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Pediatric Dysphagia: Complex Case Studies of Patients with ...


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Pediatric Dysphagia: Complex Case Studies of Patients with ...

  1. 1. Pediatric Dysphagia: Complex Case Studies of Patients with Aerodigestive Disorders (2421) Jennifer Perez, M.S., CCC-SLP Children’s Hospital Boston, Boston, MA Alana Lowry, M.S., CCC-SLP Fletcher Allen Healthcare, Burlington, VT Kara Fletcher Larson, M.S., CCC-SLP South Shore Hospital, Weymouth, MA
  2. 2. 3 SLP’s = 1 Vision <ul><li>All have worked and trained extensively at Children’s Hospital Boston </li></ul><ul><li>Clinical specialty in pediatric dysphagia </li></ul><ul><li>High volume of pediatric videofluoroscopy </li></ul><ul><li>Level 3 NICU </li></ul><ul><li>Exposure to wide range of medical/surgical pediatric diagnoses </li></ul><ul><li>Working within the division of ORL </li></ul>
  3. 3. Children’s Hospital Boston <ul><li>Acute, urban pediatric hospital </li></ul><ul><li>Associated with Harvard Medical School </li></ul><ul><li>397 bed comprehensive pediatric healthcare center </li></ul><ul><ul><li>Medical/Surgical ICU </li></ul></ul><ul><ul><li>Cardiac ICU </li></ul></ul><ul><ul><li>Neonatal ICU (Level III) </li></ul></ul><ul><li>204 specialized clinical programs </li></ul>
  4. 4. Aero-digestive Disorders <ul><li>Congenital Anomalies of Larynx </li></ul><ul><li>Upper Airway Obstruction </li></ul><ul><li>Esophageal Obstructive Disorders </li></ul>
  5. 5. Center for Aero-Digestive Disorders (CADD)
  6. 6. Center for Aero-Digestive Disorders <ul><li>Multi-disciplinary clinic including pediatric specialists from: </li></ul><ul><ul><li>Otolaryngology (ORL) </li></ul></ul><ul><ul><li>Gastroenterology </li></ul></ul><ul><ul><li>Pulmonary </li></ul></ul><ul><ul><li>Surgery </li></ul></ul><ul><ul><li>Radiology </li></ul></ul><ul><ul><li>Speech-Language Pathology </li></ul></ul><ul><ul><ul><li>Pediatric Dysphagia specialists </li></ul></ul></ul><ul><ul><ul><li>Voice specialists </li></ul></ul></ul><ul><ul><li>Neurology </li></ul></ul><ul><ul><li>Genetics </li></ul></ul>
  7. 7. Center for Aero-Digestive Disorders <ul><li>Providing comprehensive services to infants and children with complex problems involving the: </li></ul><ul><ul><li>Airway </li></ul></ul><ul><ul><li>Pulmonary tract </li></ul></ul><ul><ul><li>Upper digestive tract </li></ul></ul>
  8. 8. Center for Aero-Digestive Disorders <ul><li>Frequently treated medical conditions: </li></ul><ul><ul><li>Asthma </li></ul></ul><ul><ul><li>Congenital anomalies of the esophagus and trachea </li></ul></ul><ul><ul><li>Croup </li></ul></ul><ul><ul><li>Gastroesophageal reflux </li></ul></ul><ul><ul><li>Iatrogenic or acquired tracheal stenosis </li></ul></ul><ul><ul><li>Laryngeal cleft </li></ul></ul><ul><ul><li>Laryngomalacia </li></ul></ul><ul><ul><li>Lymphatic malformation </li></ul></ul><ul><ul><li>Pneumonia </li></ul></ul><ul><ul><li>Stridor </li></ul></ul><ul><ul><li>Subglottic stenosis </li></ul></ul><ul><ul><li>Tracheoesophageal fistula/esophageal atresia </li></ul></ul>
  9. 9. Center for Aero-Digestive Disorders <ul><li>Logistics—How the CADD works: </li></ul><ul><ul><li>Once per week, patients see ORL, GI, Pulmonary in clinic </li></ul></ul><ul><ul><ul><li>Speech-Pathology available for consultation </li></ul></ul></ul><ul><ul><li>Following clinic, team members meet to discuss and collaborate on findings and determine follow-up plan </li></ul></ul><ul><ul><li>Once per month, CADD conference of various disciplines for review of most complex patients/cases </li></ul></ul>
  10. 10. Importance of the speech-language pathologist on the Aero-Digestive team <ul><ul><li>Clinical screening for feeding & swallowing safety </li></ul></ul><ul><ul><li>Objective assessment of swallow function pre- and/or post-surgical intervention </li></ul></ul><ul><ul><li>Comprehensive feeding evaluation/therapy for sensory-based feeding issues, oral motor delays secondary to complex medical/surgical history </li></ul></ul>
  11. 11. Center for Aero-Digestive Disorders <ul><li>Criteria for referral for objective swallowing evaluation: </li></ul><ul><ul><li>Coughing, choking or sputtering (during or after feeds) </li></ul></ul><ul><ul><li>Wet/junky/congested breath sounds (during or after feeds) </li></ul></ul><ul><ul><li>Chronic wheezing or stridor </li></ul></ul><ul><ul><li>Chronic pneumonia, croup, bronchitis </li></ul></ul><ul><ul><li>Oxygen desaturation/cyanosis (during or after feeds) </li></ul></ul><ul><ul><li>Apnea/bradycardia, etc. with feeds </li></ul></ul><ul><ul><li>Color change with feeds </li></ul></ul><ul><ul><li>Chronic fever of unclear etiology </li></ul></ul>
  12. 12. Objective Assessment of Swallow Function FEES vs. VFSS <ul><li>FEES </li></ul><ul><li>Fiberoptic Endoscopic </li></ul><ul><li>Evaluation of Swallowing </li></ul><ul><li>No radiation exposure </li></ul><ul><li>Invasive-Difficulty maintaining cooperation in children </li></ul><ul><li>12 mo - 4 yrs </li></ul><ul><li>Does not provide information regarding interaction of oral, pharyngeal and esophageal phases of swallowing </li></ul><ul><li>Can assess breastfeeding </li></ul><ul><li>Can view secretions </li></ul><ul><li>Equipment is portable </li></ul><ul><li>Can assess VPI and vocal cord issues </li></ul><ul><li>VFSS </li></ul><ul><li>Videofluoroscopic Swallow Study </li></ul><ul><li>(Modified Barium Swallow Study) </li></ul><ul><li>Exposure to radiation </li></ul><ul><li>Non-invasive </li></ul><ul><li>Dynamic assessment of oral, pharyngeal, and esophageal phases of swallowing </li></ul><ul><li>Cannot assess breastfeeding </li></ul><ul><li>Readily available in most medical centers </li></ul><ul><li>Gold standard assessment of swallowing physiology </li></ul>Adapted from: Arvedson & Lefton-Greif, 1998
  13. 13. Management of Dysphagia <ul><li>Establish safe oral feeding plan : </li></ul><ul><ul><li>? Need for non-oral supplementation </li></ul></ul><ul><ul><li>Diet modification </li></ul></ul><ul><ul><li>Modify delivery method </li></ul></ul><ul><ul><li>Limit volume/length of time </li></ul></ul><ul><ul><li>Determine safest positioning </li></ul></ul><ul><ul><li>Counseling families </li></ul></ul><ul><ul><li>**Collaboration with other medical providers in CADD is essential for decision-making and establishing these dietary changes** </li></ul></ul>
  14. 14. Congenital Anomalies of the Larynx: Laryngeal Cleft
  15. 15. Laryngeal Cleft <ul><li>Definition: </li></ul><ul><ul><li>Communication between the posterior larynx and esophagus </li></ul></ul><ul><ul><li>Failure of the tracheoesophageal septum to develop </li></ul></ul>
  16. 16. Case Study # 1: <ul><li>-5 week old baby boy </li></ul><ul><li>-Clinical symptoms: </li></ul><ul><li>-coughing while drinking thin liquids </li></ul><ul><li>-pneumonia </li></ul><ul><li>-losing weight due to poor feeding </li></ul><ul><li>-Admitted to pulmonary service at CHB </li></ul><ul><li>-NG-tube placed </li></ul><ul><li>-Referred for VFSS </li></ul>
  17. 17. Case Study # 1 (cont): <ul><li>- VFSS Results : </li></ul><ul><li>-silent aspiration with thin liquids </li></ul><ul><li>-consistent deep laryngeal penetration with nectar-thick liquids </li></ul><ul><li>-nasopharyngeal reflux </li></ul><ul><ul><li>- assumed dysphagia due to discoordinated suck-swallow-breathe pattern </li></ul></ul><ul><li>-VFSS repeated no improvement x 2 </li></ul><ul><li>-G-tube placed at 4 months old </li></ul>
  18. 18. Case Study # 1 (cont): <ul><li>-At 11 months of age: </li></ul><ul><li>-Baby assessed for laryngeal cleft (DL/B) </li></ul><ul><li>-found to have Type 1 laryngeal cleft </li></ul><ul><li>-Surgically repaired </li></ul><ul><li>-Repeat VFSS 4 weeks post surgery </li></ul><ul><li>-continued aspiration of thin liquids </li></ul><ul><li>-Repeat VFSS 12 weeks post surgery </li></ul>
  19. 19. VFSS Clip # 2 Post-Surgery
  20. 20. Case Study # 1 (cont): <ul><li>-Repeat VFSS 12 weeks post surgery </li></ul><ul><li>-no aspiration with thin liquids </li></ul><ul><li>-Now: Child 3 years old </li></ul><ul><li>-G-tube removed </li></ul><ul><li>-stable respiratory status </li></ul>
  21. 21. Classification of Laryngeal Clefts <ul><li>According to length: </li></ul><ul><ul><li>Type I: interarytenoid only </li></ul></ul><ul><ul><li>Type II: partial cricoid </li></ul></ul><ul><ul><li>Type III: complete cricoid </li></ul></ul><ul><ul><li>Type IV: extending into thoracic trachea </li></ul></ul>
  22. 22. Types of Laryngeal Clefts <ul><li>Types I and II </li></ul><ul><ul><li>Diagnosis may take months to years </li></ul></ul><ul><li>Types III and IV </li></ul><ul><ul><li>Diagnosed on the first day of life due to severity </li></ul></ul><ul><ul><li>High morbidity associated with Type IV </li></ul></ul>
  23. 23. Classification of Laryngeal clefts Benjamin and Inglis, 1989
  24. 24. <ul><li>Type I </li></ul>Complete cricoid cleft Cleft extending to thoracic trachea Interarytenoid and supra glottic cleft Type I A Type I Type I B Type II Type I Type I C Type III Type II Type II Type III Type III Type III Type IV Classification Petterson Armitage Benjamin Partial cricoid cleft extending below the level of the vocal cords Cleft extending to cervical trachea
  25. 25. Photos courtesy of Dr. Reza Rahbar, ORL/CHB
  26. 26. Associated Anomalies <ul><li>Aero-Digestive: </li></ul><ul><ul><li>Tracheoesophageal fistulas: 20-37% </li></ul></ul><ul><ul><li>Tracheomalacia, laryngomalacia, subglottic stenosis </li></ul></ul><ul><ul><li>Hypoplastic lungs, transposition of the great arteries </li></ul></ul><ul><ul><li>Bifid uvula, cleft lip, cleft palate </li></ul></ul>
  27. 27. Associated Syndromes <ul><li>Pallister Hall syndrome: </li></ul><ul><ul><li>Hypothalamic hamartoblastoma , Hypopituitarism, Polydactyly, Imperforate anus </li></ul></ul><ul><li>Opitz-Frias syndrome: </li></ul><ul><ul><li>Cleft lip palate, Hypertelorism, Airway cleft, Hypospadias </li></ul></ul><ul><li>VACTERL </li></ul><ul><ul><li>Heart and vertebral anomalies , Esophageal atresia , Polydactylie, Absent left radius, Anorectal anomalies </li></ul></ul>
  28. 28. Associated Syndromes <ul><ul><li>At Children’s Hospital Boston, of 81 patients with diagnosis of laryngeal cleft (2003-2008): </li></ul></ul><ul><ul><li>TEF (s/p repair) (N=6) </li></ul></ul><ul><ul><li>Multiple Congenital Anomalies (N=13) </li></ul></ul><ul><ul><ul><li>Trisomy 21 (N=3) </li></ul></ul></ul><ul><ul><ul><li>Fetal Alcohol syndrome (N=1) </li></ul></ul></ul><ul><ul><ul><li>Tetralogy of Fallot (N=2) </li></ul></ul></ul><ul><ul><ul><li>Waardenburg (N=1) </li></ul></ul></ul><ul><ul><ul><li>Scimitar (N=1) </li></ul></ul></ul><ul><ul><ul><li>CHARGE association (N=2) </li></ul></ul></ul><ul><ul><ul><li>Opitz (N=1) </li></ul></ul></ul><ul><ul><ul><li>VACTERL (N=2) </li></ul></ul></ul>
  29. 29. Incidence of Laryngeal Clefts (all types) <ul><li>Live birth: 1 in 10,000-1 in 20,000 </li></ul><ul><li>Incidence of laryngeal cleft increases to 0.6% in patients with co-existing TEF </li></ul><ul><li>Strong association with other anomalies, but also can exist in isolation </li></ul>
  30. 30. Symptoms of Laryngeal Cleft <ul><li>Cough* </li></ul><ul><li>Wheezing* </li></ul><ul><li>Stridor </li></ul><ul><li>Feeding Difficulty </li></ul><ul><li>Aspiration </li></ul><ul><li>Respiratory Distress </li></ul><ul><ul><li>*Most common referral symptoms </li></ul></ul><ul><ul><li>* Symptoms are non-specific </li></ul></ul>
  31. 31. Diagnosis of Laryngeal Cleft <ul><li>High index of suspicion based on: </li></ul><ul><ul><li>Clinical presentation </li></ul></ul><ul><ul><ul><li>Medical history/Systematic chart review </li></ul></ul></ul><ul><ul><li>Interpretation of preoperative studies </li></ul></ul><ul><ul><ul><li>Chest radiograph, VFSS/FEES, Upper GI series </li></ul></ul></ul><ul><ul><li>Thorough endoscopic airway evaluation under general anesthesia </li></ul></ul><ul><ul><ul><li>Direct laryngoscopy, bronchoscopy, esophagoscopy </li></ul></ul></ul><ul><ul><ul><li>Endoscopist MUST perform diligent interarytenoid palpation </li></ul></ul></ul>
  32. 32. Diagnosis of Laryngeal Cleft <ul><li>Airway endoscopy (in which laryngeal cleft is suspected) is needed for definitive diagnosis </li></ul><ul><li>VFSS does not identify laryngeal cleft, but can confirm symptoms of aspiration due to overall neuromuscular discoordination or isolated pharyngeal dysphagia </li></ul>
  33. 33. Increasing Incidence: Why? <ul><li>Literature review documents incidence of Type I laryngeal cleft is higher than in the past </li></ul><ul><li>7.6 % (Chien et al, 2006) </li></ul><ul><li>6.2 % (Parsons et al, 1998) </li></ul><ul><li>7.1 % (Watters & Russell, 2003) </li></ul><ul><li>-Rising incidence may be due to: </li></ul><ul><li>-type of patients referred to aerodigestive clinics </li></ul><ul><li>-higher index of suspicion for presence of laryngeal cleft at time of airway endoscopy (ORL now looking more specifically for laryngeal cleft) </li></ul>
  34. 34. Surgical Repair of Laryngeal Cleft <ul><li>Conventional Treatment: </li></ul><ul><ul><li>Invasive surgery requiring incision in the neck and opening of the larynx </li></ul></ul><ul><li>Endoscopic Repair: </li></ul><ul><ul><li>Laser and suture </li></ul></ul><ul><li>Robotic Repair: </li></ul><ul><ul><li>Work through the oral cavity and in the restricted confines of the airway without impeding breathing </li></ul></ul><ul><ul><li>First performed by Dr. Reza Rahbar at Children’s Hospital Boston </li></ul></ul>
  35. 35. Photos courtesy of Dr. Reza Rahbar, Children’s Hospital Boston Endoscopic Repair of Laryngeal Cleft
  36. 36. SLP Perspective: Typical course for patient <ul><li>PCP or other specialist refers for MBS due to chronic pulmonary symptoms of unclear etiology associated with feeding </li></ul><ul><li>VFSS: Documentation of aspiration with liquids </li></ul><ul><li>SLP modifies diet (i.e,. Nectar-thick liquids) to promote swallowing safety, minimize aspiration </li></ul><ul><ul><li>-(patient may need NG-tube) </li></ul></ul><ul><li>SLP contact PCP to consider referral to CADD </li></ul><ul><li>Patient seen in CADD by ORL, GI, Pulmonary </li></ul>
  37. 37. SLP Perspective: Typical course for patient (cont.) <ul><li>6. CADD team completes direct laryngoscopy/bronchoscopy to confirm diagnosis of laryngeal cleft </li></ul><ul><li>Team decides: </li></ul><ul><ul><li>-conservative management or surgical repair?? </li></ul></ul><ul><li>IF SURGICAL REPAIR: </li></ul><ul><li>8. Maintain diet of thickened liquids for 6-8 weeks after surgery </li></ul><ul><li>Repeat VFSS at 8-12 weeks after surgery, and repeat every 3 months as needed </li></ul><ul><li>If no aspiration, child resumes full oral diet </li></ul>
  38. 38. Who to consider for laryngeal cleft: <ul><li>Any infant or child with normal development (i.e., no neurogenic, medical and genetic etiology) who presents with “isolated swallowing dysfunction” </li></ul><ul><li>Any infant or child with history of Tracheoesophageal fistula (TEF) and aspiration on VFSS </li></ul><ul><li>Any infant or child with a history of chronic aspiration that does not improve over time or with traditional intervention </li></ul>
  39. 39. Conservative Monitoring <ul><li>If no surgical intervention, conservative management of dysphagia: </li></ul><ul><ul><li>Diagnose and treat symptoms of GER </li></ul></ul><ul><ul><li>SLP works with family to modify diet </li></ul></ul><ul><ul><li>Close follow-up with CADD </li></ul></ul>
  40. 40. Our experience at CHB <ul><li>2003-2008 </li></ul><ul><ul><li>81 patients referred for evaluation of cough, feeding difficulty, and aspiration </li></ul></ul><ul><ul><li>74 of 81 patients found to have significant laryngeal cleft (Type I or Type II) </li></ul></ul><ul><ul><li>25 of 74 = conservative monitoring with medical therapy </li></ul></ul><ul><ul><li>49 of 74 = endoscopic CO2 laser repair of Type I and Type II laryngeal cleft </li></ul></ul><ul><ul><ul><li>(age range: 8 mo-15 years) </li></ul></ul></ul>
  41. 41. Our experience at CHB <ul><li>- Type I cleft: 100 % reported feeding difficulty with thin liquids </li></ul><ul><li>-Wheezing and chronic cough most common respiratory symptoms </li></ul><ul><li>-86% patients with aspiration on pre-surgical VFSS </li></ul><ul><li>- 71% patients with Type I =no aspiration on VFSS post-repair </li></ul><ul><li>- 82% patients with Type II =no aspiration on VFSS post repair </li></ul><ul><li>-Overall improvement noted in decreasing cough, normal VFSS, decreasing need for hospitalization </li></ul>
  42. 42. Case Study # 2: <ul><li>-5 month old baby girl </li></ul><ul><li>-Clinical symptoms: </li></ul><ul><li>-coughing while drinking thin liquids </li></ul><ul><li>-wheezing (worsens with feeding) </li></ul><ul><li>-frequent spit ups with feeds </li></ul><ul><li>-Referred for VFSS by PCP </li></ul>
  43. 43. Case Study # 2 (cont): <ul><li>- VFSS Results : </li></ul><ul><li>-silent aspiration with thin and nectar-thick liquids </li></ul><ul><li>-no aspiration with honey-thick liquids </li></ul><ul><li>-no aspiration with purees </li></ul><ul><li>-Discuss results with PCP who refers to CADD </li></ul><ul><li>- Nutrition assessment </li></ul><ul><li>-Airway endoscopy reveals Type 1 laryngeal cleft </li></ul><ul><li>-Patient undergoes surgical repair </li></ul><ul><li>-Repeat VFSS 3 months after laryngeal cleft repair </li></ul>
  44. 44. VFSS Clip # 2 Post-op
  45. 45. What the SLP has learned… <ul><li>*Child with aspiration on VFSS who has otherwise normal development, who presents with any chronic respiratory symptoms is a candidate for CADD work-up </li></ul><ul><li>*Maintain pre-surgical diet at least 6-8 weeks after surgery </li></ul><ul><li>*Do not repeat VFSS too soon after surgery-typically wait 8-12 weeks (minimize radiation exposure) </li></ul><ul><li>*Collaboration with Aero-digestive team is essential! </li></ul>
  46. 46. Fletcher Allen Health Care <ul><li>Rural, community hospital </li></ul><ul><li>Level I Trauma Center </li></ul><ul><li>Level III 20-bed NICU </li></ul><ul><li>10-bed NTU </li></ul><ul><li>30-bed Pediatric Floor </li></ul><ul><li>PICU </li></ul>
  47. 47. Vermont’s Academic Medical Center <ul><li>In alliance with UVM </li></ul><ul><li>Teaching hospital </li></ul><ul><li>Vermont Oncology Center </li></ul><ul><li>Innovative care </li></ul><ul><li>Acute Rehabilitation Center-Fanny Allen </li></ul>
  48. 48. What is Upper Airway Obstruction (UAO)? <ul><li>An obstruction present in the upper airway due to anatomic or physiologic anomalies disrupting respiration and the oral feeding process </li></ul><ul><li>Can be tracheal, laryngeal or pharyngeal in nature </li></ul><ul><li>Miller & Willging, 2007 </li></ul>
  49. 49. Upper vs. Lower Airway Obstruction <ul><li>Pattern of stridor is key for differential diagnosis of UAO </li></ul><ul><li>Inspiratory stridor </li></ul><ul><li>Expiratory stridor </li></ul><ul><li>Biphasic stridor </li></ul>Koufman, J & Block, C. American Journal of Speech-Language Pathology Vol.17 327-334 November 2008.
  50. 50. Upper Airway Obstruction: Classifications <ul><li>Congenital: </li></ul><ul><li>Choanal atresia </li></ul><ul><li>Cleft lip </li></ul><ul><li>Micrognathia </li></ul><ul><li>Laryngomalacia </li></ul><ul><li>Neoplasm </li></ul><ul><li>Laryngeal cleft </li></ul><ul><li>Acquired: </li></ul><ul><li>Croup </li></ul><ul><li>Epiglottitis </li></ul><ul><li>Tracheitis </li></ul><ul><li>Foreign body ingestion </li></ul><ul><li>Inhalation injury </li></ul>The following four slides adapted from Andrews, T. Airway obstruction in craniofacial anomalies: In: Myer, C, Cotton, R, Shott, S. eds. The Pediatric Airway: An Interdisciplinary Approach, Philadelphia, PA: JB Lippencott; 1995:249. Most recently published in Miller & Willging, 2007.
  51. 51. Upper Airway Obstruction: Classifications <ul><li>Acute: </li></ul><ul><li>Cases are seen in emergency department </li></ul><ul><li>Cases of UAO are acquired </li></ul><ul><li>Infection and croup </li></ul><ul><li>Chronic: </li></ul><ul><li>Pierre Robin sequence </li></ul><ul><li>Treacher-Collins syndrome </li></ul><ul><li>Apert syndrome </li></ul><ul><li>Beckwith-Wiedemann syndrome </li></ul>
  52. 52. Etiologies of UAO <ul><li>Infection/Inflammation </li></ul><ul><li>Accounts for 90% of UAO </li></ul><ul><li>Croup </li></ul><ul><li>Epiglottitis </li></ul><ul><li>Tracheitis </li></ul><ul><li>Retropharyngeal abscess </li></ul><ul><li>Peritonsillar abscess </li></ul><ul><li>Accident/Trauma: </li></ul><ul><li>Foreign body ingestion </li></ul><ul><li>Inhalation injury </li></ul><ul><li>Burns </li></ul><ul><li>External trauma to neck </li></ul><ul><li>Post-intubation </li></ul>
  53. 53. Types of UAO <ul><li>Nasopharyngeal: </li></ul><ul><li>Adenoid hypertrophy </li></ul><ul><li>Choanal atresia </li></ul><ul><li>Midface hypoplasia </li></ul><ul><li>Oropharyngeal: </li></ul><ul><li>Macroglossia </li></ul><ul><li>Pharyngeal hypotonia </li></ul><ul><li>Cleft Lip/Palate </li></ul><ul><li>Laryngeal type </li></ul><ul><li>anomalies: </li></ul><ul><li>Laryngeal cleft </li></ul><ul><li>Vocal fold paralysis </li></ul><ul><li>Neoplasm </li></ul><ul><li>Laryngeal Web </li></ul><ul><li>Laryngomalacia </li></ul>
  54. 54. Role of Laryngeal-Pharyngeal Reflux and GERD in UAO <ul><li>Erythema </li></ul><ul><li>Post-glottic edema </li></ul><ul><li>Vocal fold edema </li></ul><ul><li>Decreased sensation </li></ul><ul><li>Do protonics reverse sensory deficits? </li></ul><ul><li>Kirby & Noel, 2007 </li></ul>
  55. 55. Case Study 1 Pediatric Patient with Congenital UAO: oropharyngeal and laryngeal types
  56. 56. Case 1 (timeline:13 months) <ul><li>Baby born at 41 6/7 weeks gestational age </li></ul><ul><li>3232 grams </li></ul><ul><li>Rapid progression of Persistent Pulmonary Hypertension (PPHN) </li></ul><ul><li>Dx Pneumothorax and underwent Thoracentisis </li></ul><ul><li>Intubated and on high frequency oscillator at FAHC </li></ul><ul><li>Transferred to CHB on Day of life (DOL) #2 for ECMO </li></ul>
  57. 57. A baby on ECMO
  58. 58. Case 1 Timeline <ul><li>DOL 0-2 at FAHC </li></ul><ul><li>DOL 2-16 at CHB </li></ul><ul><li>DOL 16 through 10/08/07 at FAHC </li></ul><ul><li>10/08/07-11/20/07 at CHB </li></ul><ul><li>11/20/07 readmitted to FAHC </li></ul>
  59. 59. Case 1 at FAHC <ul><li>First Feeding Team consult on DOL 19 </li></ul><ul><li>Baby with stridor at rest and feeding </li></ul><ul><li>Bronchoscopy revealing significant airway prolapse, laryngomalacia and vocal fold immobility </li></ul><ul><li>Baby on O2 nasal cannula </li></ul><ul><li>Weight loss </li></ul><ul><li>Breastfeeding attempts were made </li></ul>
  60. 60. Case 1 at CHB <ul><li>Baby re-intubated after trial CPAP, but worsening respiratory distress </li></ul><ul><li>Undergoes supraglottoplasty for severe laryngomalacia </li></ul><ul><li>Dx subglottic stenosis </li></ul><ul><li>Fails extubation </li></ul><ul><li>Tracheostomy (Shiley 3.5 uncuffed) </li></ul><ul><li>Ng tube for poor feeding and aspiration </li></ul>
  61. 61. Case 1 at FAHC <ul><li>Returns to Vermont for trach teaching </li></ul><ul><li>Does baby need G tube? </li></ul><ul><li>Cultural differences: Somalian family with 6 kids </li></ul><ul><li>Mother breast fed all children </li></ul><ul><li>Baby now showing signs of oral defensiveness </li></ul>
  62. 62. VFSS results reveal <ul><li>Discoordination of sucking skill </li></ul><ul><li>Trach= “open system” </li></ul><ul><li>Laryngeal penetration with thin and nectar thick liquid-what are the implications? </li></ul><ul><li>Microaspiration </li></ul><ul><li>Silent in nature </li></ul><ul><li>Oral defensiveness </li></ul><ul><li>GERD </li></ul>
  63. 63. VFSS: Looking at the whole picture <ul><li>Oral defensiveness </li></ul><ul><li>Poor growth </li></ul><ul><li>Recurrent clinical signs aspiration with breast milk exiting trach site during nippling </li></ul><ul><li>Baby undergoes G tube placement for supplemental feedings </li></ul>
  64. 64. Case 1 VFSS 2 Results Reveal <ul><li>5 weeks later </li></ul><ul><li>Trach </li></ul><ul><li>Passy Muir Speaking Valve in place </li></ul><ul><li>No documentation of aspiration with thin liquid </li></ul><ul><li>Balance PO (per mouth) and PG (per gavage) feedings </li></ul>
  65. 65. Case 1 Re-admitted to FAHC <ul><li>5 weeks later </li></ul><ul><li>Recurrent upper respiratory infections </li></ul><ul><li>Post-tussive emesis </li></ul><ul><li>Bilateral Infiltrates on CXR </li></ul><ul><li>Cultural differences: everyone eats out of same bowl with hands (and germs) </li></ul>
  66. 66. Case 1 Treatment Plan: “Interim Feeding Plan” <ul><li>When baby is healthy with no URI, emesis, PO feed thin liquids via bottle </li></ul><ul><li>If baby is ill, with URI, emesis, make temporary, alternate modifications to feeding. This could include NPO </li></ul><ul><li>Less conservative, would be nectar thick liquids and/or begin spoon feeding at 6 mo. </li></ul><ul><li>Rely on G tube as primary means of nutrition and hydration </li></ul>
  67. 67. Case 1: Happy Outcomes <ul><li>Vocal fold mobility returns </li></ul><ul><li>Baby decannulated </li></ul><ul><li>Full PO feeder of thin liquids via sippy cup and bottle and soft solids </li></ul><ul><li>Will keep G tube through winter months as back-up for “Interim Feeding Plan” </li></ul><ul><li>Achieving developing milestones in timely age-appropriate manner </li></ul>
  68. 68. What we learn (and questions to ask) <ul><li>Should breastfeeding be allowed if baby with s/s aspiration? </li></ul><ul><li>Is any aspiration allowed? </li></ul><ul><li>The importance of PMV to restore healthy upper airway and “closed system” </li></ul><ul><li>VFSS is only ONE window in time and only a small piece to our overall assessment </li></ul><ul><li>How to embrace and manage cultural differences </li></ul>
  69. 69. Case Study 2 Pediatric Patient with Congenital UOA: acute, oropharyngeal type
  70. 70. Case 2 (timeline: 9 months) <ul><li>10 month old admitted to FAHC with respiratory distress and neck edema </li></ul><ul><li>Tracheostomy </li></ul><ul><li>Biopsied revealed ganglioneuroblstoma </li></ul><ul><li>Baby received cycle of carboplatin and etoposide, which decreased size of tumor temporarily </li></ul><ul><li>Mass increased in size, compromising her airway and necessitating intubation </li></ul>
  71. 71. Case 2 at CHB <ul><li>Transferred to CHB for debulking of the mass </li></ul><ul><li>Subsequent pathology reveals a benign cervical right neck neuroectodermal lesion </li></ul><ul><li>Most notable complication after debulking is poor feeding </li></ul><ul><li>VFSS in Boston reveals silent aspiration of puree and all liquid consistencies </li></ul>
  72. 72. Case 2 <ul><li>MRI reveals residual tumor in posterior fossa and neck after debulking </li></ul><ul><li>Large enhancing mask in the right parapharyngeal space </li></ul><ul><li>Neurosurgery deferred further resection due to low growth potential and stable size on MRI </li></ul><ul><li>Sub total resection: implications for long term and feeding? </li></ul>
  73. 73. Case 2 VFSS results reveal <ul><li>Silent aspiration with purees and liquids </li></ul><ul><li>Delayed initiation of swallow </li></ul><ul><li>Significant pharyngeal weakness and poor pharyngeal constriction </li></ul><ul><li>Post-swallow residue </li></ul>
  74. 74. Case 2: Treatment plan <ul><li>NPO recommended to PCP </li></ul><ul><li>Baby underwent G tube placement given likely long-term nature of impairment </li></ul><ul><li>Parents extremely frustrated by NPO status </li></ul><ul><li>Oral Stimulation program and tactile play with textures in absence of PO intake </li></ul>
  75. 75. What we learn (and questions to ask) <ul><li>Understanding prognosis is essential to make recommendations </li></ul><ul><li>Is any aspiration allowed? </li></ul><ul><li>How do we balance the need for NPO status with developmental feeding needs and family desires to feed the child? </li></ul><ul><li>Is NPO a feasible and reasonable recommendation? </li></ul>
  76. 76. Case Study 3 Pediatric patient with Congenital UAO: laryngeal anomaly type
  77. 77. Case 3 (timeline: 3 months) <ul><li>Born at 41 weeks gestational age </li></ul><ul><li>4095 grams; LGA </li></ul><ul><li>C-section due to fetal distress </li></ul><ul><li>Respiratory distress at birth </li></ul><ul><li>Consulted Feeding Team at DOL 11 (recommended NPO) </li></ul><ul><li>Stridor </li></ul><ul><li>Intubated </li></ul>
  78. 78. Case 3 <ul><li>Feeding Team re-consulted 3 weeks later after successful extubation </li></ul><ul><li>s/p epiglottoplasty and supraglottoplasty for redundant tissue </li></ul><ul><li>Baby with weak cry, aphonic at times </li></ul><ul><li>S/s aspiration during nippling </li></ul><ul><li>Inspiratory stridor continues </li></ul><ul><li>MRI was normal </li></ul>
  79. 79. Laryngoscopy results reveal <ul><li>Larynx remarkable for enlarged arytenoid cartilages </li></ul><ul><li>Paradoxical VF movement with each inspiration </li></ul><ul><li>A-P collapse of trachea was observed dynamically </li></ul><ul><li>No clear pulsatile compression of trachea was seen </li></ul>
  80. 80. VFSS results reveal <ul><li>Day after laryngoscopy </li></ul><ul><li>Aspiration with thin liquids </li></ul><ul><li>No evidence of aspiration with nectar thick liquids </li></ul><ul><li>Ng tube in place </li></ul>
  81. 81. Case 3: Treatment Plan <ul><li>Recommendations for baby to nipple nectar thick liquids via slow flow nipple </li></ul><ul><li>PO ad lib when baby showing interest in nippling </li></ul><ul><li>Gavage feedings continue to supplement until adequate volumes consumed PO </li></ul><ul><li>Repeat VFSS in 6-8 weeks </li></ul>
  82. 82. Case 3: Differential Diagnosis <ul><li>Paradoxical Vocal Fold Movement (PVFM) </li></ul><ul><li>Known an a functional or non-organic disorder </li></ul><ul><li>Etiology suggests psychopathology </li></ul><ul><li>Laryngeal dystonia? </li></ul><ul><li>Neurogenic etiology? </li></ul>
  83. 83. Case 3: Literature review <ul><li>Heatley & Swift, 1996 report a case of 4 month old with PVCD related to underlying GERD </li></ul><ul><li>Omland & Brondbo, 2008 report 4 cases infant of BVCP vs PVCM </li></ul><ul><li>EMG can provide further diagnostic info if the laryngeal muscles have normal electrical activity </li></ul>
  84. 84. Case 3 <ul><li>PVFM classified as Cortical or Upper Motor Neuron OR Nuclear and Lower Motor Neuron </li></ul><ul><li>In infant with no clear organic etiology, can’t be classified under above </li></ul><ul><li>GERD </li></ul><ul><li>Laryngeal Dyskinesia (lack of abductory movement with inhalation) </li></ul>
  85. 85. Final Thoughts on what we learn… <ul><li>How do we best manage aspiration in pediatrics? </li></ul><ul><li>Least restrictive, safest diet </li></ul><ul><li>“ Do no Harm” principle </li></ul><ul><li>Is any aspiration allowed? </li></ul><ul><li>Colin Wallis, MD, Pediatric Pulmonologist, of Great Ormond Street Hospital asks “Is there damage from aspiration?” not is it occurring </li></ul>
  86. 86. Final Thoughts <ul><li>Upper airway obstruction is an area where the medical SLP needs expertise </li></ul><ul><li>Teaming with ENT, Pulmonary, GI, Radiology is essential </li></ul><ul><li>Continuing Education for SLP and always asking questions is essential </li></ul>
  87. 87. Esophageal Etiologies of Pediatric Dysphagia
  88. 88. South Shore Hospital <ul><li>Regional Medical Center SE MA </li></ul><ul><li>4,800 births/year </li></ul><ul><li>380 inpatient beds </li></ul><ul><li>Level 2 Trauma Center </li></ul><ul><li>10 Level 3 NICU </li></ul><ul><li>20 Level 2 NICU </li></ul><ul><li>Pediatric Floor </li></ul><ul><li>Outpatient Pediatric Feeding Clinic </li></ul><ul><li>Clinical Affiliations </li></ul>
  89. 89. Anatomy of the Esophagus <ul><li>Refer to the esophagus as the “proximal” and “distal” portions. </li></ul><ul><li>Proximal third of the esophagus- striated muscle </li></ul><ul><li>Distal third of the esophagus (and remainder of the gut)-smooth muscle </li></ul>
  90. 90. Anatomy & Physiology of the Esophagus <ul><li>After the upper esophageal sphincter relaxes, a reflexive, peristaltic “primary stripping wave” is initiated. </li></ul><ul><li>This propels the bolus distally </li></ul><ul><li>Amplitude of this wave is higher in the distal esophagus </li></ul><ul><li>Traveling at 3-5 cm per second (Rubenstein, J.) </li></ul>
  91. 91. Anatomy & Physiology of the Esophagus <ul><li>Stripping wave initiated by the oropharyngeal swallow causing the LES to relax </li></ul><ul><li>Remains open until the stripping wave has arrived at the LES </li></ul><ul><li>Secondary stripping waves originate from within the proximal esophagus and propel any residual bolus into the stomach </li></ul>
  92. 92. Esophageal Dysphagia Occurs When: <ul><li>Amplitude of the stripping wave is too low </li></ul><ul><li>Velocity of the wave is too slow or too fast </li></ul><ul><li>The wave is so fast the UES and LES contract simultaneously </li></ul><ul><li>The UES or LES fail to relax </li></ul><ul><li>There is mechanical obstruction that cannot be overcome by the stripping wave </li></ul>
  93. 93. Etiologies of Esophageal Dysphagia <ul><li>Differential diagnosis from oropharyngeal etiologies </li></ul><ul><li>Co-occurring oropharyngeal and esophageal etiology </li></ul><ul><li>Careful history is important </li></ul><ul><li>Child should be asked to “point” with one finger to the area they feel there is “hang up” of the bolus </li></ul>
  94. 94. Etiologies of Esophageal Dysphagia <ul><li>2 Main Etiologies of Esophageal Dysphagia </li></ul><ul><li>Mechanical Causes </li></ul><ul><li>Motility Disturbances </li></ul><ul><li>Food impaction is more frequent with mechanical causes </li></ul><ul><li>Patients with mechanical obstruction can typically still swallow liquids </li></ul><ul><li>More pronounced solid food dysphagia </li></ul>
  95. 95. Etiologies of Esophageal Dysphagia <ul><li>Mechanical disturbance- usually localize the “hang up” of the food distal to the larynx </li></ul><ul><li>Oropharyngeal etiology localize the “hang up” proximal to the larynx </li></ul><ul><li>Often not possible to determine etiology and hence diagnostic testing is indicated </li></ul><ul><li>If SLP finds evidence of an esophageal disorder a recommendation is made for appropriate referral to a specialist </li></ul>
  96. 96. Case Study of a Pediatric Patient with Mechanical Obstruction
  97. 97. VACTERL or VATER Association <ul><li>Acronym used to describe a series of characteristics which have been found to occur together </li></ul><ul><li>V =vertebrae, which are abnormal (70% patients) </li></ul><ul><li>A =anal atresia/ imperforate anus (55% patients) </li></ul><ul><li>C = cardiac defects (75% patients) </li></ul><ul><li>T= tracheoesophageal fistula (TEF), an abnormal connection between the trachea and the esophagus </li></ul><ul><li>E= esophageal atresia, (EA) the esophagus does not connect to the stomach </li></ul><ul><li>R= renal defects </li></ul><ul><li>L= limb (arm) defects </li></ul>
  98. 98. VACTERL Association <ul><li>Babies diagnosed with VACTERL have at least 3 or more of the anomalies. </li></ul><ul><li>Wide range of manifestations </li></ul><ul><li>EA with TEF occurs in 70% patients </li></ul><ul><li>EA/TEF can also occur as an isolated defect </li></ul><ul><ul><li>EA/TEF as an isolated condition occurs in 1 in 3,000-5,000 births </li></ul></ul><ul><li>Typically have normal development and intelligence </li></ul>
  99. 99. Esophageal Atresia (EA) & Tracheoesophageal Fistula (TEF) <ul><li>Presence of EA suspected in the post natal period </li></ul><ul><li>Newborn presents with excessive salivation </li></ul><ul><li>Coughing, choking, sneezing. </li></ul><ul><li>Upon oral feeding the infant struggles and may become cyanotic </li></ul><ul><li>Respiratory distress </li></ul>
  100. 100. EA & TEF <ul><li>Congenital EA represents failure of the esophagus to develop as a continuous passage. </li></ul><ul><li>TEF represents an abnormal opening between the trachea and the esophagus </li></ul><ul><li>DX is made, all oral feedings are stopped </li></ul><ul><li>Surgical repair within the first days of life </li></ul>
  101. 101. Case Study <ul><li>4 year 11 month old male with VACTERL Association </li></ul><ul><li>Followed since infancy through Swallowing Disorders Program </li></ul><ul><li>Post natal diagnosis of EA/TEF s/p surgical repair on DOL#3 </li></ul><ul><li>Cardiac diagnosis of Tetrology of Fallot s/p repair (multiple staged repairs) </li></ul><ul><li>G-tube placed in infancy s/p EA/TEF repair </li></ul>
  102. 102. Case Study <ul><li>History of aspiration with thin and nectar thick liquids on VFSS first identified at 6 months of age. </li></ul><ul><li>Cleared to orally feed purees only. </li></ul><ul><li>G-tube to meet hydration, nutritional needs </li></ul><ul><li>No improvement with swallow function over the years </li></ul><ul><li>Referred to CADD clinic at 3 years of age </li></ul>
  103. 103. Case Study <ul><li>Dx with type 1 laryngeal cleft (LC) </li></ul><ul><li>S/P endoscopic repair of LC at 3 years 6 months old </li></ul><ul><li>Pre-surgically patient eating diet of purees, soft dissolvables and solids (hamburger, pasta, grilled chicken, green beans) in limited volume. </li></ul><ul><li>All liquids via g-tube </li></ul>
  104. 104. Case Study <ul><li>6 months s/p LC repair patients VFSS revealed improvement in underlying swallow function </li></ul><ul><li>Patient cleared to take thin and nectar thick liquids </li></ul><ul><li>Patient co-existing sensory based feeding disorder </li></ul><ul><li>Volume of solids limited due to texture sensitivity, hyper-sensitive gag reflex </li></ul><ul><li>Reduced efficiency with chewing </li></ul><ul><li>Receiving motor/sensory based feeding therapy </li></ul>
  105. 105. Case Study <ul><li>Patient returns for repeat VFSS at 4 years 11 months due to complaints “food is getting stuck” </li></ul><ul><li>Patient points to area below the larynx/chest area </li></ul><ul><li>Mother describes patient as “forceful swallow” with facial grimacing, decreased interest in solids, decreased volume of solids </li></ul><ul><li>Asymptomatic with liquids </li></ul>
  106. 106. Videofluoroscopic Results Reveal: <ul><li>No evidence of aspiration with thin liquids </li></ul><ul><li>No evidence of aspiration with solids which pass from the pharynx through the UES </li></ul><ul><li>Normal pharyngeal phase of swallowing </li></ul><ul><li>(confirm this on video clip) </li></ul>
  107. 107. Videofluoroscopic Results Reveal: <ul><li>Esophageal phase reveals moderate anastomotic narrowing at the site of the EA repair </li></ul><ul><li>Piece of chicken impacted at the site of the anastomosis </li></ul><ul><li>Liquid wash downs required to clear the solid </li></ul><ul><li>Continued ingestion of solids can lead to “back up” </li></ul>
  108. 108. Treatment Plan <ul><li>Attending radiologist at VFSS paged the GI physician to discuss dx of a stricture </li></ul><ul><li>Patient placed on diet of liquids/purees only until surgical decision </li></ul><ul><li>Patient underwent dilatation of the esophagus </li></ul><ul><li>1 week s/p dilatation patient resumed and tolerated solid foods. </li></ul><ul><li>Avoid hard to chew solids & high risk choking foods </li></ul><ul><li>Avoid “gummy” foods </li></ul><ul><li>Smaller, more frequent meals </li></ul>
  109. 109. What we learn…….. <ul><li>We must look beyond the pharyngeal swallow </li></ul><ul><li>Most neonates with EA/TEF have some degree of esophageal dysmotility </li></ul><ul><li>Strictures at the site of the anastomosis are common and may require mulitple dilatations </li></ul>
  110. 110. What we learn…….. <ul><li>Solid food dysphagia often the first sign the stricture has re-occurred </li></ul><ul><li>As children mature they are “in tune” to their esophagus and can describe symptoms </li></ul><ul><li>Many older children will self-restrict their diet </li></ul><ul><li>VFSS is an effective procedure for identifying the stricture </li></ul>
  111. 111. What we learn…….. <ul><li>VFSS is only one instrumental evaluation that may give rise to esophageal dysphagia </li></ul><ul><li>Further diagnostic testing may include: </li></ul><ul><ul><li>Upper GI series </li></ul></ul><ul><ul><li>Esophagoduodenscopy (EGD) </li></ul></ul>
  112. 112. Case Study of a Pediatric Patient with Motility Disturbance
  113. 113. Case Study <ul><li>3 month old infant referred for VFSS by pediatric otolaryngologist (ORL) </li></ul><ul><li>Presenting symptoms coughing and choking during feeding </li></ul><ul><li>Mother reports “gasping” during bottle feeds </li></ul><ul><li>PCP placed infant on Zantac with no improvement in symptoms </li></ul>
  114. 114. VFSS Results <ul><li>Documentation of silent aspiration with thin, nectar thick and honey thick liquid </li></ul><ul><li>Build up of residue above the upper esophageal sphincter </li></ul><ul><li>Failure of the UES to relax </li></ul><ul><li>Aspiration results from a build-up/ spill over of liquid due to failure of the UES to relax </li></ul>
  115. 115. VFSS Results <ul><li>Coordination of suck swallow breathe altered </li></ul><ul><li>Airway protection is compromised </li></ul><ul><li>Co-occurrence of oropharyngeal dysphagia along with an esophageal phase dysphagia </li></ul>
  116. 116. Treatment Plan <ul><li>Attending radiologist at VFSS paged referring ORL physician </li></ul><ul><li>Patient made NPO by referring physician </li></ul><ul><li>Patient admitted to the hospital </li></ul><ul><li>GI service consulted </li></ul>
  117. 117. Cricopharyngeal Dysfunction <ul><li>Cricopharyngeal dysfunction as a cause of oropharyngeal dysphagia in infancy </li></ul><ul><li>Multi-phase dysphagia </li></ul><ul><li>Results from 2 conditions: </li></ul><ul><li>Failure of relaxation of the upper esophageal sphincter (UES) and/or </li></ul><ul><li>Cricopharyngeal incoordination </li></ul><ul><li>(Mihailocivc, T, & Perisic, V.N., 1992) </li></ul>
  118. 118. Cricopharyngeal dysfunction <ul><li>Manifested by swallowing difficulties </li></ul><ul><li>Nasopharyngeal regurgitation </li></ul><ul><li>Coughing, choking </li></ul><ul><li>Aspiration pneumonia </li></ul><ul><li>Diagnosis typically made by radiography and motility studies </li></ul><ul><li>Cricopharyngeal dysfunction caused by either primary UES motility disorder or </li></ul><ul><li>May be associated with central nervous system diseases </li></ul>
  119. 119. Case Study Con’t… <ul><li>Patient admitted to the hospital for nasogastric tube feedings </li></ul><ul><li>GI work up revealed cricopharyngeal achalasia </li></ul><ul><li>Patient underwent esophageal dilatation during admission </li></ul><ul><li>D/C home full oral feeder with resolution of symptoms </li></ul><ul><li>f/u with GI physician </li></ul>
  120. 120. Repeat VFSS <ul><li>8 weeks s/p dilatation patient returned for repeat VFSS </li></ul><ul><li>Full PO feeding of thin liquid via bottle in the interim </li></ul><ul><li>Spoon feeding had been initiated </li></ul><ul><li>No intermittent respiratory symptoms </li></ul><ul><li>VFSS reveals qualitative improvement but continued pooling at the UES </li></ul><ul><li>Aspiration with thin liquids documented </li></ul><ul><li>No aspiration with spoon feeding of purees but pooling </li></ul>
  121. 121. Treatment Plan <ul><li>Medical team met to discuss the case </li></ul><ul><li>Cleared to continue on thin liquids with pacing strategy in place </li></ul><ul><li>Alter pacifier for non-nutritive sucking to help clear the residue with subsequent swallows </li></ul><ul><li>Nectar thick liquids not indicated due to increased build up </li></ul><ul><li>Return to GI to discuss need for re-dilatation </li></ul>
  122. 122. Treatment Options for Cricopharyngeal Achalasia <ul><li>Cricopharyngeal myotomy </li></ul><ul><ul><li>Surgical division of the muscle </li></ul></ul><ul><ul><li>Renders the muscle inactive </li></ul></ul><ul><ul><li>Often as “last option” </li></ul></ul><ul><li>Dilatation </li></ul><ul><ul><li>Single balloon catheter dilatation proven to be successful </li></ul></ul><ul><ul><li>Less invasive than myotomy </li></ul></ul><ul><ul><li>Results apparent 24-48 after procedure </li></ul></ul><ul><ul><li>Patient may require repeat dilatations </li></ul></ul><ul><ul><li>Botox injection to the UES </li></ul></ul><ul><ul><li>More commonly used in the adult population </li></ul></ul><ul><ul><li>Efficacy of botox use to LES in pediatrics </li></ul></ul><ul><ul><li>Botox effect may wear off over time </li></ul></ul><ul><ul><li>Need to re-inject </li></ul></ul><ul><ul><li>Risk of injecting the wrong site/muscle </li></ul></ul>
  123. 123. Case study Con’t…. <ul><li>Different treatment modality trialed </li></ul><ul><li>Patient received Botox injection to the UES </li></ul><ul><li>Due to complications from Botox injection patient required hospital admission for NG tube placement </li></ul><ul><li>Decision was made for g-tube placement and repeat dilatation to the UES due to on-going dysphagia and aspiration </li></ul>
  124. 124. Case Study Con’t…. <ul><li>Improvement in underlying swallow function s/p second dilatation </li></ul><ul><li>Aspiration resolved </li></ul><ul><li>Patient progressed to mashed and soft table foods (1 year of age) </li></ul><ul><li>Soft solids pass through site of dilatation without hold up </li></ul><ul><li>Cricopharyngeal muscle still prominent but with improved coordination </li></ul>
  125. 125. What we learn <ul><li>ASHA Guidelines for Speech Language Pathologists Performing Videofluoroscopic Swallow Studies (2004) states </li></ul><ul><li>“ Clinicians should be aware that oropharyngeal swallow function is often altered in patients with esophageal motility disorders and dysphagia.” </li></ul><ul><li>“ SLP’s have knowledge and skills to recognize patient signs and symptoms associated with esophageal dysphagia </li></ul>
  126. 126. What we learn <ul><li>Results of the instrumental oropharyngeal swallow evaluation should include an esophageal screening. </li></ul><ul><li>Results of the esophageal screening may reveal a structural or motor abnormality suggesting that a referral to a Gastroenterologist may be indicated. (Easterling, 2007, Perspectives) </li></ul><ul><li>“ SLP’s should have sufficient knowledge of esophageal structure and function to make an appropriate referral and plan cooperative management </li></ul>
  127. 127. Seeking Resources <ul><li>Accessibility to a pediatric medical center that offers multidisciplinary team approach such as The Center for Aerodigestive Disorders. </li></ul><ul><li>Underscores the need for graduate training in pediatric dysphagia </li></ul><ul><li>Course work and clinical practicum </li></ul><ul><li>Collaboration among hospital and community based SLP’s for carry over of safe and successful feeding plan in the home setting. </li></ul>
  128. 128. Questions?
  129. 129. How to contact us: <ul><li>[email_address] </li></ul><ul><li>[email_address] </li></ul><ul><li>[email_address] </li></ul>
  130. 130. References <ul><li>ASHA 2008 Upper Airway Obstruction References </li></ul><ul><li>Andrews, T. Airway obstruction in craniofacial anomalies: In: Myer, C, Cotton, R, Shott, S. eds. The Pediatric Airway: An Interdisciplinary Approach, Philadelphia, PA: JB Lippencott; 1995:249. (Most recently published in Miller & Willging, 2007.) </li></ul><ul><li>Koufman, J & Block, C. American Journal of Speech-Language Pathology Vol.17 327-334 November 2008 </li></ul><ul><li>Omland, T, Brondbo, K (2008) Paradoxical vocal cord movement in newborn and congenital idiopathic vocal cord paralysis: two of a kind? European Archives of Otorhinolaryngology 265:803-807. </li></ul><ul><li>Mathers-Schmidt, B (2001) Paradoxical Vocal Fold Motion: A Tutorial on a Complex Disorder and the Speech-Language Pathologist’s Role. American Journal of Speech-language Pathology 10: 111-125. </li></ul><ul><li>Heatley, D, Swift, E (1996) Paradoxical vocal cord dysfunction in an infant with stridor and Gastroesophageal reflux. International Journal of Pediatric Otorhinolaryngology 34: 149-151. </li></ul><ul><li>Ibrahim, W, Gheriani H, Almohamed, A, Raza, T (2007) Paradoxical vocal cord motion disorder: past, present and future. 83: 164-172 </li></ul><ul><li>Haibeck, L, Mandell, D (2008) The Aerodigestive Clinic: Multidisciplinary Management of Pediatric Dysphagia. ASHA Division 13 Perspectives on Swallowing and Swallowing Disorders 17: 101-109. </li></ul><ul><li>Arvedson, J (2008) Food for Thought on Pediatric Feeding and Swallowing. ASHA Division 13 Perspectives on Swallowing and Swallowing Disorders 17: 110-118. </li></ul><ul><li>Miller, C, Willging, JP (2007) The Implications of Upper-Airway Obstruction on Successful Infant Feeding. Seminars in Speech and Language 28:190-203. </li></ul><ul><li>Zaichkin, J (2002) Newborn Intensive Care: What every parent needs to know. 2nd edition. NICU INK Book Publishers, Santa Rosa, CA. </li></ul><ul><li>Kirby, M, Noel, R. (2007) Nutrition and Gastrointestinal Tract Assessment and Management of Children with Dysphagia. Seminars In Speech and Language 28: 180-189. </li></ul>
  131. 131. References <ul><li>Arvedson, J. & Lefton-Greif, M. 1998. Pediatric Videofluoroscopic Swallow Studies: A Professional Manual with Caregiver Guidelines. San Antonio, TX: Communication Skill Builders </li></ul><ul><li>Rahbar, R. et al. The presentation and management of laryngeal cleft: A 10-year experience. Arch Otolaryngol Head Neck Surg. 2006; 132: 1336-1341. </li></ul><ul><li>Parsons, DS. et al. Type I posterior laryngeal clefts. Laryngoscope. 1998; 108: 403-410. </li></ul><ul><li>Watters K., J. Russel. Diagnosis and management of type I laryngeal cleft. International Journal of Pediatric Otorhinolaryngology. 2003; 67: 591-596. </li></ul><ul><li>Chien, W. et al. “Type I laryngeal cleft: Establishing a functional diagnostic and management algorithm.” International Journal of Pediatric Otorhinolaryngology. 2006, 70: 2073-2079. </li></ul><ul><li>Benjamin B. A Inglis. Minor congenital laryngeal clefts: diagnosis and classification. Ann Otol Rhinol Laryngol 1989; 98: 417-420. </li></ul><ul><li>Bosely, M. et al. The utility of fiberoptic endoscopic evaluation of swallowing (FEES) in diagnosing and treating children with Type I laryngeal clefts. International Journal of Pediatric Otorhinolaryngology. 2006; 70: 339-343. </li></ul>
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