to randomized trials Progress through a hierarchy Give specific feedback Build in success

42 Treatment: A Dynamic Process Target voice may change over time Surgery Refinement of best voice Maturity

43 Environment Enhancement Accommodations Amplification Positioning / Seating Control environmental noise

44 EG History of L-transposition of the great arteries, status post double switch procedure at 18 years of age Weak breathy strained voice quality after surgery Left vocal fold palsy - wide paramedian position.

45 EG Voice pre-surgery CAPE-V Overall = 56 MPT = 9 seconds Voice post-surgery CAPE-V Overall = 15 MPT = 18 seconds QuickTime and a decompressor are needed to see this picture.

46 EG Aerodynamic measurements post surgery Mean airflow.16 lit/sec (<.20) Mean peak air pressure 7.54 cm H20 (4-8) Aerodynamic Resistance 45 (31-45)

47 DW Former 28 week preemie Left vocal fold palsy dx at 11 years Suspect longstanding; hyperfunction observed 7 years before Weak, breathy voice quality Vocal hyperfunction

48 DW Left vocal fold fat injection medialization Pre-post perceptual and acoustic measures essentially unchanged Voice therapy undertaken

49 CASE: ZR History of tracheoesophageal fistula, repaired as a newborn infant. Status post prior LTR, anterior graft Bilateral VF immobility, only limited movement R arytenoid Complete glottic closure and a fairly good voice quality for a brief duration One month post decannulation

50 CASE: ZR Sustained /a/ Speech

51 JF Prematurity 24 weeks Subglottic stenosis Tracheostomy at 81 days s/p LTR, anterior and posterior grafts s/p cordotomy R TVC Long term plan for decannulation

52 JF Oral communicator using buccal speech

53 JF Communication options discussed Immediate therapy Reduce excessive strain lip synch Progressed well with artificial larynx training and articulation therapy

54 JF Decannulation accomplished Initially aphonic continued with artificial larynx Gradually established functional voice

55 GC Congenital glottic and subglottic stenosis Status post LTR ADHD

56 GC Before voice therapy CAPE-V Overall Rating = 72 Low pitched, harsh voice Variable loudness Visible neck tension After short course of therapy CAPE-V = 53 Reduced jitter