A review of Esophageal Disorders

1. Esophageal DisordersEsophageal Disorders
Shima Ghavimi, MDShima Ghavimi, MD
Internal MedicineInternal Medicine
PGY2PGY2

2. Esophageal DisordersEsophageal Disorders
Anatomic &Anatomic &
StructuralStructural
Motility disorderMotility disorder
RefluxReflux
Pill induced andPill induced and
eosinophiliceosinophilic
esophagitisesophagitis

3. Structural DisorderStructural Disorder
 Hiatal herniaHiatal hernia
 Rings and WebsRings and Webs
 DiverticulaDiverticula
 TumorsTumors

4. HiatalHiatal HerniaHernia
Herniation of viscera,Herniation of viscera,
Most commonlyMost commonly
stomach intostomach into
mediastinum throughmediastinum through
the esophageal hiatusthe esophageal hiatus
of the diaphragm.of the diaphragm.

5.  Type 1 or sliding hiatal hernia:Type 1 or sliding hiatal hernia:
 95% of total95% of total
 Gastroesophageal junction and gastricGastroesophageal junction and gastric
cardia slide upwardcardia slide upward
 As a result of weakening of theAs a result of weakening of the
phrenoesophageal ligamentphrenoesophageal ligament

7.  Type II, III, and IV are all subtypes of paraType II, III, and IV are all subtypes of para
esophageal herniaesophageal hernia
 Type II and type III the gastric fundus alsoType II and type III the gastric fundus also
herniatesherniates
 Difference: in type II GE junction remainsDifference: in type II GE junction remains
fixed at the hiatusfixed at the hiatus
 Type III: mixed sliding /paraesophagealType III: mixed sliding /paraesophageal
herniahernia

8.  Type IV: viscera other than the stomachType IV: viscera other than the stomach
herniates, MC the colonherniates, MC the colon

10. Rings and WebsRings and Webs
 A lower esophageal mucosal ring (B ring)A lower esophageal mucosal ring (B ring)
thin membranous narrowing at thethin membranous narrowing at the
squamocolumnar mucosal junctionsquamocolumnar mucosal junction
 Its origin is unknownIts origin is unknown
 Usually asymptomaticUsually asymptomatic
 When lumen diameter isWhen lumen diameter is less than 13 mmless than 13 mm
can causecan cause episodic solid food dysphagiaepisodic solid food dysphagia
and isand is called Schatzki ring.called Schatzki ring.

11.  Is the MCC of intermittent food impactionIs the MCC of intermittent food impaction
 Also known asAlso known as Steakhouse syndromeSteakhouse syndrome
 Symptomatic rings treated by dilatationSymptomatic rings treated by dilatation

12.  Web-like constrictions higherWeb-like constrictions higher in thein the
esophagus can be ofesophagus can be of congenitalcongenital oror
inflammatoryinflammatory in originin origin
 When circumferential causeWhen circumferential cause intermittentintermittent
dysphagia to solidsdysphagia to solids similar to schatzkisimilar to schatzki
ringsrings
 Symptomatic proximal esophagealSymptomatic proximal esophageal
webs+IDA in middle aged women is calledwebs+IDA in middle aged women is called
Plummer-VinsonPlummer-Vinson syndsynd..

14. DiverticulaDiverticula
 Categorized by location:Categorized by location:
 EpiphrenicEpiphrenic
 Hypopharyngeal (zenker)Hypopharyngeal (zenker)
 Mid esophagealMid esophageal

15.  Epiphrenic and zenkerEpiphrenic and zenker are bothare both falsefalse
diverticuladiverticula(herniation of mucosa and sub(herniation of mucosa and sub
mucosa through muscular layer of themucosa through muscular layer of the
esophageal layer)esophageal layer)
 Result fromResult from increased intraluminalincreased intraluminal
pressure A/W distal obstructionpressure A/W distal obstruction

16.  In Zenker Herniation occurs at the naturalIn Zenker Herniation occurs at the natural
weakness which is calledweakness which is called killian trianglekillian triangle

18.  Small zenkerSmall zenker diverticula is usuallydiverticula is usually
asymptomaticasymptomatic if it enlarge enough to retainif it enlarge enough to retain
saliva and food it can cause dysphagia,saliva and food it can cause dysphagia,
halitosis, aspiration.halitosis, aspiration.
 Treatment: surgeryTreatment: surgery

19. Epiphrenic diverticulaEpiphrenic diverticula usually A/Wusually A/W
achalasia or distal esophageal strictureachalasia or distal esophageal stricture
Mid esophageal diverticulaMid esophageal diverticula :May:May
be caused by traction from adjacentbe caused by traction from adjacent
inflammation( classically TB)inflammation( classically TB)
 Both are asymptomaticBoth are asymptomatic until they enlargeuntil they enlarge
and causeand cause dysphagia and regurgitationdysphagia and regurgitation

20. TUMORSTUMORS
 Esophageal cancer occurs in aboutEsophageal cancer occurs in about
4.5:100,000 in the U.S4.5:100,000 in the U.S
 Mortality is 4.4:100,000Mortality is 4.4:100,000
 Two types:Two types: adenocarcinomaadenocarcinoma andand SCCSCC
 RFs:RFs: adenocarcinomaadenocarcinoma strongly related tostrongly related to
thethe GERDGERD and Barrett metaplasiaand Barrett metaplasia
 SCC :SCC : smoking, alcohol , caustic injury,smoking, alcohol , caustic injury,
HPV infectionHPV infection
 Harrison principle of internal medicine 18Harrison principle of internal medicine 18thth
editionedition

21.  Typical presentation:Typical presentation: progressive solidprogressive solid
food dysphagia and weight lossfood dysphagia and weight loss
 Associated symptoms:Associated symptoms: odynophagiaodynophagia,, ironiron
deficiency anemiadeficiency anemia, and, and with midwith mid
esophageal tumors: hoarseness from leftesophageal tumors: hoarseness from left
recurrent laryngeal nerve injuryrecurrent laryngeal nerve injury

22. GERDGERD
 It has been estimated that 15% of adults inIt has been estimated that 15% of adults in
the United States are affected by GERD.the United States are affected by GERD.
Harrison principle of internal
medicine 18th
edition

23. SymptomsSymptoms
 Typical symptoms: heart burn andTypical symptoms: heart burn and
regurgitationregurgitation
 Less common : chest pain and dysphagiaLess common : chest pain and dysphagia
 Extra esophageal symptomsExtra esophageal symptoms (asthma,(asthma,
globus, laryngitis, cough, throatglobus, laryngitis, cough, throat
clearing)clearing)
Atypical symptoms :dyspepsia, epigastric
pain, nausea, bloating

24. complicationscomplications
 EsophagitisEsophagitis
 BleedingBleeding
 StrictureStricture
 Barret esophagusBarret esophagus
 Esophageal adenocarcinomaEsophageal adenocarcinoma

26. DiagnosisDiagnosis
 Typical symptoms such as heart burn,Typical symptoms such as heart burn,
regurgitation or both is enough toregurgitation or both is enough to
diagnose GERD.diagnose GERD.
 A favorable response to PPI is alsoA favorable response to PPI is also
supportive evidence for GERD and issupportive evidence for GERD and is
reasonable first step in patients withoutreasonable first step in patients without
alarm symptoms.alarm symptoms.

27.  Endoscopy is first step in patient withEndoscopy is first step in patient with
alarms symptoms (dysphagia, anemia,alarms symptoms (dysphagia, anemia,
vomiting or weight loss, age >50)vomiting or weight loss, age >50)
 If patients do not respond to PPI and haveIf patients do not respond to PPI and have
negative Upper endoscopy to make anegative Upper endoscopy to make a
definitive diagnosis ambulatory PHdefinitive diagnosis ambulatory PH
monitoring is usually performed.monitoring is usually performed.

29. Medical TreatmentMedical Treatment
 Lifestyle modificationsLifestyle modifications
1.1. Weight loss is recommended for GERDWeight loss is recommended for GERD
patients who are overweight or have hadpatients who are overweight or have had
recent weight gain.recent weight gain.
2. Head of bed elevation and avoidance of2. Head of bed elevation and avoidance of
meals 2–3h before bedtime should be meals 2–3h before bedtime should be
recommended for patients with nocturnalrecommended for patients with nocturnal
GERD.GERD.

30.  Routine global elimination of food that canRoutine global elimination of food that can
trigger reflux (including chocolate,trigger reflux (including chocolate,
caffeine, alcohol, acidic and/or spicycaffeine, alcohol, acidic and/or spicy
foods) is not recommended in thefoods) is not recommended in the
treatment of GERD.treatment of GERD.
 Acid suppression via PPI’s for 8 weeks,30-Acid suppression via PPI’s for 8 weeks,30-
60 min before meal60 min before meal

31. For patients with partial response to once
daily therapy, tailored therapy with
adjustment of dose timing and/or twice daily
dosing should be considered.

32.  Non-responders to PPI should be referredNon-responders to PPI should be referred
for evaluationfor evaluation
Maintenance therapy with the lowest
effective dose if needed.

33.  without erosive disease bedtime H2
blocker therapy can be added to daytime
PPI therapy in selected patients evidence
of night-time reflux if needed.
PPIs are safe in pregnancy.

34. Indications for SurgeryIndications for Surgery
Failed optimal medical management
Noncompliance with medical therapy
High volume reflux
Severe esophagitis by endoscopy
Benign stricture
Barrett's columnar-lined epithelium
(without severe dysplasia or carcinoma)
Up-to-dateUp-to-date

35. Barrett esophagusBarrett esophagus
 Is a complication of GERDIs a complication of GERD
 Normal squamous epithelium of the distalNormal squamous epithelium of the distal
esophagus is replaced by columnaresophagus is replaced by columnar
epitheliumepithelium

36.  Is premalignantIs premalignant
 Annual incidence of esophagealAnnual incidence of esophageal
adenocarcinoma is 0.5%adenocarcinoma is 0.5%
 10% of patients with chronic GERD10% of patients with chronic GERD
symptoms have Barrett on endoscopysymptoms have Barrett on endoscopy

37.  The diagnosis of BE
is suggested by
endoscopic findings
and is confirmed
histologically by the
presence of
specialized intestinal
metaplasia with acid-
mucin–containing
goblet cells

40. Esophageal MotilityEsophageal Motility
DisordersDisorders

41. AchalasiaAchalasia

42. AchalasiaAchalasia
 Incidence of 1:100,000Incidence of 1:100,000
population annuallypopulation annually
 usually presents between agesusually presents between ages
30 to 6030 to 60
 male=femalemale=female
 No racial predilectionNo racial predilection
American college of gastroenterology guidelineAmerican college of gastroenterology guideline

43. PathophysiologyPathophysiology
 Degeneration of NO producing inhibitory neuronsDegeneration of NO producing inhibitory neurons
 loss of ganglionic cells in the myenteric plexus (distalloss of ganglionic cells in the myenteric plexus (distal
to proximal)to proximal)
 vagal fiber degenerationvagal fiber degeneration
 that affect relaxation of LESthat affect relaxation of LES
 Basal LES pressure risesBasal LES pressure rises

44. underlying cause: unknownunderlying cause: unknown
Autoimmune, viral immune, or
neurodegenerative.

45. Clinical PresentationClinical Presentation
dysphagiadysphagia (most patients report solid and liquid(most patients report solid and liquid
food dysphagia)food dysphagia)
regurgitationregurgitation of food retained in the prox.of food retained in the prox.
Dilated esophagusDilated esophagus
chest painchest pain (squeezing, retrosternal, radiates(squeezing, retrosternal, radiates
to jaw, neck, arms or back and worsen withto jaw, neck, arms or back and worsen with
food)food)
weight lossweight loss
nocturnal cough and recurrent aspirationnocturnal cough and recurrent aspiration
Sensation of heartburn in 30%Sensation of heartburn in 30% ,assumed to,assumed to
be related retained food fermentation and lacticbe related retained food fermentation and lactic
acid formationacid formation

46. DDXDDX
 DESDES
 Chagas(endemic in central brazil,Chagas(endemic in central brazil,
Venezuela, NorthernVenezuela, Northern
argentina)argentina)reduvid(kissing)reduvid(kissing)
bugsbugstransmits Tryponosomatransmits Tryponosoma
CruziCruzidestruction of autonomic gangliondestruction of autonomic ganglion
cells in heart, urinary tract, gut andcells in heart, urinary tract, gut and
respiratory tractrespiratory tract

47.  Pseudoachalasia: tumor infiltration ,MCPseudoachalasia: tumor infiltration ,MC
seen with carcinoma of the gastric fundusseen with carcinoma of the gastric fundus
or distal esophagusor distal esophagus
 More likely with advanced age, abruptMore likely with advanced age, abrupt
onset of symptoms(<1yr) and weight lossonset of symptoms(<1yr) and weight loss

48. Diagnostic Work UpDiagnostic Work Up
plain film (air-fluid level, wide mediastinum,plain film (air-fluid level, wide mediastinum,
absent gastric bubble)absent gastric bubble)
Barium swallow (Barium swallow (Bird beak sign)Bird beak sign)
Primary screening test (95% accurate)Primary screening test (95% accurate)
endoscopy (rule out GE junction tumors)endoscopy (rule out GE junction tumors)
esophageal manometry (absent peristalsis,esophageal manometry (absent peristalsis, ↓↓
LES relaxation, & resting LES >45 mmHg)LES relaxation, & resting LES >45 mmHg)

50. Manometric FeaturesManometric Features
 Incomplete LESIncomplete LES
relaxationrelaxation
 Elevated restingElevated resting
pressure (>45pressure (>45
mmHg)mmHg)
 Aperistalsis ofAperistalsis of
esophageal bodyesophageal body

51. TreatmentTreatment
Achalasia is a chronic condition without
cure
Goal of treatments:
 reduce LES pressure andreduce LES pressure and
 increase stomach emptyingincrease stomach emptying

52. Pharmacologic therapyPharmacologic therapy
 Is the least effective therapyIs the least effective therapy
 CCB and long acting nitrates are the MCCCB and long acting nitrates are the MC
medications to usemedications to use
 Nifedipine, it should be used (10–30 mg)
sublingually 30–45 min before meals for best
response

53. Sublingual isosorbide dinitrate
Effective in 30–65%
5 mg only 10–15 min before meals.

54.  The phosphodiesterase-5-inhibitor, sildenafil,
has also been shown to lower the LES in
achalasia.
 Headache, hypotension, and pedal edema are
common limiting factors in their use, also they
do not provide complete relief of symptoms

55. Reserved for patients with achalasia who
1. Cannot or refuse to undergo more
definitive therapies (PD or surgical
myotomy)
2.Who have failed botulinum toxin
injections.

56. Botulinum ToxinBotulinum Toxin
 prevents ACH release at NM junctionprevents ACH release at NM junction
Approximately 50% of patients relapse
and require repeat treatments at 6–24-
month intervals
.

57. Serious side effects are uncommon
16–25% rate of developing chest pain and
rare complications, such as mediastinitis
and allergic reactions related to egg
protein

59. Pneumatic DilatationPneumatic Dilatation
Is the most effective nonsurgical option
 All patients considered for PD must also
be candidates for surgical intervention in
the event of esophageal perforation
needing repair.

60.  After dilation radiographic testing by
gastrograffin study should be done to
exclude esophageal perforation.
 Vomiting after procedure is possible.
Developing severe chest pain with or
without fever after discharge needs an
immediate medical attention.

61.  Efficacy ranging from 32-98%Efficacy ranging from 32-98%
 Major complication: perforation withMajor complication: perforation with
incidence of 1-5%incidence of 1-5%
 Success increases with repeat dilatationsSuccess increases with repeat dilatations

62. MyotomyMyotomy
 Usually performed in conjunction withUsually performed in conjunction with
fundoplicationfundoplication
>90% initial response; 85% at 10 years; 70%>90% initial response; 85% at 10 years; 70%
at 20 years (85% at 5 years with min. inv.at 20 years (85% at 5 years with min. inv.
techniques)techniques)
 <1% mortality; <10% major morbidity<1% mortality; <10% major morbidity

63. EsophagectomyEsophagectomy
Indication:
1. End-stage” achalasia or sigmoid
esophagus
 PD may be less effective
 A surgical myotomy may be an initial approach before
consideration for esophagectomy.

64. 2.who have failed PD and/or myotomy and
who are good candidates for surgery
Dysphagia requiring dilation may occur in
up to 50% of patients after esophagectomy.

66. Spastic Motility Disorders of theSpastic Motility Disorders of the
EsophagusEsophagus

67. Spastic Motility Disorders of theSpastic Motility Disorders of the
EsophagusEsophagus
 Diffuse Esophageal SpasmDiffuse Esophageal Spasm
 Nutcracker EsophagusNutcracker Esophagus

68. EpidemiologyEpidemiology
 Any age (mean 40 yrs)Any age (mean 40 yrs)
 Female > MaleFemale > Male

69. Clinical PresentationClinical Presentation
 Dysphagia to solids and liquidsDysphagia to solids and liquids
 intermittent and non-progressiveintermittent and non-progressive
 present in 30-60%, more prevalent in DES (in most studies)present in 30-60%, more prevalent in DES (in most studies)
 Chest PainChest Pain
 swallowing is not necessarily impairedswallowing is not necessarily impaired
 can mimic cardiac chest paincan mimic cardiac chest pain

70. Diffuse Esophageal SpasmDiffuse Esophageal Spasm
 Manometry showsManometry shows
intermittent , highintermittent , high
amplitude (>30mmHg) ,amplitude (>30mmHg) ,
simultaneous and non-simultaneous and non-
peristaltic contractionsperistaltic contractions
in response toin response to
swallowing.swallowing.
 Diagnosis is made byDiagnosis is made by
clinical presentation,clinical presentation,
and typical findings onand typical findings on
barium swallow and/orbarium swallow and/or
manometry followingmanometry following
exclusion of otherexclusion of other
disorders.disorders.

71. Nutcracker EsophagusNutcracker Esophagus
 high pressure peristaltichigh pressure peristaltic
contractionscontractions
 avg pressure in wetavg pressure in wet
swallows is >220 mmswallows is >220 mm
HgHg
 33% have long duration33% have long duration
contractions (>6 sec)contractions (>6 sec)

72. TreatmentTreatment
 Symptomatic reliefSymptomatic relief
 CCBs are first-line treatmentCCBs are first-line treatment
 Trazodone and imipramine could beTrazodone and imipramine could be
helpfulhelpful
 Botox injection maybe considered whenBotox injection maybe considered when
these therapies are unsuccessfulthese therapies are unsuccessful

73. Hypomotilty DisordersHypomotilty Disorders
 primary (idiopathic)primary (idiopathic)
 defined asdefined as
low contraction wave pressures (<30 mm Hg)low contraction wave pressures (<30 mm Hg)
aging produces gradual decrease in contractionaging produces gradual decrease in contraction
strengthstrength

74. Hypomotilty DisordersHypomotilty Disorders
 secondarysecondary
 sclerodermascleroderma
 in >75% of patientsin >75% of patients
 aperistalsis in manometryaperistalsis in manometry
 Smooth muscle atrophy and fibrosisSmooth muscle atrophy and fibrosisloss of peristalsis andloss of peristalsis and
weakening of LES with refluxweakening of LES with reflux
 other “connective tissue diseases”other “connective tissue diseases”
 CRESTCREST
 polymyositis & dermatomyositispolymyositis & dermatomyositis
 diabetesdiabetes
 60% with neuropathy have abnormal motility on testing (most60% with neuropathy have abnormal motility on testing (most
asx)asx)
 otherother
 hypothyroidism, alcoholism, amyloidosis, narcoticshypothyroidism, alcoholism, amyloidosis, narcotics

75. Eosinophilic EsophagitisEosinophilic Esophagitis
 Prevalence is 1:1000Prevalence is 1:1000
 More common in white maleMore common in white male
 Consider in cases of Dysphagia and foodConsider in cases of Dysphagia and food
impaction regardless of the presence orimpaction regardless of the presence or
absence of heartburnabsence of heartburn
Atypical chest pain and heartburn that isAtypical chest pain and heartburn that is
refractory to PPI.refractory to PPI.

76.  History of food allergy, asthma, eczemaHistory of food allergy, asthma, eczema
or allergic rhinitisor allergic rhinitis
 Endoscopic finings: multiple esophagealEndoscopic finings: multiple esophageal
rings, linear furrows and punctaterings, linear furrows and punctate
exudatesexudates
 Histologic confirmation: >15 eos per HPFHistologic confirmation: >15 eos per HPF

77. GERD must be excluded by ambulatory
pH monitoring with lack of response to a
therapeutic trial of a PPI twice a day for 6
weeks.

78. TreatmentTreatment
-Swallowed aerosolized corticosteroid
-In refractory cases may need a combination
of esophageal dilation, systemic
corticosteroids, or a food elimination diet

79. Pill induced esophagitisPill induced esophagitis
Symptoms: odynophagia, dysphagia, and
sometimes retrosternal chest pain.
Tetracycline, iron sulfate,
bisphosphonates, potassium, NSAIDs,
and quinidine
Diagnosis is suspected by medication
review and is confirmed by endoscopy.

80. Treatment typically includes temporary
cessation of the culprit medication or
taking the medication with a large bolus of
water and avoiding a recumbent posture
for 30 to 60 min.

82.  Especial thanks to:Especial thanks to:
 Dr. LaiyemoDr. Laiyemo
 Dr. AgaziDr. Agazi
 Dr. HemmingsDr. Hemmings

83. 02/28/1602/28/16 LDLT 5th caseLDLT 5th case 8383

84. ReferencesReferences
 Harrison principle of internal medicineHarrison principle of internal medicine
 MKSAPMKSAP
 Up-to-dateUp-to-date
 MedscapeMedscape
 American gastroenterology guidelinesAmerican gastroenterology guidelines
 John Hopkin modulesJohn Hopkin modules