Esophageal Dysphagia: Esophageal Dysphagia: Pediatric Case StudiesPediatric Case Studies

Ajay Kaul, MBBS, MDAjay Kaul, MBBS, MDAssociate Professor of PediatricsAssociate Professor of Pediatrics

Division of Pediatric Gastroenterology, Hepatology and NutritionDivision of Pediatric Gastroenterology, Hepatology and NutritionCincinnati Children’s Hospital Medical CenterCincinnati 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):Mucosal (Inflammatory):• Eosinophilic esophagitis• Infection:Fungal (candida), Viral (CMV, Herpes, VZV)• Burns: coin/button battery / caustic ingestion, pill esophagitis

Neuromuscular (Motility):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:Functional/Sensory:• Visceral hyperalgesia

ESOPHAGEAL DYSPHAGIAESOPHAGEAL DYSPHAGIA

PHYSICALPHYSICAL BEHAVIORALBEHAVIORAL

FUNCTIONALFUNCTIONAL

Its not all physical!!Its not all physical!!

Case #1Case #1• 14 month old, exclusively breast fed, 14 month old, exclusively breast fed, neurodevelopmentally normal, oral aversionneurodevelopmentally normal, oral aversion• history of significant reflux: fussy/cries/archeshistory of significant reflux: fussy/cries/arches• tried frequent burping, upright after feeds, H2 Btried frequent burping, upright after feeds, H2 B• Reflux Sx better but still not eating, referred to SLPReflux Sx better but still not eating, referred to SLP• failed attempts at weaning and intro of baby foodsfailed attempts at weaning and intro of baby foods• no weight gain over past 3-5 monthsno weight gain over past 3-5 months• mom tired and exhausted from frequent nursingmom tired and exhausted from frequent nursing wants to go back to work, feels like she failedwants to go back to work, feels like she failed• Upper GI contrast study normal (with NG tube)Upper GI contrast study normal (with NG tube)• VFSS: only 2 swallows evaluated, “normal” VFSS: only 2 swallows evaluated, “normal” • Hospitalized for NG feeds: continued Sx: NJ feedsHospitalized for NG feeds: continued Sx: NJ feeds• Doc wants infant evaluated for a fundo/G-tubeDoc wants infant evaluated for a fundo/G-tube

Case #1Case #1Questions:Questions:1.1. Is he safe to take oral feeds?Is he safe to take oral feeds?2.2. Is this GERD? Is this GERD? 3.3. What is your next step: empiric therapies What is your next step: empiric therapies or further investigations?or further investigations?3. What empiric therapies?3. What empiric therapies?4. What investigations?4. What investigations?5. Is it behavioral?5. Is it behavioral?

Case #1Case #11.1. Is he safe to take oral feeds?Is he safe to take oral feeds?

• probably safe as did well on probably safe as did well on breast feeds without coughing/chokingbreast feeds without coughing/choking• Neurodevelopmentally normalNeurodevelopmentally normal• No history of pneumoniasNo history of pneumonias• UGI showed no structural abnormality,UGI showed no structural abnormality, normal stripping waves in esophagusnormal stripping waves in esophagus• Continue nursing (no need for NPO)Continue nursing (no need for NPO)• Concerns: lack of weight gain andConcerns: lack of weight gain and mother’s conditionmother’s condition

Case #1Case #12. Is this GERD?2. Is this GERD?

• Most likelyMost likely• Did not respond to traditional therapyDid not respond to traditional therapy• Definitely not a ‘happy spitter’Definitely not a ‘happy spitter’

3. What is your next step?3. What is your next step?• FEES to look at VF, airway protective FEES to look at VF, airway protective reflexes, sensationreflexes, sensation• Endoscopy to rule out esophagitis Endoscopy to rule out esophagitis (GERD vs other causes) (GERD vs other causes) • Impedance-pH study to characterizeImpedance-pH study to characterize GER GER

Case #1Case #1• Endoscopy showed normal mucosa Endoscopy showed normal mucosa & a patulous lower esophageal& a patulous lower esophageal sphincter, no esophagitissphincter, no esophagitis

• Impedance-pH study was normalImpedance-pH study was normal with no evidence for esophageal with no evidence for esophageal dysmotilitydysmotility

Case #1Case #1

1.1. Is GERD still the cause?Is GERD still the cause?

2.2. Why is the infant still orallyWhy is the infant still orally aversive?aversive?

Pain PathwayPain Pathway

AcidAcid ReceptorReceptor

Primary Afferent NeuronPrimary Afferent Neuron

EsophagusEsophagus

Acid contact with esophageal mucosaAcid contact with esophageal mucosa

Peripheral Sensitization of PANPeripheral Sensitization of PAN

Alteration in Dorsal Horn neurons Alteration in Dorsal Horn neurons

Change in DH Change in DH outlastsoutlasts duration of injurious stimulus duration of injurious stimulus

‘‘Central Sensitization’Central Sensitization’• reduced reduced activation thresholdactivation threshold of DH neurons to of DH neurons to a given sensory stimulus a given sensory stimulus • increase in increase in responsivenessresponsiveness of DH neurons to of DH neurons to a given sensory inputa given sensory input• enlargement enlargement of the receptive field for sensory inputof the receptive field for sensory inputPreviously innocuous sensory input evokes painPreviously innocuous sensory input evokes pain

VISCERAL HYPERSENSITIVITYVISCERAL HYPERSENSITIVITY

Case #1Case #1Does this infant need more therapy/meds Does this infant need more therapy/meds or fundo/G tube?or fundo/G tube?

• Trial on a PPI, continued therapyTrial on a PPI, continued therapy• Scheduled for fundo with G tube in Scheduled for fundo with G tube in 2 weeks2 weeks

Case #1Case #14. Is there a behavioral component?4. Is there a behavioral component?

• likely ‘learned response’ playing rolelikely ‘learned response’ playing role• may benefit from feeding therapymay benefit from feeding therapy

Case #1Case #1Follow-up:Follow-up:• Few days after starting on a PPI Few days after starting on a PPI (2 mg/kg/dose) twice a day (off label), & (2 mg/kg/dose) twice a day (off label), & with continued therapy, the symptoms with continued therapy, the symptoms started to resolve and the infant appearedstarted to resolve and the infant appeared to be happier and started accepting some to be happier and started accepting some stage 1 baby foods with slow weight gainstage 1 baby foods with slow weight gain• Collective decision was made to hold offCollective decision was made to hold off on the fundo/G tube surgeryon the fundo/G tube surgery

Case #2Case #2• 12 month old infant with spastic CP, Sz dis,12 month old infant with spastic CP, Sz dis, has a tracheostomy, retching and gagginghas a tracheostomy, retching and gagging with bolus g-tube feeds, not gaining weightwith bolus g-tube feeds, not gaining weight• G-tube, fundoplication, pyloroplastyG-tube, fundoplication, pyloroplasty at 6 months ageat 6 months age• used to nurse before surgery but nowused to nurse before surgery but now orally aversive & exclusively G-tube fedorally aversive & exclusively G-tube fed• anterior loss and drooling salivaanterior loss and drooling saliva• some swallows notedsome swallows noted• parents interested to push oral feedsparents interested to push oral feeds

Case #2Case #2Questions:Questions:

1.1. Is he safe to take oral feeds?Is he safe to take oral feeds?2.2. How will you assess safety of swallow?How will you assess safety of swallow?3.3. What should your next step be?What should your next step be?4.4. Why is the infant retching and gagging now?Why is the infant retching and gagging now? 5.5. How can we decrease the retching and How can we decrease the retching and

gagging?gagging?6.6. Are there behavioral issues involved or is it all Are there behavioral issues involved or is it all

a physical problem?a physical problem?

Case #2Case #21. Is he safe to take oral feeds?Is he safe to take oral feeds?

• how alert is he at baseline? how alert is he at baseline? • how frequent are his seizures? how frequent are his seizures? • what medications is he on? what medications is he on? • is his neuro status deteriorating?is his neuro status deteriorating?• does he show signs of aspiration:does he show signs of aspiration: coughing/choking/wet sounding/ coughing/choking/wet sounding/ tracheal suctioning?tracheal suctioning?• is there history of pneumoniais there history of pneumonia?

Case #2Case #21.1. Is he safe to take oral feeds?Is he safe to take oral feeds?

• how alert is he at baseline? how alert is he at baseline? AlertAlert• how frequent are his seizures? how frequent are his seizures? RareRare• what medications is he on? what medications is he on? No changeNo change• is his neuro status deteriorating? is his neuro status deteriorating? NoNo• does he show signs of aspiration:does he show signs of aspiration: coughing/choking/wet sounding/ coughing/choking/wet sounding/ NoNo tracheal suctioning?tracheal suctioning?• is there history of pneumonia? is there history of pneumonia? NoNo

Case #2Case #2 2. How will you assess safety of swallow?2. How will you assess safety of swallow?

• Clinical examClinical exam• VFSSVFSS• FEESFEES• Dye test: put few drops of food coloring Dye test: put few drops of food coloring

in mouth and check for colored in mouth and check for colored secretions at trach sitesecretions at trach site

Case #2Case #2 2. How will you assess safety of swallow?2. How will you assess safety of swallow?

• Clinical exam: Clinical exam: few swallows, drooling, few swallows, drooling, refuses tastesrefuses tastes

• VFSS: VFSS: not possiblenot possible• FEES: FEES: VF movement normal, some VF movement normal, some

pooling and penetration of secretions, pooling and penetration of secretions, normal sensation with no aspirationnormal sensation with no aspiration

• Dye test: Dye test: no colored secretions suctioned no colored secretions suctioned from tracheostomyfrom tracheostomy

Case #2Case #2 3. What is your next step?3. What is your next step?

• Start therapyStart therapy• Address retching and gaggingAddress retching and gagging• BothBoth

Case #2Case #2 4. Why is the infant retching and gagging?4. Why is the infant retching and gagging?

• ““Vagal Pinch” from fundoVagal Pinch” from fundo• Gas bloat: inability to vent airGas bloat: inability to vent air• Volume intolerance from smaller Volume intolerance from smaller

stomach capacitystomach capacity• Loss of fundal accomodation reflex Loss of fundal accomodation reflex

(passive relaxation of fundus to (passive relaxation of fundus to accommodate feed)accommodate feed)

• Delayed stomach emptying of feedsDelayed stomach emptying of feeds• Dumping of liquids (especially with Dumping of liquids (especially with

pyloroplasty)pyloroplasty)

FundoplicationFundoplication

loss of fundus results in smaller volume of loss of fundus results in smaller volume of stomach and decreased capacity of stomach stomach and decreased capacity of stomach to relax and accommodate a large mealto relax and accommodate a large meal

Post-cardiacPost-cardiacsurgerysurgery

Post-fundo

Vagal nerve traumaVagal nerve trauma

• delayed gastric emptyingdelayed gastric emptying• dumping of liquidsdumping of liquids• esophageal dysmotilityesophageal dysmotility• vocal fold dysfunctionvocal fold dysfunction

Case #2Case #2 Dumping:Dumping:

rapid movement of liquid feed into intestinesrapid movement of liquid feed into intestines

hyperglycemiahyperglycemia

increased insulin productionincreased insulin production

hypoglycemiahypoglycemia

Counter-regulatory hormones (including epinephrine)Counter-regulatory hormones (including epinephrine)

Retching, gagging, jittery, sweating, tachycardia, crampsRetching, gagging, jittery, sweating, tachycardia, cramps

Retching/GaggingRetching/Gaggingwith feedswith feeds

Oral AversionOral Aversion

Retching/GaggingRetching/Gaggingwith feedswith feeds

Oral AversionOral Aversion

Case #2Case #2 5. How can we decrease the retching and 5. How can we decrease the retching and

gagging?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 #2Case #2

Failed dietary manipulations & meds:Failed dietary manipulations & meds:

• Upper GI contrast studyUpper GI contrast study• EndoscopyEndoscopy• Impedance-pH studyImpedance-pH study

Case #2Case #2

Upper GI contrast study:Upper GI contrast study:

• fundoplication wrap notedfundoplication wrap noted• paraesophageal herniaparaesophageal hernia• noted to retch and gag after noted to retch and gag after

barium bolus given through G tubebarium bolus given through G tube

Paraesophageal HerniaParaesophageal Hernia

CHESTCHEST

ABDOMENABDOMEN

Case #2Case #2

Case #2Case #2

EGD:EGD:

• fundoplication and G tube noted fundoplication and G tube noted • paraesophageal hernia presentparaesophageal hernia present• otherwise normal mucosaotherwise normal mucosa

Paraesophageal HerniaParaesophageal Hernia

FundoplicationFundoplication

Retching/GaggingRetching/Gagging

Paraesophageal Paraesophageal HerniaHernia

Oral Oral AversionAversion

Tight FundoplicationTight Fundoplication

Balloon dilationBalloon dilation

Impedance –pH studyImpedance –pH study

• no episodes of reflux noted during no episodes of reflux noted during 24 hour study on bolus feeds24 hour study on bolus feeds• retching/gagging not related to refluxretching/gagging not related to reflux• dysmotility noted on waveform with dysmotility noted on waveform with swallowsswallows

Case #2Case #26. Is there a behavioral component to Sx?6. Is there a behavioral component to Sx?

• most cases have a behavioral overlapmost cases have a behavioral overlap• ‘ ‘learned behavior’ imprintinglearned behavior’ imprinting• Sx may not completely resolve afterSx may not completely resolve after addressing the primary physical causeaddressing the primary physical cause• overtime, as oral intake improves overtime, as oral intake improves without discomfort, the brain ‘unlearns’without discomfort, the brain ‘unlearns’ negative behaviorsnegative behaviors

Case #2: follow-upCase #2: follow-up

• paraesophageal hernia was repairedparaesophageal hernia was repaired• started tolerating continuous then bolusstarted tolerating continuous then bolus G-tube feeds, later on pureed diet G-tube feeds, later on pureed diet by G-tubeby G-tube• gaining weightgaining weight

Case #3Case #3• 2 yr old M, only taking smooth textures x 6mo2 yr old M, only taking smooth textures x 6mo• gags with solids especially meatsgags with solids especially meats• was taking table foods until 18 months until he was taking table foods until 18 months until he choked on a hot-dogchoked on a hot-dog• hx of eczema, asthma, allergieshx of eczema, asthma, allergies• no pneumonias; no hx of FB ingestionno pneumonias; no hx of FB ingestion• neurodevelopmentally normalneurodevelopmentally normal• occ choking/gagging then vomiting in AMocc choking/gagging then vomiting in AM• gaining weightgaining weight• Test Feed: self-fed liquids and pureed without Test Feed: self-fed liquids and pureed without problem, but refused meatsproblem, but refused meats

Case #3Case #3Questions:Questions:1.1. Is this behavioral (learned response)?Is this behavioral (learned response)?2.2. Is he safe to take oral feeds?Is he safe to take oral feeds?3.3. Is there a physical cause for dysphagia?Is there a physical cause for dysphagia?4.4. Is it oro-pharyngeal or esophageal Is it oro-pharyngeal or esophageal dysphagia?dysphagia?5. What is your next step?5. What is your next step?

Case #3Case #3

1.1. Is this behavioral (learned response)?Is this behavioral (learned response)? • Most likely a componentMost likely a component• Not sure ‘sole’ problemNot sure ‘sole’ problem

Case #3Case #3

2. Is he safe to eat by mouth?2. Is he safe to eat by mouth?

• Most likely safe for the foods Most likely safe for the foods he is takinghe is taking• Most likely not aspiratingMost likely not aspirating

Case #3Case #33. Is there a physical cause for dysphagia:3. Is there a physical cause for dysphagia: oropharyngeal or esophageal?oropharyngeal or esophageal?

• ProbablyProbably• Not likely CNS disorderNot likely CNS disorder• Most likely esophagealMost likely esophageal

Case #3Case #35. What would you do next?5. What would you do next?

• VFSSVFSS• FEESFEES• Neck/Chest X-RayNeck/Chest X-Ray• Upper GI contrast studyUpper GI contrast study• EGD (endoscopy)EGD (endoscopy)• CT scan of neck/chestCT scan of neck/chest

Case #3Case #35. What would you do next?5. What would you do next?

• VFSS VFSS • FEESFEES• Neck/Chest X-RayNeck/Chest X-Ray• Esophagram/UGI: Esophagram/UGI: normalnormal• EGD (endoscopy): EGD (endoscopy): abnormalabnormal• CT scan of neck/chestCT scan of neck/chest

Eosinophilic EsophagitisEosinophilic Esophagitis

Esophageal Bx: typical features of EE

Case #3: Follow upCase #3: Follow up

• started on swallowed fluticasone, PPIstarted on swallowed fluticasone, PPI• skin allergy test identified no food allergensskin allergy test identified no food allergens• repeat EGD in 3 months showed resolutionrepeat EGD in 3 months showed resolution of EE changesof EE changes• oral intake of solids improved over next feworal intake of solids improved over next few months and by 6 months was taking meatsmonths and by 6 months was taking meats without any dysphagiawithout any dysphagia• did not receive any OT/ST/BTdid not receive any OT/ST/BT

Dysphagia. 2007 Jan;22(1):44-8. Epub 2006 Oct 6.

                                                

Eosinophilic esophagitis in infants and toddlers with feeding disordersPentiuk SP, Miller CK, Kaul A.

Department of Pediatrics, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio 45229 Cincinnati, Ohio 45229

Challenges in the Management Challenges in the Management of Pediatric Dysphagiaof Pediatric Dysphagia

• gaps in knowledge, field still evolving, currentgaps in knowledge, field still evolving, current information mostly extrapolated from adult studiesinformation mostly extrapolated from adult studies• clinical research in pediatrics, ethicsclinical research in pediatrics, ethics• growth and development: a dynamic processgrowth and development: a dynamic process of changing anatomy and physiology of changing anatomy and physiology • what is normal?what is normal?• congenital abnormalities increase complexitycongenital abnormalities increase complexity• history is second-hand, affected by parental history is second-hand, affected by parental perspectivesperspectives• pt. cooperation during exam, investigations and pt. cooperation during exam, investigations and therapy suboptimal: ? reliability of resultstherapy suboptimal: ? reliability of results• parental factors: concepts and myths, parental factors: concepts and myths, socio-economic and cultural factors, expectationssocio-economic and cultural factors, expectations

Interdisciplinary approachInterdisciplinary approachin the management of in the management of Pediatric DysphagiaPediatric Dysphagia

• PhysiciansPhysicians• NursesNurses• Therapists: SLP, OT, BehavioralTherapists: SLP, OT, Behavioral• DieticiansDieticians• Social workers, InterpretersSocial workers, Interpreters• Support (office) staffSupport (office) staff

Child Is Child Is NOTNOT a aCompressed AdultCompressed Adult

‘‘Mini Me’Mini Me’

I have a differentset of issues!!

That’s a wrap!