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Treatment of Achalasia: Lessons Learned with Chagas' Disease

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Treatment of Achalasia: Lessons Learned with Chagas' Disease Diseases of the Esophagus (2008) 21, 461–467 DOI: 10.1111/j.1442-2050.2008.00811.x Original article Treatment of achalasia: lessons learned with Chagas’ disease F. A. M. Herbella,1 J. L. B. Aquino,2 S. Stefani-Nakano,3 E. L. A. Artifon,4 P. Sakai,4 E. Crema,5 N. A. Andreollo,6 L. R. Lopes,6 C. de Castro Pochini,7 P. R. Corsi,7 D. Gagliardi,7 J. C. Del Grande1 1Department of Surgery, Division of Esophagus and Stomach, Federal University of Sao Paulo, Sao Paulo; 2Department of Surgery, School of Medicine, Catholic University, Campinas; 3Santa Casa de Goiânia, Goiania; 4Department of Surgery, Gastrointestinal Endoscopy Unit, University of Sao Paulo Medical School, Sao Paulo; 5Department of Surgery, Universidade Federal do Triângulo Mineiro, Uberaba, MG; 6Department of Surgery, Division of Esophagus and Stomach and Gastrocentro, State University of Campinas, Campinas; 7Department of Surgery, Santa Casa de Sao Paulo Medical School, Sao Paulo, Brazil SUMMARY. Chagas’ disease (CD) is highly prevalent in South America. Brazilian surgeons and gastroenter- ologists gained valuable experience in the treatment of CD esophagopathy (chagasic achalasia) due to the high number of cases treated. The authors reviewed the lessons learned with the treatment of achalasia by different centers experienced in the treatment of Chagas’ disease. Preoperative evaluation, endoscopic treatment (forceful dilatation and botulinum toxin injection), Heller’s myotomy, esophagectomy, conservative techniques other than myotomy, and reoperations are discussed in the light of personal experiences and review of International and Brazilian literature. Aspects not frequently adopted by North American and European surgeons are emphasized. The review shows that nonadvanced achalasia is frequently treated by Heller’s myotomy. Endoscopic treatment is reserved to limited cases. Treatment for end-stage achalasia is not unanimous. Esophagectomy was a popular treatment in advanced disease; however, the morbidity/mortality associated to the procedure made some authors seek different alternatives, such as Heller’s myotomy and cardioplasties. Minimally invasive approach to esoph- ageal resection may change this concept, although few centers perform the procedure routinely. KEY WORDS: achalasia, Chagas’ disease, esophagus, surgery. INTRODUCTION national and Brazilian literature. Aspects not fre- quently adopted by North American and European Chagas’ disease (CD) (American trypanosomiasis) is surgeons are emphasized. a common disease in South America. Although the incidence of CD is declining,1 an impressive number of individuals still suffer from the disease. Chagas’ disease CD esophagopathy (CDE) leads to slow esoph- Carlos Chagas’ work is unique in the history of medi- ageal emptying due to nonrelaxation of the lower cine – it is the only instance in which a single investi- esophageal sphincter, similar to idiopathic (primary) gator has described the infection, agent, vector, 2 achalasia. manifestations, epidemiology and some of the hosts This article reflects the lessons learned by different of a pathogenic parasite. Only a year after Chagas’ Brazilian centers highly experienced in the treatment paper was published, a committee of Brazilian of CDE. Different treatment options are discussed in medical experts suggested the name CD for American the light of personal experiences and review of inter- trypanosomiasis.3 CD has a wide distribution in Central and South Address correspondence to: Dr Fernando A. M. Herbella, America. It is endemic in 21 countries, with 16–18 Division of Esophagus and Stomach, Federal University of Sao Paulo, Rua Napoleao de Barros, 715 2o. andar, Sao Paulo, million persons infected and 100 million people at Brazil 04024-002. Email: [email protected] risk. It is locally transmitted in Argentina, Belize, Author’s contribution: FAH: conception and design, writing of Bolivia, Brazil, Colombia, Costa Rica, Ecuador, El the final version of the manuscript. All authors: data collection, writing parts of the manuscript, approval of the final version of Salvador, French Guiana, Guatemala, Guyana, the manuscript. Honduras, Mexico, Nicaragua, Panama, Paraguay, © 2008 Copyright the Authors Journal compilation © 2008, Wiley Periodicals, Inc. and the International Society for Diseases of the Esophagus 461 462 Diseases of the Esophagus Peru, Suriname, and Venezuela. It is sometimes Manometrically, there are no significant differ- transmitted in the United States as well.4,5 ences between CDE and achalasia.2 In Brazil, esoph- The disease is caused by Trypanosoma cruzi, a flag- ageal manometry is not performed routinely other ellated protozoan which is transmitted to humans by than for research or academic purposes. Exceptions a blood-sucking insect which deposits its infective to the rule are incipient cases without esophageal feces on the skin at the time of biting, or directly by dilatation. Some authors; however, perform esoph- transfusion of infected blood, which is rare nowa- ageal manometry routinely in order to define end- days. Humans and a large number of species of stage achalasia based on the presence of simultaneous domestic and wild animals constitute the reservoir, waves with amplitudes lower than 20 mm Hg.8 and the vector insect infests poor housing and thatched roofs.4 Endoscopic treatment Commonly, there are no acute clinical manifesta- tions. In about one-third of infected cases, a chronic Dilatation form develops some 10–20 years later, causing irre- Endoscopic forceful balloon dilatation of the lower versible damage to the heart, esophagus and/or colon. esophageal sphincter was traditionally indicated as Injury to these organs is characterized by: (i) dilated the initial treatment for patients with absent or cardiomiopathy and conduction system abnormali- minimal dilatation of the esophagus. However, a shift ties, most frequently right bundlebranch block or left to surgical treatment in these patients can be noticed anterior fascicular block; (ii) achalasia-like esophag- in Brazil as well as in the United States.9 At the opathy with marked esophageal dilatation; and present time, dilatation is rarely used as a primary (iii) megacolon, particularly of the sigmoid segment, and definitive treatment. Currently, indications for usually complicated by fecal impaction or sigmoid endoscopic dilatation include: (i) primary treatment volvulus.4–6 The heart is the most commonly affected in patients unfit or unwilling for surgery; (ii) recur- organ (60%). The colon and the esophagus are affected rence of symptoms after myotomy; and (iii) preopera- in approximately 20% of the cases, with 60% of the tive as a mean to improve nutrition status before a patients developing concomitant cardiopathy.6 major operation.10 There is neither vaccine nor recommended drug Patients are kept on clear fluids for 3 days before available to prevent Chagas’ disease. Also specific the procedure. Cleansing of the esophagus with a treatment for the chronic phase of the disease is non- large tube may be necessary at the day of the test as existent. well. Correct placement of the catheter may be challeng- TREATMENT OF CHAGAS’ DISEASE ing in a massive dilated esophagus. At the time of ESOPHAGOPATHY IN BRAZIL endoscopy, the esophagogastric junction is identified with the aid of air inflation. The endoscope is then Preoperative evaluation advanced with counterclockwise rotation movements Careful preoperative clinical evaluation is essential in and its tip facing up, in an attempt to follow the patients with CDE. Most of the individuals are curvature of the esophagus bellow the diaphragm. undernourished because of late presentation to The stomach is accessed and a guide wire is left in medical care. A significant number of patients present place. The balloon is placed at the esophagogastric with subclinical pulmonary complications of the junction with the aid of the guide wire and fluoros- disease due to chronic aspiration and a major opera- copy. Interestingly, some groups perform dilatation tion may be anticipated. CD may affect, apart from at the endoscopy suite without the aid of fluoroscopy. the esophagus, the heart and the colon. For this In these centers, the balloon is positioned based on reason, different from idiopathic achalasia, a cardiac the distance from the nares calculated during endos- and colonic workup is also necessary. copy. The correct position of the balloon is assured Barium esophagram is performed in all patients with direct visualization with the endoscope and the due to the fact that therapy may be guided based on balloon insufflation is done under direct vision. These the presence and grade of the esophageal dilatation.7 groups report similar results compared to fluoros- Esophageal dilatation is a common feature of CDE, copy guidance. Esophageal perforation rate is also rendering the name megaesophagus, as it is known in comparable. Latin America. The esophagus is dilated in 70–100% Air insufflation is progressive. The balloon is of the patients with CDE, with massive dilatations inflated to 5 psi for 1 to 3 minutes and deflated. If the (more than 10 cm) in 10–40% of the cases.2 Timed- patient is able to tolerate the dilatation new cycles are barium esophagram is not popular in Brazilian initiated with 7 and 10 psi. If the patient is not able to centers. tolerate the procedure it is discontinued even if Upper digestive endoscopy is always performed to incomplete in order to diminish the risk of esophageal rule out malignancy or premalignant lesions of the perforation.
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