Achalasia Management in 2018 - Excellence

Achalasia Management in 2018

Subhash Chandra MBBS CHI Health Clinic Gastroenterology

Assistant Professor Creighton University, School of Medicine

Aug. 25th 2018

Disclosures No relevant financial disclosures.

Esophageal Motility

Esophageal Motility Disorder

Impaired EGJ relaxation

• Achalasia type I, II & III

• EGJ outflow obstruction

Impaired coordination

• Distal esophageal spasm

Impaired contractility

• Scleroderma

• Ineffective esophageal manometry

Hyper-contractile esophagus

• Jackhammer

• Nutcracker

Chagas disease

Presenter

Presentation Notes

three dominant mechanisms: 1) transient LES relaxations (tLESRs), without anatomic abnormality, 2) LES hypotension, again without anatomic abnormality, or 3) anatomic distortion of the EGJ inclusive of (but not limited to) hiatus hernia. hypotensive LES and ineffective esophageal motility

Achalasia

• Described in 1674. • 10/100,000 in USA. • Failure to relax. • Functional loss of myenteric plexus ganglion cells in the distal

esophagus and lower esophageal sphincter. • Autoimmune/genetic/infection. • Loss of nitric oxide and vasoactive intestinal peptide

neurotransmitters • Unopposed cholinergic stimulation leads to impaired LES

relaxation, hypercontractility of the distal esophagus, and rapidly propagated contractions in the distal esophagus.

• Progressive disease. • EGJOO < type III < type II < type I

J Clin Gastroenterol. 2010 Jul;44(6):407-10 Dis Esophagus. 2012 Apr;25(3):209-13

Presenter

Presentation Notes

indolent viral infection (HSV1, measles, HPV) in conjunction with a genetically susceptible host more likely to have concomitant autoimmune diseases than the general population inflammatory reaction is associated with a T-cell lymphocyte infiltrate that leads to a slow destruction of ganglion cells. GWAS Studies are not available yet.

Clinical Presentation

• Esophageal Symptoms

• Dysphagia (90%)

• Heartburn (75%)

• Regurgitation or vomiting (45%)

• Noncardiac chest pain (20%)

• Epigastric pain (15%)

• Odynophagia (<5%)

• Extra-esophageal symptoms

• Cough or asthma (20%-40%)

• Chronic aspiration (20%-30%)

• Hoarseness or sore throat (33%)

• Unintentional weight loss (10%)

Diagnosis Rule out mechanical causes

Diagnostic tests:

1. Esophagogram.

2. EGD.

3. High resolution esophageal manometry (gold standard).

4. Functional Lumen Imaging Probe.

Esophagogram

Am J Gastroenterol. 2000 Oct;95(10):2720-30

Presenter

Presentation Notes

more accurate in identifying achalasia and segregating the disorder from other forms of severe motor dysfunction

JAMA. 2015;313(18):1841-1852.

Presenter

Presentation Notes

Distal Contractile Integral

Scleroderma

Functional Lumen Imaging Probe

Presenter

Presentation Notes

EGJ-DI have a stronger association with symptoms and esophageal retention than manometric measures of LES pressures. FLIP may better characterize EGJ function because the degree of luminal opening is conceptually a more important determinant of bolus flow than LES relaxation Intraoperative use of FLIP during laparoscopic Heller myotomy or per-oral endoscopic myotomy Lower EGJ-CSA at a 30-mL fill volume of an 8-cmFLIP in those with a poor symptomatic outcome (n. 13) than those with a good outcome (n. 50) final intraoperative EGJ-DI (at 40-mL distention volume with an 8-cm FLIP) of 4.5–8.5 mm2/mm Hg was less likely to have dysphagia or GERD symptoms at >6 months follow-up after POEM (n . 21) or laparoscopic Heller myotomy (n . 11). Feasibility and clinical effectiveness of using the Eso-FLIP (Crospon) achalasia hydraulic dilation balloon

Normal Achalasia

Gastroenterology. 2012 Aug;143(2):328-35

Treatment Temporary 1. Medications

2. Botulinum toxin injection

Definitive 1. Pneumatic dilation

2. Heller’s myotomy

3. Peroral endoscopic myotomy

4. Hydraulic dilation

Medical Therapy • Calcium channel blockers

• Nitrates

• 5′-Phosphodiesterase inhibitors

• Limiting adverse effects

• Not good as long term treatment option

• Variable absorption

• Reserved for poor candidates for definitive tx.

Botulinum Toxin • Block acetylcholine release

• Injection into the lower esophageal sphincter

• 2/3rd respond

• Effect lasts about 6 month

• Almost all relapse

• Cause fibrosis, making myotomy difficult with repeated injection

• Primarily reserved for patients who are not candidates for definitive therapy

Pneumatic Dilation

• 62%-90% respond, better with graded dilation, comparable to LHM

• 1-4% perforation

• 1/3rd 5 year recurrence Am J Gastroenterol. 2012;107(12):1817-25

N Engl J Med. 2011 May 12;364(19):1807 Am J Gastroenterol. 1993 Jan;88(1):34-8

Clin Gastroenterol Hepatol. 2006 May;4(5):580-7

Heller’s Myotomy • Surgical incision of the circular muscles.

• Anterior approach, incising about 7 cm, 5 cm of distal esophagus and 2 cm into gastric cardia.

• Laparoscopic as good as open for clinical response.

• Superior to single pneumatic dilation in clinical efficacy.

• Low morbidity.

• Prior endoscopic therapies increases risk of complications and decrease clinical response rate.

• Combined with partial fundoplication, decrease reflux (48% vs 9%).

Ann Surg. 2004;240(3):405-412

Per-Oral Endoscopic Myotomy

Per-Oral Endoscopic Myotomy • Over 90% response, including type III

Compared to Heller’s myotomy:

• Comparable to better clinical response, likely from longer myotomy.

• Similar adverse events, operative time but a reduced length of hospital stay.

• Over 2/3rd with previous Heller’s respond.

• Second POEM is feasible.

• Higher acid reflux, up to 40% at 2-5 years.

Dis Esophagus. 2016 Oct;29(7):807-819 Surg Endosc. 2017 Jun 29.

Rev Esp Enferm Dig. 2017 Aug;109(8):578-586

Intraoperative FLIP • EGJ cross-sectional area, mm2

Yes No P value

Clinical response 89 (78–107) 72 (49-80) 0.01

GERD 100 (91-104) 79 (57-94) 0.02

Surg Endosc (2016) 30:2886–2894

An ideal range for final EGJ distensibility: 4.5 – 8.5 mm2/mmHg.

Surg Endosc. 2015 Mar; 29(3): 522–528

Surg Endosc. 2015 Mar;29(3):522-8

Normal

Achalasia

Good response

Incomplete response

Gastroenterology. 2012 Aug;143(2):328-35

Presenter

Presentation Notes

FLIP may better characterize EGJ function because the degree of luminal opening is conceptually a more important determinant of bolus flow than LES relaxation Intraoperative use of FLIP during laparoscopic Heller myotomy or per-oral endoscopic myotomy Lower EGJ-CSA at a 30-mL fill volume of an 8-cmFLIP in those with a poor symptomatic outcome (n. 13) than those with a good outcome (n. 50) final intraoperative EGJ-DI (at 40-mL distention volume with an 8-cm FLIP) of 4.5–8.5 mm2/mm Hg was less likely to have dysphagia or GERD symptoms at >6 months follow-up after POEM (n . 21) or laparoscopic Heller myotomy (n . 11). Response: EGJ cross-sectional area (mm2) 89.0 (78.5–106.7) 72.4 (48.8–80.0) 0.0 GERD: EGJ cross-sectional area (mm2) 99.5 (91.2–103.7) 79.3 (57.1–94.2) 0.02

Modality Effectiveness Durability Procedural issues

Medication <50% (minimal clinical) NA • NA

Botox 66% 6 mnts • 30 min procedure • Minimally invasive

Pneumatic Dilation Up to 90% 2-5 yrs

• 30-min procedure • 1-3 procedures • Fluoroscopy • 1-4% perforation

LHM 88-95% 5-10 yrs • OR with GA • 90 min procedure • 1-2 d hospital stay

POEM 90-95% ?

• OR/endoscopy suit/GA • 90 min procedure • 1-2 d hospital stay • GERD

Documents

Achalasia Management in 2018 - Excellence