Achalasia Mr Yuen Soon Laparoscopic Tutor Consultant Oesophagogastric and Laparoscopic Surgeon

Definition ach·a·la·sia (āk'ə-lā'zhə) n. The failure of a ring of muscle fibers, such as a sphincter of the esophagus, to relax. [New Latin : a-1 + Greek khalasis, relaxation (from khalān, to loosen).]

Definition Achalasia is primary a disorder of motility of the lower oesophageal or cardiac sphincter. The smooth muscle layer of the oesophagus has impaired peristalsis and failure of the sphincter to relax causes a functional stenosis or functional oesophageal stricture.

Who is this

Who described this?

Who is this?

First described by Sir Thomas Willis 1672 Described as Cardiospasm by Von Mikulicz 1881 Ernest Heller performed the first operation 1913 History

Term Achalasia cioned by Hurt and Rake 1929 First laparoscopic Hellers performe by Shimi in UK 1991 Botox introduced 1994

Clinical Features 1/ Equal sex distribution Occurs at all ages especially after seventh decade

Clinical Symptoms Dysphagia Regurgitation (80-90%) Chest Pain (17-63%) Heartburn/Cough/Recurrent Chest Infection/Weight loss

Dysphagia Inability to swallow –Non prgressive –Constant Due to –motility dysfunction –Cardiac spasm

Regurgitation Food refluxing from distal to proximal oesophagus –Usually stale food –Predisposes to Halitosis Chest infections Sometimes mistaken for heartburn

Chest pain Mechanism unclear –Oesophageal Distention –Oesophageal irritation –Tertiary contraction No correlation with manometry 84% resolved with Manometry Heterogenous cause

Pathophysiology Loss of nerve cells in the oesophagus Fibrosis and inflammation Hypertrophy and degeneration of oesophageal muscle Loss of Nitric Oxide deficiency Preservation of Acetyl Choline Nerves and other promoters of muscle tone Eosinophils

Huh?!? What does that all mean –Oesophageal Motility –Sphincter dysmotility

Viral Autoimmune Allergy But truly no one knows Aetiology

Barium Swallow Endoscopy and Biopsy Manometry Investigations

Barium Swallow Characteristic Findings –Aperistalsis of Distal Oesophagus –Bird Beaking –Dilatation or tortuousity

GI Motility online (May 2006) | doi: /gimo53 Figure 1 Esophagrams of a patient with early achalasia pre- and posttreatment.

GI Motility online (May 2006) | doi: /gimo29 Figure 1 a: Barium esophagram showing a dilated, tortuous esophagus and a ”bird's beak” appearance of the lower esophageal sphincter (LES).

Stages of Achalasia 2-3 cm is normal 4-5 cm is stage two and bird beak looking 5-7 cm is stage three 8+ cm is sigmoid or stage 4.

Endoscopy To ensure no other causes of symptoms Usual findings –Excess stale food in oesophagus –Candidiasis

Manometry Characteristic findings –Absence of peristalsis –Pressure maybe hypertonic (Vigourous Achalasia) –Pressure maybe hypotonic –May have distal barrier function (Non relaxing sphincter)

GI Motility online (May 2006) | doi: /gimo22 Figure 2 Esophageal manometric findings in achalasia.

GI Motility online (May 2006) | doi: /gimo22 Figure 3 Contour plot topographic analysis of esophageal motility in achalasia.

GI Motility online (May 2006) | doi: /gimo22 Figure 4 Esophageal manometric findings in vigorous achalasia.

GI Motility online (May 2006) | doi: /gimo22 Figure 5 Esophageal manometric findings in achalasia variant with preserved LES relaxation.

Differential Diagnosis Secondary Achalasia –Cancer –Infection –Allergy Other Oesophageal Dysmotilities –Diffuse Oesophageal Spasm –Presbyoesophagus –Scleroderma

Achalasia Allgrove's syndrome (AAA syndrome)10, 36 Hereditary cerebellar ataxia37 Familial achalasia38 Sjögren's syndrome39 Sarcoidosis40 Postvagotomy41 Autoimmune polyglandular syndrome type II11 Achalasia with generalized motility disorder Multiple endocrine neoplasia (MEN) IIb (Sipple's syndrome)12, 42 Neurofibromatosis (von Recklinghausen's Disease)13 Chagas' disease (Trypanosoma cruzi) Paraneoplastic syndrome (Anti-Hu antibody)17, 18 Parkinson's disease8 Amyloidosis43, 44 Eosinophilic gastroenteritis45, 46 Fabry's disease47 Down syndrome Hereditary cerebellar ataxia37 Achalasia with associated Hirschsprung's disease15 Hereditary hollow visceral myopathy16 Achalasia associated with cancer. (Cancer-associated achalasia may be due to local invasion of the esophageal myenteric plexus or as a part of a paraneoplastic syndrome.) Squamous cell carcinoma of the esophagus Adenocarcinoma of the esophagus Gastric adenocarcinoma Lung carcinoma Leiomyoma Lymphoma Breast adenocarcinoma Hepatocellular carcinoma Reticulum cell sarcoma Lymphangioma Metastatic renal cell carcinoma Mesothelioma Metastatic prostate carcinoma Pancreatic adenocarcinoma

Treatment Conservative Medical –Drugs –Botox –Dilatation Surgical –Hellers –Oesophagectomy

Conservative Dietetic Support Enteral forms of feeding Stent

Medical Drugs –Seldom long lasting –Seldom effective –Nitrates (GTN) –Calcium Channel Antagonist (Nifedipine) –Sildenafil (Viagra)

Medical Dilatation –60% success at a year and 24% at 5 years following single dilatation –Symptoms reoccur in 50% within 5 years –In general 60% have good results at 5 years with one or more dilatation

Medical Predictors of good outcome –Low residual pressure –Older patients Complications –Perforation 3-7% (0-21% Range) –Reflux 2% –Higher rate of complication if followed by myotomy

Medical Botox –High quality symptom relief 1 month % 6 month % –Duration of response upto 15 months –50% will need other forms of treatment within 2 years –Reduces Sphincteric pressure by 40%

Botox Increases operative complications Recommended only for –Elderly –Low pressure sphincter

Dilatation vs Botox AuthorAnnese 89 Vaezi 86 Mikaeli 36 * Ghoshal 87 Muehldorfer 88 BoTPDBoTPDBoTPDBoTPDBoTPD * These authors used 200 U of Dysport. NR: not reported; LESP: lower esophageal sphincter pressure. Modified from Zhao and Pasricha. 67 Response rate 1 month8/8 14/2015/2013/2018/206/88/109/1210/12 12 months 6/8NR7/2014/203/2010/193/86/8NR Reduction in LESP 1 month-49%-72%-6 %-66%-24%-26%-53%-62%-44%-51% 12 months NR Reduction in retention 1 month47%59%33.6%50.2%NR 12 months NR 13.4%54.6%NR

Surgery Laparoscopic Long myotomy 6-8cm above and 3 below Good long term results for dysphagia %

Surgery Reflux 17-28% to 6% if antireflux procedure added Oesophageal perforations 1-5% Pneumothorax 3%

Medical vs Surgical Dilation (n = 32)Surgery (n = 42) LESP, lower esophageal sphincter pressure. Source: Modified from Csendes et al. 34 Type of procedure Mosher dilation (12–15 psi for 10–20 seconds)Myotomy + antireflux (Dor) Clinical response65%95% LESP at 5 years (% baseline) 16 mm Hg (50%)10 mm Hg (25%) GE junction at late follow-up (% baseline)7.2 mm (270%)9 mm (321%) Esophageal diameter at late follow-up (% base line)29 mm (70%)26 mm (50%) Reflux8%28% Perforation5.60%— Deaths00 Need for surgery22%—

Surveillance Do we need it Rise in Squamous cancers of oesophagus –33-100x ie 3.4/1000 patient years vs 0.1/1000 patient years –If done then needs chromoendoscopy from 10 years after symptoms starts

Questions

Other Dysmotilities Motor disorderManometric abnormality Achalasia Absent, incomplete, or abnormally timed LES relaxation Absent peristalsis May have elevated resting LES and intraesophageal pressuresLES Diffuse esophageal spasm >20% of swallows result in simultaneous contractions May have multipeaked and/or prolonged contractions Amplitude of the contractions may be increased, normal, or decreased Hypertensive peristalsis (nutcracker esophagus) Peristaltic contractions of increased amplitude (>180 mmHg) and/or increased duration (>8 sec) Hypertensive LESLES Increased resting LES pressure (>40 mm Hg above intragastric pressure)LES Hypotensive peristaltic contractions (ineffective peristalsis) Decreased amplitude (<30 mmHg) peristaltic or nonperistaltic contractions in distal esophagus with 30% of wet swallows With or without hypotensive LESLES Hypotensive LES and increased frequency of TLESRLESTLESR LESLES pressure <10 mmHg TLESRs cannot be evaluated by usual manometric studies