Esophagus

High Yield Anatomy and Physiology

• Layers of esophagus
• Mucosa
• Submucosa
• Muscularis propia
• NO SEROSA
• Esophageal blood supply
• Cervical - Inferior thyroid artery
• Thoracic - Vessels directly off aorta
• Abdominal - Left gastric and inferior phrenic arteries
• Upper esophageal sphincter = Cricopharyngeus (innervated by superior laryngeal nerve)
• Killian’s Triangle
• Triangular area in the wall of the pharynx located superior to the cricopharyngeus muscle and inferior to the inferior constrictor muscles
• Potentially weak spot where a pharyngoesophageal diverticulum (Zenker’s diverticulum) is more likely to occur Esophageal Perforation
• Can occur due to external trauma (rare), iatrogenic trauma (EGD, dilations, TE echo), increased luminal pressure (retching/Boerhaave), malignancy, chemical ingestion
• Diagnosis
• CXR — may have any combination of pleural effusion, pneumomediastinum, subcutaneous emphysema, pneumothorax, sub diaphragmatic air . However, CXR may also be entirely normal .

• Contrast esophagography is probably the study of choice (some may say oral contrast CT). Use water soluble first (Gastrografin) followed by dilute barium if no perforation is seen with gastrografin. If the patient is aspiration risk, use only dilute barium.

• Most common site of perforation? — distal esophagus in the left posterolateral aspect 2-3cm above GE junction
• Most common Iatrogenic location is at cricopharyngeus
• Treatment — Will vary based on location of injury, physiologic status of patient, damage to surrounding tissues, and underlying esophageal pathology .
• Resuscitate and start antibiotic for empiric coverage of Gram
• rods, oral flora, anaerobes, and fungus (e.g. ampicillin, ceftriaxone, metronidazole, and fluconazole)
• Options include non-operative management for contained leaks, drainage alone, T-tube drainage, esophageal exclusion and diversion, esophageal stents/clips, primary repair with buttress, and esophagectomy with either immediate or delayed reconstruction
• Isolated cervical esophageal injury? → Open neck and place drains
• Thoracic perforation
• Primary repair preferred if patient can tolerate — Left thoracotomy, debride devitalized tissue, myotomy to visualize full extend of mucosal injury, repair in 2 layers (inner absorbable, outer permanent), cover with well vascularized tissue (intercostal, omental, or latissimus flap), leak test, place NG past repair, drain chest, close. Also, consider placing enteral access .
• Consider underlying pathology — consider esophagectomy for malignancy, caustic perforation, or burned out megaesophagus from achalasia
• If perforation from achalasia and esophagus normal → perform contralateral myotomy
• If severely devitalized esophagus and patient unstable — exclusion and diversion
• Closure of perforation, drainage, and cervical esophagostomy for proximal diversion
• Placing T-tube into defect and draining externally as controlled fistula
• J-tube enteral access for these situations

Esophageal Motility Disorders Figure 1 - Esophageal Manometry Findings

• Achalasia — Incomplete relaxation of the LES (hypertonic) WITH aperistalsis or hypotonic esophageal contractions .
• Manometry findings, 3 Types (See Image):
• high, or normal, LES basal pressure
• Incomplete LES relaxation
• Hypotonic or absent peristalsis
• Imaging
• Bird’s beak sign on barium swallow with esophageal dilation
• Caused by degenerative loss of nitric oxide producing inhibitory neurons within the LES, mixed etiology autoimmune, genetic, infectious
• Causes can be idiopathic or secondary to Chagas’ disease (Trypanosoma Cruzi)
• Pseudoachalasia = achalasia caused by malignancy

• Treatment: Minimally invasive Heller myotomy with partial fundoplication (6 cm on esophagus, 2 cm onto stomach)

• While endoscopic therapies are available (Pneumatic dilation, Botulinum toxin injection), these are less effective and increase the rate of later surgical complication, thus should be avoided for those that are good surgical candidates .
• If they perforate during a dilation make sure you do the myotomy after repairing the esophagus
• Isolated hypertensive LES
• Manometry findings:
• High basal LES pressure
• Complete LES relaxation
• Normal peristalsis
• Tx: Ca channel blockers, nitrates, Heller
• Diffuse esophageal spasm
• Manometry findings
• Normal LES pressure and relaxation
• High amplitude, uncoordinated esophageal contractions (>30mmHg simultaneous contractions is >10% of swallows)
• Tx: Ca channel blockers, nitrates . Surgery is less effective . Needs long segment myotomy in extreme cases .
• Nutcracker esophagus
• Manometry findings
• Generally normal LES pressure and relaxation
• High amplitude, coordinated esophageal contractions
• Tx: Ca channel blockers, nitrates . Surgery is less effective . Needs long segment myotomy in extreme cases . Esophageal Diverticula
• Zenker’s (Cervical) Diverticulum — due to dysfunction of superior esophageal sphincter muscles causing increased intraesophageal pressure

• False pulsion diverticulum

• Division of upper esophageal sphincter is key to preventing continued symptoms, recurrence, and post operative fistula
• Diverticulum >3cm — Endoscopic division of upper esophageal sphincter, creating a common lumen between diverticulum and esophagus is an effective option
• For diverticulum <3cm — need open myotomy (via left neck incision) with or without diverticulectomy (resection or suspension of diverticula)
• Epiphrenic esophageal diverticula
• Pulsion diverticulum
• Associated with esophageal motility disorders
• Treatment: Diverticulectomy and treatment of underlying motility disorder (generally requires Heller myotomy)
• Thoracic, mid-esophageal, diverticula
• Different in that it is often a TRACTION diverticula (True diverticula), commonly associated with adjacent inflammatory conditions (e.g. tuberculosis, malignancy), although can also be pulsion caused by mobility disorder
• If symptomatic — VATS diverticulectomy and myotomy Barrett’s Esophagus Definition: Intestinal metaplasia of the lower esophagus (Squamous → Columnar)
• Mucosal reaction to lower esophageal injury due to reflux of gastric acid .
• 30-60x increased risk of esophageal adenocarcinoma
• What is Surveillance?
• EGD annually with biopsies → if 2 consecutive years negative for dysplasia → EGD every 3 years
• 4 quadrant biopsies every 1-2cm of involved segment

• Low-grade dysplasia on biopsy? → repeat endoscopy with biopsy in 6 months

• High-grade dysplasia (HGD) on biopsy? → repeat biopsy and confirm with expert GI pathologist → endoscopic mucosal resection (EMR) if HGD confirmed
• Answer used to be Esophagectomy — however, the rate of progression to invasive cancer may be lower than originally thought . Esophageal and Esophagogastric Junction Cancer
• Histologically classified as Squamous Cell Cancer (SCC) or Adenocarcinoma
• both are more common in men
• SCC more common in Asia and Eastern Europe
• Adenocarcinoma is more common in N . American and W . Europe
• Tobacco and ETOH strong risk factors for SCC
• Obesity, GERD, Barretts are major risk factors for Adenocarcinoma
• Often advanced stage at time of diagnosis
• Work-up
• H&P, labs, endoscopy with biopsy (+bronch if tumor above carina), CT chest/abdomen .
• EUS with FNA of suspicious nodes and PET/CT also recommended for staging
• Staging Pearls
• T Stage
• T1
• 1a — Invades lamina propria or muscularis mucosa
• 1b — Invades submucosa (important distinction because rich submucosal lymphatic system
• T2 — Invades muscularis propria
• T3 — Invades adventitia (remember, no serosa)

• T4 — Invades surrounding structures

• 4a - Resectable (invades pleura, pericardium, diaphragm)
• 4b - Unresectable (invades aorta, vertebrae, trachea)
• N Stage
• N1 — Involves 1-2 nodes
• N2 — Involves 3-6 nodes
• N3 — 7 or more nodes
• M1 - distant metastasis
• Grade is also important for management decisions (e .g . EMR vs esophagectomy for small superficial lesions, neoadjuvant vs surgery first, etc.)
• Stage
• I — T1, N0, M0
• II — Up to T3, N0, M0 or T2, N1, M0
• III — Up to T4, N3, M0
• IV — Distant mets
• Management
• Randomized trials have shown preoperative chemoradiation (CROSS study) and perioperative chemotherapy (MAGIC Trial) improves survival in patient with resectable esophageal and esophagogastric CA
• Thoracic esophageal CA >5cm from cricopharyngeus, abdominal esophageal CA, and EGJ CA → should be considered for esophagectomy for resectable lesions
• Cervical or cervicothroacic esophageal CA <5cm for cricopharyngeus → Definitive chemoradiation, No esophagectomy
• NCCN Recommendations
• HGD, Tis or select T1a tumors (<2cm and well to moderate differentiation with no e/o lymph node metastasis) → Endoscopic resection +/- ablation

• T1b, N0 tumors → Esophagectomy (Some say treat select T1b (small superficial T1b without NVI with EMR/ablation, but controversial)

• young patients, and those with high grade T1 lesions may be candidates for neoadjuvant chemoradiation
• T2 or greater or any N+ → Neoadjuvant chemoradiation followed by esophagectomy if resectable
• Unresectable (T4b or M1) → Definitive chemoradiation
• Fluorouracil or Taxane based therapy for perioperative and definitive chemo
• Surgical approaches
• Transthoracic Esophagectomy
• Ivor-Lewis esophagectomy — Laparotomy and right thoracotomy with upper thoracic esophagogastric anastomosis — good for distal tumors
• Stomach mobilized and used as conduit, preservation of right gastric and right gastroepiploic artery
• McKeown esophagectomy is similar except anastomosis made higher (cervical anastomosis) — better for more proximal lesions
• Transhiatal Esophagectomy
• Laparotomy and left cervical incision with cervical anastomosis
• Advantages: Avoid morbidity of thoracotomy, leak with cervical anastomosis better tolerated than thoracic leak
• Disadvantages: Potentially smaller lymph node harvest, Large mid thoracic level tumors may be difficult to mobilize
• Equal long term survival as Transthoracic approach

• Minimally invasive techniques are also options in experienced hands (thoracoscopic, laparoscopic, and robotic mobilization)

• Patient had previous gastric resection → Colon interposition conduit
• Adjuvant therapy
• IN GENERAL (Some Caveats, but for Boards):
• SCC does not need adjuvant therapy if R0 resection (regardless of nodal status)
• Adenocarcinoma generally get adjuvant chemo, except when:
• T1, NO and R0 resection and did NOT receive neoadjuvant therapy Quick Hits
• Anatomic areas of esophageal narrowing? — at the cricopharyngeus muscle, aortic arch, left mainstem bronchus, LES
• Esophagus most vulnerable to injury at these sites
• Primary blood supply to gastric conduit after esophagectomy?
• Right gastroepiploic
• Patient with dysphagia and you note skin thickening on palms/soles?
• Tylosis — Autosomal dominant condition linked to chromosome 17q25 associated with palmoplantar keratoma
• 40-90% risk of SCC of esophagus by age 70 — annual Upper GI starting at age 20
• SCC of head and neck, esophagus, and pancytopenia
• Fanconi Anemia (not syndrome)
• Patient with locally advanced esophageal cancer is undergoing neoadjuvant chemoradiation, has severe dysphagia and is malnourished → what feeding tube are you going to place?

• Jejunal feeding tube — NO G-TUBE OR PEG — Preserve gastric conduit

• Dysphagia with well circumscribed, ovoid 6cm mass on barium swallow in wall of mid esophagus?
• Esophageal leiomyoma — Most common benign tumor of
• Treatment: For symptomatic tumors or tumors >5cm → Enucleation via VATS or thoracotomy (Right sided approach for mid esophageal lesions, Left sided approach for distal lesions)
• DO NOT Biopsy — Creates mucosal scarring and makes enucleation more dangerous/difficult
• Patient with longstanding GERD and now with dysphagia . EGD demonstrates a narrowed ring of mucosal just above the GE junction .
• Schatzki’s ring, found at the squamocolumnar junction
• Dilation and PPI, NO RESECTION
• Approaches to esophagus by level
• Cervical - Left neck
• Mid thoracic - Right chest
• Distal - Left chest

Figures