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Achalasia

John Rosen, MD Miguel Saps, MD Ann & Robert H. Lurie Children’s Hospital of Chicago Reviewed by Diana Riera , MD of the Professional Education Committee. Achalasia. Cases. 14 year-old male with chest discomfort and progressive dysphagia to solids then liquids

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Achalasia

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  1. John Rosen, MD Miguel Saps, MD Ann & Robert H. Lurie Children’s Hospital of Chicago Reviewed by Diana Riera, MD of the Professional Education Committee Achalasia

  2. Cases • 14 year-old male with chest discomfort and progressive dysphagia to solids then liquids • 5 year-old female with regurgitation of undigested food, decreased oral intake, and weight loss Achalasia

  3. Presentation • Typically child or adult with progressive symptoms • Can present at any age • Mean age at presentation 8.8 years • Mean duration of symptoms prior to diagnosis 23 months • 1:10000 incidence, <5% present in infancy Achalasia

  4. Presentation • Differential Diagnosis • Esophageal obstruction (tumors in adults) • Surgical complications • Chagas disease • Eosinophilic esophagitis • Anorexia nervosa • Other motility disorders • Scleroderma • Rumination • Peptic stricture Achalasia

  5. Presentation • Associated disorders • Trisomy 21 • Allgrove/Triple A Syndrome • (achalasia, alacrima, ACTH deficiency) • Rozychi Syndrome • (achalasia, deafness, short stature, vitiligo, and muscle wasting) • Sarcoidosis • Hirschsprung disease • Pyloric stenosis • Hodgkin disease • Pierre-Robin sequence Achalasia

  6. Classification • Idiopathic esophageal motility disorder • Lack of esophageal peristalsis • Partial or incomplete LES relaxation • May have increased LES pressure • Absent or abnormal inhibitory innervation in distal esophageal myenteric plexus • Mixed inflammatory process precedes neural degeneration • Etiologic theories include: • Infection: similar clinical and manometric findings in Chagas disease • Autoimmune: nonspecific esophageal antimyenteric antibodies • Genetic: clustered in families and syndromic associations Achalasia

  7. DiagnosisImaging • Chest x-ray • widened mediastinum • lack of air in stomach • dilated esophagus with air-fluid levels • screening test only • Barium (contrast) esophagram • esophageal dilation • sigmoid shaped esophagus • tapering/beaking at gastroesophageal junction • sensitivity >90% • PPV up to 96% Achalasia

  8. DiagnosisImaging • Timed barium esophagram • height of column at 1 and 5 min is delayed • Both BE and timed BE correlate poorly with severity of symptoms • Esophageal transit scintigraphy • T99m sulfur colloid nuclide • PPV is 95% Achalasia

  9. DiagnosisOther • Esophagogastroduodenoscopy (EGD) • excludes other causes of dysphagia (ie. EoE) • diagnostic in 1/3 cases • stasis esophagitis • Esophageal manometry • gold standard • lack of esophageal peristalsis is hallmark • incomplete LES relaxation in 85-100% of children • nonspecific abnormality may progress to achalasia Achalasia

  10. DiagnosisManometry Normal High-resolution Manometry Achalasia

  11. DiagnosisHRM - Achalasia Achalasia Type Esophageal Body I (Classic) No contractions II Pan-esophageal pressurization III Spastic contraction Achalasia

  12. DiagnosisChicago Classification • Type I • classic achalasia • mean Integrated Relaxation Pressure (IRP) > upper limit of normal • 100% failed peristalsis • Type II • achalasia with esophageal compression • same as Type I plus • panesophageal pressurization with >20% of swallows • Type III • spastic achalasia • mean IRP > upper limit of normal • no normal peristalsis • preserved fragments of distal peristalsis or premature (spastic) contractions with >20% of swallows Achalasia

  13. Treatment • No cure • Pneumatic dilation • Esophageal myotomy • per-oral esophageal myotomy (POEM) • Less effective • calcium channel blockers • nitrates • sildenafil • botulinum toxin Achalasia

  14. TreatmentPneumatic Dilation • Controlled tear of lower esophageal sphincter (LES) • Postdilation pressure of LES predicts success • some advocate multiple dilations in (1-3 week) time span • Possible postdilation GER or esophagitis • Perforation risk 0.5-6% • severe chest pain, unexplained tachycardia, fever • XR: subcutaneous/mediastinal emphysema, left pleural effusion • diagnosis with water soluble contrast esophagram • treatment with TPN, PPI, IV antibiotics • surgery may be needed if mediastinal contamination or sepsis Achalasia

  15. TreatmentMyotomy • Heller is most common • 10-100% effective in children • 90% short term success rate • can be done laparoscopic • primary treatment in most children • Sometimes fundoplication is done at the same time • potential complications • recurrent or persistent dysphagia • GERD (up to 50% of children) • secondary to fundoplication, incomplete myotomy, or stricture • POEM (Per-Oral Endoscopic Myotomy) • 85% short term success rate Achalasia

  16. Postoperative Prognosis • Continued risk of aspiration, dysphagia, GERD • No change in esophageal motor function • Esophageal malignancy • occurs even after years symptom free • cause unknown • squamous cell carcinoma frequency of 0.02%-5% • occurs at mean age 48 years Achalasia

  17. Summary • Achalasia is an idiopathic esophageal motility disorder with lack of both peristalsis and LES relaxation • Diagnosis is by esophageal manometry • Myotomy is the primary treatment in children • Treatment may relieve symptoms, but does not address underlying disorder • Long-term risk of esophageal adenocarcinoma is unchanged regardless of treatment Achalasia

  18. References Boudewijn F. Kessing, , Albert J. Bredenoord, André J.P.M. Smout. Erroneous Diagnosis of Gastroesophageal Reflux Disease in Achalasia. ClinGastroenterol and Hepatol. 2011 Dec;9(12):1020-24. Bredenoord AJ, Fox M, Kahrilas PJ, Pandolfino JE, Schwizer W, Smout AJ & The International High Resolution Working Group. Chicago classification criteria of esophageal motility disorders defined in high resolution esophageal pressure topography. NeurogastroenterolMotil. 2012; 24:57-65. Chiu PW, Wu JC, Teoh AY, Chan Y, et al. Peroral endoscopic myotomy for treatment of achalasia: from bench to bedside (with video). GastrointestEndosc. Oct 2012. PAP. Gold B, Frem J. Other Neuromuscular Disorders; Achalasia. In: Kleinmen R, Goulet O, Mieli-Vergani G et al. eds. Walker’s Pediatric Gastrointestinal Disease. 5th ed. Sheldon, CT: People’s Medical Publishing House; 2008: 77-81. Nurko S., et al: Other motor disorders. In: Walker W., Durie P., Hamilton J., ed. Pediatric Gastrointestinal Disease, Pathophysiology, Diagnosis and Management, Vol. 1, 3rd ed.. St. Louis, MO: Mosby; 2000:317-350. Pandolfino JE, Kwiatek MA, Nealis T, Bulsiewicz W, Post J, Kahrilas PJ. Achalasia: A new clinically relevant classification by high-resolution manometry. Gastroenterol. 2008; 135(1):1526-33. Patti MG, Albanese CT, Holcomb GW 3rd, Molena D, Fisichella PM, Perretta S, Way LW. Laparoscopic Heller myotomy and Dor fundoplication for esophageal achalasia in children. J Pediatr Surg. 2001 Aug;36(8):1248-51. Sood M, Rudolph C. Achalasia and other motor disorders. In: Wyllie R, Hyams J. eds. Pediatric Gastrointestinal and Liver Disease. 4th ed. Online; Elsevier; 2012. Walzer N, Hirano I. Achalasia. GastroenterolClin N Amer. 2008; 37(4):807-25. Achalasia

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