Esophageal achalasia and adenocarcinoma in Barrett's esophagus: a report of two cases and a review of the literature.

Two cases of a rare combination of conditions, achalasia and adenocarcinoma in Barrett's esophagus are reported. Cancer developed 26 years after the onset of gastroesophageal reflux in one and 30 years after esophagomyotomy in the other. Twenty-one cases of Barrett's esophagus and achalasia have now been reported; adenocarcinoma developed in six patients. Only one has survived more than five years after treatment. Long-term surveillance of patients with achalasia is recommended.

Current status of cricopharyngeal myotomy for cervical esophageal dysphagia.

OBJECTIVE: We have reviewed our experience with cricopharyngeal myotomy for a variety of conditions causing cervical esophageal dysphagia to clarify its indications and results as well as to determine what, if any, ancillary procedures are indicated. METHODS: Eighty-three patients underwent cricopharyngeal myotomy between January 1970 and January 1995, 54 of whom had a pharyngoesophageal diverticulum. The remainder suffered from a variety of motor disorders of the upper esophageal sphincter. Clinical follow-up evaluation was obtained in 71 of the 83 patients (86%). RESULTS: Good or excellent results were obtained in 87% of the patients with pharyngoesophageal diverticula, 100% after myotomy plus diverticulectomy, 87% after myotomy plus diverticulopexy and 67% after myotomy alone. Of patients with hypertensive upper esophageal sphincter, 100% had good or excellent results, whereas only 60% with nonspecific esophageal motor disorders were so evaluated. None of the patients with bulbar palsy or miscellaneous conditions had good or excellent results. CONCLUSIONS: We recommend cricopharyngeal myotomy for all patients with a pharyngoesophageal diverticulum coupled with diverticulopexy for the majority, reserving diverticulectomy for those with recurrent pouches or extremely large pouches (6-8 cm in diameter). Good or excellent results can be expected after myotomy in patients with a hypertensive upper esophageal sphincter. Myotomy is rarely indicated for patients with dysphagia secondary to bulbar palsy. The role of cricopharyngeal myotomy for patients with non-specific esophageal motor disorders remains controversial.

Reoperation after failed antireflex surgery. Review of 101 cases.

Between January 1970 and July 1994, 101 patients underwent reoperation for a failed antireflux procedure. These patients had previously had 160 upper gastrointestinal tract operations, usually a Nissen fundoplication or one of its modifications (87). The chief reason for failure of the original antireflux procedure was faulty surgical technique (65). An incorrect diagnosis accounted for most of the remaining failure (22). Of patients who had follow-up studies, 80% were improved by reoperation, which consisted of takedown or refashioning of the original wrap in the majority of patients (63). A more radical approach is justified after two failed reoperations. Our current preference is for vagotomy, antrectomy, and Roux-en-Y diversion coupled, when indicated, with resection of the esophagogastric junctional area.

Achalasia and squamous cell carcinoma of the esophagus: analysis of 241 patients.

Achalasia of the esophagus is presumed by many to be a premalignant lesion leading to an increased risk of squamous cell carcinoma. There is disagreement, however, as to the precise risk of malignant degeneration and there is no consensus as to either the need for close surveillance of achalasia patients or the surveillance technique that should be employed. A review of the available literature on the subject has disclosed a wide range of reported cancer risks in achalasia patients, from zero to 33 times that of the normal population. Cancers, when discovered, are often unresectable and the median survival when they are resectable is low. A personal experience with 241 achalasia patients treated during the past quarter of a century disclosed that 9 had carcinoma, for a prevalence of 3.7%. Carcinoma developed in 3 of these 9 while they were under our observation. This translates into one cancer per 1,138 patient-years of follow-up, an incidence of 88 per 100,000 population, and a risk 14.5 times that of the age-adjusted and sex-adjusted general population. Because of the low postresection survival rate if treatment is delayed until carcinoma of the esophagus becomes symptomatic, closer surveillance of achalasia patients is recommended than has been the case. Because it seems unlikely that close endoscopic surveillance will prove to be cost-effective, periodic (every 2 to 3 years) blind brush biopsy warrants further study as a means of surveillance.

Vagotomy, antrectomy, and Roux-en-Y diversion for complex reoperative gastroesophageal reflux disease.

OBJECTIVE: Failure of conventional surgical therapy for treatment of patients with gastroesophageal reflux disease (GERD) taxes the ingenuity of the esophageal surgeon. This study defines the role of vagotomy, antrectomy, and Roux-en-Y diversion coupled, when necessary, with resection of the esophagogastric junction as an alternative to other surgical procedures currently employed for these complicated cases. SUMMARY BACKGROUND DATA: Currently, the operation in question rarely is performed in the United States. Other procedures, such as interposition of short or long segments of intestine and total esophagectomy with gastric pull-up, are preferred. However, surgeons from Scandinavia, Great Britain, and Europe have published widely on the subject, some even preferring its use as a primary procedure in GERD. METHODS: This report reviews the indications and results of the operation in 36 patients who underwent operation between January 1970 and January 1994. Follow-up evaluation was available for review in 33 patients observed from 1 to 20 years postoperatively (average, 6 2/3 years). Of these patients, 32 had undergone 66 previous operative procedures on the distal esophagus and stomach ranging from 1 to 6 per patient. There were no hospital deaths, but complications developed in nine patients (25%); only half of these complications were major. Of patients available for follow-up, 85% were improved by the operation, 24 of the 33 having excellent or good results. CONCLUSIONS: The operation of vagotomy, antrectomy, and Roux-en-Y diversion, embodying the principles of acid suppression and alkaline diversion, has proved to be a successful alternative to other operative procedures currently favored in the United States for the treatment of the complex reoperative patient with GERD.

Ten to 20-year clinical results after short esophagomyotomy without an antireflux procedure (modified Heller operation) for esophageal achalasia.

To determine the long-term clinical results after modified esophagomyotomy without an antireflux procedure for esophageal achalasia, the status of all patients undergoing this operation with a minimum follow-up time of 10 years was reviewed; 81 such patients were operated on between January 1970 and January 1981. Thirteen patients were lost to follow-up review permitting clinical evaluation during the past year of 68 patients (84%) observed for a median of 13.6 years. Fifty-nine patients (87%) were improved by operation; 90% of the patients who underwent a primary procedure were improved, whereas only 73% of patients undergoing reoperation benefited. Kaplan-Meier analysis of the results of all 81 patients disclosed an improvement rate of 98.5% at 5 years, 95.6% at 10 years, 85.8% at 15 years, and 67.3% at 20 years. When the level of improvement or lack thereof was analyzed, the percentage of excellent results decreased from 54% to 32% (P = 0.02). The percentage of good results remained the same, whereas fair or poor results together increased from 20% to 37% (P = 0.05). Neither age, sex, esophageal caliber, duration of symptoms, or previous therapy appeared to influence these results. We conclude that limited esophagomyotomy without an antireflux procedure results in persistent long-term improvement for the patient with esophageal achalasia. The level of improvement, however, decreases with the passage of time, presumably because of persistent disease in the body of the esophagus leading to impaired esophageal emptying in some patients and late reflux esophagitis in other patients owing to poor esophageal clearance.

Barrett's ulcer: a surgical disease?

Between 1973 and 1990, 285 patients with Barrett's esophagus were treated at the Lahey Clinic. Of these patients, 73 had adenocarcinoma in Barrett's esophagus either when first seen or while under surveillance. Of the remaining 212 patients with benign Barrett's esophagus, 30 had endoscopic evidence of a Barrett's ulcer, for a prevalence of 14%. Initial treatment consisted of aggressive medical therapy, including H2 antagonists and antacids as well as the usual dietary and antireflux measures. In 2 to 4 months, 27 patients underwent repeat endoscopy. Continued endoscopic evaluation in this group totaled 109 patient-years, with a range of 2 months to 13 years (median 2.3 years). Complete healing occurred in 23 of the 27 patients (85%) in 2 to 14 months (median 4 months). Recurrent ulcers developed in seven patients, and these ulcers healed with further medical therapy in five patients. Antireflux procedures were performed in four of six patients with nonhealing Barrett's ulcers, 1 to 1.5 cm in size, and all healed. Two patients refused to have an operation. In our experience, the majority of Barrett's ulcers heal with medical therapy. We reserve surgical intervention for otherwise suitable candidates for operation when no evidence of healing is found within 4 months of medical therapy or for the complications of Barrett's ulcer, namely, perforation, uncontrollable hemorrhage, or malignant degeneration, which were not encountered in this series.

Acid-provoked esophageal spasm as a cause of noncardiac chest pain.

A total of 394 patients with noncardiac chest pain underwent both basal esophageal manometry and combined esophageal motility and acid perfusion studies between 1986 and 1988. On basal esophageal manometry, 275 patients had a normal response, 64 patients had findings of high-amplitude peristalsis or "nut-cracker" esophagus, and 11 patients exhibited changes of diffuse esophageal spasm. Of the 275 patients who had normal findings on basal esophageal manometry, 90 patients (33%) had a positive response on combined esophageal motility and acid perfusion studies, that is, reproduction of chest pain with associated abnormal motility changes. The present study focuses on the 90 patients with acid-provoked esophageal spasm. On acid perfusion study, these 90 patients had a 46.2% rise in deglutition response and a 95% increase in duration compared with a 3.2% and a 4.3% change in values for the control group of healthy volunteers. Of the group with acid-induced spasm, 90.1% had excessive dysmotility changes (repetitive waves, multiple peaks, spontaneous or simultaneous contractions) compared with an incidence of 12.5% in the control group.

Barrett's esophagus. Prevalence and incidence of adenocarcinoma.

Between January 1973 and January 1989, 241 patients with Barrett's esophagus were treated at the Lahey Clinic Medical Center, Burlington, Mass. Of these patients, 65 presented with adenocarcinoma in Barrett's esophagus for a prevalence rate of 27%. Of 176 patients followed up for a total of 497 patient-years, adenocarcinoma developed in five patients for an incidence of one per 99 patient-years. The development of adenocarcinoma during endoscopic surveillance 1, 2, 2, 4, and 10 years after the initial diagnosis of Barrett's esophagus emphasizes the importance of long-term endoscopic and histologic surveillance. All five patients had severe dysplasia before adenocarcinoma developed. Yearly endoscopic follow-up examination is recommended for all patients with Barrett's esophagus unless mild dysplastic changes are found, in which case surveillance should be increased. Patients with severe dysplasia who are otherwise acceptable candidates for operation should be advised to have esophageal resection.

Adenocarcinoma in Barrett's esophagus. A clinicopathologic study of 65 cases.

The natural history of Barrett's esophagus, particularly the prevalence and incidence of malignant changes in it, remains controversial. Furthermore the prognosis of surgically treated patients with carcinoma in Barrett's esophagus has not been elucidated fully. To examine these and other issues, the records of 65 patients with carcinoma in Barrett's esophagus presenting at the Lahey Clinic Medical Center from January 1973 to January 1989 were reviewed. During this period, 241 patients with documented Barrett's esophagus were seen, for a prevalence of carcinoma of 27%. Adenocarcinoma in Barrett's esophagus accounted for 30% of the surgically treated carcinomas of the thoracic esophagus during this period. All but four of these patients were men. Symptoms of chronic reflux were present in less than one half of the patients and dysphagia was often the presenting symptom. In eight patients the carcinoma was discovered on routine surveillance endoscopy, and in four patients progression of disease from benign columnar epithelium to dysplasia to carcinoma was documented. Tumors developed in six patients who had undergone previous antireflux surgery, and in four other patients a second carcinoma developed in residual Barrett's epithelium after a previous resection. Of the 65 patients, 61 (94%) were considered to have operable disease, all of whom underwent resection. Two patients (3.3%) died within 30 days of operation. The resected specimens were staged as follows: stage 0, 4; stage I, 10; stage II, 17; stage III, 25; stage IV, 4. Of the resected specimens, 73% showed areas of dysplasia adjacent to the tumor. The overall adjusted actuarial 5-year survival rate was 23.7%. The 3-year survival rate was 100% for patients with stage 0 carcinoma, 85.7% for patients with stage I carcinoma, 53.6% for patients with stage IIA carcinoma, 45% for patients with stage IIB carcinoma, 25.2% for patients with stage III carcinoma, and 0% for patients with stage IV carcinoma. The premalignant nature of Barrett's esophagus requires endoscopic surveillance to detect early carcinoma because symptoms often occur late or are absent. Antireflux surgery does not protect against the development of carcinoma. All of the Barrett's epithelium must be resected because a second carcinoma may develop in residual columnar epithelium. Severe dysplasia should be considered an indication for resection. Although operability and resectability rates are high, long-term survival is not. Early detection is mandatory if long-term survival is to be achieved.

Effect of antireflux operation on Barrett's mucosa.

Regression of Barrett's epithelium after antireflux operations remains a controversial topic. We evaluated the effect of antireflux procedures in patients with Barrett's esophagus on the regression of columnar epithelium and dysplasia and its potential protective effect on the subsequent development of carcinoma. Of the 241 patients with Barrett's esophagus treated at the Lahey Clinic from 1973 to 1989, 37 patients underwent an antireflux operation. Regression was defined as histological evidence of regenerating squamous mucosa that completely or partially replaced the columnar epithelium. Improvement in lower esophageal sphincter pressure to 12 mm Hg or greater occurred in 19 of 26 patients (73%) who had perioperative manometry. Symptomatic relief of esophagitis occurred in 34 of 37 patients (92%). Four patients had partial regression with regenerating squamous mucosa juxtaposed with areas of columnar epithelium. Carcinoma developed in 3 of 37 patients (8.1%). One patient had recurrence of severe symptoms of reflux esophagitis before development of carcinoma. Patients with Barrett's esophagus who have undergone a successful antireflux operation with symptomatic relief and evidence of improvement in lower esophageal sphincter pressures rarely show regression of Barrett's mucosa and may still be at risk for development of carcinoma. Therefore, the indications for antireflux operation in Barrett's esophagus should remain the same as for other patients with gastroesophageal reflux, but yearly endoscopic and histological surveillance should be continued postoperatively.

Esophageal reconstruction for complex benign esophageal disease.

We report the cases of 35 patients with complex benign esophageal disease who required radical surgical reconstruction. These patients had undergone 63 previous esophageal operations. Twenty-seven patients required esophagogastrectomy, four had esophageal exclusion before colon interposition, two had cardioplasty, and two without stricture did not require resection. Reconstruction was achieved by esophagogastrostomy in six patients, colon interposition in eight, and acid suppression and alkaline diversion in 21. One patient died of pneumonia 2 weeks after esophagogastrostomy. The overall rate of postoperative improvement was 70%, but the condition of 86% of patients was improved after the acid-suppression and alkaline-diversion procedure, which is the reconstructive procedure we prefer in properly selected patients with complex benign esophageal disease.

Limited esophagogastrectomy for carcinoma of the cardia. Indications, technique, and results.

Between 1970 and 1988, 149 patients with carcinoma of the cardia were operated on at the Lahey Clinic. Of these patients, 127 (85%) underwent resection; 23 (18.1%) were of a palliative nature. More than 75% had Stage III and IV disease. One patient (0.8%) died within 30 days of the operation of a myocardial infarct. Two other patients failed to leave the hospital. Of 25 postoperative complications, 14 (11%) were considered major. Palliation of dysphagia was successful in 80% of patients. The actuarial 5-year survival rate was 22.4%. Of patients with Stage I and II disease, 36.6% survived for 5 years, and of patients with Stage III disease, 22.5% survived. No patient with Stage IV disease lived for longer than 1 year. It is concluded that limited esophagogastrectomy can be performed in most patients with carcinoma of the cardia with low mortality and morbidity and with satisfactory long-term survival.

Giant leiomyoma of esophagus.

This case report of a 37-year-old man with giant leiomyoma details some of the problems encountered in diagnosing and treating this rare form of the disease. Initial studies at another institution were interpreted as demonstrating achalasia, and thoracotomy was later undertaken because of a mistaken diagnosis of a pericardiac mass, which led to a biopsy and a resulting esophagopleural fistula. Subsequent esophagectomy and drainage of empyema space were employed to remove the tumor, which had ulcerated and bled, as well as to drain the empyema cavity. The tumor measured 20.5 cm long and weighed 540 gm. Gastrointestinal continuity was reestablished by colon interposition, and the patient has been well for the succeeding 6 years.

Reoperative achalasia surgery.

Forty-six patients with esophageal achalasia required reoperation between January 1970 and January 1986. Three of these patients required a second reoperative procedure, for a total of 49 reoperations. Indications for reoperation were inadequate myotomy, 17; gastroesophageal reflux, 14; concomitant antireflux operation, six; incorrect diagnosis, four; carcinoma of the esophagus, four; megaesophagus, three; and paraesophageal hernia, one. Various procedures were employed at the time of reoperation, including revision of the myotomy, takedown or revision of a previously performed wrap, fundoplication, and resection. Of the 48 patients available for follow-up study over an average postoperative period of 5 years, the condition of 38 (79%) was considered to have been improved by reoperation. The best results were obtained by revision or takedown of a previous wrap (an improvement rate of 88.9%) and radical resective procedures (89% to 100%). We conclude that for good results to be achieved after reoperative achalasia procedures, the preoperative diagnosis must be accurate, the operation should be performed early before the development of megaesophagus, and a short but complete esophagomyotomy must be performed, preferably without the addition of an antireflux procedure. Elimination or revision of a previously performed fundoplication can be expected to be followed by good results. The precise indications for radical resective procedures have yet to be defined clearly, but their wider application to carefully selected patients with postoperative achalasia seems justified.

Use of fiberoptic endoscopy in diagnosis and therapy of upper gastrointestinal disorders.

The future role of therapeutic endoscopy appears bright and depends on the possibilities of technology and the ingenuity of those performing the procedures. New techniques are being devised, and the therapeutic applications are being evaluated continually. Such issues as appropriate indications, efficacy, and cost-effectiveness remain important considerations for the practitioner dealing with gastrointestinal disorders.

Overview of the current management of carcinoma of the esophagus and cardia.

Esophagogastrectomy with esophagogastrostomy provides the best palliation for patients with carcinoma of the esophagus and cardia. It affords better palliation, longevity and overall survival than do other forms of therapy either singly or in combination. Treatment span is short, the patient is able to return to a reasonably normal life-style relatively quickly and the debilitating effects of dysphagia are relieved in most patients. Between January 1970 and July 1984 at the Lahey Clinic Medical Center, the overall operability rate was 80.3% and the resectability rate 87.2%. Four of 191 patients who underwent resection died for a hospital death rate of 2.1%. The overall 5-year survival rate was 16.3% and 81.2% of patients had successful palliation of dysphagia.

Dysplastic Barrett's esophagus in scleroderma.

A review of 107 patients with Barrett's esophagus revealed three patients who concomitantly had scleroderma. Two of the three patients had pathological evidence of high-grade dysplasia of the columnar-lined epithelium, and the third patient had nondysplastic columnar-lined epithelium in the distal esophagus. Patients with scleroderma often have an incompetent lower esophageal sphincter, poor or absent distal esophageal peristalsis, and reflux esophagitis, all of which are believed to predispose to Barrett's esophagus. The importance of Barrett's esophagus is its potential for malignant transformation. Identification of such patients permits aggressive medical treatment and endoscopic and pathological surveillance.

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.