Distal oesophageal spasm

Section: UGI Sub-section: Oesophagus Curriculum: Curriculum, page 95

Definition

• Poorly understood hypermotility disorder
• Resulting in repetitive, simultaneous high amplitude contractions of oesophageal body
  • DES describes a specific abnormal esophageal motor pattern characterized by spastic or premature contractions in the distal esophagus

Incidence

• Difficult to determine, probably 5 x rarer than achalasia.
• F>M

Aetiology

• Poorly understood
• Motor abnormality in lower 2/3 oesophagus
• Muscular hypertrophy and degeneration of vagal branches
• Get repetitive, simultaneous, high amplitude contractions

Clinical

• Squeezing chest pain – may radiate to jaw/arm (can mimic angina)
• Dysphagia
• Regurgitation common, acid reflux isn’t
• Triggered by stress, exercise, associated with other GI complaints

Pathology

• ? Can be thought of being part of a spectrum to achalasia
• Peristalsis is progressively lost

Investigations

• Barium swallow abnormal in 70%
  • 30% have classic “corkscrew oesophagus”
    • Segments of spasm & irregular (tertiary) peristalsis
  • Epiphrenic (or mid-oesophageal) diverticulum is sometimes present (Oesophageal Diverticulum)
  • Can get bird beak similar to achalasia
• Oesophageal Manometry
  • Abnormal peristalsis with simultaneous high amplitude contractions (>10% & <100%) and long contractions
    • High amplitude, non-peristaltic
  • Diffuse spasm C.F. segmental (shorter segment)
  • Abnormalities usually confined to distal 2/3
  • LOS may be normal or similar to that seen in achalasia
  • NB: Findings intermittent so manometry may miss diagnosis
• 24hr pH monitoring:
  • Useful to differentiate from dysmotility secondary to GORD
• Endoscopy
  • to rule out luminal lesions

Management

• Mainstay of management non-surgical
• Evaluate for psychiatric conditions – anxiety/depression/PTSD
  • Reassure that nature of the pain is oesophageal and not cardiac
• If dysphagia, identify trigger food/drink and eliminate from diet
• If reflux component: PPIs
• Medical therapy effective for some pts
  • Options include
    • antispasmodic agent (eg, hyoscyamine 0.25 mg three times daily as needed)
    • nitrate (eg, isosorbide dinitrate 5 to 10 mg twice to three times daily as needed)
    • calcium channel blocker (eg, nifedipine, extended release, 30 mg daily),
    • phosphodiesterase inhibitor (sildenafil 25 to 50 mg daily).
• Endoscopic tx:
  • Pneumatic dilatation to 50/60F → relieves dysphagia in 70-80%
  • Botox → some success but often not prolonged
• Surgical treatment - try to avoid!
  • Last resort for incapacitating chest pain or severe dysphagia that has failed to respond
    • Or those with pulsion diverticulum

1. Long oesophagomyotomy

• Use manometry to guide length, do laparoscopic abdominal or left thoracotomy, down onto LOS

2. Partial anterior fundoplication (Dor)

• As phreno-oesophageal ligament divided, provides reflux protection
• Results variable but can improve symptoms in 80%

Prognosis

• Progression to achalasia (complete loss of peristalsis) has been documented
• Respiratory complications may occur
• A mid-oesophageal or epiphrenic diverticulum may develop
• Secondary to the motor disorder