Section: UGI Sub-section: Oesophagus Curriculum: Curriculum, page 94

Definition

  • Oesophageal luminal narrowing secondary to inflammation, fibrosis, or neoplasia
  • Benign vs malignant
  • Concerning if rapidly progressive
  • Especially if associated weight loss

Aetiology

Extrinsic Causes

Vascular Rings

  • Developmental abnormality
    • Aberrant right subclavian artery from descending aorta
      • Most common
      • Travels behind oesophagus, incomplete ring
      • Often asymptomatic, may cause posterior compression
        • “Dysphagia Lusoria”
        • Associated with non-recurrent laryngeal nerve
    • Anomalous formation of right aortic arch with left ligamentum arteriosum and resultant retro-oesophageal left subclavian artery
      • Complete ring
    • Double aortic arch
      • Fail to remodel appropriately, which results in two aortic arches both connecting the ascending and descending aortas
      • Complete ring
    • Other causes
      • Left aortic arch with a right descending aorta and right ductus arteriosus (or ligamentum)
      • Right aortic arch with a left descending aorta and left ductus arteriosus (or ligamentum)
      • Left aortic arch with an aberrant right subclavian and a right ductus arteriosus (or ligamentum)
      • Right aortic arch with mirror-image branching and a left ductus arteriosus (or ligamentum) arising from a retroesophageal dimple pointing to the left (also described as a double aortic arch with atretic left arch)
      • Right aortic arch with retroesophageal left innominate and either a left ductus arteriosus or diverticulum

Double aortic arch Right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum Screen Shot 2020-08-22 at 2.56.55 PM.png

Pulmonary artery sling

  • Left pulmonary artery comes off right instead of main branch & courses between trachea & oesophagus
  • Associated with other cardiac defects & foregut abnormalities
  • All require repair to prevent tracheal or left PA stenosis Screen Shot 2020-08-22 at 3.02.03 PM.png

Innominate artery compression syndrome

  • This syndrome occurs when the innominate (or brachiocephalic) artery originates later along the course of the transverse arch, resulting in takeoff to the left of the trachea.
  • Anterior tracheal compression with associated tracheomalacia results when the aberrant innominate artery passes back to the right.

Intrinsic Causes

  • Peptic strictures – most common (90%)
  • Schatzki ring
  • Rings
  • Webs
    • Plummer-Vinson syndrome
  • Others
  • Anastomotic

Peptic Strictures

  • Concentric lower oesophageal ring at squamo-columnar junction
  • 2-10% of GORD pts
  • Due to chronic inflammation & fibrosis
  • Most < 1cm in length
  • Management
    • Biopsy to exclude malignancy
    • Must treat reflux initially – PPI’s, anti-reflux surgery
    • Sequential dilation with 10mm balloon
      • Perforation risk 1-2%, 90% success rate
    • Division with laser of tight band
      • May reduce need for repeated dilation
    • Stent placement – temporary as needs to be removed
    • Severe strictures may require resection.

Oesophageal Ring

  • Types of rings
    • A - normal smooth muscle contraction in the esophagus
    • B -  mucosal structure precisely at the squamocolumnar junction that is smooth and thin (≤5 mm in axial length). Owing to its location, a B ring is covered with squamous mucosa proximally and columnar epithelium distally
      • Esophageal rings are usually mucosal but in rare cases may be muscular due to hypertrophy of an A ring.
      • Schatzki ring, the most common type of esophageal ring, is a narrow mucosal B ring that is less than 12.5 mm in diameter
  • Aetiology
    • Hiatus hernia 
      • Schatzki rings are almost always associated with a hiatal hernia. In a study of 167 patients with a Schatzki ring, a hiatal hernia was detected in 97 percent
        • Lies precisely at squamocolumnar mucosal GEJ
        • Does not have a component of true oesophageal muscle
      • Aetiology not understood
        • ? Persistent apposition of two mucosal layers & fibrosis of submucosa.
        • Almost always accompanied by a small hiatal hernia -? fibrosed proximal margin of sliding HH.
    • Eosinophilic oesophagitis
      • Schatzki rings have also been associated with eosinophilic esophagitis
      • However, more often patients with eosinophilic esophagitis have multiple proximal esophageal rings, long strictured segments, linear furrows in the mucosa, and eosinophilic abscesses that appear as white papules.
  • Symptoms
    • Often asymptomatic
    • Episodic dysphagia (often large pieces of meat causing complete obstruction).
    • Often spontaneously passes after a few minutes
  • Investigations
    • Barium swallow +/- endoscopy.
    • Endoscopy
      • Biospy ? eosinophilic esophagitis.
        • Dilation in patients with eosinophilic esophagitis is usually reserved for patients with rings who have not responded to medical therapy.
  • Management
    • Endoscopic
      • Dilation - Large caliber bougie (≥50 French) or balloon dilator (18 to 20 mm)

Oesophageal Web

  • Thin, membranous structures that partially or completely compromise lumen.
    • Only involve mucosa & part of the submucosa
  • Aetiology
    • Congenital
      • Mostly lower 2/3, failure of coalescence of oesophageal vacuoles which normally leads to complete luminal patency between 25-31 days of embryologic development
      • Most likely circumferential, thick & rough
    • Acquired
      • More common, anterior cervical oesophagus, post-cricoid, thin mucosal fold
      • Associated with:
        • Plummer-Vinson Syndrome
        • Dermatological conditions
          • Bullous pemphigoid, Epidermolysis bullosa, pemphigus vulgaris, psoriasis, Stevens-Johnson syndrome
  • Mostly asymptomatic. Occasional solid food dysphagia.
  • Investigations
    • Barium endoscopy 1st investigation
    • Endoscopy may miss it high in the cervical oesophagus
  • Management
    • Membranous disruption with endoscope or bougie or balloon dilation
    • Very rarely require surgical excision with mucosectomy
    • Slight increased risk of oesophageal cancer

Plummer-Vinson Syndrome

  • also known as Paterson-Brown-Kelly Syndrome
    1. Upper oesophageal web
    2. Dysphagia
    3. Iron deficiency anaemia
      • ± associated with glossitis & angular stomatitis / cheilosis
  • Risk of Oesophageal cancer or pharyngeal Squamous cell carcinoma
  • Rare; mostly females
  • Typically seen in
    • Edentulous, middle-aged, malnourished women with atrophic oral mucosa, glossitis, spoon-shaped fingernails & Fe deficiency anaemia

Other causes

  • Eosinophilic oesophagitis
  • Infection
  • Crohn’s
  • Neoplasia, post treatment strictures (radiotherapy, EMR etc)
    • Don’t dilate malignant stricture – just pass feeding tube and workup
      • Can stent later
  • Pill-injury
    • Potassium, iron, beta-blocker, doxycycline, tetracycline, NSAIDs, bisphosphonates, quinidine
    • Stick at 2nd constriction as there is a low peristaltic wave here
  • Caustic Ingestion

Clinical Presentation

  • Dysphagia
  • Cnce lumen <13mm, meat/bread impaction
  • Regurgitate food
  • Odynophagia
  • Weight loss
  • Recurrent respiratory symptoms
  • Difficulty breathing
  • Caustic injury

Investigations

  • Gastroscopy
  • CXR
    • Exclude pneumomediastinum/pneumothorax suggestive of perforation
  • Hoarseness/stridor (caustic injury) = intubation early +/- nebulized steroids.
  • Barium Swallow
    • To diagnose a Schatzki ring; place prone turned slightly to right; patient takes in a large breath as bolus reaches GEJ.
  • CTA for anomalous anatomy

Management

Chronic Strictures

  • Endoscopic Rx
    • Don’t bougie until see re-epithelialisation.
    • Bougie strictures even if asymptomatic – may need daily for weeks
    • Dilatation if less than 2 cm
      • Remove impacted food (don’t push through ring/obstruction – perforation risk).
      • Good for Schatzki ring, surgery not indicated unless dilatation fails or intractable reflux
    • Savary Gillard Dilation
      • Over guidewire, requires passage of endoscope beyond the stricture so wire not placed blind
      • Dilate up 3 x successive sizes, bring back for repeat if needed
      • Can assess force required for dilation
      • Must do post procedure endoscopy check
    • Ballon dilation “Through the scope dilatation”
      • Don’t need to pass the stricture, can see through the balloon,
      • Don’t need to pass pharynx repeatedly
    • Webs
      • Dilatation or piece-meal excision with biopsy forceps or laser lysis, bougie or balloon

Operative

  • Slings, rings – repaired
    • If pulmonary sling not repaired get LPA narrowing & tracheal stenosis
    • Need open sternotomy + cardio-pulmonary bypass – anatomic re-positioning
  • Webs – surgery for thick webs refractory to endoscopic Rx
    • Transcervical or transthoracic approach to oesophagus
    • Longitudinal myotomy & circumferential excision of web, circumferential re-approximation of mucosa with interrupted absorbable sutures followed by longitudinal closure of muscle
  • Anti-reflux procedures ± Collis gastroplasty (as oesophageal shortening often present) –
    • Creating a gastric tube & bringing it up into the thorax
  • Resection and reconstruction- delay for at least 6 months.
    • Stomach pull-up, versus jejunal loop interposition, versus long-segment colon interposition graft.
    • Need to resect oesophagus/stomach – 1000 x increased risk of cancer