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Esophageal Dysphagia: Pediatric Case Studies

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