Disorders of the Esophagus

Definition

• a physiologic phenomenon that allows venting of swallowed air from the stomach

• TLESR occurs in response to distension of the proximal stomach

  • presence of hiatal hernia may increase frequency of TLESR

• in GERD, patients typically reflux gastric contents through a compliant GEJ during a TLESR

  • frequency of TLESRs may also be higher in GERD

• TLESRs are suppressed during deep sleep

• they are less frequent when LES relaxation is abnormal (ex. esophageal outflow obstruction)

• frequency is not related to degree of gastric acid secretion in the stomach

Risk Factors

• obesity and OSA

  • can have increased frequency of TLESRs

Treatment

• baclofen

  • GABA B receptor agonist

  • can reduce TLESR frequency

  • can reduce reflux episodes 

Risk Factors

• reduced saliva production following radiation for head and neck cancers

  • salivary bicarbonate is needed for neutralization of mucosal acidification following volume clearance of refluxate

  • increased risk of erosive esophagitis, but no evidence for increase prevalence of Barrett's esophagus 

• women have... 

  • a shorter length of esophagus regardless of height

  • lower rates of erosive esophagitis and Barrett's esophagus 

Treatment

• PPI therapy

• repeat EGD to assess for healing

Defintion

• normal EGD

• increased acid exposure on ambulatory reflux monitoring

Defintion

• Rome IV

  • typical heartburn symptoms

  • normal EGD

  • normal pH/impedance testing

  • negative association between symptoms and reflux events

Treatment

• SSRIs

Definition

• vague symptoms of constant sensation in the throat

• non-painful

• no dysphagia or odynophagia

• symptoms occur between meals

• no inlet patch or other structural abnormalities

• GERD is commonly associated with globus

• all organic causes should be ruled out prior to treating globus as a functional disorder

Signs and Symptoms

• dysphagia

Treatment

• high resolution manometry to rule out major motor disorder

  • may frequently identify a nonspecific motility disorder

• pH monitoring to establish diagnosis of GERD if present

• trial PPI

• APC or RFA ablation of cervical gastric inlet patch associated with improvement of globus symptoms in PPI non-responsive patients in small studies

  • no role for ablation in the distal esophagus

Definition

• the proximal esophagus at the level of the aortic arch is a site where swallowed pills can be held up

  • this area corresponds to a transition zone between skeletal and smooth muscle in the esophageal wall

  • may be associated with lower contractile pressures

Signs and Symptoms

• dysphagia

• retrosternal chest pain during swallowing

Risk Factors

• Doxycycline

  • can cause intense local reaction with prolonged contact with esophageal mucosa leading to erosion or ulceration

Pathology Findings

• Esophageal erosion biopsy - acute neutrophilic infiltrate with no viral inclusions

Definition

• investigate with EGD at presentation with careful inspection of GEJ to rule out malignancy causing pseudoachalasia

• perform manometry after EGD to determine subtype of achalasia

• Achalasia subtypes may present early and may not have classic findings

  • may have an elevated Integrated Relaxation Pressure (IRP) with preserved peristalsis indicating evolving achalasia 

Endoscopic Findings

• EGD

  • dilated esophagus

  • rosette appearance of lower esophageal sphincter

• High resolution manometry

  • Type 1 - classic

    • elevated IRP 

    • 100% failed peristalsis

  • Type 2

    • elevated IRP

    • 100% failed peristalsis

    • panesophageal pressurization (20% or more of swallows)

  • Type 3 - spastic

    • elevated IRP

    • premature contractions with distal latency <4.5 seconds (20% or more of swallows)

• pH monitoring

  • elevated acid exposure may reflect stasis in the setting of distal esophageal obstruction rather than true GERD

Imaging Findings

• barium swallow

  • bird's beak appearance 

Treatment

• Type 1 Achalasia

  • Heller myotomy/POEM or pneumatic dilation to 30 mm or more - first line

  • botox injection or calcium channel blocker - second line

• Type 2 Achalasia

  • Heller myotomy/POEM or pneumatic dilation to 30 mm or more - first line

• Type 3 Achalasia

  • POEM is first line

  • Botox injection of not a candidate for POEM

• Surgical myotomy

  • modified Heller approach

• Per oral endoscopic myotomy (POEM)

  • highest risk for GERD and reflux esophagitis following procedure

  • up to 19% develop reflux symptoms, 29% have reflux esophagitis, and 39% have abnormal pH testing after POEM

    • compared to 9%, 8%, and 17% respectively following laproscopic myotomy 

• Pneumatic dilation

  • reflux can occur but rates are lower than following POEM

• Botulinum toxin injection

  • in poor surgical candidates

  • reflux symptoms occur infrequently following injection

  • causes scarring and multiple injections could make subsequent surgery more difficult

• Calcium channel blocker

  • not a definitive treatment option

  • oral nifedipine TID

  • reflux symptoms occur infrequently

• Nitrates

  • not a definitive treatment option

  • unlikely to help in late-stage achalasia 

• Recurrence of symptoms after achalasia treatments/surgeries 

  • trial pneumatic balloon dilation before more invasive approaches

Definition

• elevated IRP in both supine and upright positions

• intrabolus pressurization (20% or more of swallows)

• does not meet criteria for achalasia

Definition

• normal median IRP

• 50% or more failed or 70% or more ineffective swallows

• Ineffective swallow

  • distal contractile integral < 450 mmHg-s-cm

  • fragmented swallow with peristaltic break (>5 cm)

• Failed swallow

  • distal contractile integral < 100 mmHg-s-cm

Definition

• normal IRP

• 100% failed peristalsis

Versus

• Type 1 Achalasia with elevated IRP and 100% failed peristalsis

Definition

• Rome IV criteria

  • chronic dysphagia

  • no structural abnormalities 

  • no mucosal abnormalities

  • no GERD, eosinophilic esophagitis, or motility disorders

Risk Factors

• diabetes mellitus

• HIV-AIDS

• immunosuppressive therapy

• antibiotics use

• corticosteroid use

• achalasia

• esophageal cancer

Signs and Symptoms

• odynophagia

Endoscopy Findings

• small shallow ulcerations

• yellow exudate

Pathology Findings

• multinucleated giant cells

Management

• evaluate with barium radiography with solid barium bolus

• high resolution impedance manometry for 

  • unexplained dysphagia 

  • persistent esophageal symptoms 

Definition

• Diagnosis confirmed on biopsy with >15 eosinophils/hpf

• Esophageal manometry can be done if EGD +/- barium radiography are unrevealing

Signs and Symptoms

• food impaction

• dysphagia

• intermittent retrosternal pain

Risk Factors

• Young males with solid food dysphagia

  • initial investigation with EGD with proximal and distal esophageal biopsies

  • EGD should be performed prior to initiation of PPI therapy if EoE is suspected

    • PPI can normalize eosinophil counts

Endoscopy Findings

• EGD

  • my have absent esophageal findings

  • edema - loss of vascular markings

  • rings - trachealization

  • exudate - white plaques

  • furrows - vertical lines

  • stricture

• EoE EREFS (endoscopic reference score)

  • used to standardize findings

Treatment

• PPI therapy BID

  • up to 50% will have normalization of biopsies on PPI

  • repeat EGD after 8 wks of BID PPI to evaluate for persistent eosinophilia

  • over time the beneficial effect of PPIs appears to wane

• Swallowed budesonide (0.5-1 mg BID) or fluticasone (440-880 mcg BID). Budesonide oral dispersible tablet (BOT) available in Europe

  • use in the case of persistent eosinophilia after PPI therapy

  • treat to effect with lowest effective dose

  • alternatively may trial elimination diet

  • suggestions for formulation

    • mix with cheap pancake syrup in ratio of 2.5 mL-1 teaspoon to 2 mL of budesonide

    • apple sauces does not stick to esophagus well

    • honey is too viscous and difficult to mix

  • treat esophageal candidiasis with topical antifungals

    • for recurrent esophageal candidiasis may need to discontinue swallowed steroid therapy

      • assess for underlying immunodeficiency

      • ask patient to take about 30 min before bed before brushing teeth and have a drink of water before bed (rather than take right before bed and allow steroids to sit overnight)

• Elimination Diet in EoE

  • adherence with all diets wane with time

  • elemental diet

    • 94% achieving histologic response

    • most effective but many challenges to tolerance/adherence 

  • six food elimination

    • 68% achieving histologic response

    • cow's milk

    • wheat

    • egg

    • soy

    • peanut/tree nut

    • fish/seafood

  • four food elimination

    • cow's milk, wheat, egg, soy

  • two food elimination

    • cow's milk, wheat

  • one food elimination

    • cow's milk

  • allergy-testing directed diet

    • limited clinical utility and is no longer commonly used

    • EoE is not an IgE based disease while allergy is IgE based testing 

• Dupilumab

  • anti-IL4R

  • FDA approved 5/2022

• Empiric esophageal dilation

  • no mortality associated with this

  • chest pain after dilation is common

  • does not address underlying disease process

• EoE with a fixed ring/stricture causing recurrent food impactions

  • biopsy to assess for active inflammation or eosinophilic infiltration that may indicate need to alter medication regimen

  • dilation therapy if inflammation is well controlled

  • if avoiding excessive dilation in any one treatment session, risk of perforation is <1%

  • passage of single large caliber (54-60 Fr) bougies (ie. Savary dilator) to treat a seemingly isolated distal ring in EoE can be hazardous due to unrecognized stenoses proximally

  • dilation with graduated balloon catheters is safer

    • 9 mm is the approximate outer diameter of a standard upper endoscope

    • may begin with an 8-10 mm balloon

    • given the fibrotic nature of esophageal mucosa in EoE, excessive balloon dilation (18-20 mm) is not recommended given risk of perforation

Definition

• patients with scleroderma are likely to have major motility disorders of the esophagus

Imaging Findings

• patulous lower esophageal sphincter and dilated, fluid-filled esophagus in later stages

Endoscopic Findings

• High resolution manometry

  • absent contractility

  • low IRP sphincter

Signs and Symptoms

• dysphagia

Endoscopic Findings

• esophageal rings

• narrow caliber throughout esophagus

Pathology Findings

• no or few eosinophils

• intraepithelial peripapillary lymphocytes with spongiosis

• exact definition has not be standardized in therms of number of lymphocytes per hpf

Treatment

• PPI

• topical steroids

• serial dilation when there are structural lesions

Signs and Symptoms

• dysphagia

Endoscopy Findings

• white plaques

• edema

• narrowed esophagus

• stricture

• does NOT have concentric rings

Pathology Findings

• Civette bodies

  • results from autoimmune T cell activity leading to degenerating basal cells

Definition

• heterotopic gastric mucosa seen in the proximal esophagus

• can be a risk factor for Barrett's esophagus and H pylori infection

• NOT associated with esophageal candidiasis

Definition

• Button and disc batteries

  • one of the most toxic ingestions possible

  • leaked contents can begin to damage mucosa in a hollow viscus within 15 min

  • damage can extend to the muscular layers within 30 min

  • must be retrieved emergently

Imaging Findings

• CXR

• Gastrografin swallow is not indicated for foreign body ingestion unless a leak or perforation is suspected

  • CT with oral contrast may provide more detail in this case

Treatment

• Button and disc batteries - emergent EGD

• Objects that are not sharp with no evidence of complete obstruction - non-emergent EGD

Signs and Symptoms

• repeated episodes of emesis followed by coffee ground emesis  

Treatment

• short course of PPI

• unlike for erosive esophagitis or gastric ulcer, repeat EGD to assess healing is NOT indicated

Definition

• also known as black esophagus

• occurs with reflux, compromised mucosa, and ischemia likely due to low flow states