Esophagus / Pharynx

     

    Introduction

    • Think of it as a muscular tube stretching from the upper esophageal sphincter to lower esophageal sphincter.
    • Innervation is different:
      • Upper Esophagus = Striated muscle
      • Lower Esophagus = Smooth muscle

    Dysphagia

    • Difficulty swallowing.
    • Symptoms:
      • Sensation of obstruction or difficulty passing food and/or liquids.
      • Can sense obstruction at the mouth, pharynx, or chest (esophagus).
    • Types:

    DysphagiaApproach2.png

     

    Oropharyngeal

    • Difficulty passing bolus from mouth to esophagus.
    • Can be: neuromuscular or anatomic.
    • Symptoms: (Clues to diagnosis)
      • Coughing (Aspiration, recurrent pneumonia)
      • Nasal Regurgitation (soft palate dysfunction)
      • Dysphonia, diplopia, musclar weakness  (neurologic)
    • Diagnosis:
      • Videofluoroscopy ("aka modified barium swallow").
      • (Foods of various consistencies are assessed)
    • Management:
      • Treat cause
      • Dietary (consistency etc..) and postural changes to improve swallowing
      • Consider consulting speech pathology

     

    Esophageal

    • Sense of food sticking in esophagus, which typically occurs right after swallowing.
      • (Where the patient is pointing is not very accurate)
    • Must distinguish solid from liquid:
      • Solid dysphagia (liquid OK) ==> Structural cause (intraluminal obstruction: Schatzki's ring, cancer, stricture)
      • Solid + Liquid Dysphagia ==> Underlying motility disorder (achalasia or scleroderma)
    • History
      • Heartburn/reflux (peptic stricture?)
      • Recurrent chest pain (negative cardiac workup): Diffuse esophageal spasm, achalasia
      • Weight loss: malignancy.
    • Diagnosis:
      • Must rule out a structure abnormality (esp if solid food)
      • Endoscopy is the best test for esophageal dysphagia

     

    Odynophagia

    • Painful swallowing
    • Sign of esophageal ulceration
    • Causes:
      • Infections: (Candida, Herpes, CMV)
      • Pill-Induced Esophagitis
      • etc..

    GERD

    • Reflux of stomach contents causing symptoms/comlications --> heartburn, regurgitation.
      • Retrosternal chest discomfort that improves with therapy. (20% have this daily).
      • Symptom severity DOES NOT correlate with degree 
    • Rule out cardiac cause!!!
    • Complications:
      • Esophagitis
      • Bleeding (ulcerative complications)
      • Lower Esophageal Strictures
      • Barrett's Esophagus + Adenocarcinoma
      • Strictures
    • Causes:
      • Mild GERD: Transient lower esophageal sphincter (LES) relaxations.
      • Severe GERD: Disruption of normal GE junction anatomy.
      • Conditions:
        • Xerostomia (less saliva)
        • Gastroparesis
      • Drugs: (reduce LES tone)
        • Nicotine
        • Obesity
    • Diagnosis:
      • Diagnosis of GERD

        (ONE of:)

             1.  Clinical: based on symptoms (and no ALARM sx) -->  often trial of therapy.

        • Heartburn,  regurgitation or both (+/- response to PPI) ==> enough to diagnose GERD.

             2.  Endoscopy  

                   (ONLY if empiric therapy fails, or ALARM symptoms)

             3.  Ambulatory pH monitoring
                   (ONLY if no responce to therapy, negative endoscopy, considering fundoplication)

        • Traditional: tube catheter through nose into distal esophagus (24 hrs)
        • New technology "tubeless": sensor in lower esophagus, wirelessly transmits pH (48hrs)

         
      • ALARM symptoms of mucosal injury (indications for scope)
        • Dysphagia
        • Anemia
        • Vomiting
        • Weight loss
    • Treatment:
      • Conservative:
        • Stop smoking!
        • Weight loss.
        • Avoid eating large meals (esp late at night) before lying down.
          • Small meals, wait 3-4 hrs before lying in bed.
        • Elevate head of the bed (put phone books under head of bed)
        • Avoid foods:
          • onions, peppermints, spicy foods, carbonated beverages.
        • Avoid medications that worsen it.
      • Medical:
        • 1st Line: Proton Pump Inhibitors (PPI)
          • Achieve LOWEST dose of PPI to achieve resolution of symptoms. (r/a dose in 8-12w)
          • Start once daily, can titrate to BID if needed, take 30-45m before meal.
          • If symptoms not controlled or endoscopy --> endoscopy.
          • ALL PPI's have the same efficacy (use cost, coverage)
            • If on clopidogrel, non-omeprazole recommended. (pantoprazole)
              • Interferes less with enzyme to activate clopidogrel.
        • 2nd Line H2 Blockers (i.e. ranitidine) - only if intolerant to PPI.
          • Not as effective as PPI
          • Tachyphylaxis develops (lose efficacy)
        • No Role: metoclopramide.

     

    Side Effects of PPI

    • Headache, Diarrhea, Abdo pain, constipation.
      • Reduce dose or switch to different one.
    • Long-Term use:
      • C. difficile
      • Pneumonia
      • Hip Fractures (conflicting data - acid inhibition decr. Ca++ reabsorption)
        • Still recommend Ca++ and VitD for all patients on PPI
      • Possible interaction with clopidogrel (increased cardiac events reported), use non-omeprazole.

     

    Surgery

    • Saved for refractory reflux (confirmed dx of esophagitis or pH)
      • Also intolerance to PPI therapy or don't want long-term meds.
    • Efficacy rates are similar to chronic PPI therapy.
    • Gastric fundus wraps around sub-diaphragmatic esophagus forming a collar (makes "speed bump"  - slows it down).
      • Gives functional closure of the sphincter, and still relaxes appropriately.
      • May have dysphagia, bloating (cannot belch), diarrhea. 
    • Down sides:
      • Up to 1/3 may need acid suppression after sugery.
      • Can re-open, and need re-operation (lower success rates).
      • Surgery does NOT reduce the risk of endocarcinoma of esophagus.

     

    Endoscopic

    • Done much less nowadays (2014), examples:
      • Radiofrequency ablation of sphincter (more stiff)
      • Endoscopic sewing
      • Injection of polymers into lower esophageal sphincter. (collagen)
    • Work for 6-12mo, but lose effectiveness.
    • AGA guidelines: No indication for endoscopic therapy for GERD

     

    Extraesophageal Symptoms

    • Chest pain, esophagitis, strictures, cancer.
    • Laryngitis, cough, asthma.
    • Recurrent otitis media, pulmonary fibrosis, sinusitis.

    Hypertonic Motility Disorders

    Achalasia / Pseudoachalasia

    • Loss of esophageal parastalsis
    • Failure of LES to relax appropriately when patient's swallow.
    • Thought to be caused by degeneration of myenteric plexus.  Loss of inhibitor neurons in lower esophageal sphincter (tonically contracted).
    • Symptoms:
      • Chest pains (rarely)
      • Dysphagia
      • Regurgitating fermtented food
    • Diagnostic Tests:
      • Chest XRay: Dilated esophagus with air-fluid level.
      • Barium Swallow: (First Line!  [screening test])
        • Dilated, smoothly tapering esophagus ("Bird beek" of LES).
      • Manometry (Confirm Diagnosis):
        • Aparistalsis, incomplete or absent relaxation of LES when swallows.
      • Upper Endoscopy (MUST DO, even if classic manometric findings)
        • Must rule out pseudoachalasia (tumor at the lower esophagus) causing achalasia-type dx.
        • Consider in older individual, profound weight loss, short duration of sx.
    • Treatment Options:
      • 1st line:
        • Laparoscopic myotomy. (shorter hospital stay, faster recovery, similar results to open procedures).
      • Nissen fundoplication:
        • performed post-OR to decrease post-op reflux.
      • Endoscopic Dilatation (IF not surgical candidate):
        • 5% risk of perforation, shorter duration of response.
        • Less successful in younger patients.
      • Endoscopic injection of Botulinum toxin into the LES: symptom relief, temporary 6-9mo.
        • Only for patients who cannot undergo surgery, and cannot tolerate endoscopic dilation (due to risk of perforation).
      • Medications:
        • Nitrates, CCB, inconsistent benefit (can provide some relief).

     

    Diffuse Esophageal Spasm

    • More commonly seen in older patients, intermittent high-amplitude contractions, no peristalsis.
    • Exclude cardiac disease
    • Diagnosis:
      • Clinical Presentation (Dysphagia or chest pain)
      • Barium Swallow (see DES)
    • "Corkscrew esophagus"
      • On esophogram: multiple simultaneous contractions (looks like corkscrew) - diffuse spasm.
    • "Nutcracker esophagus"
      • Extreme spasm
    • Mannometric finding: average distal esophagus pressure >220mmHg (during peristalsis)
    • Treatment:
      • Symptomatic
      • 1st Line:  Calcium Channel blockers
      • Others:
        • Trazodone
        • Imipramine (modulate visceral sensory)
        • Botox

    Hypomotility Disorders

    Scleroderma

    • Dysphagia, slower transit times.
    • Mannometry:
      • Lower amplitude contractions, often non-peristalsis.
    • Causes:
      • Opioid drug users!
      • Scleroderma (aparistalsis on mannometry, decreased pressure in LES on mannomery)
        • vs. Achalasia (Increased pressure of LES)
    • Symptoms:
      • Often get severe esophagitis --> can cause strictures
    • Treatment:
      • High-dose PPI
      • (Cannot do fundoplication b/c poor peristalsis, would cause severe dysphagia)

     

    Esophagitis

    • Ddx:
      • Infectious, pill induced, eosinophilic

    Infectious

    • Organisms:
      • Candidate albicans (most common, sometimes even in immunocompetent)
        • Dysphagia with curdy white plaques on endoscopy
      • HSV - Large superficial ulcerations, biopsy edge of ulcer.
      • CMV - Causes large isolated ulcerations, biopsy base of ulcer.
      • Others (HPV, trypanosoma cruzi, TB, treponema pallidum, etc.. )
    • Almost always in immunocompromized (steroids, azathioprine, TNF-a inhibitors, congenital/acquired, inhaled corticosteroids)
    • Symptoms:
      • Often see candida (oral thrush) - if have dysphagia, treat as esophagitis.
      • Treat cause:
        • Candida (antifungal)
        • HSV (acyclovir)
        • CMV (gancyclovir

    Pill Induced

    • Often pain with swallowing, chest pain.
    • Caused by pills stucking areas of the esophagus, producing inflammation.
    • Symptoms:
      • Odynophagia
      • Dysphagia
      • Retrosternal chest pain
    • Several places can happen:
      • Aortic arch (indentation in esophagus)
      • GE junction
      • Enlarged L atrium
    • Most common pills:
      • Tetracycline (+ derivatives)
      • Iron tablets 
      • Bisphosphonates
      • Potassium 
      • NSAIDs
      • Quinidine
    • Management:
      • HOLD drug
      • If need to take:
        • Take with large glass of water to ensure it washes out.
        • Avoid laying recombant for 30-60min after.

    Eosinophilic Esophagitis

    • Somewhat like an "allergic reaction of esophagus.
    • Eosiophilic infiltration into esophageal mucosa.
    • Solid food dysphagia --> solid foods
    • Symptoms:
      • Recurrent food impactions, or hx of solid food dysphagia.
      • May have non-specific sx: vomiting, abdo pain, failure to thrive (cannot swallow).
    • Typical presentation:
      • Often pt with esophagitis --> put on PPI x6w --> does not get better --> endoscopy biopsy --> eosinophilic.
    • Associated with asthma and allergies.
    • COMMON, male predominance, 54 in 100,000 in US.
    • Diagnosis:
      • >15 eosinophils / HPF on esophageal endoscopic biopsy (taken MID esophagus).
        • Exclude GERD (causes eosinophilia in DISTAL esophagus).
      • Endoscopy: "corrigated" esophagus, strictures, "cracks", but can be normal.
    • Management
      • 1st LINE:
        • INTENTIONALLY swallowing aerosolized corticosteroids (asthma inhaler).
        • RINSE mouth afterwards (avoid thrush)
          • Only given after given PPI trial to ensure ruled out reflux disease.
      • Children: Food elimination diets (not as effective in adults)
      • Adults: May need to dilate
      • Last Line:
        • Systemic steroids rarely needed.

     

    Esophageal Malignancy

    • Upper Esophagus --> Squamous Cell Carcinoma
    • Lower Esophagus --> Adenocarcinoma
    • Upper Endoscopy is the gold standard test for solid food dysphagia concerning for malignancy (Not CT)

     

     

    Barrett's Esophagus

    • Pre-malignant complication of GERD
    • Normal squamous epithelium of distal esophagus is replaced by specialized columnar epithelium.
    • More common in white pts with longstanding GERD.
    • Risk of esphageal carcinoma of 0.5% (30-50 fold increase risk of CA)
    • Screening/Surveillance Guidelines:
      • Do not screen for Barrett's esophagus in patients with GERD
      • Screening for BE can be considered in MEN:
        • MEN with chronic chronic (>5 years) and/or frequent (weekly or more) sx GERD
          AND
        • ≥2 RFs for BE or EAC:
          • Age >50 years
          • Caucasian
          • Central obesity (waist circumference >102 cm or waist–hip ratio (WHR) >0.9)
          • Smoking (current or past)
          • Family history of BE or EAC (1st degree relative)
      • Women do not get Berretts (very rare), only screen if many RFs
    • Diagnosis:
      • Diagnosis of Barrett's Esophagus:

          1.  Endoscopic Findings (replacement of normal mucosal surface with dark pink columnar epithelium)

             AND

          2.  Histopathology (intestinal metaplasia with acid-mucin containing goblet cells)

    • Classification:
      • Long-Segment:  > 3cm of metaplasia above the EG Junction
      • Short-Segment: < 3cm of metaplasia
    • Management:
      • Screening for Cancer:  (Based on Pathology)
      • (Screen for invasive adenocarcinoma)
      • Pathology Grade Recommended Screening
        No Dysplasia

        Repeat endoscopy 1yr post-diagnosis (to ensure not missed dysplasia),

        if second endoscopy is negative, then endoscopy q3yrs.

        Low Grade Dysplasia

        Consider Endoscopic Ablation  

        (Can also do surveillance q1yr)

        High Grade Dysplasia

        Endoscopic Ablation

      • PPI's may prevent progression (only retrospective cohort studies, no RCTs).
      • Fundoplication does not reduce risk of progression
      • If high grade dysplasia:
        • Photodynamic,  RF ablation, and Cryotherapy effective in treating high grade.
          • (risk of strictures)
        • Ablation allows Barrett's Epithelium replacement with normal

    Esophageal Carcinoma

    • Adenocarcinoma or Squamous Cell
    • last 3 decades --> 300% increase in adenocarcinoma (and less squamous cell).
      • Adenocarcinoma correlates more with GERD (dietary, size, etc..)
    • Dyspagia, weight loss = poor prognosis 
    • 5 year survival <20%, (often diagnosed in advanced stage)
    • Risk Factors:
      • Squamous Cell
        • Alcohol, Tobacco, Nitrosamine (foods), corrosive injury, Zinc/Selenium deficiency, 
        • Achalasia, HPV
      • Adenocarcinoma (more common)
        • Tobacco use, Obesity (central obesity), symptomatic GERD, Barrett's esophagus
    • Symptoms:
      • Progressive dysphagia of solid foods
      • Weight loss (less food)
      • Anemia (loss in esophagus)
      • Reflux symptoms can decrease! (tumor now constricts)
    • Diagnosis:
      • Upper Endoscopy + biopsy
    • Staging
      • CT scan (distant metastasis)
      • Local/regional staging
      • If indeterminate lesions --> PET
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