Esophageal Symptoms and Diagnostic Workup

Section 1.1: Algorithm for dysphagia

Dysphagia sorts cleanly along two axes, and the first task at the bedside is to fix the vignette to a quadrant of the grid before ordering tests. The first axis is anatomic location: oropharyngeal dysphagia is a problem of bolus initiation and transfer from mouth to upper esophagus, while esophageal dysphagia is a problem of bolus transit through the tubular esophagus to the stomach. The second axis is mechanism: mechanical narrowing produces a progressive solids-then-liquids pattern, while motility disorders affect solids and liquids equally from the start. Once the vignette is sorted by anatomy and pattern, test selection follows directly. Oropharyngeal dysphagia goes to videofluoroscopy with a speech therapist before EGD because EGD does not visualize the swallowing maneuver and a Zenker pouch can be perforated by blind intubation. Esophageal dysphagia goes to EGD with biopsies first, because EGD addresses both the structural differential and EoE simultaneously. Alarm features short-circuit the algorithm and mandate prompt EGD regardless of pattern.

Oropharyngeal dysphagia is a failure of the initiation phase of swallowing, in which the tongue, soft palate, and pharyngeal constrictors must coordinate with relaxation of the upper esophageal sphincter to drive a bolus across the cricopharyngeus. Most cases are neuromuscular: stroke, Parkinson disease, ALS, myasthenia gravis, and head and neck radiation each disrupt one or more elements of that coordinated sequence. Zenker diverticulum is the structural exception in this category because it forms at the same anatomic transition. Patients describe coughing on swallow, nasal regurgitation when palatal closure fails, drooling when oral containment is impaired, and frank aspiration when the bolus reaches an unprotected airway. The diagnostic test of choice is the modified barium swallow, also called videofluoroscopic swallow study, performed with a speech-language pathologist who watches the bolus in real time across multiple consistencies and head positions. EGD has no role in primary oropharyngeal dysphagia evaluation because it does not visualize the swallowing event and provides no information about aspiration risk or compensatory maneuvers. A cricopharyngeal bar on barium reflects failure of the upper esophageal sphincter to relax and is treated with cricopharyngeal myotomy or, in selected patients, botulinum toxin to the cricopharyngeus.

Esophageal dysphagia begins with the symptom history, because the pattern almost always tells the candidate whether to expect a structural lesion or a motility disorder before any test is ordered. Mechanical narrowing produces dysphagia first to solids and only later to liquids as the lumen narrows further; the differential includes Schatzki ring, esophageal web, peptic stricture, eosinophilic esophagitis, and esophageal malignancy. Motility disorders produce dysphagia to solids and liquids together from the outset, because the propagated wave fails on every bolus regardless of consistency; the differential here is achalasia, distal esophageal spasm, jackhammer esophagus, and scleroderma esophagus. EGD with esophageal biopsies is the first test in nearly every esophageal dysphagia vignette, because it covers the structural differential, identifies subtle eosinophilic esophagitis in mucosa that can look entirely normal at endoscopy, and rules out malignancy. Esophageal biopsies for EoE are obtained at two levels (proximal and distal, two to four samples each) even when the mucosa appears unremarkable, because EoE is a histologic diagnosis with normal endoscopic appearance in 5 to 10 percent of cases (see Ch 5 for the EoE deep dive). Barium swallow is added when EGD is non-diagnostic or when motility is suspected, because the timed barium esophagram visualizes a tertiary contraction pattern, a bird-beak narrowing, or a corkscrew morphology that points to the manometric study that follows.

New-onset dysphagia is itself an alarm symptom. Every dysphagia vignette goes to prompt workup regardless of age: EGD with biopsies for esophageal dysphagia, modified barium swallow with speech-language pathology for oropharyngeal dysphagia, and barium-first for suspected Zenker. The age-plus-alarm-features rule that decides EGD versus empiric therapy belongs to dyspepsia, not to dysphagia. What the malignancy-suspicion features do for dysphagia is shift the differential and the urgency: unintentional weight loss, iron deficiency anemia, overt or occult GI bleeding, rapid progression, and persistent vomiting raise the pretest probability that the underlying lesion is cancer rather than a benign stricture or ring. Older age and smoking or alcohol exposure are risk multipliers in that calculation. The principle is mechanism-based: progressive solid food dysphagia in a 65-year-old smoker with weight loss is esophageal cancer until proven otherwise, and the workup escalates from index EGD to immediate staging the moment biopsies confirm.

Zenker diverticulum is the canonical reason barium swallow precedes EGD. It is a false diverticulum (mucosa and submucosa only, no muscular layer) that herniates posteriorly through Killian triangle, the area of relative weakness between the oblique fibers of the inferior pharyngeal constrictor above and the transverse fibers of the cricopharyngeus below. The mechanism is functional outflow obstruction at the upper esophageal sphincter (incomplete cricopharyngeal relaxation, often called cricopharyngeal dysfunction) that drives intraluminal pressure into the wall during repeated swallowing over years. Most patients are in the seventh or eighth decade and roughly 90 percent present with dysphagia. The presentation that gives the diagnosis away is regurgitation of undigested food eaten hours or days earlier, halitosis from the trapped material, gurgling sounds during or after swallowing, chronic cough, and aspiration pneumonia from spillage of pouch contents into the airway. Diagnosis is made by barium swallow, which fills the pouch and shows a posterior outpouching at the cervical esophagus. EGD is avoided as the initial test because the scope tip can enter the pouch rather than the true lumen and perforate the diverticulum. Treatment is cricopharyngeal myotomy combined with diverticulotomy, performed open, by transoral stapled approach (which divides the wall between pouch and esophagus in a single firing), or by flexible endoscopic Z-POEM where the cricopharyngeus is divided through a submucosal tunnel.

What the boards test

The boards reward candidates who can sort a dysphagia vignette by anatomy and pattern within the first two sentences and let the test selection fall out of that sort. Oropharyngeal dysphagia is the patient who coughs at the start of the swallow, regurgitates through the nose, drools, or aspirates, and the workup is modified barium swallow with a speech therapist rather than EGD because EGD does not visualize swallowing and a Zenker pouch can be perforated. Esophageal dysphagia is the patient with food sticking after the swallow, and the workup begins with EGD plus biopsies because EGD covers structure, ulceration, malignancy, and EoE in one trip; biopsies are obtained at two levels even when the mucosa looks normal because EoE can be histologic with a normal endoscopic appearance (see Ch 5 for the EoE deep dive). Within esophageal dysphagia the pattern separates the differentials cleanly: progressive solids only points to a narrowing process (ring, web, peptic stricture, EoE, malignancy), while solids and liquids together from the start points to motility (achalasia, spasm, scleroderma). Barium swallow precedes or follows EGD when motility is suspected or when EGD is non-diagnostic. Dysphagia is itself an alarm symptom and there is no age threshold for the EGD; the cancer-suspicion features (weight loss, iron deficiency anemia, GI bleeding, rapid progression, persistent vomiting, with older age and smoking or alcohol as risk multipliers) shift the differential toward malignancy and accelerate from index EGD to staging, but the EGD was happening regardless. Zenker is the exception that proves the rule: the older patient with halitosis, regurgitation of undigested food, and gurgling gets a barium swallow first because blind EGD risks perforation of the false diverticulum at Killian triangle, and the answer in management is cricopharyngeal myotomy plus diverticulotomy by open, transoral stapled, or flexible endoscopic Z-POEM approach.

Section 1.2: Globus, rumination, and functional esophageal pain

The disorders in this section are the diagnoses that arrive in clinic after the structural and reflux workups have already returned clean. They share a brain-gut axis mechanism (visceral hypersensitivity, learned somatic responses, or a behavioral re-swallow pattern), and they share the trap that they are routinely misdiagnosed and misdosed as refractory GERD. The unifying clinical task is to recognize that PPI failure in a patient who looks like GERD on the surface is the giveaway that one of these functional or behavioral diagnoses is the real problem, and that treatment is not more acid suppression but a neuromodulator, a behavioral intervention, or in the case of rumination, diaphragmatic breathing.

Globus pharyngeus is the persistent or intermittent sensation of a lump, fullness, or tightness in the throat between meals, classically improved or unchanged by swallowing rather than worsened. The mechanism is multifactorial and includes hypertonic upper esophageal sphincter behavior, post-nasal drip, and reflux-related laryngeal sensitization in a subset, but in most patients no single physiologic abnormality is identified and the dominant driver is a heightened perception of normal pharyngeal sensation. The workup is targeted: rule out reflux and post-nasal drip with history and a focused exam, and look for a structural lesion only when alarm features (true dysphagia, weight loss, hoarseness, neck mass, smoking history) are present. Reassurance is the dominant management once the alarm screen is negative, because the natural history is benign and intervention beyond reassurance rarely improves outcome. PPI is not a default answer for globus.

Rumination syndrome is the effortless regurgitation of recently swallowed food into the mouth followed by re-swallowing or spitting, occurring without preceding nausea or retching, typically within ten to fifteen minutes of meal initiation. The mechanism is a learned, unintentional contraction of the abdominal wall and diaphragm that raises intra-abdominal pressure against a relaxed lower esophageal sphincter, producing retrograde flow of undigested gastric contents into the esophagus and pharynx. The Rome IV criteria require effortless regurgitation of recently swallowed food with re-swallowing or spitting, not preceded by retching, occurring on most days for at least three months with onset at least six months earlier. The diagnostic fingerprint on high-resolution manometry with concurrent impedance is the abdominal-strain "R-wave" pressurization: a simultaneous rise in intra-gastric pressure and intra-esophageal pressure during the postprandial period without evidence of antecedent retching, often with the lower esophageal sphincter pressure dropping at the same instant. The clinical giveaway in vignettes is failure of high-dose twice-daily PPI in a young patient whose "regurgitation" occurs immediately after every meal, whose material is recognizable as the food just eaten, and who often gains rather than loses weight. Treatment is diaphragmatic breathing taught by behavioral therapy (slow deep abdominal inspiration during the postprandial window, which is incompatible with the abdominal wall contraction that drives the rumination event) with cognitive behavioral therapy for refractory cases. Acid suppression has no role.

Functional chest pain is noncardiac chest pain after cardiac, GERD, EoE, and primary motility disorders have been excluded by appropriate testing. The mechanism is visceral hypersensitivity at the level of the esophageal afferent neurons and central pain processing pathways, in which non-painful esophageal events (low-volume reflux, normal contractions, mild distension) are perceived as pain because the gain on the pathway is set too high. Treatment is the neuromodulator: a low-dose tricyclic such as imipramine 25 to 50 mg or amitriptyline 25 to 50 mg at bedtime, an SSRI when TCAs are not tolerated, hypnotherapy, or cognitive behavioral therapy. The neuromodulator is the first-line drug class, not a higher PPI dose, because the lesion is in the pain processing pathway rather than the acid burden.

Functional heartburn and reflux hypersensitivity are the analogous brain-gut axis diagnoses on the heartburn side and are detailed in the GERD chapter. Functional heartburn is heartburn with normal acid exposure time and negative symptom-reflux association, treated with a neuromodulator. Reflux hypersensitivity is heartburn with normal acid exposure time but positive symptom-reflux association, treated with combined neuromodulator and acid suppression because the patient genuinely associates symptoms with physiologic reflux events but the volume of acid exposure is below the threshold that defines GERD (see Ch 3 for the Lyon Consensus and pH-impedance interpretation framework).

What the boards test

The boards test these disorders as the diagnoses that explain PPI failure in patients who look like GERD on the surface, and the favored vignette is the young patient on twice-daily PPI without improvement whose history actually fits one of these alternatives. Globus is the lump-in-throat sensation between meals that is unchanged or improved by swallowing rather than worsened, and the answer is reassurance after a focused screen for reflux, post-nasal drip, and alarm features rather than escalation of acid suppression. Rumination is recognized by the immediate postprandial timing within ten to fifteen minutes, the effortless and non-retching regurgitation of recently swallowed and recognizable food, the failure of PPI, and on high-resolution manometry by the abdominal-strain R-wave with simultaneous gastric and esophageal pressurization and lower esophageal sphincter relaxation; treatment is diaphragmatic breathing taught by behavioral therapy, not acid suppression and not fundoplication. Functional chest pain is noncardiac chest pain after cardiac, GERD, EoE, and motility have been excluded, and the first-line therapy is a low-dose tricyclic such as imipramine 25 to 50 mg or amitriptyline 25 to 50 mg, an SSRI as a tolerability alternative, or hypnotherapy and cognitive behavioral therapy, because the mechanism is visceral hypersensitivity rather than acid exposure. The repeating board pattern is that PPI failure in a patient who looks like GERD should prompt the candidate to reconsider whether the diagnosis is GERD at all, and the neuromodulator or the behavioral intervention is the answer rather than a higher PPI dose.

Section 1.3: Odynophagia workup

Odynophagia is mucosal injury until proven otherwise, and the differential is sorted by exposure history before a single test is ordered. The three exposure axes are pill exposure (was a tablet swallowed with too little water or in a recumbent position), immune status (HIV CD4 count, transplant status, chemotherapy, chronic inhaled or systemic steroids), and prior reflux or caustic injury (deep dive in the EoE and GI emergencies chapters, see Ch 5 for EoE and Ch 31 for caustic ingestion). Pinning the exposure narrows the differential to one or two diagnoses before EGD, and the EGD is then directed toward biopsy of the appropriate site. Caustic injury is mentioned here only by name; the staging system, the airway management, and the long-term stricture surveillance are covered in the GI emergencies chapter.

Pill esophagitis is a direct mucosal injury at the point of pill contact, most commonly at the level of the aortic arch where the left mainstem bronchus indents the esophagus and a tablet can lodge against the wall, or at the lower esophageal sphincter where a slow propagated wave can leave a pill in contact with the mucosa for minutes. The mechanism is local toxicity from the dissolved tablet contents, with the pH of the dissolved drug, its osmolality, and its tissue contact time each contributing. The classic offenders are doxycycline and tetracycline, potassium chloride (especially extended-release formulations), bisphosphonates (alendronate, risedronate), NSAIDs and aspirin, iron, clindamycin, ascorbic acid, quinine and quinidine, and dabigatran. The risk factors are taking the pill with little or no water, recumbent dosing or lying down immediately after, and an underlying motility disorder or stricture that prolongs contact time. The classic presentation is sudden retrosternal odynophagia hours to days after a specific pill, often in a patient who took the medication at bedtime with a small sip of water. EGD typically shows discrete, often shallow ulcers at the aortic arch level with normal intervening mucosa and a kissing pattern (paired ulcers on opposing walls where the tablet sat between them). Histology shows ulceration with neutrophilic and reactive epithelial inflammation, without the eosinophilic pattern of EoE or the viral inclusions of HSV and CMV. Treatment is removal of the offending agent, sucralfate slurry to coat the injured mucosa, swallowing technique counseling (take pills upright with at least 8 ounces of water and remain upright for 30 minutes), and a brief PPI course in the patient with concurrent reflux. Symptoms typically resolve within one to two weeks.

Infectious esophagitis is sorted by host immune status, which narrows the differential before EGD. Candida esophagitis is the most common in PPI users with concurrent inhaled steroids, in HIV patients with CD4 counts under 200, in solid organ and stem cell transplant recipients, in patients on long-term broad-spectrum antibiotics, and in patients with esophageal stasis from achalasia or strictures. The mechanism is overgrowth of normal oral and esophageal Candida flora when local mucosal defense (acid, motility, cell-mediated immunity) fails. Endoscopy shows white or yellow-white plaques adherent to the mucosa, typically diffuse, that do not wash off with irrigation. Brushings or biopsies show pseudohyphae and yeast forms on Gram, periodic acid-Schiff, or Grocott methenamine silver stain. Treatment is fluconazole 200 to 400 mg daily for 14 to 21 days; an echinocandin (caspofungin, micafungin, anidulafungin) is used when fluconazole resistance is documented or strongly suspected, particularly in transplant recipients with prior azole exposure.

HSV esophagitis is recognized by shallow ulcers with raised, well-demarcated edges, often described as volcano-edge ulcers, distributed in the mid to distal esophagus. The histologic fingerprint is multinucleated giant cells with intranuclear Cowdry type A inclusions in the squamous epithelial cells at the edge of the ulcer, which is why biopsies should target the ulcer edge rather than the base. Treatment is acyclovir 400 mg orally five times daily for 14 to 21 days in the immunocompromised host or 200 mg five times daily for 7 to 10 days in the rare immunocompetent case; intravenous acyclovir is used when oral intake is impossible. Foscarnet covers acyclovir-resistant HSV.

CMV esophagitis is recognized by one or more large, deep, often serpiginous ulcers (sometimes called punched-out or geographic ulcers) that can coalesce into a single broad lesion, typically in the distal esophagus. The histologic fingerprint is owl-eye intranuclear inclusions and basophilic cytoplasmic inclusions in endothelial cells and stromal fibroblasts in the submucosal tissue, which is why CMV biopsies must be taken from the ulcer base rather than the edge. The exposure pattern that points toward CMV is HIV with CD4 under 100, solid organ transplantation, and chemotherapy-induced neutropenia. Treatment is intravenous ganciclovir 5 mg/kg twice daily induction with transition to oral valganciclovir for completion; foscarnet is the alternative for ganciclovir-resistant disease.

Host stratification is the recurring board pattern that ties the infectious differential together. A PPI user with concurrent inhaled corticosteroids and a fluconazole-responsive course points to Candida. A transplant recipient or a patient with HIV CD4 under 100 with deep solitary ulcers in the distal esophagus points to CMV. A volcano-edge shallow ulcer with multinucleated giant cells in epithelial cells at the edge points to HSV. The decision to skip empiric fluconazole and proceed directly to EGD is driven by severity of odynophagia, immunocompromised status, no response to a fluconazole trial, or clinical features suggesting a viral or pill etiology rather than Candida.

What the boards test

Odynophagia tests on three axes that the candidate should run before any test is ordered: the pill axis, the immune-status axis, and the prior-injury axis (deep reflux or caustic, with caustic detailed in Ch 31). Pill esophagitis is recognized by sudden retrosternal odynophagia hours to days after a specific pill (doxycycline, tetracycline, potassium chloride, bisphosphonates, NSAIDs, iron, clindamycin, ascorbic acid, quinine and quinidine, dabigatran), classically in a patient who took the pill with little water or while recumbent; EGD shows kissing ulcers at the aortic arch level with normal intervening mucosa, and treatment is removal of the agent, sucralfate, swallowing technique counseling (upright, 8 ounces of water, remain upright 30 minutes), and a short PPI course when reflux is concurrent. Infectious esophagitis sorts by host: PPI plus inhaled steroid favors Candida (white plaques, pseudohyphae, fluconazole 200 to 400 mg daily for 14 to 21 days, echinocandin if azole-resistant); HIV CD4 under 100 or transplant favors CMV (one or more deep serpiginous ulcers, owl-eye intranuclear inclusions in endothelial cells and stromal fibroblasts, biopsy the ulcer base, IV ganciclovir then oral valganciclovir, foscarnet for resistant); and immunocompromise generally also favors HSV (shallow volcano-edge ulcers, multinucleated giant cells with intranuclear Cowdry inclusions in squamous epithelial cells at the ulcer edge, biopsy the edge, acyclovir 400 mg five times daily for 14 to 21 days, foscarnet for acyclovir-resistant). The biopsy site distinguishes HSV from CMV and is high-yield: HSV is the epithelial-edge diagnosis and CMV is the stromal-base diagnosis, because the inclusion bodies live in different cells. The decision to skip empiric fluconazole and proceed directly to EGD is driven by severity, immunocompromise, lack of response to fluconazole, or a presentation that fits viral or pill esophagitis rather than Candida.

Section 1.4: Post-surgical and systemic dysphagia

The dysphagias in this section are the ones the standard EGD-then-manometry workup misses unless the right history is asked. The diagnosis lives in the surgical history, the autoimmune panel, the radiation field, or the neurologic exam, not in the endoscopic photos. Post-fundoplication and post-bariatric anatomy each create predictable failure modes that map to specific procedures and to specific complications. Scleroderma esophagus, Sjogren, amyloid, and chemoradiation each compromise either peristalsis, salivary flow, muscular integrity, or mucosal architecture in ways that change how the swallow proceeds. The neuromuscular disorders (myasthenia gravis, ALS, polymyositis and dermatomyositis) attack the oropharyngeal phase and are recognized by the associated systemic findings and electromyography rather than the swallow study alone. The connecting principle is that the diagnosis often lives outside the GI tract, and the candidate's job is to ask the question that surfaces it.

Post-fundoplication complications follow predictable mechanical patterns once the antireflux barrier has been surgically constructed. The Nissen fundoplication wraps 360 degrees of gastric fundus around the distal esophagus to create an antireflux barrier; partial fundoplications (Toupet at 270 degrees posterior, Dor at 180 degrees anterior) trade some reflux control for less postoperative dysphagia and less gas-bloat. The slipped wrap occurs when the wrap migrates onto the proximal stomach rather than encircling the distal esophagus, producing the paradoxical combination of recurrent reflux (because the wrap no longer reinforces the lower esophageal sphincter) plus dysphagia (because the wrap now creates a relative obstruction at the gastric body). The tight wrap presents with early postoperative dysphagia, often immediately after surgery, because the wrap is too snug around the esophagus and the bolus cannot pass; treatment is pneumatic dilation in the early postoperative period or surgical revision if dilation fails. The gas-bloat syndrome is the inability to belch, leading to abdominal distension and sometimes bloating-related discomfort, because the wrap acts as a one-way valve that prevents transient lower esophageal sphincter relaxations from venting swallowed air. Telescoping occurs when the gastroesophageal junction slips upward through an intact wrap, distorting the anatomy in a way that reproduces both reflux and dysphagia. Approximately 10 to 30 percent of patients resume antireflux medication within 5 years of fundoplication. Workup begins with barium swallow rather than EGD because barium shows the wrap geometry and the relationship of the gastroesophageal junction to the wrap; EGD follows for direct mucosal assessment. PPI is reintroduced for slipped wraps with reflux, dilation is the answer for the tight wrap, and surgical revision is reserved for refractory cases.

Post-bariatric dysphagia divides predictably by the operation. Sleeve gastrectomy creates a tubular stomach by stapling along the greater curvature, and the anatomic vulnerability is at the incisura angularis, where a kink, twist, or stricture at the staple line produces a fixed obstruction with progressive dysphagia and food intolerance. Sleeve gastrectomy also causes or exacerbates reflux rather than relieving it, because reduced gastric volume, loss of the angle of His, disrupted sling fibers at the gastroesophageal junction, and a higher intragastric pressure environment combine to drive reflux upward; this is why sleeve gastrectomy is generally avoided in morbidly obese patients with established GERD, and Roux-en-Y gastric bypass is the antireflux bariatric operation of choice in that population. Roux-en-Y gastric bypass produces dysphagia most often through anastomotic stricture at the gastrojejunal anastomosis, presenting weeks to months after surgery with progressive solid food intolerance; through-the-scope balloon dilation is the treatment, often requiring multiple sessions. Marginal ulcer at the gastrojejunal anastomosis presents with epigastric pain, dysphagia, and GI bleeding, and the predictable risk factors are smoking (the strongest modifiable factor), NSAID use, and Helicobacter pylori infection. Treatment is high-dose PPI plus reversal of the modifiable factors (tobacco cessation, NSAID discontinuation, H. pylori eradication if positive), with surgical revision reserved for refractory or perforating ulcers.

Scleroderma esophagus is the canonical disorder of failed peristalsis with a hypotensive lower esophageal sphincter, and the dual hit is what drives the clinical phenotype. The mechanism is fibrotic replacement and dysfunction of esophageal smooth muscle, which produces absent or markedly weak contractions in the distal two thirds of the esophagus (the smooth muscle segment) while sparing the striated upper third. The same fibrotic process drops lower esophageal sphincter resting pressure, which removes the antireflux barrier just as the peristaltic pump is failing. The result is severe gastroesophageal reflux because acid pours back unimpeded and there is no peristaltic wave to clear it, and the downstream consequences are erosive esophagitis, peptic stricture, and Barrett esophagus in 5 to 35 percent of patients (esophageal adenocarcinoma is rare despite the Barrett rate). On manometry the pattern is absent contractility (DCI under 100 mmHg.cm.s on essentially all swallows) plus a hypotensive lower esophageal sphincter, distinguishing it from achalasia (where the lower esophageal sphincter fails to relax with elevated IRP) and from absent contractility with a normal lower esophageal sphincter pressure (which is not scleroderma). Diagnosis of the underlying connective tissue disease integrates the cutaneous and Raynaud findings; ANA is positive in over 90 percent and anti-centromere antibody is characteristic of the limited cutaneous (CREST) phenotype. Treatment is aggressive acid suppression with twice-daily PPI (over 90 percent symptom response), nocturnal head-of-bed elevation, dilation when peptic stricture develops, and motility-conscious counseling. Fundoplication is generally avoided because peristalsis cannot push a bolus past a wrap, and dysphagia rates after fundoplication in scleroderma are unacceptable; partial wraps in carefully selected patients are sometimes considered but are not the default.

Sjogren syndrome produces dysphagia by removing the salivary contribution to swallowing rather than by damaging the esophagus directly. The mechanism is autoimmune lymphocytic infiltration of salivary and lacrimal glands, which produces xerostomia and reduces the lubrication and bolus formation that swallowing depends on. Patients describe difficulty initiating swallow with dry foods such as bread or crackers, often improved by water sips and worse at the end of the day. Treatment is salivary stimulation with pilocarpine 5 mg four times daily or cevimeline 30 mg three times daily (muscarinic agonists that increase salivary flow), saliva substitutes, careful oral hygiene to prevent the dental decay that follows xerostomia, and humidification of dry environments. The reduced salivary clearance is also a refractory-reflux clue when Sjogren and GERD coexist, because saliva normally contributes to esophageal acid clearance.

Amyloid causes dysphagia through infiltrative deposition of insoluble fibrils in the esophageal muscularis and submucosa. The two principal forms in adults are AL (light chain) amyloid in the setting of plasma cell dyscrasia, and ATTR (transthyretin) amyloid in either the hereditary or wild-type form. The hint at the bedside is macroglossia, which suggests AL amyloid in particular and warrants serum and urine immunofixation, free light chains, and a skeletal survey or marrow biopsy in the appropriate clinical setting. Dysphagia from amyloid reflects infiltration of the muscularis with replacement of contractile elements, and the manometric pattern is variable. Treatment is directed at the underlying amyloidogenic process (chemotherapy or autologous stem cell transplant for AL amyloid; tafamidis, patisiran, or inotersen for ATTR amyloid) rather than at the esophagus itself.

Chemoradiation injury to the esophagus has two phases that the candidate must distinguish. Acute radiation mucositis develops during or shortly after thoracic or head and neck radiation and presents with severe odynophagia, dysphagia, and dehydration; supportive care, topical anesthetics, opioid analgesia for severe cases, and nutritional support are the management. Late radiation stricture develops months to years after radiation and presents with progressive solid food dysphagia from fibrotic narrowing in the radiation field. Proactive swallow therapy during and after radiation reduces the rate of stricture and improves long-term swallowing function. Endoscopic dilation, often serial, is the treatment for established stricture; complete obstruction may require combined antegrade and retrograde rendezvous techniques.

The neuromuscular dysphagias attack the oropharyngeal phase and are recognized by the associated systemic findings rather than the swallow alone. Myasthenia gravis is autoimmune blockade of the postsynaptic acetylcholine receptor at the neuromuscular junction, and the clinical signature is fatigability that worsens with repeated effort and improves with rest, often with ptosis and diplopia. Anti-acetylcholine receptor antibodies are positive in the majority of generalized cases, with anti-MuSK antibodies covering a subset of seronegative patients. Treatment is pyridostigmine for symptomatic relief (an acetylcholinesterase inhibitor that prolongs acetylcholine action at the synapse), with immunosuppression and thymectomy for selected patients. ALS produces oropharyngeal dysphagia through degeneration of bulbar motor neurons, with fasciculations, tongue atrophy, and progressive limb weakness as the supporting findings; electromyography shows the denervation pattern and confirms the diagnosis. Polymyositis and dermatomyositis are inflammatory myopathies presenting with symmetric proximal muscle weakness (difficulty rising from a chair or lifting the arms overhead), elevated creatine kinase, anti-Jo-1 antibodies in a subset, and oropharyngeal dysphagia in roughly half of patients with bulbar involvement. Dermatomyositis adds the cutaneous findings (heliotrope rash on the eyelids, Gottron papules over the knuckles, shawl sign on the upper chest and back) and carries an associated malignancy risk that warrants age-appropriate cancer screening, with gastric, lung, ovarian, breast, and lymphoma topping the list.

What the boards test

The post-surgical and systemic dysphagias test on a single principle: the diagnosis lives in the history outside the EGD report, and the candidate has to ask the right question. Post-fundoplication dysphagia presents in three patterns. The slipped wrap gives recurrent reflux plus dysphagia and is treated with PPI and consideration of revision. The tight wrap gives early postoperative dysphagia and is treated with pneumatic dilation. Gas-bloat is the inability to belch with abdominal distension and reflects the wrap acting as a one-way valve. Barium swallow is the first test in any of these, because it shows the wrap geometry that EGD cannot. Post-bariatric dysphagia divides cleanly: sleeve gastrectomy strictures at the incisura angularis and is dilated, and sleeve causes rather than relieves reflux, which is why sleeve is avoided in obese patients with established GERD and Roux-en-Y gastric bypass is the antireflux operation of choice for that population. Roux-en-Y produces gastrojejunal anastomotic stricture that is dilated, and marginal ulcer at the gastrojejunal anastomosis in the smoker on NSAIDs (with H. pylori on the differential) is treated with high-dose PPI plus reversal of the modifiable risk factors. Scleroderma esophagus is recognized on manometry by absent peristalsis (DCI under 100 mmHg.cm.s on essentially all swallows) combined with a hypotensive lower esophageal sphincter, and the dual hit is what drives severe reflux, peptic stricture, and Barrett in 5 to 35 percent of patients; management is twice-daily PPI, head-of-bed elevation, and dilation for stricture, while fundoplication is generally avoided because peristalsis cannot push past a wrap. Sjogren removes salivary lubrication and is treated with pilocarpine or cevimeline plus saliva substitutes, and is a refractory-reflux clue because saliva contributes to esophageal acid clearance. Amyloid causes infiltrative dysphagia, and macroglossia in particular hints at AL amyloid and prompts a paraproteinemia workup. Chemoradiation produces acute mucositis during radiation and late stricture months to years after, with proactive swallow therapy reducing the long-term rate of fibrotic stricture and dilation as the answer for established stricture. The neuromuscular trio is recognized by the systemic exam: myasthenia by fatigable weakness, ptosis, and anti-AChR or anti-MuSK antibodies (treated with pyridostigmine and immunosuppression); ALS by fasciculations, tongue atrophy, and electromyographic denervation; polymyositis and dermatomyositis by symmetric proximal weakness, elevated creatine kinase, anti-Jo-1, and the dermatomyositis rashes that prompt age-appropriate cancer screening because of the malignancy association with gastric, lung, ovarian, breast, and lymphoma cancers.