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Query: UMLS:C0014848 (achalasia)
 2,804 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

AIMS: The management and surveillance of achalasia remains controversial at the present time. The aim of this study was therefore to evaluate the results of endoscopic management and subsequent surveillance of patients with achalasia presenting to a specialist unit. METHODS: A prospective cohort of 40 patients with a radiological and manometric diagnosis of achalasia who presented to this unit between 1991 and 1998 were studied; the male : female ratio was 1 : 1 and the median age 38 (range 15-84) years. Twenty-one patients presented de novo, seven had previously undergone cardiomyotomy and 12 were referred following unsuccessful dilatation. RESULTS: Some 36 patients were treated with balloon dilatation (Microvasive achalasia balloon, 35/40 mm). Results were graded 1-4 (1, asymptomatic; 2, symptomatic but significantly improved; 3, symptomatic with no change; and 4, symptomatic but worse); 29 of 36 patients were grade 1 at subsequent follow-up and the remaining seven were grade 2 (median follow-up 17 (range 5-96) months). There was a single complication of oesophageal perforation which was treated conservatively with full recovery. Following intervention, patients were enrolled in a prospective surveillance programme of chromoendoscopy at 2-year intervals; in a total of 74 patient-years' follow-up, two superficial squamous cell carcinomas (SCCs) and one adenocarcinoma (following cardiomyotomy) were detected, giving a relative risk of one cancer in 25 patient-years. CONCLUSIONS: Balloon dilatation is a safe and effective treatment for achalasia even in patients who have had previous unsuccessful dilatations or cardiomyotomy. There is a high risk of SCC and the adenocarcinoma may have resulted from previous refluxogenic therapy, so all patients with achalasia should be followed up with surveillance endoscopy.
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PMID:Achalasia: management, outcome and surveillance in a specialist unit 1071 70

The diagnosis of achalasia is based on the patient's symptoms and on manometric studies although other diseases present similar symptomatology. To present an elderly woman clinically and manometrically diagnosed with achalasia of the cardia who was treated by dilatation. When the patient relapsed and was surgically treated she was found to have and esophageal adenocarcinoma. When considering a diagnosis of achalasia, alternative diagnoses (especially neoplasias) should be borne in mind, especially in unusual situations, such as rapid onset or in the elderly.
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PMID:[Pseudoachalasia: a diagnosis to consider in the assessment of dysphagia]. 1126 Dec 27

The authors report a case of primary adenocarcinoma arising in the jejunal limb of a Roux-en-Y esophago-jejunostomy in a 76-years-old Caucasian woman. She was affected by esophageal achalasia and in another surgical department underwent gastric resection for adenocarcinoma 6 months before admission. A total gastrectomy performed was due to severity of achalasia symptoms conditioning a high degree of malnutrition. Two years after discharge the patient underwent surgery for total dysphagia, epigastric pain and inexorable weight loss. Histological response was adenocarcinoma of the long arm of Roux-en-y loop. Primary malignancies of the jejunum and ileum are extremely rare and many pathogenetic hypotheses were done but none of them received clinical or experimental demonstration. Symptoms are not specific and diagnosis is difficult and late, x-rays and endoscopy leading to it, US, CT and MNR are useful for differential diagnosis and staging. Prognosis is severe. Surgical therapy is large resection, if possible, but often it is only palliative.
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PMID:[Primary adenocarcinoma of the jejunum arising in the anastomotic loop of a Roux-en-Y esophago-jejunostomy: a case report]. 1081 73

Although adenocarcinoma of the cardia is extremely rare in adolescent patients, the endoscopist should be alert to this disease in patients of any age with dysphagia, even if symptoms, and results of a barium study, upper endoscopy, and esophageal manometry are suggestive of primary achalasia, especially if family history is negative for achalasia. In addition, secondary achalasia should be suspected in patients who do not respond to therapy with botulinum toxin within 2 months. Because none of the mentioned tests can distinguish between primary achalasia and secondary forms due to carcinoma of the cardia, biopsy specimens should be obtained. It appears that, although there is a minimal risk for complications, a diagnostic procedure such as biopsy would be appropriate when the information obtained could be essential. In some cases EUS can be an additional diagnostic tool, because lesions of the submucosa and the surrounding area can be identified by EUS.
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PMID:Gastric adenocarcinoma mimicking achalasia in a 15-year-old patient: a case report and review of the literature. 1117 37

Reported incidence rates of carcinoma in patients with achalasia and the prevalence of achalasia in patients with esophageal cancer vary widely in the literature. The prognosis of an "achalasia-carcinoma" is generally considered poor, although systematic studies assessing the incidence, prevalence, and prognosis of patients with "achalasia-carcinoma" are scant. We investigated the incidence of esophageal cancer in a large series of patients with known achalasia, assessed the prevalence of achalasia in patients presenting with esophageal cancer, and evaluated the prognosis of these patients compared to that of patients with esophageal cancer without achalasia. Between 1982 and 1998 a total of 124 patients with primary achalasia were treated and followed at our department. During the same time period 1366 patients presented with esophageal cancer (879 esophageal squamous cell carcinomas, 487 adenocarcinomas). Of the 124 patients with primary achalasia, 4 developed a carcinoma during a mean follow-up of 5.6 years (i.e., an incidence of one carcinoma per 173.6 patient-years of follow-up). Altogether, 13 of 879 patients (1.5%) presenting with esophageal squamous cell carcinoma and 1 of 487 patients (0.2%), presenting with esophageal adenocarcinoma had a history of primary achalasia. Seven patients with achalasia-carcinoma (50%) had early-stage disease (stage I, IIA, or IIB). There was no difference in the prognosis of patients with resected achalasia-carcinoma versus those with esophageal carcinoma but no achalasia. Thus in our population of patients with long-standing achalasia the risk for developing an esophageal cancer was increased about 140-fold over that of the general population. With liberal use of surveillance, carcinoma could often be detected at an early stage in these patients, with a prognosis that was not worse than that of patients with squamous cell esophageal cancer but no achalasia.
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PMID:Achalasia and esophageal cancer: incidence, prevalence, and prognosis. 1137 10

The authors reviewed the clinical records of 1460 patients with esophageal anastomoses, the operations performed in their institute between 1973 and 2000. Surgical outcome was assessed with incidence of anastomotic leaks and overall mortality. Hand-sewn anastomoses were performed by inserting single layer interrupted monofil steel wire (507), PDS (232) or Vicryl (65) sutures and circular stapler (EEA, Ethicon) was used in 656 patients. 453 (65 cervical, 293 thoracic, and 95 abdominal) anastomoses performed between 1995 and 2000 were analyzed separately. During this 6-year period, the majority of operations (88%) were performed because of esophageal malignancy (squamous cell carcinoma of the esophagus or adenocarcinoma of the cardia). Surgery was performed for benign disease (e.g. stricture, perforation, and stage IV achalasia) in 12%. The incidence of anastomotic leak was the highest (20%) after cervical anastomosis, compared to a 4.4% or 1% associated with thoracic or abdominal anastomoses. These differences are statistically significant (p < 0.001). The rate of anastomotic leakage was strongly connected to surgical technique: it was 15% in hand-sewn anastomoses and only 4.4% with stapled anastomosis. During the 6-year period overall mortality was 6.6%t.
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PMID:[Esophageal anastomosis--based on the experience with 1460 operations]. 1143 62

Two unusual cases of achalasia with endoscopic and histologic documentation of Barrett's esophagus are presented. One patient had Barrett's esophagus at the time of initial endoscopy for achalasia, before any treatment. The other patient developed specialized columnar epithelia in the esophagus after treatment with pneumatic dilation. Each patient had evidence of low-grade dysplasia. Including these two patients, 30 cases of Barrett's esophagus in patients with achalasia have been reported in the literature. In 73% (22 of 30) of the cases, Barrett's esophagus was detected after esophagomyotomy. In 20% (6 of 30) of the cases of achalasia and Barrett's esophagus, adenocarcinoma developed. The current two cases are unusual because Barrett's esophagus in achalasia generally develops from gastroesophageal reflux after esophagomyotomy. No other patients have been reported to develop Barrett's esophagus after pneumatic dilation alone. Patients with achalasia and Barrett's esophagus may be at a particularly high risk for developing dysplasia and adenocarcinoma.
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PMID:Barrett's esophagus and achalasia. 1190 48

We report an unusual hepatoid adenocarcinoma in Barrett's esophagus with achalasia, which developed in a 44-year-old Japanese woman. The patient received an esophago-gastrectomy after diagnosis of the tumor and achalasia at the lower esophagus, 4 months before her death due to multiple metastatic tumors of the liver. The main granular tumor removed surgically was a hepatoid adenocarcinoma, mainly composed of clear cancer cells (alpha-1 antitrypsin, albumin and alpha-fetoprotein positive), with elements of choriocarcinoma and tubular adenocarcinoma. Non-neoplastic specialized columnar epithelium was present extensively near the oral side of the tumor edge in the esophagus, indicating Barrett's esophagus. This unusual tumor was therefore considered to have originated in Barrett's esophagus. The gastroesophageal reflux was presumed to have occurred for a long period, as there was a well-preserved fundic gland in the stomach and a history of frequent vomiting from the patient's youth, accounting for the appearance of achalasia.
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PMID:Hepatoid adenocarcinoma in Barrett's esophagus associated with achalasia: first case report. 1194 Feb 19

Pseudoachalasia is an esophageal motor disorder usually associated with malignancy that has clinical, radiographic, and manometric findings that are often indistinguishable from primary achalasia. There are few reports examining the histologic features of the associated neoplasms and their relationship with the esophageal myenteric plexus. We studied the clinical and pathologic features of 13 cases of pseudoachalasia seen at our institution between 1979 and 1999. Detailed clinical and radiographic data were obtained from medical records. In all cases available histologic material was reviewed to confirm the presence and type of associated neoplasm. When possible, the relationship of the neoplasm to the esophageal myenteric plexus was examined. In selected cases immunohistochemical stains were performed to further evaluate this relationship. All patients had clinical, radiographic, and manometric features similar to primary achalasia. The cohort included seven men and six women, age range 24-79 years (median 61 years). Associated neoplasms included esophageal adenocarcinoma arising in Barrett's esophagus (n = 1), adenocarcinoma of the esophagogastric junction (n = 7), metastatic renal cell carcinoma to the esophagogastric junction (n = 1), breast adenocarcinoma (n = 1), pulmonary small cell carcinoma (n = 1), pleural malignant mesothelioma (n = 1), and mediastinal fibrosis (n = 1). The mechanism of pseudoachalasia was consistent with neoplastic infiltration of the esophageal myenteric plexus in 11 cases. Neoplastic cells surrounded myenteric ganglion cells, which appeared normal in number and morphology. In the patient with pulmonary small cell carcinoma, there was no evidence of neoplastic infiltration of the esophagogastric junction, and anti-ANNA-1 antibody was detected, suggesting a paraneoplastic syndrome. Tissue obtained at the time of esophagomyotomy revealed lymphocytic myenteric inflammation and marked depletion of ganglion cells identical to that seen in primary achalasia. The mechanism pseudoachalasia in the patient with breast adenocarcinoma is uncertain, as there was no evidence of direct involvement of the esophagogastric junction. In summary, we describe 13 cases of pseudoachalasia resulting in a clinical syndrome indistinguishable from primary achalasia. The most common mechanism is direct involvement of the esophageal myenteric plexus by neoplastic cells. Rarely, a distant neoplasm may cause this syndrome as a paraneoplastic process.
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PMID:The pathogenesis of pseudoachalasia: a clinicopathologic study of 13 cases of a rare entity. 1202 84

Dysphagia is the characteristic cardinal symptom of diseases of the esophagus. Etiologically, consideration must first be given to carcinoma of the esophagus. Among the cancers affecting this organ, adenocarcinoma of the distal part of the esophagus is the most common. The most important differential diagnosis in patients who have difficulty swallowing is peptic stricture of the distal esophagus arising from long-standing reflux disease. Further possible causes are motility disorders of the esophagus including, in particular, achalasia and--more rarely--diverticula, foreign bodies, membranes or rings, benign tumors or extraluminal processes.
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PMID:[Peptic stenosis, motility disorder or carcinoma. What is ate the bottom of dysphagia?]. 1219 75


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