Editing Oesophageal motility disorders (section)

== '''Classification by aetiology:''' ==

* '''Primary     disorders:'''

=== '''Achalasia''' ===

*** Failure of LOS to relax       with swallowing, associated with lack of co-ordinated peristalsis in       distal oesophagus
*** '''Risk       factors'''
**** Usually diagnosed between        25-60yo
**** M:F equal
*** '''Aetiology'''
**** '''Primary'''
***** Unknown aetiology
***** Possibly autoimmune -         possibly triggered by a virus, possibly HSV-1
**** '''Secondary''' (or more accurately,        diseases that can produce manometry findings similar to primary        achalasia)
***** Chagas disease (infection         with protozoan parasite ''Trypanosoma cruzi'', common in Central/South         America)
***** Malignancy
***** Amyloidosis
***** Neurofibromatosis
***** Eosinophilic oesophagitis
***** MEN 2B
***** Juvenile Sjogren's         syndrome
***** Chronic idiopathic         intestinal pseudo-obstruction
***** Anderson-Fabry disease
*** '''Pathophysiology'''       (of primary       achalasia)
**** Degeneration of ganglion        cells in the myenteric plexus in the oesophageal wall. Leads to reduced        neuromuscular function of the oesophagus, possibly secondary to        destruction of nerves to LOS
**** Loss of inhibitory neurons        is especially important
**** Oesophageal dilation is a        result of chronic obstruction and compensatory remodelling. This is a        sign of more advanced disease
**** Subtle impaired upper        oesophageal sphincter relaxation is also often seen
**** Histologic examination        reveals decreased numbers of neurons, and those that remain are        surrounded by inflammation
**** Increased risk of SCC, but        not by very much, 3% over 13 years in one series
*** '''Presentation'''
**** Insidious onset
**** Classic triad - dysphagia        (91%), regurgitation (90%), weight loss
**** Also heartburn,        post-prandial choking and nocturnal coughing
**** Dysphagia often begins        with liquids and progresses to solids
**** Recurrent aspiration -        pneumonia, lung abscess, bronchiectasis
**** Severity assessed using        Eckardt score (dysphagia, regurgitation, retrosternal pain, weight loss        rated 0-3)
*** '''Natural       history'''
**** Late disease characterised        by very dilated oesophagus
*** '''Diagnosis'''
**** Manometry
***** Elevated integrated         relaxation pressure >=15mmHg and absence of normal peristalsis
***** Type I (classic): 100%         failed peristalsis as indicated by a distal contractile integral         <100mmHg/s/cm, and without pan-oesophageal pressurisation
***** Type II: 100% failed         peristalsis and pan-oesophageal pressurisation seen in >20% of         swallows
***** Type III (spastic): no         normal peristalsis and premature contractions with distal latency         <4.5s and DCI>450mmHg/s/cm seen in >20% of swallows
**** Barium swallow - bird's        beak, sphincter spasm with delayed emptying, dilatation of oesophageal        body, lack of peristaltic waves in the body and failure of relaxation        of LOS
**** Gastroscopy - rule out        pseudoachalasia - GOJ tumours, neuropathy, strictures. Note 'popping'        feeling as you pass through LOS.
***** Can use the 'Content,         Anatomy, Resistance and Stasis' (CARS) score - which I can't find         online in July 2024
****


'''Classification     based on area of achalasia''' ("Chicago classification" - v4.0 is latest     (2020))

** Cannot be applied to people      with previous foregut surgery
** Type I - minimal oesophageal      function, dilated oesophagus, bird's beak
***

* Type II
** Absence of peristalsis,      bird's beak
** Best response to treatment
**
* Type III
** Impaired LOS relaxation but      also uncoordinated spastic activity in distal 2/3 of oesophageal body -      corkscrew appearance on oesophagram. Swallow results in      lumen-obliterating spasms. Chest pain often prominent.
** Endoscopic myotomy preferred
**

* '''Management'''
** Goals: treatment is directed      at relieving the obstruction in LOS - doesn't address the decreased      motility in the oesophageal body - therefore all are palliative
** Outcomes are best in type II      achalasia; however, no treatment can fix the underlying issue
** '''Treatment      approach by achalasia subtype'''
*** Type I and II: myotomy       (surgical or POEM), or balloon dilation
*** Type III: POEM (since can       do a longer myotomy)
*** If not a surgical candidate       can do botox
*** If can't even do botox,       medical therapy
** '''Medical''' - note these therapies are      really only limited to patients who cannot have more invasive techniques
*** Nitrates
**** Isosorbide mononitrate,        5-10mg, 15mins prior to meals
*** CCBs
**** Nifedipine 10-30mg 30mins        prior to meals
*** Phosphodiesterase-5       inhibitors
**** Sildenafil
** '''Endoscopic'''
*** Botulinum toxin 100U Botox       A injected into four quadrants just above Z-line
**** Wears off after three        months
**** Response worsens within a        year
**** Limited use to patients        who are not candidates for pneumatic dilation or Heller myotomy
**** 89% remission at one year        with balloon dilatation, compared with 38% with botox
*** Pneumatic dilatation
**** Balloon device (30, 35 or        40mm) placed across LOS and inflated to 7-15psi until waist is fully        flattened, and held for 15-60 seconds
**** Graded approach is best
**** Look out for occult        perforation - barium swallow if worried
**** GORD happens 15-30% of        patients post-dilation - give PPI
**** Frequently requires        multiple treatments
*** Per-oral endoscopic myotomy
**** Novel technique
**** Equivalent short-term        outcomes to myotomy
**** Divide muscular layer of        the distal oesophagus, LOS and cardia. Good results in short-term, but        unclear if these will be durable.
***** POEM has also shown good         results for sigmoid oesophagus
** '''Surgical:'''
*** Modified Heller myotomy -       good, durable results
**** 5cm proximal to GOJ, 3cm        distal
**** EATI trial - pneumatic        dilatation and lap Heller myotomy had roughly equivalent results at two        years, although 25% of dilatation patients needed repeat treatments
**** Addition of a partial        antireflux procedure (Toupet/Dor) is controversial but probably        warranted
*** Oesophagectomy for       megaoesophagus, sigmoid oesophagus, failure of more than one myotomy, or       reflux stricture not amenable to dilatation
**** Can be a transhiatal        oesophagectomy in most patients, but total oesophagectomy with        trans-thoracic dissection may be warranted in the setting of        mega-oesophagus
** Surveillance
*** Regular gastroscopy       recommended to exclude carcinoma

=== '''Diffuse     oesophageal spasm''' ===

** Also known as distal      oesophageal spasm
** Epidemiology
*** Uncommon and poorly       understood
*** More common in women and       those with multiple medical issues
** Presentation
*** Symptoms of chest pain and       dysphagia, often related to eating, mimicking angina
**** Often pronounced during        times of heightened emotional stress
**** Regurgitation of        oesophageal contents and saliva
** Pathophysiology
*** Premature and rapidly       propagated contractions in the distal oesophagus
*** Muscular hypertrophy and       degeneration of the branches of the vagus
** Dx manometry - repetitive,      simultaneous, high amplitude contractions
**
** Medical treatment is      mainstay
*** Start with PPI therapy
*** If no improvement, add       peppermint oil before meals as a smooth muscle relaxant
**** ML says doesn't work, but        can try Fisherman's Friend
*** Then trial antispasmodic       (hyoscyamine) or nitrate
*** Patients with predominant       chest pain symptoms who still don't improve could trial TCA       (amitriptyline 10-25mg nocte, not PRN, for four weeks)
** Surgical: (if refractory to      medical therapy, trial a stepwise intervention approach)
*** Botox
*** Bougie dilatation up to 50       or 60Fr provides relief for severe dysphagia in 70-80% of cases
*** POEM/surgical myotomy
**** Indications for surgery -        incapacitating chest pain or dysphagia who have failed medical therapy.
***** Need to release the         entire length of the abnormal motility
***** Extend down as far as         LOS, but not generally onto the stomach
***** Generally do a Dor         fundoplication at the same time to protect against reflux
***** Reportedly improves         symptoms in 80%
***** POEM has been reported to         have a success rate of 93%

=== '''Hypercontractile     oesophagus/nutcracker oesophagus/jackhammer oesophagus''' ===

** Hypertensive peristalsis or      high-amplitude peristaltic contractions
** Also uncommon
** Seen in patients of both      genders and all ages
** Presentation: (overall      similar to diffuse oesophageal spasm)
*** Most painful motility       disorder
*** Chest pain, dysphagia
*** Regurgitation/reflux are       uncommon
** Diagnosis
*** Manometry - see below
*** Normal LOS with relaxation
***
** Treatment
*** Similar to diffuse       oesophageal spasm
*** Good early results from       POEM

=== '''Hypertensive     lower oesophageal sphincter''' ===

** Also known as      oesophago-gastric junction outflow obstruction
** Thought by some to be early      achalasia - the difference is that effective peristalsis is present here
** Diagnosis
*** Manometry
*** Barium swallow may show       narrowing at GOJ with delayed emptying
** Medical:
*** Not as useful
** Endoscopic:
*** Botox injections -       temporary
*** Balloon dilatation - can be       longer-term relief
*** Several studies supporting       use of POEM
** Surgical
*** Indicated for those with       severe symptoms who fail to respond to endoscopic treatments
*** Laparoscopic Heller myotomy
*** Patients with normal       oesophageal motility need a partial antireflux procedure too

=== '''Ineffective     oesophageal motility''' ===

** Contraction abnormality of      the distal oesophagus
** Usually associated with GORD      or scleroderma
** These manometric findings      can be seen in 15-20% of 'normal' people
** Could be secondary to      chronic inflammation to the oesophageal body
** Symptoms - reflux,      dysphagia, regurgitation
** Diagnosis
*** Manometry - >50% of       swallows deemed 'ineffective'
*** Barium swallow -       nonspecific abnormalities of oesophageal contraction
** Treatment
*** Treat GORD/scleroderma
*** Altered motility appears to       be irreversible once it develops
*** Low likelihood of a good       result from surgery





Chicago Classification algorithm:



[[Category:UGIS]]