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Esophageal Dysphagia: Pediatric Case Studies
 

Esophageal Dysphagia: Pediatric Case Studies

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    Esophageal Dysphagia: Pediatric Case Studies Esophageal Dysphagia: Pediatric Case Studies Presentation Transcript

    • 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
      • 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
    • PHYSICAL BEHAVIORAL FUNCTIONAL Its not all physical!!
    •  
    • 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
    • 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?
    • 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
    • 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
    • Case #1
      • Endoscopy showed normal mucosa
      • & a patulous lower esophageal
      • sphincter, no esophagitis
      • Impedance-pH study was normal
      • with no evidence for esophageal
      • dysmotility
    • Case #1
      • Is GERD still the cause?
      • Why is the infant still orally
      • aversive?
    • Pain Pathway Acid Receptor Primary Afferent Neuron Esophagus
    • 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
    • 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
    • Case #1
      • 4. Is there a behavioral component?
        • likely ‘learned response’ playing role
        • may benefit from feeding therapy
    • 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
    •  
    • 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
    • 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?
    • 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 ?
    • 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
    • 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
    • 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
    • Case #2
      • 3. What is your next step?
        • Start therapy
        • Address retching and gagging
        • Both
    • 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)
    • Fundoplication loss of fundus results in smaller volume of stomach and decreased capacity of stomach to relax and accommodate a large meal
    • Post-cardiac surgery Post-fundo Vagal nerve trauma
      • delayed gastric emptying
      • dumping of liquids
      • esophageal dysmotility
      • vocal fold dysfunction
    • 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
    • Retching/Gagging with feeds Oral Aversion
    • Retching/Gagging with feeds Oral Aversion
    • 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
    • Case #2
      • Failed dietary manipulations & meds:
        • Upper GI contrast study
        • Endoscopy
        • Impedance-pH study
    • Case #2
      • Upper GI contrast study:
        • fundoplication wrap noted
        • paraesophageal hernia
        • noted to retch and gag after barium bolus given through G tube
    • Paraesophageal Hernia CHEST ABDOMEN
    • Case #2
    •  
    • Case #2
      • EGD:
        • fundoplication and G tube noted
        • paraesophageal hernia present
        • otherwise normal mucosa
    • Paraesophageal Hernia
    • Fundoplication Retching/Gagging Paraesophageal Hernia Oral Aversion
    • Tight Fundoplication
    • Balloon dilation
    • 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
    •  
    • 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
    • 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
    •  
    • 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
    • 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?
    • Case #3
      • Is this behavioral (learned response)?
        • Most likely a component
        • Not sure ‘sole’ problem
    • Case #3
      • 2. Is he safe to eat by mouth?
        • Most likely safe for the foods
        • he is taking
        • Most likely not aspirating
    • Case #3
      • 3. Is there a physical cause for dysphagia:
      • oropharyngeal or esophageal?
        • Probably
        • Not likely CNS disorder
        • Most likely esophageal
    • 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
    • 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
    • Eosinophilic Esophagitis Esophageal Bx: typical features of EE
    • 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
        •                                                    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.
    • 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
    • Interdisciplinary approach in the management of Pediatric Dysphagia
      • Physicians
      • Nurses
      • Therapists: SLP, OT, Behavioral
      • Dieticians
      • Social workers, Interpreters
      • Support (office) staff
    • Child Is NOT a Compressed Adult ‘ Mini Me’ I have a different set of issues!!
    • That’s a wrap!