Acalásia idiopática com resposta duradoura ao tratamento farmacológico: relato de caso / Idiopathic acalasia with term response to medical treatment: case report

A acalásia é caracterizada pela aperistalse do corpo esofágico e pelo relaxamento incompleto ou ausente do esfíncter esofagiano inferior. De acordo com sua etiologia, pode ser classificada em chagásica ou idiopática. O tratamento pode ser farmacológico, endoscópico ou cirúrgico. O tratamento farmacológico é considerado pouco eficaz, pois seus efeitos não são permanentes, além de causar efeitos colaterais pronunciados em muitos casos, como cefaleia e hipotensão. As medicações de escolha são os bloqueadores dos canais de cálcio e os nitratos. Os autores relatam o caso de uma paciente com diagnóstico de acalásia idiopática cujo tratamento farmacológico tem sido eficaz ao longo de cinco anos. Tal modalidade foi instituída considerando a cronicidade da doença, a idade avançada da paciente e a presença de comorbidades.

Achalasia is characterized by esophageal body aperistalsis and incomplete or absent relaxation of the lower esophageal sphincter. According to its ethiology, it is classified in chagasic or idiopathic. The treatment options are pharmacological, endoscopical or surgery. Pharmacological treatment has low effectiveness, because clinical results are not permanent and can cause pronounced side effects, such as headache and hypotension. The drugs of choice are calcium channel blockers and nitrates. The authors report a case of a patient diagnosed with idiopathic achalasia whose pharmacological treatment has been effective for five years long. Such choice was made considering the chronic aspects of the disease, patients advanced age and comorbidities presented.

Acalasia, experiencia de 6 años. Hospital "Dr. Rafael Ángel Calderón Guardia" (2001-2007) / Achalasia, a 6 Years Study at the Calderon Guardia Hospital

Objetivos: Caracterizar la población de pacientes vistos con esta entidad en el Hospital "Dr. Rafael Angel Calderón Guardia" durante los últimos 6 años y determinar su presentación clínica, método(s) de diagnóstico utilizado (s), tratamiento brindado y evolución durante el primer año postratamiento. Materiales y métodos: Se analizaron los expedientes clínicos de hospitalización de los pacientes con acalasia atendidos desde enero de 2001 hasta enero de 2007; luego se revisaron las notas de evolución de la consulta externa de Gastroenterología durante el año posterior a la terapia brindada. Resultados: Durante el periodo se analizaron 30 pacientes en total. Hubo una discreta predominancia del género masculino y la edad promedio en el momento del diagnóstico fue de 50,37 años. El 100% de los pacientes presentó disfagia de larga data y los síntomas asociados más frecuentes fueron la pérdida de peso y el dolor torácico. Los métodos diagnósticos más utilizados fueron la manometría esofágica, la endoscopía y el esofagograma. El tratamiento que más se empleó fue la dilatación neumática seguida de la cirugía. El 50% de los pacientes reinició o persistió con disfagia durante el año siguiente a su tratamiento. La incidencia de complicaciones fue baja y no hubo perforación esofágica. Conclusiones: Las características generales y la presentación clínica de los pacientes coincidieron con lo descrito en la bibliografía. El tratamiento que más se brindó fue la dilatación neumática. La mitad de los pacientes presentaron o continuaron con síntomas postratamiento.

Objectives: To determine the general characteristics of patients with diagnosis of achalasia seen during the last 6 years at the Dr. Rafael Angel Calderon Guardia Hospital, their clinical presentation, diagnostic methods utilized, treatments given and the presence of disphagia within the following year after therapy. Methods: We analized the clinical records of patients with achalasia seen from january 2001 to january 2007. We also reviewed out patient clinic notes, looking for the persistence or recurrence of disphagia during the first year after therapy. Results: 30 patients were found and included in the study. There was a slight male gender predominance, and an average age of 50,37 years. All the patients had long standing dysphagia, the other most frequent symptoms were weight loss (43,33%) and chest pain (13,33%). The more commonly used diagnostic methods were esophageal manometry, endoscopy and barium esophagogram. Pneumatic dilation was the most frequently utilized treatment (46,67%) followed by surgery (26,67%). Half the patients recurred or continue having dysphagia during the year following treatment. The rate of complications was low and there were no esophageal perforations or mediastinitis. Conclusions: The general characteristics and clinical presentation of the patients agreed with those mentioned in the literature. Esophagic manometry was the most used diagnostic test and esophageal dilation was the preferred treatment. The rate of dysphagia within a year posttreatment was high.

Effect of botulinum toxin injection for achalasia in Thai patients.

BACKGROUND: Achalasia is a disorder of the esophagus. The lower esophageal sphincter fails to relax and increases the loss of body peristalsis. It is an uncommon disease worldwide. Data regarding its treatment are derived mostly from North America and European countries. Few data regarding this treatment were available in Asia and no data about using botulinum toxin injection for this disease was available in Thailand. OBJECTIVE: To evaluate the efficacy of botulinum toxin in achalasia in Thai patients. MATERIAL AND METHOD: Eleven achalasia adult Thai patients with a mean age of 56.5 +/- 16.9 were studied. There are nine females and two males. The duration of symptom before treatment was 27.5 +/- 34.5 months. All patients receiving botulinum toxin injection at Siriraj hospital between 2001 and 2006 were retrospectively reviewed. Pretreatment of baseline lower esophageal sphincter, symptom score and body weight were compared. Time to second botulinum toxin injection or the need to receive treatment for recurrence was recorded to evaluate the time of recurrence. Adverse events from this procedure were collected. RESULTS: Eleven patients were involved in this study. One patient that received 40 units of botulinum toxin showed no response after a six months follow up. The other ten patients received botulinum toxin 80 units for each session and were enrolled in this study. All ten patients demonstrated good response to the first botulinum toxin injection and subsequent injections. Four patients received only one session of botulinum toxin injection during study period. Meanwhile, five patients received two sessions and only one patient required four sessions. Symptom score of all ten patients improved significantly compared with pretreatment score (7.3 +/- 1.3 for pretreatment and 0.4 +/- 0.5, 0.9 +/- 0.7 and 1.6 +/- 1.3 after 2 weeks, 3 months and 6 months, respectively). Body weight increased significantly when compared with pretreatment (47.7 +/- 6.5 Kg for pretreatment and 49.2 +/- 5.8, 50.5 +/- 6.4, and 50.7 +/- 5.8 Kg after 2 weeks, 3 months, and 6 months, respectively). Previous treatments prior botulinum toxin injection do not seem to influence the effect of this treatment. Mean time of recurrence is 444 +/- 132 days (270-718 days). Minor adverse events such as chest pain and reflux symptoms were seen in this therapy. CONCLUSION: Botulinum toxin injection in Thai achalasia patients is an effective, simple, and safe treatment. These results showed the similar outcomes as in Caucasian patients.

Acalasia. Estudio de 27 casos en el Hospital Calderón Guardia / Acalasia. Study of 27 cases in the Hospital Calerón Guardia

Se estudiaron en forma retrospectiva los expedientes clínicos de 27 pacientes diagnosticados con acalasia en el Hospital Dr. R. A. Calderón Guardia (HCG) vistos durante el período de 11 años comprendido entre 1991 y 2001, encontrando una tasa de 0.3 por 100.000 habitantes la cual es menor que la informada en la literatura. Esto posiblemente debido a que la ausencia en nuestro medio de manometría esofágica dificulta el diagnóstico de pacientes en estadio temprano o con enfermedad leve o moderada. La presentación clínica fue muy similar a la reportada por otros autores con una relación hombre mujer de 1.7 a 1 y edad promedio de 42 años. Los síntomas con un promedio de evolución de 7 años, fueron el más frecuente, la disfagia que se presentó en todos los casos seguida de vómito, dolor toráciso, pérdida de peso y datos de reflujo gastroesofágico. La esofagoscopía y la esofagografía mostraron hallazgos de enfermedad avanzada como dilatación esofágica importante, retención de restros alimentarios y cardias con espasmo que se superó fácilmente al presionar con el endoscopio. El tratamiento que se administró fue solamente médico (Nifedipina o Isorbide) a 6 pacietes, dilataciones y tratamiento médico a 7 y quirúrgico (esofagomiotomía) a 14, a tres de estos se les agregó un procedimiento anti-reflujo. La evolución se valoró con base en datos clínicos, encontrando de todos los pacientes contratamiento médico y dilataciones continúan sintomáticos y con tratamiento. De los operados hay uno que ha requerido dilataciones 5 y 9 años después de la intervención, dos que no tuvieron procedimiento anti-reflujo, están en tratamiento por este problema y el resto se encuentran asintomáticos sin que se presentaran complicaciones quirúrgicas. Palabras claves: Acalasia, esófago enfermedades, esofagocardiomiotomía.

Inyección intraesfinteriana de toxina botulínica en pacientes con acalasia esofágica / Intrasphincteric injection of botulinum toxin in patients with achalasia

Eight patients received 80 units of BoTx. Assessment of response was based on changes in the symptom scores (0-9) and esophageal manometric studies. Results: Six out of 8 patients (75 percent) had sustained clinical improvement after therapy. This effect was maintained for a mean time of 17.8 months. The symptom score decreased from a mean of 6.7 to 0.5 (p < 0.01) and after treatment, LES pressure decreased from 63 to 25.5 mm Hg (p = 0.07).l There were no serious adverse effects. Five of the six responders have relapsed. Two of these patients received a second BoTx infection with satisfactory results, two went to surgery and one refused other type of therapy and died of pneumonia. Conclusions: Intrasphincteric BoTx injection is a simple, safe and effective method of treatment in patients with achalasia, with a duration of response averaging 1.5 years. Its use may be suggested in some patients with high surgical risk and those who refuse a more invastive therapy. It is also useful in malnourished patients to attain an adequate nutritional status before surgery

La toxina botulínica es efectiva en el tratamiento a corto plazo de acalasia esofagica. Resultados preliminares de un estudio randomizado / The botulinum toxin is effective in the short-term treatment of esophageal achalasia. Preliminary results of a randomized trial

Botulinum toxin (BoTox) is a potent inhibitor of the release of acetylcholine from terminal nerves and has been used succesfully in spastic disorders of skeletal muscle. Its used for the treatment of disorders of gastrointestinal smooth muscle has recently been explored. In this study we evaluated the efficacy of transendoscopic injection of BoTox in 13 symptomatic patients with achalasia G II (Siewert classification) without previous treatment of an angoing randomized-controlled trial. Patients were blindy randomized to administrate: a) 8OU of BoTox were injected in four quadrants (1 ml in each quadrant-20 U/ml) (n=8) normal saline solution as placebo injected in the same way (n=5). Patientes who did not respond were retreated in an open design with the same schedule of BoTox. BoTox or placebe were injected directly into the lower esophageal sphincter (LES), located by manometric and endoscopic procedures, via sclerotherapy injector. Response to treatment was assessed by changes in symptoms score, weight, LES pressure, barium esophagograms and endoscopy. All determinations were repeated at basal and after 7-30-60 and 90 days of treatment. Post treatment response was considered positive if at one month, 3 of 4 parameters were improved. No evidence of response to BoTox were assessed in 3 patients. At 90 days, 10 patients remain well and data are as follows: (X + SD) symptoms score: (Pre: 3.23 + 0.44) (Post: 1.31 + 0.95); LES pressure (mmHg) (Pre: 53,15 + 66.31 + 7.49); per cent reduction of esophageal diameter 55 per cent (p< 0.0001) (pair T-test). Relaxation of LES did not change after treatment. There were no side effects related to BoTox injection. Conclusions: Endoscopic intrasphincteric BoTox injection is safe, simple and effective in the short term treatment for achalasia. Further studies are necessary for evaluation of long term effects.

Comparison of the effects of sublingual isosorbide dinitrate and cardiomyotomy on esophageal emptying in patients with chagasic megaesophagus

Os efeitos da cardiomiotomia cirúrgica e do dinitrato de isossorbitol sobre o esvaziamento esofagiano em 18 portadores de megaesôfago chagásico. O esvaziamento esofagiano foi medido três vezes em cada paciente: duas vezes antes e uma vez após a realizaçäo da cardiomiotomia; 5mg de dinitrato de isossorbitol foi administrado por via sublingual 5 minutos antes de um dos estudos pré-operatórios. Os resultados revelaram que o dinitrato de isossorbitol e a cardiomiotomia aceleram o esvaziamento esofagiano com intensidade semelhante, embora a cardiomiotomia determine completo esvaziamento do esôfago mais freqüentemente do que o dinitato de isossorbitol

Use of isosorbide dinitrate for the symptomatic treatment of patients with Chagas' disease achalasia: a double-blind, crossover trial

1. A randomized, double-blind, placebo-controlled trial was carried out to determine the efficacy of isosorbide dinitrate (ISD) on diasphagia in patients with Chagasic achalasia. 2. Twenty-three patients with Chagas' disease and dysphagia entered the study ans 20 (87%) complelted the two 7-day treatment periods. Subjects were given either 5 mg ISD (12 patients) or placebo (11 patients) by the sublingual route for the first 7 days. On the 8 th day, patients crossed over and began another 7-days period during which they received the opposite, identical-appearing tablets. 3. Scores attributed by uniformed investigators for the frequency and severity of dysphagia were signidicantly lower (P,0.05) following ISD treatment than after the placebo period or for the pretreatment condition. A significantly higher degree of improvement of dysphagia was experienced by the patients during ISD treatment than during the placebo period. Fourteen patients experienced meal-related headaches during ISD, but not placebo treatment. The extent of improvement in general well-being due to ISD was the same when the drug given in the first or second test period. 4. Our results indicate that ISD, 5 mg by the sublingual route, is effective in alleviating dysphagia in patients with Chagasic achalasia but its usefulness is limited by the high rate of headache as a side effect

O dinitrato de isossorbitol no esvaziamento esofagiano do megaesôfago chagásico: uma proposta terapêutica? / Isosorbide dinitrate on esophageal emptying of chagasic megaesophagus: a therapeutic approach? (editorial)

O dinitrato de isossorbitol no esvaziamento esofagiano do megaesôfago chagásico: uma proposta terapêutica?