Esophageal Motility Disorders Flashcards
MC Categories of Esophageal Motility Disorders
- Achalasia
- Diffuse esophageal spasm
- Nutcracker esophagus
- Hypertensive LES
- Scleroderma
A common cause of esophageal dysmotility that must be ruled out before a diagnosis of altered esophageal motor function is made
GERD
Other less common diseases that may affect esophageal motility include
- Connective tissue disorders
- DM
- Dermatomyositus
- Amyloidosis
- Chronic ideopathic intenstial pseudo-obstruction
- Alcoholism
- Chaga’s disease
Definition of achalasia
Primary esophageal motility disorder charcterized by:
Aperistalsis of distal esophagus
Failure of complete LES relaxation
Early achalasia findings may include:
Disordered peristalsis with normal LES function
Pathophysiology of Achalasia
Degeneration of ganglion cells in esophageal myoenteric plexus
- Destruction of inhibitory neurons (contain NO and VIP) due to inflammatory process
- Initiating event may be viral, and inflammatory process autoimmune in origin
- Elevated antibody titers to herpes virus
- Predominant T-lymphocyte inflammatory infiltrates
- Autoantibodies to myoenteric plexus
Natural hisotry of achalasia
- Loss of esophageal proppulsion and lack of LES relaxation
- Stasis of food bolus until intraesophageal pressure great enough allow transit into stomach
- Esophageal dilation
- Stasis of food bolus until intraesophageal pressure great enough allow transit into stomach
MC presentation of achalasia
Dysphagia
- Progressive
- Liquids and solids
- Exacerbated by emotional stress and fast eating
- Regurgitation of undigested food and aspiration: MC in advanced phase of achalsia when esophagus is dilated
- Chest pain and indigesion common: often misleading to diagnosis of GERD
2 classic barium swallow findings for achalsia
Bird’s beak narrowing of GEJ
Air-fluid level in distal esophagus
Role of endoscopy in diagnostic evaluation of suspected achalsia
Rule out pseudoachalsia due to esophageal, gastric, or pancreatic cancer involving distal esophagus or esophageal diaphragmatic hernia
Gold standard diagnostic test for achalasia
Esophagel mannometry
- Aperistalsis
- Failure of LES relaxation
- LES hypertensive
- Resting esophageal pressure elevated and non-peristaltic
- Simultaneous contractions seen in reponse to swallow
- Esophagus very dilated: low amplitude
- Vigerous achalsia: high amplitude and duration simultanous contractions (rare)
Medical treatment options and success for achalasia
- Options: reduce LES tone
- anticholangerics
- nitrates
- Ca channel blockers
- Beta blockers
- PDE inhibitors
- Success: low efficacy with high rates of adverse effects
Success of endoscopic balloon diation for achalasia
50-90% with multiple sessions in experienced hands
- Risk of perforation: 2-5% per dilation
- Less effective in young patients
RCT results (endoscopic dilation vs surgery) for achalsia
Near complete relief of symptoms at 5-year follow-up:
- Endoscopic balloon dilation: 51%
- Surgery: 95%
Success of intersphincteric botulinum toxin injection for achalasia
60-70% (short lived)
- Requires repeated injections
- Tx may lead to GERD
Standard of Care treatment for achalasia
Heller myotomy with fundoplication
- Laparoscopic distal esophageal myotomy
- myotomy 6 cm with extension of at leat 1.5 cm onto stomach
- Partial fundoplication (Dor or Toupet)
- RCT demonstrated higher postop GERD without fundoplication
Surgical treatment options for end-stage achalsia or failure of Heller myotomy with fundoplication
Esophagectomy with reconstruction (gastric pull-up or colonic interposition)
Most frequent reason for performing esophagectomy in achalasia
Iatrogenic perforation
- Other indication: esophageal cancer or dysplasia
Achalasia a known risk factor for what
Esophageal cancer
- Endoscopic surveillence offered even after surgical intervention and resolution of symptoms
Diagnosis
Achalasis
- Aperistalsis in esophageal body
- Failure of relaxation of LES
Definition of Diffuse Esophageal Spasm (DES)
Rare esophageal motility disorder characterized by:
- non-peristaltic, simultaneous contractions of distal esophagus
- occur either spontaneously or in response to swallow
- intact LES function and relaxation
Pathophysiology of DES
Unknown
Hypotheses:
- dysfunction of inhibitoary nerves (similar to achalasia)
- early stage of myoenteric plexus denervation disease
% of cases of DES that will progress to achalsia
3-5%
MC presentation of DES
- Chest pain
- rest
- after swallow
- associated with hih-amplitude and long-duration esophageal contractions
- Dysphagia
- non-progressive (different from achalasia which is progressive)
- liquids and solids
- intermittent
UGI findings of DES
- Vary in appearance:
- normal
- Altered progression of bolus due to uncoordinated, simultaneous contractions
- “corkscrew appearance”
Associated esophageal condition that may occur with DES
Esophageal pulsion diverticulum
Manometric findings for DES
- More than 20% simultanous contractions (> 30 mmHg) in response to wet swallow
- Nl LES function
Non-surgical treatment options for DES
- Medical:
- Improve dysphagia:
- Ca channel blockers
- Nitrates
- PDE inhibitors
- Improve chest pain:
- low-dose TCA
- Improve dysphagia:
- Interventional:
- Botulinum toxin injection
- Balloon dilation
Surgical treatment for DES
Long myotomy of esophagus + partial fundoplication (GERD reduction)
Diagnosis
Diffuse Esophageal Spasm
- Simultaneous contractions of esophageal body
- Nl LES tone and relxation
Monometric characteristics of nutcracker esophagus occur in __% of pateint with non-cardiac chest pain
27-48% (i.e. 25-50%)
Patient population most commonly affected by Nutcracker Esophagus
Females
Psychiatric disorders
Proposed pathophysiology of Nutcracker Esophagus
- Imbalance between excitatory and inhibitory innervation
- Increased esophageal muscle thickness
- Increased muscle thickness may be responsible for high-amplitude peristalsis
MC presenting symptoms of Nutcracker Esophagus
- Chest pain (MC)
- Dysphagia
- Depression
- Anxiety
Manometric findings of Nutcracker Esophagus
- High-amplitude (>180 mmHg) contractions in distal esophagus
- Prolonged contractons (>6sec) common but not required for diagnosis
- Peristaltic waves normally propogated, repeative (>2), and persistent
- May have hypertensive LES (> 40 mmHg)
Diagnosis
Normal Esophageal Manometry
Diagnosis:
Nutcracker Esophagus
- High-amplitude (>180 mmHg) esophageal contraction
- Propogated
- Prolonged (> 6 sec)
- Repetative (>2 peaks)
- Increased LES tone (>40 mmHg)
Treatment options for Nutrcacker Esophagus
Similar to DES
- Anticholinergic and smooth-muscle relaxing agents (Nitrates, Ca channel blockers, PDE inhibitors)
- TCA (decreases chest pain)
- Botulinum toxin, esophageal dilation, surgery (long myotomy) in select cases
Definition of Hypertensive LES
Resting LES pressure > 45 mmHg (or > 95th percentile in nl subjects)
Other manometric findings associated with Hypertensive LES
- 50% of patients with peristaltic waves characteristic of nutcracker esophagus
- Some patients have incompete relaxation of LES and elevated intrabolus pressures suggesting outflow obstruction
MC symptoms of nutcracker esophagus
Dysphagia
Chest pain
Indigestion
Other esophageal anatomic disorder commonly associated with hypertensive LES
Large paraesophageal hernia with abnormal esophageal exposure to gastric acid
- 24-hr pH monitoring test used to identify these patinets
Treatment options for hypertensive LES
Medial managment
Distal esophageal myotomy with partial fundoplication (Heller)
Results in smooth muscle atrophy and subsequent replacement with scar
Scleroderma
Manometric findings of scleroderma
- Peristaltic dysfunction (normal peristalsis of upper 1/3 esophagus d/t striated muscle)
- Weakening of LES
Barium esophagram findings of scleroderma
- Slightly dilated esophagus
- Weak or absent peristalsis
- Free reflux often is demonstrated
Diagnosis
Achalasia
Bird’s beak (dilated esophagus)
Diagnosis
Nutcracker Esophagus
Corkscrew
Diagnosis
Normal esophagram
