Esophageal Dysphagia: Pediatric Case Studies

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Esophageal Dysphagia: Pediatric Case Studies

  1. 1. Esophageal Dysphagia: Pediatric Case Studies Ajay Kaul, MBBS, MD Associate Professor of Pediatrics Division of Pediatric Gastroenterology, Hepatology and Nutrition Cincinnati Children’s Hospital Medical Center
  2. 2. <ul><li>Structural/Anatomic: </li></ul><ul><li>Strictures (congenital strictures or acquired: foreign body or caustic ingestion, </li></ul><ul><li>gastroesophageal reflux, eosinophilic esophagitis, epidermolysis bullosa, TEF) </li></ul><ul><li>Extrinsic compression (aberrant subclavian artery, vascular ring, cardiomegaly) </li></ul><ul><li>Severe Kyphoscoliosis </li></ul><ul><li>Tight fundoplication </li></ul><ul><li>Mucosal (Inflammatory): </li></ul><ul><li>Eosinophilic esophagitis </li></ul><ul><li>Infection:Fungal (candida), Viral (CMV, Herpes, VZV) </li></ul><ul><li>Burns: coin/button battery / caustic ingestion, pill esophagitis </li></ul><ul><li>Neuromuscular (Motility): </li></ul><ul><li>Cricopharyngeal achalasia (with / without Chiari malformation) </li></ul><ul><li>Achalasia </li></ul><ul><li>Post TEF / EA, Fundoplication, cardiac surgery </li></ul><ul><li>Connective tissue diseases: dermatomyositis, scleroderma </li></ul><ul><li>Colonic interposition/ reverse gastroplasty or pull up for esophageal atresia </li></ul><ul><li>Functional/Sensory: </li></ul><ul><li>Visceral hyperalgesia </li></ul>ESOPHAGEAL DYSPHAGIA
  3. 3. PHYSICAL BEHAVIORAL FUNCTIONAL Its not all physical!!
  4. 5. Case #1 <ul><li>14 month old, exclusively breast fed, </li></ul><ul><li>neurodevelopmentally normal, oral aversion </li></ul><ul><li>history of significant reflux: fussy/cries/arches </li></ul><ul><li>tried frequent burping, upright after feeds, H2 B </li></ul><ul><li>Reflux Sx better but still not eating, referred to SLP </li></ul><ul><li>failed attempts at weaning and intro of baby foods </li></ul><ul><li>no weight gain over past 3-5 months </li></ul><ul><li>mom tired and exhausted from frequent nursing </li></ul><ul><li>wants to go back to work, feels like she failed </li></ul><ul><li>Upper GI contrast study normal (with NG tube) </li></ul><ul><li>VFSS: only 2 swallows evaluated, “normal” </li></ul><ul><li>Hospitalized for NG feeds: continued Sx: NJ feeds </li></ul><ul><li>Doc wants infant evaluated for a fundo/G-tube </li></ul>
  5. 6. Case #1 <ul><li>Questions: </li></ul><ul><li>Is he safe to take oral feeds? </li></ul><ul><li>Is this GERD? </li></ul><ul><li>What is your next step: empiric therapies </li></ul><ul><li>or further investigations? </li></ul><ul><li>3. What empiric therapies? </li></ul><ul><li>4. What investigations? </li></ul><ul><li>5. Is it behavioral? </li></ul>
  6. 7. Case #1 <ul><ul><li>Is he safe to take oral feeds? </li></ul></ul><ul><ul><ul><li>probably safe as did well on </li></ul></ul></ul><ul><ul><ul><li>breast feeds without coughing/choking </li></ul></ul></ul><ul><ul><ul><li>Neurodevelopmentally normal </li></ul></ul></ul><ul><ul><ul><li>No history of pneumonias </li></ul></ul></ul><ul><ul><ul><li>UGI showed no structural abnormality, </li></ul></ul></ul><ul><ul><ul><li>normal stripping waves in esophagus </li></ul></ul></ul><ul><ul><ul><li>Continue nursing (no need for NPO) </li></ul></ul></ul><ul><ul><ul><li>Concerns: lack of weight gain and </li></ul></ul></ul><ul><ul><ul><li>mother’s condition </li></ul></ul></ul>
  7. 8. Case #1 <ul><li>2. Is this GERD? </li></ul><ul><ul><li>Most likely </li></ul></ul><ul><ul><li>Did not respond to traditional therapy </li></ul></ul><ul><ul><li>Definitely not a ‘happy spitter’ </li></ul></ul><ul><ul><li>3. What is your next step? </li></ul></ul><ul><ul><li>FEES to look at VF, airway protective </li></ul></ul><ul><ul><li>reflexes, sensation </li></ul></ul><ul><ul><li>Endoscopy to rule out esophagitis </li></ul></ul><ul><ul><li>(GERD vs other causes) </li></ul></ul><ul><ul><li>Impedance-pH study to characterize </li></ul></ul><ul><ul><li>GER </li></ul></ul>
  8. 9. Case #1 <ul><li>Endoscopy showed normal mucosa </li></ul><ul><li>& a patulous lower esophageal </li></ul><ul><li>sphincter, no esophagitis </li></ul><ul><li>Impedance-pH study was normal </li></ul><ul><li>with no evidence for esophageal </li></ul><ul><li>dysmotility </li></ul>
  9. 10. Case #1 <ul><li>Is GERD still the cause? </li></ul><ul><li>Why is the infant still orally </li></ul><ul><li>aversive? </li></ul>
  10. 11. Pain Pathway Acid Receptor Primary Afferent Neuron Esophagus
  11. 12. Acid contact with esophageal mucosa Peripheral Sensitization of PAN Alteration in Dorsal Horn neurons Change in DH outlasts duration of injurious stimulus ‘ Central Sensitization’ <ul><li>reduced activation threshold of DH neurons to </li></ul><ul><li>a given sensory stimulus </li></ul><ul><li>increase in responsiveness of DH neurons to </li></ul><ul><li>a given sensory input </li></ul><ul><li>enlargement of the receptive field for sensory input </li></ul><ul><li>Previously innocuous sensory input evokes pain </li></ul><ul><li>VISCERAL HYPERSENSITIVITY </li></ul>
  12. 13. Case #1 <ul><li>Does this infant need more therapy/meds </li></ul><ul><li>or fundo/G tube? </li></ul><ul><ul><li>Trial on a PPI, continued therapy </li></ul></ul><ul><ul><li>Scheduled for fundo with G tube in </li></ul></ul><ul><ul><li>2 weeks </li></ul></ul>
  13. 14. Case #1 <ul><li>4. Is there a behavioral component? </li></ul><ul><ul><li>likely ‘learned response’ playing role </li></ul></ul><ul><ul><li>may benefit from feeding therapy </li></ul></ul>
  14. 15. Case #1 <ul><li>Follow-up: </li></ul><ul><li>Few days after starting on a PPI </li></ul><ul><li>(2 mg/kg/dose) twice a day (off label), & </li></ul><ul><li>with continued therapy, the symptoms </li></ul><ul><li>started to resolve and the infant appeared </li></ul><ul><li>to be happier and started accepting some </li></ul><ul><li>stage 1 baby foods with slow weight gain </li></ul><ul><li>Collective decision was made to hold off </li></ul><ul><li>on the fundo/G tube surgery </li></ul>
  15. 17. Case #2 <ul><li>12 month old infant with spastic CP, Sz dis, </li></ul><ul><li>has a tracheostomy, retching and gagging </li></ul><ul><li>with bolus g-tube feeds, not gaining weight </li></ul><ul><li>G-tube, fundoplication, pyloroplasty </li></ul><ul><li>at 6 months age </li></ul><ul><li>used to nurse before surgery but now </li></ul><ul><li>orally aversive & exclusively G-tube fed </li></ul><ul><li>anterior loss and drooling saliva </li></ul><ul><li>some swallows noted </li></ul><ul><li>parents interested to push oral feeds </li></ul>
  16. 18. Case #2 <ul><li>Questions: </li></ul><ul><li>Is he safe to take oral feeds? </li></ul><ul><li>How will you assess safety of swallow? </li></ul><ul><li>What should your next step be? </li></ul><ul><li>Why is the infant retching and gagging now? </li></ul><ul><li>How can we decrease the retching and gagging? </li></ul><ul><li>Are there behavioral issues involved or is it all a physical problem? </li></ul>
  17. 19. Case #2 <ul><li>Is he safe to take oral feeds? </li></ul><ul><ul><li>how alert is he at baseline? </li></ul></ul><ul><ul><li>how frequent are his seizures? </li></ul></ul><ul><ul><li>what medications is he on? </li></ul></ul><ul><ul><li>is his neuro status deteriorating? </li></ul></ul><ul><ul><li>does he show signs of aspiration: </li></ul></ul><ul><ul><li>coughing/choking/wet sounding/ </li></ul></ul><ul><ul><li>tracheal suctioning? </li></ul></ul><ul><ul><li>is there history of pneumonia ? </li></ul></ul>
  18. 20. Case #2 <ul><li>Is he safe to take oral feeds? </li></ul><ul><ul><li>how alert is he at baseline? Alert </li></ul></ul><ul><ul><li>how frequent are his seizures? Rare </li></ul></ul><ul><ul><li>what medications is he on? No change </li></ul></ul><ul><ul><li>is his neuro status deteriorating? No </li></ul></ul><ul><ul><li>does he show signs of aspiration: </li></ul></ul><ul><ul><li>coughing/choking/wet sounding/ No </li></ul></ul><ul><ul><li>tracheal suctioning? </li></ul></ul><ul><ul><li>is there history of pneumonia? No </li></ul></ul>
  19. 21. Case #2 <ul><li>2. How will you assess safety of swallow? </li></ul><ul><ul><ul><li>Clinical exam </li></ul></ul></ul><ul><ul><ul><li>VFSS </li></ul></ul></ul><ul><ul><ul><li>FEES </li></ul></ul></ul><ul><ul><ul><li>Dye test: put few drops of food coloring in mouth and check for colored secretions at trach site </li></ul></ul></ul>
  20. 22. Case #2 <ul><li>2. How will you assess safety of swallow? </li></ul><ul><ul><ul><li>Clinical exam: few swallows, drooling, refuses tastes </li></ul></ul></ul><ul><ul><ul><li>VFSS: not possible </li></ul></ul></ul><ul><ul><ul><li>FEES: VF movement normal, some pooling and penetration of secretions, normal sensation with no aspiration </li></ul></ul></ul><ul><ul><ul><li>Dye test: no colored secretions suctioned from tracheostomy </li></ul></ul></ul>
  21. 23. Case #2 <ul><li>3. What is your next step? </li></ul><ul><ul><li>Start therapy </li></ul></ul><ul><ul><li>Address retching and gagging </li></ul></ul><ul><ul><li>Both </li></ul></ul>
  22. 24. Case #2 <ul><li>4. Why is the infant retching and gagging? </li></ul><ul><ul><li>“ Vagal Pinch” from fundo </li></ul></ul><ul><ul><li>Gas bloat: inability to vent air </li></ul></ul><ul><ul><li>Volume intolerance from smaller stomach capacity </li></ul></ul><ul><ul><li>Loss of fundal accomodation reflex (passive relaxation of fundus to accommodate feed) </li></ul></ul><ul><ul><li>Delayed stomach emptying of feeds </li></ul></ul><ul><ul><li>Dumping of liquids (especially with pyloroplasty) </li></ul></ul>
  23. 25. Fundoplication loss of fundus results in smaller volume of stomach and decreased capacity of stomach to relax and accommodate a large meal
  24. 26. Post-cardiac surgery Post-fundo Vagal nerve trauma <ul><li>delayed gastric emptying </li></ul><ul><li>dumping of liquids </li></ul><ul><li>esophageal dysmotility </li></ul><ul><li>vocal fold dysfunction </li></ul>
  25. 27. Case #2 Dumping: rapid movement of liquid feed into intestines hyperglycemia increased insulin production hypoglycemia Counter-regulatory hormones (including epinephrine) Retching, gagging, jittery, sweating, tachycardia, cramps
  26. 28. Retching/Gagging with feeds Oral Aversion
  27. 29. Retching/Gagging with feeds Oral Aversion
  28. 30. Case #2 <ul><li>5. How can we decrease the retching and gagging? </li></ul><ul><ul><li>Try continuous G tube feeds (not bolus) </li></ul></ul><ul><ul><li>Pureed feeds by G tube </li></ul></ul><ul><ul><li>Dumping: formula containing complex carbs (starches) </li></ul></ul><ul><ul><li>Venting of stomach </li></ul></ul><ul><ul><li>Prokinetic Medications: erythromycin, domperidone, other </li></ul></ul>
  29. 31. Case #2 <ul><li>Failed dietary manipulations & meds: </li></ul><ul><ul><li>Upper GI contrast study </li></ul></ul><ul><ul><li>Endoscopy </li></ul></ul><ul><ul><li>Impedance-pH study </li></ul></ul>
  30. 32. Case #2 <ul><li>Upper GI contrast study: </li></ul><ul><ul><li>fundoplication wrap noted </li></ul></ul><ul><ul><li>paraesophageal hernia </li></ul></ul><ul><ul><li>noted to retch and gag after barium bolus given through G tube </li></ul></ul>
  31. 33. Paraesophageal Hernia CHEST ABDOMEN
  32. 34. Case #2
  33. 36. Case #2 <ul><li>EGD: </li></ul><ul><ul><li>fundoplication and G tube noted </li></ul></ul><ul><ul><li>paraesophageal hernia present </li></ul></ul><ul><ul><li>otherwise normal mucosa </li></ul></ul>
  34. 37. Paraesophageal Hernia
  35. 38. Fundoplication Retching/Gagging Paraesophageal Hernia Oral Aversion
  36. 39. Tight Fundoplication
  37. 40. Balloon dilation
  38. 41. Impedance –pH study <ul><li>no episodes of reflux noted during </li></ul><ul><li>24 hour study on bolus feeds </li></ul><ul><li>retching/gagging not related to reflux </li></ul><ul><li>dysmotility noted on waveform with </li></ul><ul><li>swallows </li></ul>
  39. 43. Case #2 <ul><li>6. Is there a behavioral component to Sx? </li></ul><ul><ul><li>most cases have a behavioral overlap </li></ul></ul><ul><ul><li>‘ learned behavior’ imprinting </li></ul></ul><ul><ul><li>Sx may not completely resolve after </li></ul></ul><ul><ul><li>addressing the primary physical cause </li></ul></ul><ul><ul><li>overtime, as oral intake improves </li></ul></ul><ul><ul><li>without discomfort, the brain ‘unlearns’ </li></ul></ul><ul><ul><li>negative behaviors </li></ul></ul>
  40. 44. Case #2: follow-up <ul><li>paraesophageal hernia was repaired </li></ul><ul><li>started tolerating continuous then bolus </li></ul><ul><li>G-tube feeds, later on pureed diet </li></ul><ul><li>by G-tube </li></ul><ul><li>gaining weight </li></ul>
  41. 46. Case #3 <ul><li>2 yr old M, only taking smooth textures x 6mo </li></ul><ul><li>gags with solids especially meats </li></ul><ul><li>was taking table foods until 18 months until he </li></ul><ul><li>choked on a hot-dog </li></ul><ul><li>hx of eczema, asthma, allergies </li></ul><ul><li>no pneumonias; no hx of FB ingestion </li></ul><ul><li>neurodevelopmentally normal </li></ul><ul><li>occ choking/gagging then vomiting in AM </li></ul><ul><li>gaining weight </li></ul><ul><li>Test Feed: self-fed liquids and pureed without </li></ul><ul><li>problem, but refused meats </li></ul>
  42. 47. Case #3 <ul><li>Questions: </li></ul><ul><li>Is this behavioral (learned response)? </li></ul><ul><li>Is he safe to take oral feeds? </li></ul><ul><li>Is there a physical cause for dysphagia? </li></ul><ul><li>Is it oro-pharyngeal or esophageal </li></ul><ul><li>dysphagia? </li></ul><ul><li>5. What is your next step? </li></ul>
  43. 48. Case #3 <ul><li>Is this behavioral (learned response)? </li></ul><ul><ul><li>Most likely a component </li></ul></ul><ul><ul><li>Not sure ‘sole’ problem </li></ul></ul>
  44. 49. Case #3 <ul><li>2. Is he safe to eat by mouth? </li></ul><ul><ul><li>Most likely safe for the foods </li></ul></ul><ul><ul><li>he is taking </li></ul></ul><ul><ul><li>Most likely not aspirating </li></ul></ul>
  45. 50. Case #3 <ul><li>3. Is there a physical cause for dysphagia: </li></ul><ul><li>oropharyngeal or esophageal? </li></ul><ul><ul><li>Probably </li></ul></ul><ul><ul><li>Not likely CNS disorder </li></ul></ul><ul><ul><li>Most likely esophageal </li></ul></ul>
  46. 51. Case #3 <ul><li>5. What would you do next? </li></ul><ul><ul><li>VFSS </li></ul></ul><ul><ul><li>FEES </li></ul></ul><ul><ul><li>Neck/Chest X-Ray </li></ul></ul><ul><ul><li>Upper GI contrast study </li></ul></ul><ul><ul><li>EGD (endoscopy) </li></ul></ul><ul><ul><li>CT scan of neck/chest </li></ul></ul>
  47. 52. Case #3 <ul><li>5. What would you do next? </li></ul><ul><ul><li>VFSS </li></ul></ul><ul><ul><li>FEES </li></ul></ul><ul><ul><li>Neck/Chest X-Ray </li></ul></ul><ul><ul><li>Esophagram/UGI: normal </li></ul></ul><ul><ul><li>EGD (endoscopy): abnormal </li></ul></ul><ul><ul><li>CT scan of neck/chest </li></ul></ul>
  48. 53. Eosinophilic Esophagitis Esophageal Bx: typical features of EE
  49. 54. Case #3: Follow up <ul><li>started on swallowed fluticasone, PPI </li></ul><ul><li>skin allergy test identified no food allergens </li></ul><ul><li>repeat EGD in 3 months showed resolution </li></ul><ul><li>of EE changes </li></ul><ul><li>oral intake of solids improved over next few </li></ul><ul><li>months and by 6 months was taking meats </li></ul><ul><li>without any dysphagia </li></ul><ul><li>did not receive any OT/ST/BT </li></ul>
  50. 55. <ul><ul><li>                                                   Eosinophilic esophagitis in infants and toddlers with feeding disorders Pentiuk SP , Miller CK , Kaul A . </li></ul></ul>Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio 45229 Dysphagia. 2007 Jan;22(1):44-8. Epub 2006 Oct 6.
  51. 56. Challenges in the Management of Pediatric Dysphagia <ul><li>gaps in knowledge, field still evolving, current </li></ul><ul><li>information mostly extrapolated from adult studies </li></ul><ul><li>clinical research in pediatrics, ethics </li></ul><ul><li>growth and development: a dynamic process </li></ul><ul><li>of changing anatomy and physiology </li></ul><ul><li>what is normal? </li></ul><ul><li>congenital abnormalities increase complexity </li></ul><ul><li>history is second-hand, affected by parental </li></ul><ul><li>perspectives </li></ul><ul><li>pt. cooperation during exam, investigations and </li></ul><ul><li>therapy suboptimal: ? reliability of results </li></ul><ul><li>parental factors: concepts and myths, </li></ul><ul><li>socio-economic and cultural factors, expectations </li></ul>
  52. 57. Interdisciplinary approach in the management of Pediatric Dysphagia <ul><li>Physicians </li></ul><ul><li>Nurses </li></ul><ul><li>Therapists: SLP, OT, Behavioral </li></ul><ul><li>Dieticians </li></ul><ul><li>Social workers, Interpreters </li></ul><ul><li>Support (office) staff </li></ul>
  53. 58. Child Is NOT a Compressed Adult ‘ Mini Me’ I have a different set of issues!!
  54. 59. That’s a wrap!

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