Esophageal Disease PDF

Esophageal Disease Ghada Alshehri Raghad Assiri Shaima Banjar Objective Understand anatomy and physiology of the Esophagus Describe the differential diagnosis of Dysphagia Discuss Clinical features, investigations, and treatment of Esophageal Motility Disorders Understand clinical features of Gastroe...

Esophageal Disease Ghada Alshehri Raghad Assiri Shaima Banjar Objective Understand anatomy and physiology of the Esophagus Describe the differential diagnosis of Dysphagia Discuss Clinical features, investigations, and treatment of Esophageal Motility Disorders Understand clinical features of Gastroesophageal Reflux disease Discuss different type of Esophageal Diverticulum and management High light management and clinical presentation of Esophageal Perforation Discus type and management of Hiatal Hernia Describe management of benign and malignant tumor of the esophagus Understand features of Caustic Injury 1 Anatomy & physiology Of Esophagus 3 2 Differential Diagnosis of Dysphagia Esophageal motility disorders Anatomy & physiology Of Esophagus Ghada Alshehri Cont. Overview: Tube that connects the pharynx (at the level of C6) to the stomach (at the level of T11) Divided into 3 main parts (cervical, Thoracic, Abdominal) Length: ∼ 25 cm Located between trachea and spine Crosses the diaphragm at T10 through the esophageal hiatus The thoracic esophagus is extraperitoneal. The abdominal part is intraperitoneal. Cont. Cont. Constrictions of esophagus Cricopharyngeal sphincter at C6 Crossing of aortic arch at T4/5 Crossing of left main bronchus at T5/6 Crossing of diaphragm (esophageal hiatus; T10) Differential Diagnosis of Dysphagia Cont. Esophageal motility disorders Esophageal Motility Disorders overview: Esophageal hypermotility Disorders Hypertensive esophageal contractions: vigorous esophageal contractions with distal contractile integral (DCI) > 8000 mm Hg/sec/cm ○ Hypercontractile esophagus (jackhammer esophagus or nutcracker esophagus): hypertensive propagative esophageal contractions in which at least 20% of swallows have a DCI > 8000 mm Hg/sec/cm Premature esophageal contractions: contractions with a distal latency of < 4.5 seconds between them ○ Distal esophageal spasm (DES; corkscrew esophagus): premature and uncoordinated nonpropagative esophageal peristalsis in at least 20% of swallows Hypertensive and propagative esophageal contractions are seen in hypercontractile esophagus. Premature and nonpropagative esophageal contractions are seen in distal esophageal spasm. Esophageal hypomotility Disorders Achalasia Esophageal Motility Disorders overview: Etiology: poorly understood The Chicago classification divides esophageal motility into four categories according to findings on high-resolution manometry. Chicago Classification of Esophageal Motility Disorders (version 3.0) Incomplete LES relaxation Achalasia Esophagogastric junction outflow obstruction (EGJ outflow obstruction) Major motility disorders Absent contractility Distal esophageal spasm Hypercontractile or jackhammer esophagus Minor motility disorders Ineffective esophageal motility Fragmented peristalsis Normal esophageal motility Clinical features Intermittent dysphagia to liquids (and potentially, solids) Episodic retrosternal chest pain Reflux symptoms (e.g., heartburn, regurgitation) Globus sensation Upper respiratory symptoms (e.g., hoarseness, recurrent cough) Symptoms aggravated by stress and/or hot and cold food/drinks Dysphagia predominantly to liquids is suggestive of an esophageal hypermotility disorder. Diagnosis Approach Rule out immediately life-threatening causes of chest pain Investigate for more common causes of dysphagia. - Upper endoscopy with biopsies - Esophageal pH monitoring to evaluate for GERD - Esophageal barium swallow Obtain high-resolution esophageal manometry (HRM) if the index of suspicion for esophageal hypermotility disorders is high or if endoscopy and esophageal barium swallow are inconclusive. Cont. Imaging Imaging is the mainstay of diagnosis for esophageal disorders. Laboratory studies may be considered to rule out other diagnoses Upper endoscopy Indication: preferred first-line test in the workup of dysphagia predominantly to solids Esophageal barium swallow Indication: preferred first-line test in the workup of dysphagia to liquids or both liquids and solids Findings Hypercontractile esophagus: usually normal Distal esophageal spasm: multiple nonperistaltic contractions, which resemble pseudodiverticula (corkscrew appearance; rosary bead esophagus) May appear normal between acute episodes of DES Cont. High-resolution esophageal manometry (HRM) Indications: normal upper endoscopy and barium swallow in a patient with dysphagia Gold standard for diagnosing esophageal motility disorders Procedure: measures the amplitude, length, and duration of the peristaltic waves via a nasogastric tube fitted with numerous pressure sensors Findings Distal esophageal spasm: premature contractions in at least 20% of swallows Hypercontractile esophagus: hypertensive esophageal contractions in at least 20% of swallows HRM may be normal between acute episodes Conventional esophageal manometry Indication: suspected esophageal hypermotility disorder if HRM is not available Procedure: Measures the propagation, speed, and vigor of the peristaltic wave via an esophageal catheter fitted with pressure sensors every 3–6 cm Results are presented in a line tracing display. Findings Distal esophageal spasm: ≥ 10% of swallows have simultaneous (nonprogressive) contractions with a mean amplitude ≥ 30 mm Hg. Hypertensive peristalsis: progressive peristaltic waves with amplitude ≥ 220 mm Hg Findings may be normal between episodes. (not used any more) Management Lifestyle modifications Sitting upright during and after meals Taking small bites, chewing food thoroughly, and eating slowly Drinking between bites Pharmacological therapy Indications Often combined with lifestyle modifications for adequate symptom control Smooth muscle relaxants and visceral analgesic agents are used as abortive therapy for acute episodes. Avoiding extremely hot or cold foods Medications for esophageal hypermotility: there is no consensus regarding the best initial therapy Avoiding bread, meat, and rice, as they worsen dysphagia Smooth muscle relaxants Stopping medications that affect esophageal motility (e.g., opioids) Nitrates (e.g., isosorbide dinitrate) PDE5 inhibitor (e.g., sildenafil) Calcium channel blockers Nifedipine Diltiazem Consider PPI if GERD is suspected or confirmed Peppermint oil Tricyclic antidepressants (e.g., imipramine) or an SSRI (e.g., trazodone) Cont. Invasive therapy Indications Inadequate symptomatic improvement with pharmacological therapy Intolerable side effects of pharmacological therapy Endoscopic options Endoscopic botox injection: first-line invasive procedure for hypermotility disorders Temporarily effective (∼ 6 months) Repeat procedures are required. Infection is a very serious potential complication of botox injection. Second-line options: Consider in patients with DES and impaired esophagogastric junction relaxation. Peroral endoscopic myotomy (POEM) Endoscopic pneumatic dilation Surgery: extended/long LES myotomy Indication: persistent symptoms despite pharmacological and endoscopic therapy Procedure May be performed laparoscopically or via an open abdominal/thoracoabdominal approach An incision extending from the LES into the esophageal body is created. Typically combined with a fundoplication procedure to minimize gastroesophageal reflux Evidence is currently lacking on the advantages of surgical intervention over endoscopic therapy. Nutcracker Esophagus known as hypercontractile esophagus is a disorder characterized by excessive contractility. It is described as an esophagus with hypertensive peristalsis or high amplitude peristaltic contractions. It is seen in patients of all ages, M=F, and is the most common of all esophageal hypermotility disorders. Pathophysiologic process is not well understood. it is associated with hypertrophic musculature that results in high amplitude contractions of the esophagus and is the most painful of all esophageal motility disorders. Signs and symptoms: Patients with nutcracker esophagus present in a similar fashion to those with DES and frequently complain of chest pain and dysphagia. Odynophagia is also noted, but regurgitation and reflux are uncommon.. Ambulatory monitoring can help distinguish this disorder from DES. This is of critical importance because a subset of DES patients with dysphagia can be helped with esophagomyotomy, but surgery is of questionable value in patients with a nutcracker esophagus. The treatment of nutcracker esophagus is medical :Calcium channel blockers, nitrates, and antispasmodics may offer temporary relief during acute spasms. Bougie dilation may offer some temporary relief of severe discomfort but has no long-term benefits. Patients with nutcracker esophagus may have triggers and are counseled to avoid caffeine, cold, and hot foods. Distal esophageal spasm An s a poorly understood hypermotility esophageal disorder characterized by repetitive, uncoordinated, nonprogressive contraction waves of the distal esophagus. Signs and symptoms: chest pain, dysphagia, regurgitation while acid reflux is not. F>M and is often found in patients with multiple medical complaints. The basic pathology is related to a motor abnormality of the esophageal body that is most notable in the lower two thirds of the esophagus. Muscular hypertrophy and degeneration of the branches of the vagus nerve in the esophagus have been observed. As a result, the esophageal contractions are repetitive, simultaneous, and of high amplitude. The treatment for DES is discussed previously Achalasia Etiology: Primary achalasia (most common): cause is unknown Secondary achalasia (pseudoachalasia): the presentation and manometric findings of a mechanical cause of obstruction (e.g., a malignancy) that mimics achalasia ○ Esophageal cancer ○ Stomach cancer and other extraesophageal cancers (symptoms may be due to mass effect or paraneoplasia) ○ Chagas disease ○ Amyloidosis Pathophysiology: Atrophy of inhibitory neurons in the Auerbach plexus → lack of inhibitory neurotransmitters (e.g., NO, VIP) → inability to relax and increased resting pressure of the LES, as well as dysfunctional peristalsis → esophageal dilation proximal to LES Clinical features: Dysphagia to solids and liquids; can be progressive or paradoxical Regurgitation Retrosternal pain and cramps Weight loss Cont. Diagnosis Esophageal barium swallow: supportive and/or confirmatory test ○ Bird-beak sign: dilation of the proximal esophagus with stenosis of the gastroesophageal junction ○ Delayed barium emptying or barium retention Upper endoscopy: to rule out pseudoachalasia ○ Usually normal ○ May show retained food in esophagus or increased resistance of LES during passage with endoscope ○ If malignancy is suspected, biopsy and endoscopic ultrasound are indicated Esophageal manometry: confirmatory test of choice Cont. Management: If a low surgical risk Pneumatic dilation ○ Endoscope-guided graded dilation of the LES that tears the surrounding muscle fibers with the help of a balloon ○ The success rate at one month is ∼ 85%; perforation risk is ∼ 2%. LES myotomy (Heller myotomy): a surgical procedure in which the lower esophageal sphincter is incised longitudinally to re-enable passage of food or liquids to the stomach. If a high surgical risk Botulinum toxin injection in the LES ○ A good choice for patients who are poor surgical candidates ○ More than 50% of patients require treatment again within 6–12 months. If other measures are unsuccessful: nitrates or calcium channel blockers Thank you ! References: 1. 2. 3. Amboss Medescape Sabiston Textbook of Surgery Gastroesophageal reflux disease (GERD) Raghad Assiri What is Gastroesophageal reflux disease (GERD)? A condition in which reflux causes troublesome symptoms (typically including heartburn or regurgitation) and/or esophageal injury/complications Etiology of GERD 1 LES dysfunction 3 Abnormal gastric emptying 2 Impaired clearance of reflux from the esophagus 4 External factors contribute to the development of GERD. Clinical Manifestations Typical symptoms Retrosternal burning pain (heartburn) Regurgitation Dysphagia, odynophagia Atypical symptoms Pressure sensation in the chest Belching, bloating Dyspepsia, epigastric pain Nausea Halitosis Extraesophageal symptoms Chronic nonproductive cough Nighttime cough Hoarseness Dental erosions Diagnosis: Twenty-four–hour pH monitoring Esophagogastroduodenoscopy (EGD) Esophageal manometry Barium esophagram Treatment Medication H2-receptor antagonists Proton pump inhibitors (PPIs) Surgery Esophagogastric fundoplication Definition: an antireflux procedure in which the gastric fundus is wrapped around the lower esophagus and secured with stitches to form a cuff Complications Barrett esophagus (BE) intestinal metaplasia of the esophageal mucosa induced by chronic reflux. Conditions that may develop include : Reflux esophagitis Iron deficiency anemia Asthma, aspiration Recurrent pneumonia, and laryngitis. Esophageal Diverticula (Esophageal pouches) Esophageal diverticula Pathophysiology: Inadequate relaxation of the esophageal sphincter and increased intraluminal pressure → outpouching of the esophageal wall → pulsion diverticulum False diverticula contain only mucosa and submucosa Inflammation of the mediastinum with scarring and retraction → traction diverticulum True diverticula contain all layers of the wall Calcification esophageal diverticulum : Location in the esophagus: Near the upper esophageal sphincter (Zenker diverticulum) Near the midpoint of the esophagus (traction diverticulum) Immediately above the lower esophageal sphincter (epiphrenic diverticulum) Clinical presentation Dysphagia Regurgitation of undigested food Halitosis Aspiration Coughing after food intake Retrosternal pressure sensation and pain Weight loss Neck mass Diagnosis Barium swallow with dynamic continuous fluoroscopy. An upper endoscopy is not required to confirm the diagnosis but is recommended to exclude a concurrent malignancy. Zenker diverticulum Most common type Zenker diverticulum at Killian triangle (a triangular weak point in the dorsal muscular wall of the hypopharynx, between the thyropharyngeal and cricopharyngeal parts of the inferior pharyngeal constrictor muscle) Zenker diverticulum Treatment Treatment involves surgery, as there is no effective medical therapy, with success rates ranging from 90% to 100%. a. Cricopharyngeal myotomy, with diverticulectomy or diverticulopexy, is classically performed through a left neck incision. The cricopharyngeus muscle is divided, and the diverticulum can either be resected or the fundus of the diverticulum can be sewn to the prevertebral fascia to facilitate its drainage. b. Endoscopic myotomy avoids an incision and shortens the hospitalization. An operating laryngoscope is used to expose the neck of the diverticulum, and a myotomy is performed using an endoscopic linear stapler. With this technique, the diverticulum becomes part of a common channel with the cervical esophagus Mid-esophageal diverticula Most mid-esophageal diverticula are asymptomatic and do not require treatment. For those with symptoms and a mid-esophageal diverticulum, treatment consists of a myotomy and a diverticulectomy. Epiphrenic diverticula Treatment Therapy is directed at the associated motility disorder that is usually present, and any complication of the diverticulum a. Small asymptomatic epiphrenic diverticula not associated with a motility disorder may be followed clinically. b. Complicated epiphrenic diverticula and those with an associated esophageal motility disorder usually require surgery. Esophageal myotomy and diverticulectomy is the initial surgical option. Esophageal Perforations Esophageal Perforation A breakage in the esophageal wall that result in leakage of air or content into the mediastinum or/and pleural cavity, peritoneum. Most common cause of perforation is iatrogenic by (Endoscopy EGD), dilatation, intubation. >50% Boerhaave’s syndrome. 15% Foreign body ingestion. 12% Accidental ingestion of caustics. 9% Other underlying disease and malignancy. 1% Differential Diagnosis Mallory–Weiss syndrome is massive upper gastrointestinal hemorrhage caused by a tear through the mucosa of the distal esophagus or gastroesophageal junction. causes are vomiting, paroxysms of coughing, blunt abdominal trauma, and straining during a bowel movement. Boerhaave syndrome Boerhaave syndrome, is a spontaneous perforation of the esophagus that results from a sudden increase in intraesophageal pressure combined with negative intrathoracic pressure. Clinical Features of esophageal perforation Mackler triad Vomiting and/or retching Severe retrosternal pain that often radiates to the back Subcutaneous or mediastinal emphysema: crepitus in the suprasternal notch and neck region or crunching/crackling sound on chest auscultation (Hamman sign) Other features Dyspnea, tachypnea, tachycardia Dysphagia Signs of sepsis History of recent endoscopy Delayed presentations: critically ill with sepsis and multiorgan dysfunction Diagnosis: Neck or a chest x-ray is first conducted Contrast Esophagography CT scan Flexible endoscopy Chest x-ray Widened mediastinum Pneumomediastinum, pneumothorax, pneumoperitoneum, subcutaneous emphysema Pleural effusion Contrast Esophagography Confirmatory test: Contrast leak on contrast esophagography (gold standard) reveals the location and size of the rupture. Management Initial approach ABCDE survey Establish airway and supplemental oxygen as needed. IV fluid resuscitation Nothing by mouth (NPO) and supply nutritional support Broad-spectrum IV antibiotics IV proton pump inhibitor Parenteral analgesics. Management Surgery Indications Hemodynamic instability Patients who do not fulfill the criteria for conservative management Clinical deterioration during conservative management Surgical repair Closure of the ruptured esophageal segment Last resort: esophagectomy;is reserved for perforations due to cancer or untreatable obstructions. Endoscopic intervention Esophageal stent placement Endoclip Fibrin glue application Reference https://next.amboss.com/us/article/wg0hx2#L34968ccd1471bd591eeef62475c1c0ad https://next.amboss.com/us/article/Eg08C2#L84a3544a474445c8573e76bff2b41ccb https://next.amboss.com/us/article/vg0AC2#L4ccef2d0b1c561ef59a16b9ba75113f5 Hiatal Hernia Shaima Banjar What is Hiatal hernia? Abnormal protrusion of any abdominal structure/organ, most often a portion of the stomach, into the thoracic cavity through a lax diaphragmatic esophageal hiatus. Types of Hiatal hernia Type I: sliding hiatal hernia Most common type (95% of cases) The GEJ and the gastric cardia slide up into the posterior mediastinum. The gastric fundus remains below the diaphragm (hourglass stomach) RF: Age, increase intra abdominal pressure, smoking Type II: paraesophageal hiatal hernia Part of the gastric fundus herniates into the thorax. The GEJ remains in its anatomical position below the diaphragm. RF: Age, women > men, increase intra abdominal pressure, chronic esophagitis, fiber depleted diet Types of Hiatal hernia Type III: mixed hiatal hernia Mix of type l and ll The GEJ and portion of the gastric fundus prolapse through the hiatus. Type IV: complex hiatal hernia Herniation of any abdominal structure other than stomach (e.g., spleen, omentum, or colon). Rarest type Clinical features 1 Clinical manifestations Symptoms of GERD Epigastric/substernal pain Early satiety Nausea, and Retching 2 Complications GERD symptoms Gastric volvulus Bleeding from gastric ulceration, gastritis, or erosions within the incarcerated hernia pouch Respiratory complications Clinical features 3 Diagnosis Barium swallow: most sensitive test. Assesses type and size of hernia Endoscopy: Z-line is squamocolumnar junction. Chest X-ray CT Thorax: recommended for urgent preoperative evaluation of complicated type II, III, and IV hernias. Esophageal manometry: to measure the pressure of LES Management Benign & malignant Esophageal Tumors Benign tumors Benign tumors of the esophagus are rare lesions that constitute less than 1% of esophageal neoplasms. the majority of ‘benign’ tumors are not epithelial in origin Leiomyoma: arise from the muscular layer of the esophagus and are the most common benign tumor of esophagus—75% of cases. Usually located in distal two thirds of esophagus. Esophageal cyst: 20% of benign esophageal tumors. - Congenital Foregut cyst ( Major)- lined with squamous, or columnar epithelium,may - contain smooth muscle, cartilage, or fat. ESOPHAGEAL DUPLICATION CYST. Associated with vertebral and spinal cord abnormalities. Acquired epithelial cyst- Arise in lamina propria Fibrovascular polyps: originate from the mucosal layer and occur in the proximal cervical esophagus. Most benign esophageal tumors are small and asymptomatic Carcinoma of the esophagus The eighth most common cancer in the world. It is a disease of mid to late adulthood,with a poor survival rate. Only 5–10 percent of those diagnosed will survive for five years. Adenocarcinoma: affects the lower third, etiological factors are GERD and obesity Squamous cell carcinoma: originate in squamous epithelium, usually affects the upper two-thirds, Common aetiological factors are tobacco and alcohol Early stages Often asymptomatic May manifest with swallowing difficulties or retrosternal discomfort. Clinical features Benign tumor: usually asymptomatic Symptoms include dysphagia, chest pain, regurgitation, esophageal obstruction, and bleeding. Malignant Tumor: Early stages Often asymptomatic May manifest with swallowing difficulties or retrosternal discomfort. Dysphagia , weight loss, pain, and anemia due to ulceration of the tumor with resultant bleeding. Diagnosis Esophagogastroduodenoscopy Best initial and confirmatory Test Direct visualization of the tumor Allows biopsy of any suspicious lesions. Barium Swallow : indicated if there's a stricture Diagnosis Endoscopic ultrasonography Used to determine the infiltration depth and register regional lymph node disease. Full metastatic workup (CXR, bone scan, CT scan, LFTs) Staging: TNM system T: tumor size and extension (T0-T4) N: lymph node metastasis (N0-N3) M: distant metastasis (M0-M1) Determine the cancer stage (1-4) Management Benign Tumor: surgical resection by enucleation. Surgery (open thoracotomy) is indicated for : large (greater than 4 cm) tumors lesions that are growing lesions that are symptomatic Malignant Tumor : Curative Indication: Locally invasive disease that has not invaded surrounding structures High-grade metaplasia in Barrett syndrome Method: Neoadjuvant chemoradiation: as definitive treatment in patients with proven complete response (e.g., during endoscopy) Surgical resection: Endoscopic submucosal resection for removal of superficial, epithelial lesions Subtotal or total esophagectomy with gastric pull-through procedure or colonic interposition Palliative Indication: patients with advanced disease (majority of patients) Method: Chemoradiation Endoscopic dilatation and stenting of the esophagus are palliative measures used for obstructing or near-obstructing tumors that are unresectable Caustic Injury CORROSIVE agents can be taken in suicide or by mistake. Caused by acid (sulfuric acid, hydrochloric acid) or alkali (sodium hydroxide, disk batteries). The severity and extent depend on the following factors: -concentration of the agent -volume ingested -Duration of contact -PH of the solution * Alkali burns are worse than acidic !? Severity 1 2 3 First-degree burn Second-degree burn Third-degree burn Superficial mucosal, focal or diffuse erythema, edema, and hemorrhage. No scar formation. mucosal and submucosal damage, ulcerations, exudates, and vesicle formation. Scar transmural, deep ulcers and black discoloration and perforation of the wall. Scar and stricture. Severity 1 2 Early Management Late Management Prehospital care Emergency department care Consultation Medication Endoscopy Esophageal dilatation Esophageal surgery Reference 1. 2. 3. 4. 5. 6. 7. Daniel P Raymond, MD, Carolyn Jones, MD. Surgical management of esophageal perforation Feb 2018. UpToDate Bailey & Love’s Short Practice of Surgery (2018). https://next.amboss.com/us/article/wg0hx2?q=esophageal%20perforation. George Triadafilopoulos, MD.Caustic esophageal injury in adults 2018. https://next.amboss.com/us/article/Dg01x2?q=hiatal%20hernia#Z496ee96043ad1b953dc0742fd7a99ab4 Michael Rosen, MD, Jeffrey Blatnik, MD.Surgical management of paraesophageal hernia 2017 Thanks!