Esophageal DisordersDr. Salem M. Bazarah, MD, M.Ed, FACP, FRCPC, FRCPC(GI) & PhDAss. Prof. & Consultant Gastroenterologist, Hepatologist & Interventional Endoscopist King Abdul Aziz UniversityDirector, Liver Transplant Program & Department of Internal Medicine DSFH

Esophageal DisordersMotilityAnatomic & StructuralRefluxInfectiousNeoplasticMiscellaneous

Esophageal AnatomyUpper Esophageal Sphincter (UES)Lower Esophageal Sphincter (LES)Esophageal Body (cervical & thoracic)18 to 24 cm

Normal Phases of SwallowingVoluntaryoropharyngeal phase bolus is voluntarily moved into the pharynxInvoluntaryUES relaxationperistalsis (aboral movement)LES relaxation

Normal Phases of SwallowingBetween swallowsUES prevents air entering the esophagus during inspiration and prevents esophagopharyngeal refluxLES prevents gastroesophageal refluxperistaltic and non-peristaltic contractions in response to stimulicapacity for retrograde movement (belch, vomiting) and decompression

Normal SwallowingCortical Swallowing AreasSwallowing CenterMotor NucleiOropharynx & EsophagusFrontal cortexBrainstem

Esophageal Motility Disorders

Motility Disordersupper esophagealUES disordersneuromuscular disordersesophageal bodyachalasiadiffuse esophageal spasmnutcracker esophagusnonspecific esophageal dysmotilityLESachalasiahypertensive LESprimary disordersachalasiadiffuse esophageal spasmnutcracker esophagusnonspecific esophageal dysmotilitysecondary disorderssevere esophagitissclerodermadiabetesParkinsonsstroke

Diagnostic Tools

cineradiology or videofluoroscopy (MBS)barium esophagramesophageal manometryendoscopy

Normal Manometry

Motility DisordersBased on ManometryAchalasia Inadequate LES relaxationDiffuse Esophageal Spasm Uncoordinated contractionNutcracker Esophagus HypercontractionIneffective Esophageal Motility Hypocontraction

Achalasia

Achalasiafirst clinically recognized esophageal motility disorderdescribed in 1672, treated with whale bone bougieterm coined in 1929epidemiology1-2 per 200,000 populationusually presents between ages 25 to 60male=femaleCaucasians > othersaverage symptom duration at diagnosis: 2-5 years

PathophysiologyDegeneration of NO producing inhibitory neuronsloss of ganglionic cells in the myenteric plexus (distal to proximal)vagal fiber degenerationunderlying cause: unknownautoimmune? (antibodies to myenteric neurons in 50% of patients)that affect relaxation of LESBasal LES pressure rises

Mechanical End Resultdual disorderLES fails to appropriately relaxresistance to flow into stomachnot spasm of LES but an increased basal LES pressure often seen (55-90%)loss of peristalsis in distal 2/3 esophagus

Clinical Presentationclinical presentationsolid dysphagia 90-100% (75% also with dysphagia to liquids)post-prandial regurgitation 60-90%chest pain 33-50%pyrosis 25-45%weight lossnocturnal cough and recurrent aspiration

Diagnostic Work Upplain film (air-fluid level, wide mediastinum, absent gastric bubble, pulmonary infiltrates)barium esophagram (dilated esophagus with taper at LES) Bird peak good screening test (95% accurate)endoscopy (rule out GE junction tumors, esp. age>60)esophageal manometry (absent peristalsis, LES relaxation, & resting LES >45 mmHg)

Manometric FeaturesIncomplete LES relaxationElevated resting pressure (>45 mmHg)Aperistalsis of esophageal body

Treatment of Achalasia

Goalsreduce LES pressure and increase emptying

Nitrates and Calcium Channel BlockersIsosorbide dinitrate Reduces LES Pressure 66% for 90 minNifedipine Reduces LES pressure 30-40% for > 60 minutes

50-70% initial response;

Botulinum Toxinprevents ACH release at NM junction 90% initial response; 60% at 1 yearNeeds repetitive sessions

Pneumatic DilatationBalloon dilatation to 300 psi disrupt circular muscle 60-95% initial success; 60% at 5 years recent series suggest 20-40% will require re-dilation Success increases with repeat dilatationsrisk of perforation 1-13% (usually 3-5%); death 0.2-0.4%

Surgical Treatmentsurgical myotomy (open or minimally-invasive)>90% initial response; 85% at 10 years; 70% at 20 years (85% at 5 years with min. inv. techniques)

Spastic Motility Disorders of the Esophagus

Spastic Motility Disorders of the EsophagusDiffuse Esophageal Spasm Nutcracker EsophagusHypertensive LESNonspecific Esophageal Dysmotility

EpidemiologyAny age (mean 40 yrs)Female > Male

Clinical PresentationDysphagia to solids and liquidsintermittent and non-progressivepresent in 30-60%, more prevalent in DES (in most studies)Chest Pain constant % across the different disorders (80-90%)swallowing is not necessarily impairedcan mimic cardiac chest painPyrosis (20%) and IBS symptoms (>50%)Symptoms and Manometry correlate poorly

Diffuse Esophageal Spasmfrequent non-peristaltic contractionssimultaneous onset (or too rapid propagation) of contractions in two or more recording leadsoccur with >30% of wet swallows (up to 10% may be seen in normals)

Nutcracker Esophagushigh pressure peristaltic contractionsavg pressure in 10 wet swallows is >180 mm Hg33% have long duration contractions (>6 sec)may inter-convert with DES

Hypertensive LESNonspecific Esophageal Dysmotilityhigh LES pressure>45 mm Hgnormal peristalsisoften overlaps with other motility disorders

abnormal motility patternfits in no other categorynon-peristalsis in 20-30% of wet swallowslow pressure waves (

Diagnosis of Spastic Motility Disorders of the EsophagusManometryBarium Esophagram EndoscopyPH monitoring

Spastic Motility Disorders of the Esophagus

treatmentreassurancenitrates, anticholinergics, hydralazine - all unprovencalcium channel blockers - too few data with negative controlled studies in chest painpsychotropic drugs trazodone, imipramine and setraline effective in controlled studiesdilation - anecdotal reports, probable placebo effect

Manometry in Esophageal SymptomsNon-Cardiac Chest PainDysphagiaJE Richter, Ann Int Med, 1987

Hypomotilty Disordersprimary (idiopathic)aging produces gradual decrease in contraction strengthreflux patients have varying degrees of hypomotilitymore common in patients with atypical reflux symptomsusually persists after reflux therapydefined aslow contraction wave pressures ( of wet swallows

Hypomotilty Disorderssecondarysclerodermain >75% of patientsprogressive, resulting in aperistalsis in smooth-muscle regionincompetent LES with refluxother connective tissue diseasesCRESTpolymyositis & dermatomyositisdiabetes60% with neuropathy have abnormal motility on testing (most asx)otherhypothyroidism, alcoholism, amyloidosis

Non ischemic Chest Painremains poorly understood (functional chest pain)enthusiastic investigation finds numerous associations in studiespsychiatric disorders (depression, panic or anxiety disorder)esophageal disorders (GERD, motility disorders)musculoskeletal disorderscardiac disease (microvascular, MVP, tachyarrhythmias)

Non ischemic Chest PainGERD is by far the most common, diagnosable, esophageal cause50-60% of patients have heartburn or acid regurgitation symptoms50% have abnormal esophageal pH studies (not always correlating to sxs)very low incidence of endoscopic findingsPPI Test may be best and most cost-effective approacha small subset of patients with non-GERD NCCP display a variety of esophageal motility disorderssymptoms and motility findings correlate poorlyesophageal hypersensitivity/hyperalgesia may explain the symptoms

GERD36-77% of all Americans experienceGERD 7% have daily GERD symptoms 14-20% weekly symptoms 15-50% monthlySymptoms include: heartburn, acidregurgitation, water brash, dysphagia,atypical symptoms (asthma, globus,laryngitis, cough, throat clearing)

PathophysiologyLower esophageal sphincter dysfunctionDelayed gastric emptyingEsophageal dysmotility+/- hiatal herniaRepetitive mucosal injury / esophagitisBarretts Esophagus

Medical TreatmentLifestyle modifications avoid coffee, fatty foods, smoking; lose weight, raise head of bed, eliminate late night mealsAcid suppressin via PPIs

Indications for SurgeryFailed medical managementNeed for lifelong medical therapyHiatal herniaAtypical symptoms with (+) pH probeComplications Barretts esophagus (5-15% develop BE) Erosive esophagitis

Surgical TreatmentPre-operative evaluation Esophagram EGD Manometry (resting LES >5, length >2cm) 24-hr esophageal pH monitoring

Surgical TreatmentLaparoscopic Nissen FundoplicationGoals of antireflux surgery: Recreate Angle of His Reconstitute LES with wrapPredictors of good surgical outcome: typical symptoms (heartburn, regurg) abnormal pH score, but NML motility clinical response to acid suppressiontherapy

Other New TreatmentsStretta...radiofrequecy ablation of LESEnteryx, Gatekeeper...implantedbiopolymer into LESEndocinch, Plicator...endoscopic suturingto recreate LES

GERD Controversies Are meds better than antireflux surgery?Does antireflux surgery allow regression of Barretts esophageal better than meds?Which is more cost effective?Does symptom relief correlate with esophageal acid exposure?Where do the newer endoscopic therapies stand?

Quiz?51 yrs old lady presented with chest pain , difficulty to swallow, post prandial vomitingEndoscopy failed to intubate the esophagusPPI givenSymptoms improve

*LDLT 5th case*

GERD Medical Vs Surgical TherapyIn 1992, VA Cooperative study found open Nissenfundoplication better than antacids, H2 blockers incontrolling GERDIn 2001, VA Coop study follow-up at 10 years showed62% of surgical arm used acid suppression meds forsymptom controlFew deaths due to esoph cancer, but study wasunderpowered to detect difference

GERD Medical Vs Surgical TherapyA multicenter Nordic study evaluated treatmentfailures of Omeprazole to Nissen fundoplication failure defined as: mod/severe heartburn,dysphagia or regurg; grade 2 esophagitis; > 8 wkspost-op requiring PPIAt 12 months surgery was favoredBut at five year follow-up, open surgery appearedsuperior, but when allowing for escalating doses ofPPI, each strategy was similar for symptom controlLundell et al. Gastroenterology 114:A207, 1998.Lundell et al. JACS 192:172-179, 2001

GERD Medical Vs Surgical TherapyUK study evaluated laparoscopic Nissen toPPI therapy in 217 randomized patients withchronic GERDAt three months, LNF group had improvedLES pressure, DeMeester acid eposurescore, GI symptom and general well-beingscore as compared to PPI group, and lastedto twelve monthsMahon et al. Brit Journ Surg 92:695-699, 2005.

Regression Of Barretts PPI compared to LNF in 35 non-randomizedpts with low-grade dyspasia detected onsurveillance EGD12 of 19 (63%) in PPI group had regression ofLGD to Barretts compared to 15 of 16 (93%)of LNF pts at 12 and 18 monthsIs biliopacreatic reflux to blame for BE?Rossi et al. Annals of Surgery 243:58-63, 2006.

DO Symptoms Correlate with Treatment (Success/Failure)24 hr pH and DeMeester acid scorescompared in 70 pts on no meds, on PPIs, orafter antireflux surgeryLES pH decreased most by LNF18 of 30 PPI pts asymptomatic but hadpathologic pH probe testing19 LNF pts complained of heartburn/regurg,only two had positive pH probeJenkinson et al. Brit Jour Surg 91:1460-1465, 2004.

Hiatal Hernia

Pathophysiology & ClassificationType I - slidingType II - paraesophagealType III - para and sliding componentType IV - other viscera involved

Clinical Presentationpostprandial fullness (63%),Reflux (31%), Dysphagia (34%), Bleeding (24%)Regurgitation/vomiting (36%)Dyspnea (11%)

Work Up

Surgical TreatmentEffective repair includes: Excision of hernia sac Reduction of hernia contents Repair of crural defect Fundoplication, gastropexy, PEG, esophageal lengthening (Collis gastroplasty)

Upper Esophageal Motility Disorders

Overviewcause oropharyngeal dysphagia (transfer dysphagia)patients complain of difficulty swallowingtracheal aspiration may cause symptomspharyngoesophageal neuromuscular disordersstrokeParkinsonspoliomyelitisALSmultiple sclerosisdiabetesmyasthenia gravisdermatomyositis and polymyositisupper esophageal sphincter (cricopharyngeal) dysfunction

Overviewcricopharyngeal hypertensionelevated UES resting tonepoorly understood (reflex due to acid reflux or distension)cricopharyngeal achalasiaincomplete UES relaxation during swallowmay be related to Zenkers diverticula in some patientsclinical manifestationslocalizes as upper (cervical) dysphagiawithin seconds of swallowingcoughing, choking, immediate regurgitation, or nasal regurgitationdiagnosis: swallow evaluation & modified barium swallow

**Functionally the esophagus can be considered in three zones (UES, body, LES). The upper esophageal sphincter (UES) is tonically closed and contracts during inspiration, preventing air from entering into the gastrointestinal tract, and reflux. It relaxes during swallow, belching and vomiting. The lower esophageal sphincter (LES) maintains a steady baseline tone (~20 mm Hg) to prevent gastric contents from entering the esophagus. The LES also contracts during periods of increased intra-abdominal pressure, preventing reflux due to the pressure build up in the abdomen. The esophagus has 2 muscle layers: the inner circular layer and the outer longitudinal layer. The longitudinal muscle shortens the esophagus, while the circular muscle forms lumen-occluding ring contractions. The combination of these localized contractions is responsible for peristalsis. The proximal esophagus contains striated muscle and the distal esophagus smooth muscle, with a long transition zone between. Striated muscle is faster.*Primary peristalsis is a wave that strips the esophagus proximal to distal, pushing the food bolus aborally toward the stomach. In the body, the wave travels at 3-4 cm/s. Secondary peristalsis is induced by esophageal distension from retained bolus, reflux, or swallowed air. Its role is to clear the esophagus of retained food. Tertiary contractions are simultaneous (nonperistaltic), dysfunctional contractions and have no known physiologic role and are seen more often in the elderly and can be induced by stimuli (stress, reflux, etc.).*Primary peristalsis is triggered by the swallowing center. Myogenic propagation occurs in the body of the esophagus.**These can be further divided into hypomotlity and hypermotlity disorders.*From S&F, 2002. Notice transition zone (mid-esophagus) with smaller amplitude contractions.*Achalasia means failure to relax. Incomplete LES relaxation without aperistalsis can be an early manifestation of achalasia, but infrequently.**This condition is often split from the rest because the response to swallows is non-peristaltic. *1/3 of pts with non-specific dysmotility will have LES hypertension.*Some anecdotal evidence that the drugs can improve manometric findings, but clinical trials have been disappointing.In the trazodone (100-150 mg/d) study, a significant improvement was seen but it was not dependent on manometric improvement. A JAMA trial of dilation found a positive effect equaled by a placebo dilator.*These disorders tend to be considered separately from disorders of the body and LES because they are usually due to problems with striated muscle or its extrinsic innervation.