Esophageal Disorders (PEARLS)

The NCCPA™ Gastroenterology and Nutrition PANCE Content Blueprint covers 6 topics under the category of esophageal disorders

Esophagitis (ReelDx)	ReelDx Virtual Rounds (Esophagitis) Esophagitis You are called to see a 54-year-old with chest pain radiating to the jaw Patient Gender: Female Age: 54 years Vitals Temperature: 98.5 F/36.9 C Blood Pressure: 150/74 Heart Rate: 70 Respiratory Rate: 16 Signs and Symptoms Chest pain; 1 hour radiating to the left jaw Click here to work through this patient case simulation. Patient will present as → Scenario A: An immunocompromised patient on high-dose steroids presents with odynophagia (painful swallowing) and dysphagia; oral thrush is visible. This is Candida esophagitis. Scenario B: A patient on HIV therapy (especially didanosine or zalcitabine) or a patient taking a bisphosphonate without adequate water presents with sudden-onset odynophagia and substernal chest pain — pill esophagitis. Scenario C: A young atopic man has dysphagia for solids and food impaction episodes; endoscopy shows esophageal rings and “trachealization” — eosinophilic esophagitis. Esophagitis is simply inflammation that may damage the tissues of the esophagus. It can be divided into two types: 1. Non-infectious Reflux esophagitis: mechanical or functional abnormality of the LES Medication-induced esophagitis: think NSAIDS or bisphosphonates Eosinophilic Esophagitis (EoE): Pt with Asthma symptoms and GERD not responsive to antacids. Allergic, eosinophilic infiltration of the esophageal epithelium. The most common cause of dysphagia and food impaction in young men Diagnosed with a biopsy A barium swallow will show a ribbed esophagus and multiple corrugated rings Radiation: radiosensitizing drugs include doxorubicin, bleomycin, cyclophosphamide, cisplatin Dysphagia lasting weeks-months after therapy Radiation exposure of 5000 cGy is associated with increased risk for stricture Corrosive: Ingestion of alkali or acid from attempted suicide 2 Infectious - odynophagia (pain while swallowing food or liquids) is the hallmark sign This occurs mainly in patients with impaired host defenses. Primary agents include Candida albicans, herpes simplex virus, and cytomegalovirus. Symptoms are odynophagia and chest pain Fungal: Infectious Candida: linear yellow-white plaques with odynophagia or pain on swallowing. Viral: HSV: shallow punched-out lesions on EGD CMV esophagitis: large, shallow, linear ulcerations; CD4 typically <100 EBV, Mycobacterium tuberculosis, and Mycobacterium avium intracellular are additional infectious causes DX: Upper endoscopy (EGD) with biopsy — gold standard for most types of esophagitis TX: Treat the underlying condition Candida: treat with fluconazole 100 mg PO daily HSV: treat with acyclovir CMV: treat with ganciclovir or valganciclovir Corrosive: treat with steroids Eosinophilic: treated by removing foods that incite an allergic response, topical steroids via inhaler Medication-induced: to prevent bisphosphonate-related esophagitis, treat by drinking pills with at least 4 ounces of water, avoid lying down for at least 30-60 minutes after ingestion Barium swallow of the esophagus on the left side shows multiple rings associated with eosinophilic esophagitis.
Gastroesophageal reflux disease	Patient will present as → a 55-year-old male with complaints of heartburn, belching, and epigastric pain which is aggravated by drinking coffee, eating fatty foods, and lying down. He says it gets better when he takes antacids. GERD is the retrograde flow of gastric acid into the esophagus due to lower esophageal sphincter (LES) relaxation, causing mucosal injury and symptoms Retrosternal pain/burning shortly after eating, worse with carbonation, greasy foods, spicy foods, and lying down DX: Patients with typical symptoms of GERD may be given a trial of PPI therapy. Patients who do not improve or have long-standing symptoms or symptoms of complications should be studied: Endoscopy with biopsy —the test of choice, but not necessary for typical uncomplicated cases. Indicated if refractory to treatment or is accompanied by dysphagia, odynophagia, or GI bleeding. Upper GI series (barium contrast study)—this is only helpful in identifying complications of GERD (strictures/ulcerations) PH Probe is the gold standard for diagnosis (but usually unnecessary) 24-hour ambulatory pH monitoring (with or without impedance): gold standard for diagnosing abnormal acid exposure; most useful for atypical symptoms or before anti-reflux surgery Esophageal manometry: evaluates LES pressure and motility; required before fundoplication Treatment: H2 receptor blockers, proton pump inhibitors, diet modification (avoid fatty foods, coffee, alcohol, orange juice, chocolate; avoid large meals before bedtime); sleep with the trunk of body elevated; stop smoking Nissen fundoplication: antireflux surgery for severe or resistant cases Complications: Strictures or Barrett’s esophagus
Mallory Weiss tear	Patient will present as → a 21-year-old male with hematemesis. He is brought by his girlfriend, who reports that he and his buddies have been out drinking every night last week in celebration of his 21st birthday. He reports having vomited each night, but tonight, when he started vomiting, he noticed that there was streaking of blood. Concerned, he decided to come to the emergency department. A tear that occurs in the esophageal mucosa at the junction of the esophagus and stomach, caused by severe retching and vomiting, results in severe bleeding Presentation: History of alcohol intake and an episode of vomiting with blood Caused by forceful vomiting. Associated with alcohol use, upper endoscopy showing superficial longitudinal mucosal erosions DX: Diagnosed with upper endoscopy showing superficial longitudinal mucosal erosions TX: Most bleeding is self-limited (90%) and managed supportively (IV fluids, NPO, hemodynamic monitoring) Active bleeding at endoscopy: epinephrine injection, thermal coagulation, or endoscopic band ligation Refractory bleeding: angiographic embolization or surgery (rare) PPIs: to reduce acid exposure and promote healing Endoscopic image of Mallory-Weiss tear showing superficial longitudinal mucosal erosions
Motility disorders	Achalasia Patient will present as → a 45-year-old patient with progressive dysphagia to both solids AND liquids, regurgitation of undigested food, chest pain, and weight loss. Symptoms worsen at night with possible aspiration/cough. Barium swallow shows “bird-beak” narrowing, and esophageal manometry confirms diagnosis. Treatment is pneumatic dilation or Heller myotomy. Achalasia is a motility disorder due to degeneration of inhibitory neurons in the Auerbach’s (myenteric) plexus → failure of LES relaxation + aperistalsis This is a FUNCTIONAL obstruction, NOT a mechanical one Progressive dysphagia to solids AND liquids simultaneously AND from ONSET (key differentiator from mechanical obstruction, which starts with solids only) Classic symptoms: regurgitation of undigested food, chest pain, weight loss, nocturnal cough/aspiration DX: Barium swallow = “bird-beak” distal esophagus (classic board image finding) Gold standard diagnosis = esophageal MANOMETRY showing ↑ LES pressure + incomplete relaxation + aperistalsis Must rule out pseudoachalasia (malignancy, especially gastric/esophageal cancer) → endoscopy required TX: Pneumatic dilation: most effective non-surgical option; endoscopic balloon dilation of the LES Laparoscopic Heller myotomy + partial fundoplication (definitive): surgical incision of the LES muscle; highly effective long-term Per-oral endoscopic myotomy (POEM): minimally invasive endoscopic procedure; highly effective, now widely available Botulinum toxin injection into the LES: for elderly/poor surgical candidates; temporary relief (6–12 months) CCBs or nitrates: minimal effect; rarely used as primary treatment Barium swallow showing dilated esophagus with retained column of barium and “bird’s beaking” suggestive of achalasia. Diffuse esophageal spasm Patient presents as → a 26-year-old male is brought to the emergency department (ED) via ambulance with a sudden onset of extreme chest pain. The patient states that he had just finished his morning run and was drinking from his water bottle when the pain began. He states that the pain was like “nothing he had experienced before” and radiated to his back, neck, and ears. He called EMS and was given 325mg aspirin, sublingual nitroglycerine, and supplemental oxygen in the field resulting in near resolution of his symptoms. In the ED, his exam is completely unremarkable except for a heart rate of 110 bpm. EKG shows sinus tachycardia, troponin and CK-MB are within normal limits, and stress test is normal. You order an upper GI contrast study which shows a corkscrew esophagus.Treatment is initiated with a calcium channel blocker (e.g., diltiazem), leading to symptom improvement. Diffuse esophageal spasm is a motility disorder of the esophagus characterized by uncoordinated, simultaneous contractions with NORMAL LES relaxation due to impaired inhibitory neural signaling. Episodic chest pain + dysphagia to BOTH solids and liquids (distinguishes from mechanical obstruction) Chest pain may mimic ANGINA → normal cardiac evaluation is a classic test clue Triggers include HOT/COLD liquids, stress, or rapid eating DX: Barium swallow: classic “corkscrew” or “rosary bead” esophagus (HIGH-YIELD imaging finding) CONFIRMED WITH: esophageal MANOMETRY GOLD STANDARD — showing simultaneous, high-amplitude, non-peristaltic contractions with NORMAL LES relaxation (distinguishes from achalasia) TX: Calcium channel blockers (e.g., diltiazem) or nitrates to reduce esophageal spasm PDE5 inhibitors (sildenafil), tricyclic antidepressants (for pain modulation) Refractory cases may require botulinum toxin injection or surgical myotomy Corkscrew esophagus as seen in diffuse esophageal spasm Neurogenic dysphagia Patient presents as → a 32-year-old female who reports to your office complaining of nasal regurgitation with the ingestion of fluids. Sure enough, when you hand her a glass of water, and she sips the liquid, it regurgitates out her nose. You make an immediate referral to the neurologist. Three months later, when the patient returns to your office, she explains that she has been diagnosed with multiple sclerosis. Neurogenic dysphagia is a result of the faulty transmission of nerve impulses to the pharyngeal muscles, generally caused by an associated neuromuscular disease, such as myasthenia gravis, amyotrophic lateral sclerosis, MS, or stroke This condition is produced by weakness and incoordination of the muscles in the pharynx that propel food into the esophagus Both liquids and solids are difficult to swallow, and aspiration into the windpipe and regurgitation into the nose commonly occur Zenker diverticulum Patient presents as → a 68-year-old female seen in the emergency department with recurrent coughing spells and regurgitation after meals. Her breath is nearly unbearable upon arrival at the ED. She is also noted to have a palpable, fluctuant neck mass on physical examination. A pharyngeal pouch that develops in the proximal esophageal wall Arising through Killian's triangle (between the oblique and transverse fibers of the inferior pharyngeal constrictor); increased pharyngeal pressure + cricopharyngeal dysfunction Causes regurgitation of undigested food and liquid into the pharynx several hours after eating, foul odor of breath Diagnosed with barium esophagram (swallow) showing outpouching of barium-filled sac Treat with observation if small and asymptomatic, diverticulectomy, cricopharyngeal myotomy Outpouching of barium-filled sac as seen in Zenker diverticulum Scleroderma (Progressive Systemic Sclerosis — Esophageal Involvement) Patient presents as → a 48-year-old woman with a history of diffuse systemic sclerosis (scleroderma) who reports progressive dysphagia to solids and liquids and chronic heartburn/regurgitation. Exam shows tight, thickened skin of the hands (sclerodactyly) and telangiectasias. Workup reveals decreased LES tone and absent distal esophageal peristalsis on manometry. She is treated with proton pump inhibitors and lifestyle modification to prevent complications such as Barrett esophagus. Scleroderma esophagus is esophageal smooth muscle atrophy and fibrosis due to systemic sclerosis, causing severe hypomotility and LES incompetence Classic presentation: progressive dysphagia (solids → liquids) + severe GERD/heartburn with regurgitation (key combo tested) Mechanism: smooth muscle fibrosis → ↓ peristalsis (distal 2/3) + ↓ LES tone → acid reflux + esophageal dilation Strong association with limited cutaneous systemic sclerosis (CREST) → think Raynaud + GERD/dysphagia Key differentiator: low LES pressure (vs achalasia = high LES pressure) → boards LOVE this contrast DX: Esophageal manometry (most accurate) showing absent peristalsis + low LES pressure; barium swallow may show dilated, atonic esophagus Complications: severe GERD → Barrett esophagus → adenocarcinoma risk, strictures, aspiration TX: Aggressive GERD management with PPIs (first-line) + lifestyle changes (elevate head, small meals) Prokinetics (e.g., metoclopramide) if needed + screening for Barrett esophagus in chronic GERD patients Peptic stricture showing narrowing of the esophagus near the junction with the stomach due to chronic gastroesophageal reflux in the setting of scleroderma. Esophageal stenosis An esophageal stricture is a narrowing of the lumen of the esophagus, preventing the passage of food. Typically, it is at the distal end of the tube and is the result of scarring after chronic exposure to gastric juice due to GERD. Scarring and, consequently, stricture formation can also occur in response to other types of trauma, including swallowing of caustic solutions, chronic swallowing of pills without water, or residual scarring after surgery The patient presents with dysphagia to solids The usual treatment is dilation Esophageal stenosis (with multiple ulcers) due to chronic reflux esophagitis
Esophageal strictures	Patient will present as → a 60-year-old man with a 15-year history of GERD presents with progressive dysphagia to solids first, then liquids (mechanical pattern). He has had no difficulty with liquids until recently. He denies weight loss. Barium swallow shows a smooth, tapered narrowing in the distal esophagus. Upper endoscopy reveals a smooth, benign-appearing stricture (Schatzki ring / peptic stricture). An esophageal stricture is an abnormal tightening or narrowing of the esophagus, making it more difficult for food to travel down the tube. People with esophageal strictures may have pain or difficulty swallowing It can be caused by or associated with gastroesophageal reflux disease, esophagitis, a dysfunctional lower esophageal sphincter, disordered motility, or a hiatal hernia Strictures can form after esophageal surgery and other treatments such as laser therapy or photodynamic therapy Dysphagia to solids that is only gradually progressive is suggestive of an esophageal stricture The majority of esophageal strictures result from benign peptic strictures from long-standing gastroesophageal reflux disease (GERD), which accounts for 70 to 80% of adult cases Esophageal stricture types: Peptic strictures: most common benign stricture; from long-standing GERD → scarring and fibrosis; smooth on barium; treatment: endoscopic dilation + PPI therapy Schatzki ring: thin, web-like stricture at the GEJ (see example) (squamocolumnar junction); produces intermittent dysphagia for solids (“steak-house syndrome” — episode while eating steak); treatment: endoscopic dilation Esophageal web (Plummer-Vinson syndrome): upper esophageal web + iron-deficiency anemia + dysphagia in middle-aged women; risk factor for squamous cell carcinoma of the esophagus; treatment: iron supplementation + dilation Malignant stricture: progressive dysphagia + weight loss; irregular/shouldered appearance on barium; requires biopsy Caustic/lye stricture: after ingestion of alkali or acid substances Diagnosed by upper endoscopy to determine the underlying cause, exclude malignancy, and perform therapy (dilation) if needed Barium contrast esophagram (barium swallow) can be used as the initial test (prior to upper endoscopy) in patients with clinical features of proximal esophageal lesion or known complex (tortuous) stricture Treat with endoscopic dilation Esophageal web on barium swallow: The arrowhead points to the incompletely opened upper esophageal sphincter. The arrow points to the jet phenomenon of the barium contrast when passing through the constricted area.
Esophageal varices (ReelDx)	ReelDx Virtual Rounds (Esophageal varices) Esophageal varices You are called to see a 34-year-old with abdominal pain and hematemesis Patient Gender: Male Age: 34 years Vitals Heart Rate: 63 Respiratory Rate: 14 Pulse Oximetry: 100% Blood Pressure: 89/48 Signs and Symptoms Abdominal pain; vomiting blood Click here to work through this patient case simulation. Patient will present as → a 64-year-old man with a history of alcoholism, tobacco use, and hypertension presents to the general surgery clinic, where he was referred for further evaluation of blood in his stool. He reports occasional abdominal pain relieved transiently with meals and one episode of painful vomiting. Recently, his stools have been black. Spider angiomas but no palmar erythema or hepatosplenomegaly are observed on the exam. Dilated veins in the distal esophagus or proximal stomach caused by elevated pressure in the portal venous system, typically from cirrhosis Presentation: Often asymptomatic until hematemesis Etiology: Portal hypertension (from cirrhosis), Budd-Chiari syndrome (from occlusion of hepatic veins) DX: Perform emergent upper GI endoscopy (once the patient is stabilized) in all patients with GI bleed ⇒ diagnostic and can be therapeutic Serum labs: hemoglobin and hematocrit, platelet count Screening is indicated when cirrhosis or portal hypertension is diagnosed When high-risk varices are diagnosed, prophylaxis should be started, and further screening is not necessary Otherwise, screening should be repeated every 2 to 3 years for patients without varices and every 1 to 2 years for patients with small varices Treatment: Therapeutic endoscopy – endoscopic banding and IV octreotide Serum labs: hemoglobin and hematocrit, platelet count Prevention of rebleeds (70% of rebleeds are within 1 year of initial bleed, and 1/3 are fatal) Nonselective beta-blockers - propranolol, nadolol (treatment of choice in primary prophylaxis to prevent rebleeds)