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Congenital diseases of the oesophagus. Diverticula and strictures

1. FISTUAL AND ATRESIA

Trachea-oesophageal fistulas

  • Most common congenital oesophageal anomaly
  • Classification – into 5 types
    • Type C comprises 85% of cases
  • Associated with VACTERL anomalies and maternal hydraminos
  • Should be recognised in first 24hrs of birth

Clinical features

  • Regurgitates feeding
  • Continuous pouring of saliva from mouth
  • Cough, cyanosis

Investigations

  • Obstruction is revealed while passing NGT
  • Contrast study – reveals fistula
  • CXR, echo

Treatment

  • Feeding via gastrostomy
  • Right sided thoracotomy (opposite side to aortic arch
    • Fistula resected and lower end anastomosed to blind upper segment

Oesophageal atresia

  • Oesophagus doesn’t develop properly – congenital absence/closure of a normal body opening
  • Proximal and distal oesophagus don’t communicate
  • Clinical features – gastric distension, neonate’s inability to swallow

2. DIVERTICULA

  • Outpouchings of the wall that contains layers of the oesophagus
    • True (congenital) – involve all layers of gut
    • False (acquired) – involve only mucosa and submucosa

Pulsion diverticulum (push from inside)

  • False type – only involve mucosa and submucosa
  • Due to increased intra-luminal oesophageal pressue – due to various motility disorders
  • Pharyngeal pouch (  Zenker’s diverticulum ) – in 30-50 year olds
    • Located immediately above the upper oesophageal sphincter (UOS)
    • Clinical features – asymptomatic, dysphagia, regurgitation, halitosis
    • Investigations – barium swallow
    • Treatment – surgery for symptomatic pts
  • Epiphrenic pulsion
    • Occurs in lower oesophagus, due to obstruction in the distal oesophagus or due to in-coordinated lower oesophageal sphincter (LOS) relaxation
    • Site is within 10cm of gastro-oesophageal junction (GOJ)
    • Clinical features – dysphagia, regurgitation, cough, weight loss, chest pain
    • Investigations – barium swallow, CT chest, endoscopic US
    • Treatment – diverticuloplexy and oesophageal myotomy

Traction diverticulum (pull from outside)

  • Occurs in mid oesophagus or in parabronchial region
    • Due to mediastinal granulomatous disease – TB
  • True type – contains all layers, due to traction by the healing fibrosing mediastinal lymph nodes
  • Most common on the right hand side
  • Clinical features – dysphagia, chest pain, regurgitation
  • Investigations – CT, barium, manometry, endoscopy, blood test for TB
  • Treatment
    • Treat TB
    • If diverticulum is <2cm then observ
    • If >2cm then surgery (diverticuloplexy/myotomy)

3. STRICTURES

  • Narrowing of oesophagus
  • Stomach acid refluxes into oesophagus, causes oesophagitis, eventually leads to stricture
  • Cause of strictures – six Cs
    • Corrosive injuries, Carcinoma, Columnar lined oesophagus (Barrett’s), Capsules of tetracyclines, C vitamins, Chronic reflux due to GORD

Features of corrosive lesion

  • Corrosives are the most common cause of oesophageal strictures – e.g. ingestion of alkali or acid
  • Common in lower 1/3 of oesophagus

Acute phase

  • Clinical features
    • Severe pain, shock, laryngeal oedema
    • Mediastinits, septicaemia, haemorrhage, perforation
  • Treatment
    • Neutralise
      • Acid – milk, egg whites
      • Alkali – vinegar, citrus fruit
    • 1st degree burn
      • 48 hours observation
      • Regular follow up
    • 2nd + 3rd degree burn
      • Fluid therapy, antibiotics, nutrition, resuscitation, tracheostomy, resection if necrosed

Chronic phase

  • Clinical features
    • Dysphagia, stricture, severe malnutrition
    • Recurrent respiratory infection
    • Trachea-oesophageal fistula formation
    • Malignant changes
  • Treatment
    • Regular oesophageal dilation
    • Balloon dilators
    • Oesophageal bypass and resection
    • Colon transposition

Schatzki rings

  • Concentric symmetrical narrowing at the GOJ
  • Associated with reflux disease
  • Involve only mucosa and submucosa – not muscle
  • Accompanied with hiatus hernia
  • Clinical features – dysphagia to solid food comes on abruptly with complete obstruction (when ring <12mm)
  • Investigations – barium swallow test
  • Treatment – endoscopic dilation and treat associated reflux. Ring should not be excised
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