Acute Upper-Airway Obstruction in Achalasia of the Esophagus

We present a patient with a rare complication of achalasia. The principal complaint was a severe acute respiratory distress due to tracheal compression by an achalasic esophagus relieved by nasoesophageal tube aspiration.     

Barrett''s Esophagus in a Patient with Achalasia

Barrett's esophagus has been reported in patients with achalasia who have undergone esophagomyotomy. The condition was thought to be acquired from gastroesophageal reflux secondary to the iatrogenically produced incompetent sphincter. We present the case of a patient with Barrett's esophagus and achalasia without any previous surgical intervention.     

Achalasia of the Esophagus in Association with Renal Cell Carcinoma

A case of secondary achalasia due to renal cell carcinoma is presented. The patient presented with typical clinical, radiologic, and manometric features of achalasia, and was found to have a renal cell carcinoma with metastases to lymph nodes in the region of the cardioesophageal junction. Direct esophageal involvement could not be demonstrated, however. Complete symptomatic remission was obtained with a single hydrostatic balloon dilatation of the cardioesophageal junction and was maintained until the patient's death 7 months later, perhaps as a result of regression of paraesophageal nodal metastases following radiotherapy, immunotherapy, and chemotherapy.     

Motility Studies in Fifty Patients with Achalasia of the Esophagus

Esophageal motility tests with constantly perfused polyethylene catheters were done in 50 patients with achalasia of the esophagus using 30 asymptomatic adults as control. The mean gastroesophageal sphincter pressure was 19.0 ± 1.3 mm. Hg. (mean ± SE) which was significantly higher than the control group (P < 0.001). The intraesophageal resting pressure was significantly higher than the mean fundic pressure and no correlation among resting gastroesophageal sphincter pressure and resting intraesophageal pressure was found. An incomplete relaxation of the sphincter after swallowing was found in 45 patients with achalasia as opposed to complete sphincter relaxation in normals.     

Small Cell Carcinoma of the Esophagus in a Patient with Longstanding Primary Achalasia

Achalasia is believed to be a predisposing factor for the development of esophageal cancer. Small cell carcinoma of the esophagus is a rare neoplasm, with fewer than 150 cases having been reported in the world literature, and it has been described only once previously in a patient with longstanding achalasia. We describe a case of an 85-yr-old woman with long-term primary achalasia who developed primary small cell carcinoma of the esophagus. We hypothesize that this patient's recurrent, worsening dysphagia is related to a paraneoplastic phenomenon. We discuss this association and review the literature.     

Achalasia Associated with Squamous Cell Carcinoma of the Esophagus: A Case Report

A case of achalasia associated with squamous cell carcinoma of the esophagus is presented. Microscopic examination of the resected esophagus demonstrated abundant nerve fibers hut absent ganglion cells throughout the tumor-involved segment. This finding is believed to be the cause of achalasia in this patient.     

Achalasia (The Usefulness of Manometry for Evaluation of Treatment)

Although manometry is used with increasingfrequency to evaluate the effectiveness of differenttreatments for achalasia, the criteria for a successfulmanometric response have not been well defined.Manometric responses were collected before and after 43treatments in 35 patients with achalasia in order todetermine manometric changes after different clinicaloutcomes: 15 unsuccessful outcomes and 28 successful outcomes were reported. In the latter, restingpressure of the lower esophageal sphincter decreased to12.8 mm Hg, whereas in unsuccessful outcomes this wassignificantly higher (28.2 mm Hg). A decrease of lower esophageal sphincter pressure below 17mm Hg or more than 40% of the pretreatment level wasassociated with successful outcomes. Our data suggestthat manometry is a good indicator of therapeutic effectiveness and we propose that it be usedsystematically for objective evaluation of achalasiatreatment.     

Achalasia in the elderly

Clinical and manometric data from 13 elderly subjects with idiopathic achalasia (mean age 79±2 years) were compared with findings from younger subjects with the same disease (n=79) to see if aging altered the presentation and outcome of this motor disorder. Fewer elderly subjects complained of chest pain (27% vs 53%), and the pain was significantly less severe (P<0.01). Other presenting features (including sex, duration of symptoms, and presence and severity of dysphagia) did not differ between the groups. Across all patients, age weakly and inversely correlated with residual postdeglutitive lower esophageal sphincter (LES) pressure (R=–0.34), and residual pressure was significantly lower in the older subjects (8.0±1.3 mm Hg vs 11.9±0.8 mm Hg;P=0.02). No differences in basal LES pressure or esophageal-body contraction amplitudes were present between the groups. Initial success with pneumatic dilation was similar in the two subject groups, but the number of older subjects available for analysis was too small to draw strong conclusions. These results indicate that aging decreases the elevation of LES residual pressure that occurs with achalasia. As elderly achalasia patients also present with less chest pain, the findings may be interrelated.Supported in part by grant AMO7130 from the United States Public Health Service. Dr. Todorczuk is supported by an educational grant from Smith, Kline, and French.     

Anti-Myenteric Neuronal Antibodies in Patients with Achalasia (A Prospective Study)

Achalasia is a motility disorder of theesophagus characterized by the loss of inhibitoryneurons in the distal esophagus. Although idiopathic innature, autoimmune mechanisms have been proposed, and we set out to determine the presence of myentericneuronal antibodies. We prospectively studied 18patients with well-characterized achalasia (by clinical,x-ray, and manometric evidence), nine withgastroesophageal reflux disease, and analyzed the sera from 22disease-free controls. Using double-label, indirectimmunofluorescence techniques, rat esophageal andintestinal sections were double-labeled with sera(dilutions of 1:50 to 1:400) from the three groups andwith neurofilament antibody to localize neurons. Sevenof 18 achalasia patients had sera that stained themajority of neurons within plexi in the esophageal and intestinal sections, including both NADPHdiaphorase (nitric oxide synthase) -positiveand-negative neurons. None of the gastroesophagealreflux patients or the controls showed staining.Neuronal antibodies in achalasia provide an attractive hypothesisto explain this diffuse, possibly immune-based disorder.     

Achalasia of the esophagus

56 patients with achalasia of the esophagus were reviewed in a retrospective study to compare the results of a forceful pneumatic dilation with those of a Heller esophagomyotomy. 22 of 33 patients treated with forceful dilation (67%), showed relief of dysphagia and reduction in the average esophageal diameter by barium swallow during the follow-up period (mean=6.5 years). In 2 patients (6%), forceful dilation was complicated by esophageal perforation, promptly diagnosed, and successfully treated at surgery in both patients. 21 out of 23 patients who underwent esophagomyotomy (91%) showed permanent relief of symptoms and improvement by endoscopic and radiographic criteria. There were no significant postoperative complications during the follow-up period ranging between 1.5 and 10.0 years. The results of this study indicate that esophagomyotomy constitutes a more effective therapeutic modality than forceful dilation (P<0.05). Although esophageal dilation has a place in the treatment of early achalasia, esophagomyotomy appears to be a safer and a more successful form of treatment, of particular value in advanced esophageal disease and in those instances where pneumatic dilation fails to result in immediate clinical improvement.     

Achalasia of the Esophagfus

A four-year old boy with achalasia of the esophagus was suceessfully treated with pneumatic dilatation, using the standard Rrovvne-McHardy dilator, without incident. His subsequent course over 10 months iias been uneventful.