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C. Easterling, Ph.D. CCC BRS-S ASHA 2007 Assistant Professor, University of Wisconsin-Milwaukee Esophageal Swallowing Physiology and Disorders Caryn Easterling, Ph.D. CCC BRS-S Assistant Professor Department of Communication Sciences and Disorders University of Wisconsin-Milwaukee Department of Neurology Department of Pediatric Gastroenterology Medical College of Wisconsin Research Scientist Department of Physical Medicine and Rehabilitation Milwaukee, Wisconsin Milwaukee, Wisconsin Esophageal Swallowing Physiology and Disorders What Is the Role of the What Is the Role of the Speech-Language Pathologist in the Speech-Language Pathologist in the Evaluation of Swallowing Disorders? Evaluation of Swallowing Disorders? Esophageal Swallowing Physiology and Disorders ASHA Preferred Practice Patterns ASHA Preferred Practice Patterns Swallowing Assessment refers to the review of Swallowing Assessment refers to the review of the esophageal phase of swallowing the esophageal phase of swallowing …screening of the esophageal phase” …screening of the esophageal phase” Collaboration between SLPs’ and Collaboration between SLPs’ and Radiologists’ observations, impressions, and Radiologists’ observations, impressions, and recommendations from the radiographic recommendations from the radiographic examination examination 1

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C. Easterling, Ph.D. CCC BRS-S ASHA 2007 Assistant Professor, University of Wisconsin-Milwaukee

Esophageal Swallowing Physiology and Disorders

Caryn Easterling, Ph.D. CCC BRS-SAssistant Professor

Department of Communication Sciences and DisordersUniversity of Wisconsin-Milwaukee

Department of NeurologyDepartment of Pediatric Gastroenterology

Medical College of WisconsinResearch Scientist

Department of Physical Medicine and Rehabilitation

Milwaukee, WisconsinMilwaukee, WisconsinEsophageal Swallowing Physiology and Disorders

What Is the Role of theWhat Is the Role of theSpeech-Language Pathologist in the Speech-Language Pathologist in the Evaluation of Swallowing Disorders?Evaluation of Swallowing Disorders?

Esophageal Swallowing Physiology and Disorders•• ASHA Preferred Practice Patterns ASHA Preferred Practice Patterns –– Swallowing Assessment refers to the review of the Swallowing Assessment refers to the review of the esophageal phase of swallowingesophageal phase of swallowing–– ““ …screening of the esophageal phase”…screening of the esophageal phase”•• Collaboration between SLPs’ and Radiologists’ Collaboration between SLPs’ and Radiologists’ observations, impressions, and recommendations observations, impressions, and recommendations from the radiographic examinationfrom the radiographic examinationEsophageal Swallowing Physiology and Disorders•• Obligated to Document Observations of the Obligated to Document Observations of the Swallowing Continuum in the upright positionSwallowing Continuum in the upright position

•• Play a key role in the early detection of potentialPlay a key role in the early detection of potential clinical and radiographic indicators of esophageal clinical and radiographic indicators of esophageal swallowing disordersswallowing disorders

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C. Easterling, Ph.D. CCC BRS-S ASHA 2007 Assistant Professor, University of Wisconsin-Milwaukee

•• Facilitate appropriate medical referralsFacilitate appropriate medical referrals–– (GI/ENT/Neuro/Pulmonary) for further evaluation(GI/ENT/Neuro/Pulmonary) for further evaluationEsophageal Swallowing Physiology & DisordersEsophageal Swallowing Physiology & DisordersEsophageal Swallowing Physiology and Disorders

DysphagiaDysphagia–– Symptom or patient report of swallowing difficultySymptom or patient report of swallowing difficulty

Often evaluated first using aOften evaluated first using a–– Videofluoroscopic Swallow Study (VFSS)Videofluoroscopic Swallow Study (VFSS)

–– Fiberoptic endoscopic Evaluation of Swallowing (FEES)Fiberoptic endoscopic Evaluation of Swallowing (FEES)

Key Elements for the SLP•• Careful history Careful history –– will yield the likely anatomical location and pathophysiological will yield the likely anatomical location and pathophysiological process in 80% of the casesprocess in 80% of the cases•• The site of “bolus hold-up” The site of “bolus hold-up” –– is not reliable if perceived in the neckis not reliable if perceived in the neck•• Endoscopy Endoscopy –– is virtually always indicated is virtually always indicated •• Precise diagnosis may require manometry and Precise diagnosis may require manometry and radiographic proceduresradiographic procedures•• A normal endoscopy and Ba swallow does not A normal endoscopy and Ba swallow does not adequately rule out a structural eso abnormalityadequately rule out a structural eso abnormality

Dysphagia•• The subjective location of dysphagia does not The subjective location of dysphagia does not always correspond to the anatomical location of the always correspond to the anatomical location of the pathologypathology

•• Patient’s clinical complaints and reporting Patient’s clinical complaints and reporting regarding the site of dysphagia may be unreliable regarding the site of dysphagia may be unreliable and does not correlate with the actual problemand does not correlate with the actual problem–– Eg., Distal stricture often referred proximallyEg., Distal stricture often referred proximally

(Jones et al., 1989)(Jones et al., 1989)

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C. Easterling, Ph.D. CCC BRS-S ASHA 2007 Assistant Professor, University of Wisconsin-Milwaukee

History•• Where does the food stick?Where does the food stick?•• Is one or more of the pharyngeal dysfunctions Is one or more of the pharyngeal dysfunctions present?present?–– Delay or absent swallowDelay or absent swallow–– Deglutitive nasopharyngeal regurgitationDeglutitive nasopharyngeal regurgitation–– Deglutitive coughDeglutitive cough–– Repetitive swallows to clear the pharynxRepetitive swallows to clear the pharynx•• Dysphagia for liquids or solids?Dysphagia for liquids or solids?–– Motor – bothMotor – both–– Structural - solidStructural - solid

History•• How long has dysphagia been present; How long has dysphagia been present; intermittent, progressive?intermittent, progressive?–– Long – benignLong – benign–– Long, intermittent, non progressive, solids - structuralLong, intermittent, non progressive, solids - structural–– Short, rapid progression, weight loss – CAShort, rapid progression, weight loss – CA•• What does the patient do when it “sticks”?What does the patient do when it “sticks”?–– Do they regurgitate?Do they regurgitate?–– Chest Pain?Chest Pain?Clinical Indicators of Possible Esophageal Swallowing Abnormality•• Fullness or tightening in throat or chestFullness or tightening in throat or chest•• Localized or radiating Neck, jaw, or chest painLocalized or radiating Neck, jaw, or chest pain•• Liquid or Solid Food DysphagiaLiquid or Solid Food Dysphagia•• Chronic CoughChronic Cough

•• Chronic Throat ClearingChronic Throat Clearing•• Excessive secretionsExcessive secretions•• Paradoxical Vocal Cord DysfunctionParadoxical Vocal Cord Dysfunction•• BreathlessnessBreathlessness•• HoarsenessHoarseness•• Sore throat Sore throat •• Acidic taste Acidic taste

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C. Easterling, Ph.D. CCC BRS-S ASHA 2007 Assistant Professor, University of Wisconsin-Milwaukee

MBS Indicators of Possible Esophageal Swallowing Abnormality: Indicators for medical referrals/further esophageal examination

Modified Barium Swallowing Study:Modified Barium Swallowing Study:•• IsIs Not Not DiagnosticDiagnostic ExamExam for Esophageal for Esophageal Swallowing Disorders or Gastroesophageal Reflux Swallowing Disorders or Gastroesophageal Reflux Disease (GERD)Disease (GERD)

•• Includes only observations of Includes only observations of potential potential indicatorsindicators of esophageal swallowing disordersof esophageal swallowing disordersMBS Indicators of Possible Esophageal Swallowing Abnormality: Indicators for medical referrals/further esophageal examination•• Large air column just below UESLarge air column just below UES

•• Pocket of contrast just posterior and distal to Pocket of contrast just posterior and distal to UES (Zenker’s Diverticulum)UES (Zenker’s Diverticulum)

•• Slow or obstructed esophageal clearance of Slow or obstructed esophageal clearance of contrast in the upright position (contrast in the upright position (±± tertiary tertiary contractions)contractions)

AnatomyAnatomy of the Human Esophagus

The esophagus:The esophagus:–– 22 to 26 centimeters in length 22 to 26 centimeters in length –– Length varies with body sizeLength varies with body size–– Tubular structure composed of skeletal Tubular structure composed of skeletal (proximal 1/3(proximal 1/3rdrd)) and smooth and smooth (distal 2/3rds)(distal 2/3rds) muscle. muscle.

(Christensen, 1987; Roman, 1982)(Christensen, 1987; Roman, 1982)

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C. Easterling, Ph.D. CCC BRS-S ASHA 2007 Assistant Professor, University of Wisconsin-Milwaukee

Anatomy of the Human Esophagus•• Organ of transport Organ of transport –– PeristalsisPeristalsis•• Peristalsis is the progression of a pressure wave Peristalsis is the progression of a pressure wave through the body of the esophagus through the body of the esophagus –– Body position is important in evaluation of peristalsisBody position is important in evaluation of peristalsis•• Striated esophageal muscle demonstrates both:Striated esophageal muscle demonstrates both:–– Primary peristalsis during the esophageal phase of swallowing Primary peristalsis during the esophageal phase of swallowing ….and ….and –– Secondary or bolus initiated peristalsisSecondary or bolus initiated peristalsis

(Kendall, Thompson, Day, & Garvie, 1987)(Kendall, Thompson, Day, & Garvie, 1987)

Anatomy of the Human Esophagus1/31/3 Striated Muscle 0.9 cm (4% ) Striated Muscle 0.9 cm (4% ) ------ MixedMixed 7.6 cm (34%)7.6 cm (34%)2/32/3 Smooth MuscleSmooth Muscle 14.3 cm (62%)14.3 cm (62%)

Anatomy of the Human Esophagus•• Striated muscle of the esophagus has both an Striated muscle of the esophagus has both an external and an internal layer of muscleexternal and an internal layer of muscle

–– Outer - longitudinal muscle Outer - longitudinal muscle –– Inner - circular muscle Inner - circular muscle

–– Interact in a stereotypic pattern during peristaltic Interact in a stereotypic pattern during peristaltic propulsion of the luminal contents propulsion of the luminal contents

(Hendrix, 1993)(Hendrix, 1993)

Anatomy of the Human Esophagus•• Layers of the Esophageal WallLayers of the Esophageal Wall–– Squamous EpitheliumSquamous Epithelium

–– Lamina PropriaLamina Propria

–– Muscularis mucosaMuscularis mucosa

Boundaries of the Esophagus are Defined by two Sphincters

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C. Easterling, Ph.D. CCC BRS-S ASHA 2007 Assistant Professor, University of Wisconsin-Milwaukee

Boundaries of the Esophagus are Defined by two Sphincters Upper Esophageal Sphincter (UES)/ Upper Esophageal Sphincter (UES)/

Pharyngoesophageal Sphincter (PES)Pharyngoesophageal Sphincter (PES)

Biomechanical Sphincter:–– Relaxation Relaxation

–– Traction force to open Traction force to open •• Superior and anterior pull by posterior cricoid cartilage Superior and anterior pull by posterior cricoid cartilage

away from the posterior pharyngeal wall away from the posterior pharyngeal wall •• Contraction of the suprahyoid musclesContraction of the suprahyoid muscles

–– Distension (elasticity of the UES)Distension (elasticity of the UES)•• Intrabolus pressureIntrabolus pressure

Boundaries of the Esophagus are Defined by two Sphincters•• Lower Esophageal Sphincter (LES)Lower Esophageal Sphincter (LES)

–– Passive Relaxation Passive Relaxation

–– Distention Distention

Neural Control of Swallowing•• Motor neurons involved in oropharyngeal and Motor neurons involved in oropharyngeal and esophageal swallowingesophageal swallowing–– Trigeminal (V) - striated muscleTrigeminal (V) - striated muscle–– Facial (VII) - striated muscleFacial (VII) - striated muscle–– Vagus (X) – striated muscleVagus (X) – striated muscle–– Glossopharyngeal (IX) - striated muscleGlossopharyngeal (IX) - striated muscle–– Hypoglossal (XII) nuclei - striated muscleHypoglossal (XII) nuclei - striated muscle–– Nucleus ambiguous (nA) - striated muscleNucleus ambiguous (nA) - striated muscle

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C. Easterling, Ph.D. CCC BRS-S ASHA 2007 Assistant Professor, University of Wisconsin-Milwaukee

–– Dorsal motor nucleus of the vagus nerve (dmnX) - Dorsal motor nucleus of the vagus nerve (dmnX) - smooth musclesmooth muscle

Neural Control of the Human Esophagus•• Esophageal Body: innervated extrinsically Esophageal Body: innervated extrinsically –– Autonomic nerves Autonomic nerves •• Via vagus nerve and branches of the sympathetic nervesVia vagus nerve and branches of the sympathetic nerves•• Motor function: brainstem, medulla and ponsMotor function: brainstem, medulla and pons

•• Highly dependent on the peripheral sensory Highly dependent on the peripheral sensory input from oropharyngeal structuresinput from oropharyngeal structures–– LES relaxation may occur independently of eso body LES relaxation may occur independently of eso body (LESR)(LESR)

Neural Control of the Human Esophagus•• Intrinsic innervation of the EBIntrinsic innervation of the EB–– Myenteric plexus Myenteric plexus •• Ganglionic cellsGanglionic cells•• Nerve fibersNerve fibers–– (Dodds et al., 1974; Gilbert & Dodds, 1986)(Dodds et al., 1974; Gilbert & Dodds, 1986)

Neural Control of the Human Esophagus•• UES – motor innervationUES – motor innervation–– Glossopharyngeal nerveGlossopharyngeal nerve–– Pharyngeal branch of the vagusPharyngeal branch of the vagus–– Recurrent laryngeal branch of the vagusRecurrent laryngeal branch of the vagus•• Cervical esophagusCervical esophagus–– Recurrent laryngeal nerveRecurrent laryngeal nerve•• Distal esophagusDistal esophagus–– Branches of the thoracic vagal trunksBranches of the thoracic vagal trunks

Moving the Bolus:Esophageal Peristalsis is InitiatedTwo Ways•• Primary Peristalsis – Swallow initiatedPrimary Peristalsis – Swallow initiated

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C. Easterling, Ph.D. CCC BRS-S ASHA 2007 Assistant Professor, University of Wisconsin-Milwaukee

•• Secondary Peristalsis – Distension of Secondary Peristalsis – Distension of esophageal bodyesophageal body

•• Remember position mattersRemember position mattersEsophageal Peristalsis•• Above the bolusAbove the bolus–– What is going on?What is going on?•• The circular muscle contracts and the The circular muscle contracts and the longitudinal muscle relaxes to produce a propulsive longitudinal muscle relaxes to produce a propulsive segment that travels the length of the esophagus segment that travels the length of the esophagus during the swallowduring the swallow

Esophageal PeristalsisCan it be modified?•• Peristalsis in the striated muscle is modifiedPeristalsis in the striated muscle is modified–– bolus volumebolus volume–– temperature changetemperature change

Indicating that sensory inputs from the esophagusIndicating that sensory inputs from the esophagusmodulate the central vagal output that controlsmodulate the central vagal output that controlsperistalsis in the striated muscle of the esophagusperistalsis in the striated muscle of the esophagus

(Dodds, Hogan, Reid, Stewart & Arndorfer, 1973)(Dodds, Hogan, Reid, Stewart & Arndorfer, 1973)

Ingestion of Cold Substances Causes Inhibition of Esophageal Peristalsis & Can Produce PainSingle Swallow:

Observe primary peristalsis

Frequent Swallows: Inhibit Propagation of Peristalsis into the

Distal EsophagusCompetence of the GE Junction•• Two Major ComponentsTwo Major Components

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C. Easterling, Ph.D. CCC BRS-S ASHA 2007 Assistant Professor, University of Wisconsin-Milwaukee

–– Sphincter pressure - LESSphincter pressure - LES•• Muscle toneMuscle tone•• DiaphragmDiaphragm–– Transient sphincter relaxationsTransient sphincter relaxations•• Specific mechanisms not knownSpecific mechanisms not known

LES Resting & Relaxation Pressure•• LES pressure during rest and deglutition is the LES pressure during rest and deglutition is the sum of sphincteric muscle tone and the phasic sum of sphincteric muscle tone and the phasic changes of the diaphragmchanges of the diaphragm–– LES Pressure increases with inspirationLES Pressure increases with inspiration

•• Transient lower esophageal sphincter relaxation Transient lower esophageal sphincter relaxation (TLESR’S)(TLESR’S)

Esophageal Causes of Dysphagia – Four Categories•• Motility Disorders or AbnormalitiesMotility Disorders or Abnormalities•• Structural AbnormalitiesStructural Abnormalities–– Obstructs passage of the bolusObstructs passage of the bolus•• Web, ring, or strictureWeb, ring, or stricture–– Benign or malignant tumorBenign or malignant tumor–– Numerous causes of bothNumerous causes of both

•• InfectionInfection•• Neural DysfunctionNeural DysfunctionESOPHAGEAL FUNCTION TESTINGObservations during an Esophagram (radiographic evaluation of the esophagus)•• Esophageal bolus transitEsophageal bolus transit–– Gravity assistedGravity assisted•• Esophageal clearance in the upright positionEsophageal clearance in the upright position

•• Esophageal shorteningEsophageal shortening•• UES opening (assume relaxation)UES opening (assume relaxation)•• UES closure (assume contraction)UES closure (assume contraction)•• LES opening (assume relaxation)LES opening (assume relaxation)

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C. Easterling, Ph.D. CCC BRS-S ASHA 2007 Assistant Professor, University of Wisconsin-Milwaukee

•• LES closure (assume contraction)LES closure (assume contraction)

Components of an Esophagram•• Recumbent PositionRecumbent Position•• Sufficient Bolus SizeSufficient Bolus Size•• Observation of Sufficient SwallowsObservation of Sufficient Swallows•• Abdominal Compression?Abdominal Compression?–– Gastroesophageal reflux?Gastroesophageal reflux?•• Bolus Challenge (marshmallow)?Bolus Challenge (marshmallow)?

Esophageal Motor Function& Bolus Motility •• 11°° peristaltic wave - initiated by swallow peristaltic wave - initiated by swallow•• 22° ° peristaltic wave - initiated by distended esophagus peristaltic wave - initiated by distended esophagus –– 22°° bolus residual bolus residual –– Reflux eventReflux event•• Tertiary contractions - non-propulsive eventsTertiary contractions - non-propulsive events•• Manometry and esophagram are complimentaryManometry and esophagram are complimentary–– Manometry quantitative Manometry quantitative –– Esophagram qualitativeEsophagram qualitative

Esophageal Manometry•• Measurement of pressure changes in the body ofMeasurement of pressure changes in the body of the esophagus the esophagus

•• Indirect measure of contraction and functionIndirect measure of contraction and function

•• No direct observation of bolusNo direct observation of bolusClinical Indications For Esophageal Manometry•• Evaluation of patients with dysphagiaEvaluation of patients with dysphagia–– Primary motility disordersPrimary motility disorders•• Achalasia: motor disorder of smooth muscle of esoAchalasia: motor disorder of smooth muscle of eso–– LOSS OF INTRAMURAL NEURONSLOSS OF INTRAMURAL NEURONS–– LES DOES NOT RELAXLES DOES NOT RELAX–– NON PERISTALTIC CONTRACTIONSNON PERISTALTIC CONTRACTIONS

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C. Easterling, Ph.D. CCC BRS-S ASHA 2007 Assistant Professor, University of Wisconsin-Milwaukee

•• Spastic disordersSpastic disorders–– Secondary motility disordersSecondary motility disorders•• SclerodermaScleroderma

Clinical Indications For Esophageal Manometry (cont.)•• Evaluation of patients with GERDEvaluation of patients with GERD–– Evaluate peristalsis and LES integrityEvaluate peristalsis and LES integrity•• GER severityGER severity•• Prior (after) fundoplicationPrior (after) fundoplication–– Assist in placement of pH probe (location of UES/LES)Assist in placement of pH probe (location of UES/LES)•• Evaluation of patients with non-cardiac chest Evaluation of patients with non-cardiac chest painpain

INTRALUMINAL IMPEDANCEINTRA-ESOPHAGEAL pH MONITORING•• Prolonged pH recording in the study of gastro-Prolonged pH recording in the study of gastro-esophageal reflux.esophageal reflux.

(Spencer J, 1969)(Spencer J, 1969)

•• Laryngopharyngealreflux (LPR)Laryngopharyngealreflux (LPR)

•• Globus pharyngeous Globus pharyngeous (Cook, 2007)(Cook, 2007)

DEFINITION OF REFLUX•• Defined by fall in pH to <4.0 until return Defined by fall in pH to <4.0 until return to >4.0to >4.0•• Rationale:Rationale:–– Clearly distinct from usual intra-esophageal - pH Clearly distinct from usual intra-esophageal - pH of 6-7of 6-7–– Pepsin Pepsin inactivatedinactivated at pH >4.0 at pH >4.0–– Good association between reflux Sx andGood association between reflux Sx andpH 4.0pH 4.0

REFLUX PARAMETERS•• Percent acid exposure (pH <4.0)Percent acid exposure (pH <4.0)

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C. Easterling, Ph.D. CCC BRS-S ASHA 2007 Assistant Professor, University of Wisconsin-Milwaukee

–– UprightUpright–– RecumbentRecumbent–– TotalTotal•• Total number of reflux episodesTotal number of reflux episodes•• Number of episodes >5 minutesNumber of episodes >5 minutes•• Time of single longest episodeTime of single longest episode

AMBULATORY PH MONITORING:Clinical Indications•• Reflux symptomsReflux symptoms–– Chest painChest pain–– HoarsenessHoarseness–– BronchospasmBronchospasm–– Non-ulcer dyspepsiaNon-ulcer dyspepsia•• Typical heartburn with negative workupTypical heartburn with negative workup•• Pre-operative evaluation for fundoplicationPre-operative evaluation for fundoplication•• Follow-up of anti-reflux therapyFollow-up of anti-reflux therapy–– During medical therapyDuring medical therapy–– Post-fundoplicationPost-fundoplication

SYMPTOM INDEX =Number of Times Number of Times

Symptom OccurredSymptom Occurredwhen pH <4when pH <4

AMBULATORY GER MONITORING(MII-pH)•• GER episodes identified by MII GER episodes identified by MII

•• GER episodes categorized as acid or non-acid by GER episodes categorized as acid or non-acid by pHpH–– Non-acid reflux = refluxate with pH >4Non-acid reflux = refluxate with pH >4

(<10% bile)(<10% bile)POTENTIAL MII-pH APPLICATIONS•• Diagnosis of patient with persistent symptoms while on Diagnosis of patient with persistent symptoms while on PPI RxPPI Rx

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C. Easterling, Ph.D. CCC BRS-S ASHA 2007 Assistant Professor, University of Wisconsin-Milwaukee

Elucidate the role of non-acid GERElucidate the role of non-acid GER•• Evaluation of atypical GERDEvaluation of atypical GERD

Correlate acid & non-acid GER episodes to sxCorrelate acid & non-acid GER episodes to sxQuantify proximal extent of GERQuantify proximal extent of GER

•• Evaluate postprandial GEREvaluate postprandial GERpH is blind during early postprandial period pH is blind during early postprandial period Postprandial is prime time for reflux and sxPostprandial is prime time for reflux and sx

PERSISTENT SYMPTOMS ON PPIPossible causes•• Acid not controlled by medicationsAcid not controlled by medications

~20%~20%•• Symptoms due to “non-acid” refluxSymptoms due to “non-acid” reflux

~40%~40%•• Symptoms not due to refluxSymptoms not due to reflux

~40%~40%

NON-ACID REFLUX•• Detected by combined impedance-pH Detected by combined impedance-pH monitoringmonitoring•• Does it cause injury?Does it cause injury?–– It does cause symptoms!It does cause symptoms!

•• Laryngopharyngeal reflux (LPR)Laryngopharyngeal reflux (LPR)–– Visual findings & pH monitoring lack specificityVisual findings & pH monitoring lack specificity–– Laryngeal irritation is multi-factorial Laryngeal irritation is multi-factorial –– Longer time to disappear compared to symptoms Longer time to disappear compared to symptoms (Belafsky et al., Laryngoscope; 2001)(Belafsky et al., Laryngoscope; 2001)

–– Empiric BID PPI (omeprazole etc.) best (4-6 months) Empiric BID PPI (omeprazole etc.) best (4-6 months) (Park et al., Laryngoscope; 2005)(Park et al., Laryngoscope; 2005)

Globus pharyngeus•• Globus pharyngeus: Globus pharyngeus: –– Feeling of a lump or foreign body in the throatFeeling of a lump or foreign body in the throat–– Crumb-like sensation, constriction causing chokingCrumb-like sensation, constriction causing choking–– 45% of population experiences45% of population experiences

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C. Easterling, Ph.D. CCC BRS-S ASHA 2007 Assistant Professor, University of Wisconsin-Milwaukee

•• Globus “hystericus”Globus “hystericus”–– Wilson et al., (1995,96), UKWilson et al., (1995,96), UK

Globus pharyngeus•• Cook et al., (2007)Cook et al., (2007)–– How valuable is videofluoroscopy and ambulatory pH in How valuable is videofluoroscopy and ambulatory pH in monitoring patients with globusmonitoring patients with globus–– pH helpful only in those who had GER with GPpH helpful only in those who had GER with GP–– All accompanying pharyngeal dysfunction noted on VFSSAll accompanying pharyngeal dysfunction noted on VFSS•• Cook et al., (1998) Cook et al., (1998) –– Hypertensive UESHypertensive UES–– No association with GERNo association with GER•• Consistent presence of mucous in the epipharynxConsistent presence of mucous in the epipharynx•• PPI & anti-depressivePPI & anti-depressive

ESOPHAGEAL MOTILITY ABNORMALITIES

Classification of Esophageal Motility Disorders•• Primary Primary –– those disorders not related to underlying diseasethose disorders not related to underlying disease

•• Secondary Secondary –– those related to underlying diseasethose related to underlying disease

Primary Motility/Motor Disorders•• AchalasiaAchalasia•• Diffuse esophageal spasmDiffuse esophageal spasm•• Intestinal pseudo-obstructionIntestinal pseudo-obstruction•• PresbyesophagusPresbyesophagus•• Neonatal achalasiaNeonatal achalasia

•• Affect neural as well as muscular elements of the Affect neural as well as muscular elements of the esophagus and LESesophagus and LES•• Pain and dysphagiaPain and dysphagia–– Calcium channel blockers Calcium channel blockers –– Anti-anxiety medicationAnti-anxiety medication

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C. Easterling, Ph.D. CCC BRS-S ASHA 2007 Assistant Professor, University of Wisconsin-Milwaukee

–– Botox vs. muscle relaxantsBotox vs. muscle relaxants

Secondary Motility Disorders•• Connective tissueConnective tissue–– SclerodermaScleroderma

•• Chemical or physicalChemical or physical–– Reflux esophagitis (peptic)Reflux esophagitis (peptic)–– Caustic esophagitisCaustic esophagitis–– VagotomyVagotomy–– RadiationRadiation

•• EndocrineEndocrine•• InfectionInfection–– Fungal: moniliasisFungal: moniliasis–– Bacterial: TBBacterial: TB–– Parasitic: Chagas’ diseaseParasitic: Chagas’ disease–– Viral: Herpes simplexViral: Herpes simplex

•• MetabolicMetabolic–– DiabetesDiabetes–– ETOHETOH–– AmyloidosisAmyloidosis–– Serum pH and electrolye disturbancesSerum pH and electrolye disturbances

Secondary Motility Disorders•• Neurologic diseaseNeurologic disease–– MyositisMyositis–– ParkinsonismParkinsonism–– Huntington’s choreaHuntington’s chorea–– Wilson’s diseaseWilson’s disease–– Cerebrovascular diseaseCerebrovascular disease–– Multiple sclerosisMultiple sclerosis–– ALSALS–– CNS neoplasmCNS neoplasm

ABNORMAL ESOPHAGEAL MOTILITYTraditional ClassificationsTraditional Classifications•• AchalasiaAchalasia•• Diffuse esophageal spasmDiffuse esophageal spasm•• Nutcracker esophagusNutcracker esophagus•• Hypertensive lower esophageal sphincterHypertensive lower esophageal sphincter•• Non-specific esophageal motility disorderNon-specific esophageal motility disorder

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C. Easterling, Ph.D. CCC BRS-S ASHA 2007 Assistant Professor, University of Wisconsin-Milwaukee

ABNORMAL ESOPHAGEAL MOTILITYNew ClassificationNew Classification•• Inadequate LES relaxation (RP >8 mmHg)Inadequate LES relaxation (RP >8 mmHg)–– Classic achalasia – absence of peristalsisClassic achalasia – absence of peristalsis–– Atypical patternsAtypical patterns•• Uncoordinated motilityUncoordinated motility–– Diffuse esophageal spasm (>10% simultaneous contractions, >30 mmHg)Diffuse esophageal spasm (>10% simultaneous contractions, >30 mmHg)•• HypercontractionHypercontraction–– Body = nutcracker esophagus (mean amplitude >180 mmHg)Body = nutcracker esophagus (mean amplitude >180 mmHg)–– LES = hypertensive LES (>45 mmHg)LES = hypertensive LES (>45 mmHg)•• Hypocontraction Hypocontraction –– Body = IEM (Body = IEM (30% contraction < 30 mmHg)30% contraction < 30 mmHg)–– LES = Hypotensive LES (<10 mmHg)LES = Hypotensive LES (<10 mmHg)

ABNORMAL ESOPHAGEAL MOTILITY•• Clinical ImportanceClinical Importance•• AchalasiaAchalasia–– Establish diagnosis and direct therapyEstablish diagnosis and direct therapy•• DES, nutcracker esophagus, hypertensive LES:DES, nutcracker esophagus, hypertensive LES:–– Relation to Sx usually not clearRelation to Sx usually not clear–– Only suggests therapy (no pathology known)Only suggests therapy (no pathology known)•• IEM, hypotensive LES:IEM, hypotensive LES:–– Usually indicates GERDUsually indicates GERD–– Important information prior to fundoplicationImportant information prior to fundoplication

ABNORMAL ESOPHAGEAL MOTILITYDiffuse Esophageal Spasm•• Chest pain Chest pain –– Associated with high amplitude esophageal peristaltic Associated with high amplitude esophageal peristaltic pressure wavepressure wave–– Repetitive non-propulsive contractionsRepetitive non-propulsive contractions•• Similar to early “vigorous” achalasiaSimilar to early “vigorous” achalasia–– Except normal LES relaxationExcept normal LES relaxation–– Manometric analysis Manometric analysis

Achalasia•• Lack of relaxation of the lower esophageal sphincter (LES)Lack of relaxation of the lower esophageal sphincter (LES)–– Absent or markedly decreased 10 and 20 esophageal peristalsis Absent or markedly decreased 10 and 20 esophageal peristalsis –– Disruption of motilityDisruption of motility•• Early- “vigorous” numerous tertiary contractionsEarly- “vigorous” numerous tertiary contractions–– Without normal 10 waveWithout normal 10 wave•• Late stage - dilated esophagus Late stage - dilated esophagus –– Large air-fluid levelLarge air-fluid level–– “Bird beak-like” appearance of the LES“Bird beak-like” appearance of the LES

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C. Easterling, Ph.D. CCC BRS-S ASHA 2007 Assistant Professor, University of Wisconsin-Milwaukee

•• Amyl Nitrate - smooth muscle relaxant Amyl Nitrate - smooth muscle relaxant –– Improves LES relaxationImproves LES relaxation–– Lowers barium column levelLowers barium column level–– Excludes underlying tumorExcludes underlying tumor

Barrett’s Esophagus•• 10% prevalence of adenocarcinoma in patient’s 10% prevalence of adenocarcinoma in patient’s with Barrett’swith Barrett’s•• Risk of adenocarcinoma 30 to 40 times greater Risk of adenocarcinoma 30 to 40 times greater in Barrett’s patients in Barrett’s patients •• Classic Presentation: Classic Presentation: –– High esophageal strictureHigh esophageal stricture–– GERD and/or hiatal herniaGERD and/or hiatal hernia

PREVALENCE OF BARRETT’S BY AGEIneffective Esophageal Motility (IEM)A SPECIFIC MANOMETRIC ABNORMALITYA SPECIFIC MANOMETRIC ABNORMALITY•• Contraction abnormality of the distal esophagus Contraction abnormality of the distal esophagus

where where 30% of wet swallows are30% of wet swallows are–– Low-amplitude contractionsLow-amplitude contractions

(<30 mm Hg)(<30 mm Hg) oror

–– Nontransmitted contractions Nontransmitted contractions •• Occurs in about 30% of GERD patientsOccurs in about 30% of GERD patients

Scleroderma•• Weak-to-absent distal peristalsis (smooth muscle)Weak-to-absent distal peristalsis (smooth muscle)•• Normal proximal peristalsis & UES (striated muscle)Normal proximal peristalsis & UES (striated muscle)•• Absent 1Absent 1°° and 2 and 2°° peristaltic pressure waves peristaltic pressure waves•• Dilated esophagusDilated esophagus•• Abnormal esophageal motilityAbnormal esophageal motility•• Patulous LES with free GERPatulous LES with free GER

•• Long term GERD leads to esophagitis, stricturesLong term GERD leads to esophagitis, strictures–– Possible Barrett’s esophagusPossible Barrett’s esophagus–– Adenocarcinoma of the esophagusAdenocarcinoma of the esophagus

IEM – “Presbyesophagus”•• Increased number of tertiary contractionsIncreased number of tertiary contractions•• Esophageal aperistalsisEsophageal aperistalsis

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C. Easterling, Ph.D. CCC BRS-S ASHA 2007 Assistant Professor, University of Wisconsin-Milwaukee

•• Older individualsOlder individuals•• Diabetes- weakened 1Diabetes- weakened 100 peristalsis peristalsis–– Frequent tertiary contractionsFrequent tertiary contractions

Motor Disturbances•• Non-specific dysmotilityNon-specific dysmotility•• Breaking 1Breaking 1°° peristaltic pressure wave peristaltic pressure wave •• 22° ° clearing - commonclearing - common•• Tertiary contractions – common and increase Tertiary contractions – common and increase with agewith age•• Low amplitude of peristaltic pressure waveLow amplitude of peristaltic pressure wave•• Prolonged duration of peristaltic pressure waveProlonged duration of peristaltic pressure wave

Obstruction to Passage of Bolus•• Webs, Rings, & StricturesWebs, Rings, & Strictures–– Webs - often incompleteWebs - often incomplete•• 1-2 mm in length 1-2 mm in length •• Cervical esophagusCervical esophagus–– Rings- 3-5 mm lengthRings- 3-5 mm length•• Schatzki’s ringSchatzki’s ring–– Gastroesophageal junctionGastroesophageal junction–– Strictures - longer than 1cmStrictures - longer than 1cm•• Can be very longCan be very long

Cervical Esophageal Webs•• Upper esophagusUpper esophagus•• Usually incompleteUsually incomplete•• Anterior locationAnterior location•• If complete with narrowingIf complete with narrowing–– DysphagiaDysphagia•• Often incidental, non-symptomaticOften incidental, non-symptomatic

Plummer Vinson Syndrome•• Dysphagia due to esophageal websDysphagia due to esophageal webs•• Iron deficiency anemiaIron deficiency anemia•• Pharyngoesophageal carcinomaPharyngoesophageal carcinoma

Zenker’s diverticulum•• HypopharnyxHypopharnyx

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•• Posteriorly just above upper esophageal sphincter Posteriorly just above upper esophageal sphincter –– Killian-Jamieson’s dehiscenceKillian-Jamieson’s dehiscence•• Can lead to:Can lead to:–– GlobusGlobus–– Retrograde aspirationRetrograde aspiration•• Hypothesis: Hypothesis: –– Incoordination of pharyngeal pressure wave contractionIncoordination of pharyngeal pressure wave contraction & UES relaxation& UES relaxation

Schatzki’s Ring•• At the gastroesophageal junctionAt the gastroesophageal junction•• Greater than 20 mm Greater than 20 mm –– B ringB ring•• Less than 12 mmLess than 12 mm–– Symptomatic Schatzki’sSymptomatic Schatzki’s•• Between 12-20 mm Between 12-20 mm –– ??•• Marshmallow ChallengeMarshmallow Challenge

Schatzki Ring•• Case report (Gawrieh., et.al, 2005)Case report (Gawrieh., et.al, 2005)–– Three patientsThree patients•• Schatzki Ring (2)Schatzki Ring (2)•• hypertensive peristaltic waves (1)hypertensive peristaltic waves (1)–– Cardiac syncope during swallowingCardiac syncope during swallowing–– Treatment – cardiac pacemakerTreatment – cardiac pacemaker•• Syncope when swallowing resolvedSyncope when swallowing resolved

Benign Strictures•• Peptic / GERDPeptic / GERD–– Barrett’s esophagitisBarrett’s esophagitis–– NG intubationNG intubation–– SclerodermaScleroderma•• Caustic ingestionCaustic ingestion•• Pill esophagitisPill esophagitis•• RadiationRadiation•• Rare: Rare: –– Congenital esophageal stenosisCongenital esophageal stenosis–– Crohn’s disease Crohn’s disease –– Eosinophilic esophagitis (increased dx)Eosinophilic esophagitis (increased dx)–– Graft vs. host diseaseGraft vs. host disease

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Caustic Ingestion•• Ingestion of strong acids or bases Ingestion of strong acids or bases –– Lye, DranoLye, Drano•• Long severe stricture formation Long severe stricture formation –– 1-3 months after event1-3 months after event•• Chronic lye stricturesChronic lye strictures–– Increased risk for esophageal carcinomaIncreased risk for esophageal carcinoma

Pill Esophagitis•• Tetracycline and doxycycline - most commonTetracycline and doxycycline - most common–– Superficial ulcersSuperficial ulcers–– Eventually healEventually heal•• Quinidine, potassium chloride, alendronate, aspirin, Quinidine, potassium chloride, alendronate, aspirin, NSAIDsNSAIDs–– Severe esophagitis with ulcers & stricturesSevere esophagitis with ulcers & strictures•• Direct irritant at areas of hang-up in the esophagusDirect irritant at areas of hang-up in the esophagus–– aortic archaortic arch–– left mainstem bronchusleft mainstem bronchus–– heartheart

Radiation•• High doses (5000 cGy (rads) or more)High doses (5000 cGy (rads) or more)–– Dysphagia from stricture formation Dysphagia from stricture formation –– 4-8 months after Radiation Therapy4-8 months after Radiation Therapy•• Acute esophagitis (1-4 weeks) Acute esophagitis (1-4 weeks) •• Smooth, long stricturesSmooth, long strictures•• Within radiation portalWithin radiation portal•• Differential Dx: Differential Dx: –– Recurrent tumor – irregular, nodular shapeRecurrent tumor – irregular, nodular shape

Eosinophilic Esophagitis (EE)•• 1966 – Dysphagia – proximal rings1966 – Dysphagia – proximal rings

(Kelly: JAMA 197: 143)(Kelly: JAMA 197: 143)

•• 1978 – EE in an adult1978 – EE in an adult(Landes: Gastroenterology 74:1298)(Landes: Gastroenterology 74:1298)

•• 1993 – EE in group 12 adults1993 – EE in group 12 adults(Attwood: Dig Dis Sci 38:109)(Attwood: Dig Dis Sci 38:109)

•• 2001 – Small Caliber Esophagus2001 – Small Caliber Esophagus(Vasilopoulos: Gastroenterology 55:99)(Vasilopoulos: Gastroenterology 55:99)

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C. Easterling, Ph.D. CCC BRS-S ASHA 2007 Assistant Professor, University of Wisconsin-Milwaukee

Eosinophilic Esophagitis (EE)•• Structural AbnormalitiesStructural Abnormalities–– Small caliber esophagusSmall caliber esophagus–– Corrugations/multiple concentric ringsCorrugations/multiple concentric rings–– Proximal stenosisProximal stenosis–– Whitish surface vesiclesWhitish surface vesicles

Eosinophilic Esophagitis (EE)•• Diagnostic evaluationsDiagnostic evaluations–– Endo - 93%Endo - 93%–– Radiologic - 69%Radiologic - 69%–– Structural alterations 23 - 25 patientsStructural alterations 23 - 25 patients–– GERD-like features: 2 patientsGERD-like features: 2 patients–– No unique features: 2 patientsNo unique features: 2 patients•• Solid food dysphagia is the clue!Solid food dysphagia is the clue!

Graft vs. Host Disease•• Bone marrow transplant patientsBone marrow transplant patients•• Bullae, ulcers, mucosal desquamationBullae, ulcers, mucosal desquamation•• Long stricturesLong strictures

Congenital Esophageal Stenosis•• In adults- mild formIn adults- mild form•• ChildrenChildren–– Severe form requiring surgerySevere form requiring surgery•• Ring-like indentationsRing-like indentations–– ? Remnants of cartilage or? Remnants of cartilage or–– Related to GERRelated to GER

Benign Tumors•• SubmucosalSubmucosal–– Leiomyoma Leiomyoma •• 50% - benign tumors50% - benign tumors–– NeurofibromaNeurofibroma–– HemangiomaHemangioma–– FibromaFibroma

–– LipomaLipoma

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C. Easterling, Ph.D. CCC BRS-S ASHA 2007 Assistant Professor, University of Wisconsin-Milwaukee

–– Granular cell myoblastomaGranular cell myoblastoma–– HemangiopericytomaHemangiopericytoma–– CondylomaaccuminataCondylomaaccuminata

Malignant Obstruction•• Squamous Cell CarcinomaSquamous Cell Carcinoma•• AdenocarcinomaAdenocarcinoma•• CarcinoidCarcinoid•• SarcomaSarcoma•• LymphomaLymphoma•• MetastasesMetastases

Squamous Cell Carcinoma•• 50-70% of esophageal tumors50-70% of esophageal tumors•• Dysphagia develops lateDysphagia develops late•• 5 year survival <10%5 year survival <10%

•• Risk factorsRisk factors–– SmokingSmoking–– AlcoholAlcohol

Conditions With Increased Risk of Esophageal Cancer•• Squamous CellSquamous Cell–– ENT CancerENT Cancer–– AchalasiaAchalasia–– Lye StrictureLye Stricture–– Celiac DiseaseCeliac Disease–– Plummer-VinsonPlummer-Vinson–– RadiationRadiation

•• AdenocarcinomaAdenocarcinoma–– Barrett’sBarrett’s–– SclerodermaScleroderma

Radiologic Appearance

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•• Superficial esophageal cancerSuperficial esophageal cancer•• VaricoidVaricoid•• Apple-core lesionsApple-core lesions•• InfiltratingInfiltrating•• PolypoidPolypoid•• Plaque-likePlaque-like•• UlcerativeUlcerative

Adenocarcinoma•• 30-50% of esophageal malignancies30-50% of esophageal malignancies•• Virtually always on a background of Virtually always on a background of Barrett’s esophagusBarrett’s esophagus–– 10% prevalence of adenocarcinoma in patient’s 10% prevalence of adenocarcinoma in patient’s with Barrett’swith Barrett’s–– Risk of adenocarcinoma 30-40x greater in Risk of adenocarcinoma 30-40x greater in Barrett’s than general populationBarrett’s than general population

Infection•• Viral - often in immunocompromised hostViral - often in immunocompromised host•• Fungal - often in immunocompromised hostFungal - often in immunocompromised host

Candida albicans•• Immunosuppressed 2Immunosuppressed 2°°

–– Malignancy Malignancy –– DiabetesDiabetes–– AIDSAIDS–– Debilitating illnessDebilitating illness•• Most common - opportunisitic esophagitisMost common - opportunisitic esophagitis–– OdynophagiaOdynophagia–– DysphagiaDysphagia–– Chest painChest pain•• May (but not always) also have oral thrushMay (but not always) also have oral thrush•• Longitudinal plaques or nodular lesionsLongitudinal plaques or nodular lesions•• Late Stage - “shaggy” esophagusLate Stage - “shaggy” esophagus

Herpes•• Immunocompromised patientsImmunocompromised patients–– Second most common opportunisitic esophagitisSecond most common opportunisitic esophagitis•• Herpes Simplex Virus IHerpes Simplex Virus I

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•• Acute, severe odynophagia with chest painAcute, severe odynophagia with chest pain•• Vesicles rupture- small superficial ulcersVesicles rupture- small superficial ulcers•• Clustered or spread apartClustered or spread apart

Cytomegalovirus (CMV)•• AIDS patientsAIDS patients–– Less likely in Less likely in otherother immunocompromised patients immunocompromised patients•• Severe odynophagiaSevere odynophagia•• Ulcers, fold thickening, nodularity- Ulcers, fold thickening, nodularity- –– May mimic herpesMay mimic herpes•• Large flat ulcers or elongatedLarge flat ulcers or elongated–– Indistinguishable from HIVIndistinguishable from HIV

HIV•• AIDS patients with odynophagiaAIDS patients with odynophagia•• Giant ulcersGiant ulcers•• Dfferential Dx: CMV- negative brushings for CMVDfferential Dx: CMV- negative brushings for CMVCase Presentations:Overlapping Disorders of Oropharyngeal andEsophageal Function

•• Caryn EasterlingCaryn Easterling

[email protected]@uwm.edu

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