Nutcracker oesophagus

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

Definition

• “Hypercontractile oesophagus” in Chicago classification
• Motility disorder in which peristaltic contractions are co-ordinated, but significantly elevated in amplitude
  • Distinct manometric abnormality defined by excessive peristaltic vigor, which may include excessive LES after-contraction, not associated with a mechanical obstruction

Incidence

• Most common primary oesophageal motility disorder. Seen in all ages, M=F

Aetiology

• Poorly understood but results in hypertrophied musculature that results in high amplitude contractions
• Primary:
  • Unknown
• Secondary:
  • Due to GORD

Clinical

• Chest pain – most common symptom – Most painful dysmotility disorder
• Dysphagia and odynophagia may be present
• Regurgitation and reflux uncommon

Pathology

• ? Involves abnormalities in neurotransmitters / mediators (e.g. NO) in the distal oesophagus

Investigations

• Ba swallow
  • Usually normal
• Manometry
  • Normal propagation of peristalsis, but waves in distal oesophagus have a very high amplitude & duration
  • Mean amplitude > 180mmHg, contraction may be > 400mg
  • Duration > 6 sec
  • LOS pressure normal with normal relaxation
• By definition:
  • Chest pain associated with peristaltic waves with amplitudes > 2SD above normal – may be higher than 400mmHg
• 24hr pH monitoring:
  • Useful to differentiate from dysmotility secondary to GORD

Management

• Medical therapy is primary treatment
  • Medications
    • 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).>)
  • Avoid triggers – caffeine, cold or hot foods
  • Dilation
    • Some temporary relief but no long-term benefit
  • If dysmotility is secondary to GORD
    • Rx of reflux
• Surgery
  • Not recommended

Prognosis / Natural Hx:

• Pulmonary complications
• An epiphrenic diverticulum may develop