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Multiple lower esophageal diverticula: unusual endoscopic appearance
33
Multiple lower esophageal diverticula: unusual endoscopic appearance John S. Gunther, M.D. EI Paso, Texas
An extraordinary case of multiple lower esophageal diverticula, with remarkable endoscopic documentation, is described. Current concepts of pathogenesis are reviewed. Treatment is properly directed to the primary lesion responsible for the diverticula.
A mong patients presenting with dysphagia, lower esophageal diverticula (including "epiphrenic" diverticula) are uncommon and multiple diverticula are rare. 1,2 Previous case studies of lower esophageal diverticula (LED) lack information derived from biopsy, manometry, and endoscopy although radiologic appearance has been described. 3 ,4,5 The object of this report is to describe a patient with severe dysphagia, alcoholic neuropathy, hiatal hernia with stricture, and 9 LED for which manometric data, biopsy, and endoscopic photography are available. This is the largest number of LED in a single patient reported to date. CASE REPORT A 51-year-old white man was admitted with tremulousness, pyrosis with progressive dysphagia for 6 years, and inability to swallow solids for 1 month. He had imbided a fifth of vodka daily for 10 years. There was no history of gastrointestinal bleeding, diarrhea, pancreatitis, or diabetes mellitus. Examination revealed hepatomegaly, spider angiomata, and poor coordination of the extremities with hyperpathia and decreased pain appreciation. Mild SGOT elevations were initially noted; liver biopsy disclosed fatty metamorphosis. Barium enema demonstrated sigmoid diverticulosis. Upper gastrointestinal series (Figure 1) showed hiatal hernia with stricture and multiple small LED. During his initial hospital course, diagnoses of alcohol withdrawal syndrome, Korsakoff's psychosis, alcoholic cerebellar degeneration, and peripheral neuropathy were substantiated. Esophagoscopy (Olympus EF) revealed 9 fixed out-pouchings of the distal esophagus, measuring 0.5 cm to 1.5 cm in diameter. These were arranged in linear fashion in the right and left posterolateral and posterior esophageal walls over the 5 cm proximal to a firm fibrous benign stricture at the esophagogastric mucosal junction.
Severe distal esophagitis, confirmed by multiple biopsies with routine and fungal stains, was present. No esophageal varices were seen, but a moderate sized hiatal hernia and chronic superficial gastritis of the proximal stomach were documented. Subsequently the esophageal stricture was dilated to 52 French with mercury filled bougies, and antacid therapy was instituted. Two months after admission the patient was eating all foods without dysphagia and had gained 16 pounds. A Bernstein test at that time was negative. Lower esophageal sphincter pressure was 4 mm Hg (normal > 9.5). The lower sphincter relaxed normally in response to swallows. In the body of the esophagus there was some failure of primary peristalsis but no evidence of synchronous, spontaneous, repetitive, or prolonged spastic activity. Three months following initial endoscopy, repeated endoscopy revealed the multiple diverticula to be unchanged. .However, the esophagitis and mucosa about the stricture were improved.
DISCUSSION LED comprises less than 10% of all esophageal diverticula. 6 They occur in the distal 10 cm of the esophagus and represent essentially false diverticula of the pulsion type.7,8 Mondiere 9 described LED in 1833 and suggested an etiologic role for increased intraluminal pressure. Early reports suggested an association of diverticula with cardiospasm in about 65% of cases, but later authors doubt this degree of association. 2,7,8,11 Goodman and Parnes 3 reviewed reports from 18821949 and found 126 cases of LED. It is probable that some cases of functional diverticulosis were included in that material as esophageal diverticula may be simulated radi-
From the Gastroenterology Service, William Beaumont General Hospital, EI Paso, Texas. Reprint requests: Major John S. Gunther, Me, Box 186, William Beaumont General Hospital, EI Paso, Texas 79920. VOLUME 19, NO.1, 1972
34
Figure 1. Upper gastrointestinal series
showing
hiatal
hernia
(left). Note that the esophageal
diverticula are not apparent in the empty esophagus. A spot film of the filled esophagus (right) shows multiple small diverticula just proximal to a fixed stricture.
ologically by esophageal spasm. 12 Granet 5 in a similar review in 1933 found only 33 well documented cases of LED. The relative rarity of lower esophageal as compared to pharyngoesophageal diverticula was illustrated by Harrington 9 and Lahey 13 who found ratios of 8 to 216 and 6 to 211. Habein 2 reported 201 patients with LED from the Mayo Clinic between 1944 and 1953. Of these, 97 had pulsion diverticula as determined by radiologic appearance. The male:female ratio was in excess of 2:1 with an average patient age of 59 years. Only 15 cases had multiple (up to
Figure 2. Esophagoscopic view of multiple esophageal diverticula above an inflammatory stricture.
3) diverticula. It was determined that 21 patients had hiatal hernia, 13 had diffuse esophageal spasm, 3 had cardiospasm, and 56 had "no associated lesion." Motility was not studied, and only 18 patients had esophagoscopy. Habein 2 categorized 52 cases as traction diverticula; all were small (less than 3 cm) and 2 double diverticula were found. Hiatal hernia was associated in 16 patients, diffuse spasm in 4, and 1 had cardiospasm. It is probable that this categorization of traction diverticula is erroneous in light of current concepts of etiology. 8, 14, 15, 16 Effler ll found only 7 cases of LED at the Cleveland Clinic over an 8 year period. None was esophagoscoped, but 5 were thought to have hiatal hernia. Six patients were dysphagic and operated with the discovery of hypertrophic circular muscle in the esophagus distal to the diverticulum. The etiology of LED is considered to be neuromuscular dysfunction causing elevated pressure in the esophageal lumen. 8, 15 Diverticula occur within or above an area of tone increase or sphincteric opening delay. Diffuse esophageal spasm creates higher pressure zones between spastic segments with the development of pulsion diverticula (single or multiple) which appear as blowouts of esophageal mucosa through relatively weak areas of the muscle layer. 8 The wall of the diverticulum is composed of thickened mucosa, submucosa, muscularis mucosae and adventita but lacks support from the muscle layer. Spasm of the lower esophageal sphincter may be a more frequent cause of epiphrenic diverticula than diffuse spasm, with a high pressure area being generated above the sphincter in front of the oncoming peristaltic wave. 15 The association of LED with hiatal hernia has been emphasized. 2 ,lo,1l Proposedly, the hiatal hernia with regurgitation results in lower esophageal spasm and delayed esophageal emptying. A peptic stricture above a nonspastic but incompetent sphincter could enhance development of diverticula by providing a GASTROINTESTINAL ENDOSCOPY
35
similar distal obstruction. Kay 14 has alluded to the importance of sympathetic preponderance and vagal denervation in genesis of lower esophageal sphincter dyskinesias. The present case illustrates the occurrence of hiatal hernia with partial mechanical obstruction due to peptic stricture and an unusually large number of pulsion diverticula. Manometric study revealed lack of lower esophageal spasm or sphincter hyperactivity. It is possible that the observed failure of primary peristalsis reflected alcoholic neuropathy and that earlier in the patient's course elevated pressures of spasm might have been seen. Winshi p17 and Fischer 18 described deterioration of esophageal peristalsis in patients with alcoholic neuropathy manifested by selective deterioration of esophageal peristalsis, most pronounced in the distal esophagus, usually with preservation of sphincteric function. Vagal nerve degeneration alone does not seem to fully account for the observed abnormalities, and the role of alcoholic myopathy is obscure. Several excellent reviews of the symptoms and treatment of LED are available. 2 ,4,7,8,10 In our patient, the complete clinical relief afforded by conventional bougienage and a medical reflux-preventing regimen confirms the impression of others that LED per se uncommonly account for significant symptoms and that therapeutic measures directed at concomitant motility, inflammatory, or obstructive lesions are most pertinent. 2,3,7,8,10,14,15
REFERENCES 1. MACMILLAN A S: Statistical study of diseases of the esophagus. Surg Gynec Obstet 60:394-402, 1935 2. HABEIN H C, MOERSCH H ), KIRKLIN JW: Diverticula of the lower part of the esophagus. Arch Int Med (Chicago) 97:768-777, 1956 3. GOODMAN H I, PARNES I H: Epiphrenic diverticula of the esophagus. J Thorac Surg 23:145-159, 1952 4. VINSON P P: Diverticula of the thoracic portion of the esophagus-report of forty-two cases. Arch Otolaryng 19:508-513, 1934 5. GRANET E: Epiphrenic diverticulum of the esophagus with case report. Amer J Surg 19:259-262, 1933 6. TERRACOl ), SWEET R H: Diseases of the esophagus. Philadelphia. WB Saunders Company, 1958, pp 264-271 7. HABEIN H, KIRKLIN J W, CLAGETT 0 T, MOERSCH H ): Surgical treatment of lower esophageal pulsion diverticuli. Arch Surg (Chicago) 72:10181024,1956 8. BElSEY R: Functional disease of the esophagus. J Thorac Cardiovasc Surg 52:164-188,1966 9. MONDIERE I T cited by HARRINGTON S W: The surgical treatment of pulsion diverticula of the thoracic esophagus. Ann Surg 129:606-618, 1949 10. DEBAKEY M C, CREECH 0: Surgical treatment of epiphrenic diverticulum of the esophagus. Review of the literature and report of a case treated by resection and esophagogastroscopy. I Thorac Surg 23:486-494,1952 11. EFFLER D B, BARR 0, GROVES L K: Epiphrenic diverticulum of the esophagus. Arch Surg (Chicago) 79:459-467,1959 12. VESCIA F G: Corkscrew esophagus masquerading as multiple esophageal diverticula. Gastrointestinal endoscopy 15:108-109, 1968 13. LAHEY F H: Intrapleural pulsion esophageal diverticulum. Lahey Clinic 8uI/5:2-5,1946 14. KAY E B: The inferior esophageal constructor in relation to lower esophageal disease. J Thorac Surg 25:1-15,1953 15. CROSS F S, JOHNSON G F, GEREIN A N: Esophageal diverticula-associated neuromuscular changes in the esophagus. Arch Surg (Chicago) 83:525-533,1961 16. BRADHAM R R, SEALY W C: Neuromuscular disorders of the esophagus. Ann Thorac Surg 3:460-476, 1967 17. WINSHIP D H, CAFLISCH C R, ZBORALSKE F F, HOGAN W ): Deterioration of esophageal peristalsis in patients with alcoholic neuropathy. Gastroenterology 55:173-178, 1968 18. FISCHER R A, ELLISON C W, THAYER W R, 5PIRO H M, GLASSER G H: Esophageal motility in neuromuscular disorders. Ann Intern Med 63:229248,1965 VOLUME 19, NO.1, 1972
Counsel to Authors GASTROINTESTINAL ENDOSCOPY seeks to publish original papers describing investigations and significant observations relating to the various endoscopic technics employed in the study of digestive diseases. Descriptions of new instruments or methods, innovative applications of endoscopy, analyses of experience, and extraordinary case reports are welcome subjects. Readers who are piqued or prodded by published articles are encouraged to submit brief comments that the Editor may include in the "Reflex" section. Announcements of postgraduate courses, new facilities, cooperative studies and other items of interest to endoscopists are invited. Address typescripts and editorial correspondence to the editor. Typescripts should be submitted in duplicate on white paper of standard (8 112. x 11 inches) size. All copy, including footnotes, references, and captions, should be double spaced with generous margins. The title page should include the author's names, the institution where the work was performed, and the name and address to which requests for reprints should be sent. Illustrations, including photographs and charts, should be submitted as glossy prints not less than 5 x 7 inches, preferably of uniform size. These must be unmounted and clearly labeled on the back, lightly in pencil. to indicate first author's name, figure number, and top of illustration. Radiographs and endoscopic photographs must be of high contrast for proper reproduction. A reasonable number of pertinent illustrations in black and white can be allowed without charge. Color reproduction can be arranged at cost to the author. References should be indicated by consecutive numbers in the text and enumerated at the end of the paper in the same order. Each reference should conform to the following example: Scope GE, Optic F: What we saw. Gastroenterology 50: 100 I, 1962. All authors should be listed (not "et al."). Abbreviations of journal names are to be used as in Index Medicus. Helpful information pertaining to the preparation of scientific articles will be found in Style Manual for Biological Journals, Ed. 2, published by American Institute of Biological Sciences, 3900 Wisconsin Ave., N.W., Washington, D.C. 20016. Galley proofs will be sent to the author for necessary corrections with the understanding that they are to be promptly returned. Reprints should be ordered when galley proof is returned. A purchase form will be furnished. Authors are reminded to retain in their own files a copy of the typescript and illustrations submitted.
Multiple lower esophageal diverticula: unusual endoscopic appearance John S. Gunther, M.D. EI Paso, Texas
An extraordinary case of multiple lower esophageal diverticula, with remarkable endoscopic documentation, is described. Current concepts of pathogenesis are reviewed. Treatment is properly directed to the primary lesion responsible for the diverticula.
A mong patients presenting with dysphagia, lower esophageal diverticula (including "epiphrenic" diverticula) are uncommon and multiple diverticula are rare. 1,2 Previous case studies of lower esophageal diverticula (LED) lack information derived from biopsy, manometry, and endoscopy although radiologic appearance has been described. 3 ,4,5 The object of this report is to describe a patient with severe dysphagia, alcoholic neuropathy, hiatal hernia with stricture, and 9 LED for which manometric data, biopsy, and endoscopic photography are available. This is the largest number of LED in a single patient reported to date. CASE REPORT A 51-year-old white man was admitted with tremulousness, pyrosis with progressive dysphagia for 6 years, and inability to swallow solids for 1 month. He had imbided a fifth of vodka daily for 10 years. There was no history of gastrointestinal bleeding, diarrhea, pancreatitis, or diabetes mellitus. Examination revealed hepatomegaly, spider angiomata, and poor coordination of the extremities with hyperpathia and decreased pain appreciation. Mild SGOT elevations were initially noted; liver biopsy disclosed fatty metamorphosis. Barium enema demonstrated sigmoid diverticulosis. Upper gastrointestinal series (Figure 1) showed hiatal hernia with stricture and multiple small LED. During his initial hospital course, diagnoses of alcohol withdrawal syndrome, Korsakoff's psychosis, alcoholic cerebellar degeneration, and peripheral neuropathy were substantiated. Esophagoscopy (Olympus EF) revealed 9 fixed out-pouchings of the distal esophagus, measuring 0.5 cm to 1.5 cm in diameter. These were arranged in linear fashion in the right and left posterolateral and posterior esophageal walls over the 5 cm proximal to a firm fibrous benign stricture at the esophagogastric mucosal junction.
Severe distal esophagitis, confirmed by multiple biopsies with routine and fungal stains, was present. No esophageal varices were seen, but a moderate sized hiatal hernia and chronic superficial gastritis of the proximal stomach were documented. Subsequently the esophageal stricture was dilated to 52 French with mercury filled bougies, and antacid therapy was instituted. Two months after admission the patient was eating all foods without dysphagia and had gained 16 pounds. A Bernstein test at that time was negative. Lower esophageal sphincter pressure was 4 mm Hg (normal > 9.5). The lower sphincter relaxed normally in response to swallows. In the body of the esophagus there was some failure of primary peristalsis but no evidence of synchronous, spontaneous, repetitive, or prolonged spastic activity. Three months following initial endoscopy, repeated endoscopy revealed the multiple diverticula to be unchanged. .However, the esophagitis and mucosa about the stricture were improved.
DISCUSSION LED comprises less than 10% of all esophageal diverticula. 6 They occur in the distal 10 cm of the esophagus and represent essentially false diverticula of the pulsion type.7,8 Mondiere 9 described LED in 1833 and suggested an etiologic role for increased intraluminal pressure. Early reports suggested an association of diverticula with cardiospasm in about 65% of cases, but later authors doubt this degree of association. 2,7,8,11 Goodman and Parnes 3 reviewed reports from 18821949 and found 126 cases of LED. It is probable that some cases of functional diverticulosis were included in that material as esophageal diverticula may be simulated radi-
From the Gastroenterology Service, William Beaumont General Hospital, EI Paso, Texas. Reprint requests: Major John S. Gunther, Me, Box 186, William Beaumont General Hospital, EI Paso, Texas 79920. VOLUME 19, NO.1, 1972
34
Figure 1. Upper gastrointestinal series
showing
hiatal
hernia
(left). Note that the esophageal
diverticula are not apparent in the empty esophagus. A spot film of the filled esophagus (right) shows multiple small diverticula just proximal to a fixed stricture.
ologically by esophageal spasm. 12 Granet 5 in a similar review in 1933 found only 33 well documented cases of LED. The relative rarity of lower esophageal as compared to pharyngoesophageal diverticula was illustrated by Harrington 9 and Lahey 13 who found ratios of 8 to 216 and 6 to 211. Habein 2 reported 201 patients with LED from the Mayo Clinic between 1944 and 1953. Of these, 97 had pulsion diverticula as determined by radiologic appearance. The male:female ratio was in excess of 2:1 with an average patient age of 59 years. Only 15 cases had multiple (up to
Figure 2. Esophagoscopic view of multiple esophageal diverticula above an inflammatory stricture.
3) diverticula. It was determined that 21 patients had hiatal hernia, 13 had diffuse esophageal spasm, 3 had cardiospasm, and 56 had "no associated lesion." Motility was not studied, and only 18 patients had esophagoscopy. Habein 2 categorized 52 cases as traction diverticula; all were small (less than 3 cm) and 2 double diverticula were found. Hiatal hernia was associated in 16 patients, diffuse spasm in 4, and 1 had cardiospasm. It is probable that this categorization of traction diverticula is erroneous in light of current concepts of etiology. 8, 14, 15, 16 Effler ll found only 7 cases of LED at the Cleveland Clinic over an 8 year period. None was esophagoscoped, but 5 were thought to have hiatal hernia. Six patients were dysphagic and operated with the discovery of hypertrophic circular muscle in the esophagus distal to the diverticulum. The etiology of LED is considered to be neuromuscular dysfunction causing elevated pressure in the esophageal lumen. 8, 15 Diverticula occur within or above an area of tone increase or sphincteric opening delay. Diffuse esophageal spasm creates higher pressure zones between spastic segments with the development of pulsion diverticula (single or multiple) which appear as blowouts of esophageal mucosa through relatively weak areas of the muscle layer. 8 The wall of the diverticulum is composed of thickened mucosa, submucosa, muscularis mucosae and adventita but lacks support from the muscle layer. Spasm of the lower esophageal sphincter may be a more frequent cause of epiphrenic diverticula than diffuse spasm, with a high pressure area being generated above the sphincter in front of the oncoming peristaltic wave. 15 The association of LED with hiatal hernia has been emphasized. 2 ,lo,1l Proposedly, the hiatal hernia with regurgitation results in lower esophageal spasm and delayed esophageal emptying. A peptic stricture above a nonspastic but incompetent sphincter could enhance development of diverticula by providing a GASTROINTESTINAL ENDOSCOPY
35
similar distal obstruction. Kay 14 has alluded to the importance of sympathetic preponderance and vagal denervation in genesis of lower esophageal sphincter dyskinesias. The present case illustrates the occurrence of hiatal hernia with partial mechanical obstruction due to peptic stricture and an unusually large number of pulsion diverticula. Manometric study revealed lack of lower esophageal spasm or sphincter hyperactivity. It is possible that the observed failure of primary peristalsis reflected alcoholic neuropathy and that earlier in the patient's course elevated pressures of spasm might have been seen. Winshi p17 and Fischer 18 described deterioration of esophageal peristalsis in patients with alcoholic neuropathy manifested by selective deterioration of esophageal peristalsis, most pronounced in the distal esophagus, usually with preservation of sphincteric function. Vagal nerve degeneration alone does not seem to fully account for the observed abnormalities, and the role of alcoholic myopathy is obscure. Several excellent reviews of the symptoms and treatment of LED are available. 2 ,4,7,8,10 In our patient, the complete clinical relief afforded by conventional bougienage and a medical reflux-preventing regimen confirms the impression of others that LED per se uncommonly account for significant symptoms and that therapeutic measures directed at concomitant motility, inflammatory, or obstructive lesions are most pertinent. 2,3,7,8,10,14,15
REFERENCES 1. MACMILLAN A S: Statistical study of diseases of the esophagus. Surg Gynec Obstet 60:394-402, 1935 2. HABEIN H C, MOERSCH H ), KIRKLIN JW: Diverticula of the lower part of the esophagus. Arch Int Med (Chicago) 97:768-777, 1956 3. GOODMAN H I, PARNES I H: Epiphrenic diverticula of the esophagus. J Thorac Surg 23:145-159, 1952 4. VINSON P P: Diverticula of the thoracic portion of the esophagus-report of forty-two cases. Arch Otolaryng 19:508-513, 1934 5. GRANET E: Epiphrenic diverticulum of the esophagus with case report. Amer J Surg 19:259-262, 1933 6. TERRACOl ), SWEET R H: Diseases of the esophagus. Philadelphia. WB Saunders Company, 1958, pp 264-271 7. HABEIN H, KIRKLIN J W, CLAGETT 0 T, MOERSCH H ): Surgical treatment of lower esophageal pulsion diverticuli. Arch Surg (Chicago) 72:10181024,1956 8. BElSEY R: Functional disease of the esophagus. J Thorac Cardiovasc Surg 52:164-188,1966 9. MONDIERE I T cited by HARRINGTON S W: The surgical treatment of pulsion diverticula of the thoracic esophagus. Ann Surg 129:606-618, 1949 10. DEBAKEY M C, CREECH 0: Surgical treatment of epiphrenic diverticulum of the esophagus. Review of the literature and report of a case treated by resection and esophagogastroscopy. I Thorac Surg 23:486-494,1952 11. EFFLER D B, BARR 0, GROVES L K: Epiphrenic diverticulum of the esophagus. Arch Surg (Chicago) 79:459-467,1959 12. VESCIA F G: Corkscrew esophagus masquerading as multiple esophageal diverticula. Gastrointestinal endoscopy 15:108-109, 1968 13. LAHEY F H: Intrapleural pulsion esophageal diverticulum. Lahey Clinic 8uI/5:2-5,1946 14. KAY E B: The inferior esophageal constructor in relation to lower esophageal disease. J Thorac Surg 25:1-15,1953 15. CROSS F S, JOHNSON G F, GEREIN A N: Esophageal diverticula-associated neuromuscular changes in the esophagus. Arch Surg (Chicago) 83:525-533,1961 16. BRADHAM R R, SEALY W C: Neuromuscular disorders of the esophagus. Ann Thorac Surg 3:460-476, 1967 17. WINSHIP D H, CAFLISCH C R, ZBORALSKE F F, HOGAN W ): Deterioration of esophageal peristalsis in patients with alcoholic neuropathy. Gastroenterology 55:173-178, 1968 18. FISCHER R A, ELLISON C W, THAYER W R, 5PIRO H M, GLASSER G H: Esophageal motility in neuromuscular disorders. Ann Intern Med 63:229248,1965 VOLUME 19, NO.1, 1972
Counsel to Authors GASTROINTESTINAL ENDOSCOPY seeks to publish original papers describing investigations and significant observations relating to the various endoscopic technics employed in the study of digestive diseases. Descriptions of new instruments or methods, innovative applications of endoscopy, analyses of experience, and extraordinary case reports are welcome subjects. Readers who are piqued or prodded by published articles are encouraged to submit brief comments that the Editor may include in the "Reflex" section. Announcements of postgraduate courses, new facilities, cooperative studies and other items of interest to endoscopists are invited. Address typescripts and editorial correspondence to the editor. Typescripts should be submitted in duplicate on white paper of standard (8 112. x 11 inches) size. All copy, including footnotes, references, and captions, should be double spaced with generous margins. The title page should include the author's names, the institution where the work was performed, and the name and address to which requests for reprints should be sent. Illustrations, including photographs and charts, should be submitted as glossy prints not less than 5 x 7 inches, preferably of uniform size. These must be unmounted and clearly labeled on the back, lightly in pencil. to indicate first author's name, figure number, and top of illustration. Radiographs and endoscopic photographs must be of high contrast for proper reproduction. A reasonable number of pertinent illustrations in black and white can be allowed without charge. Color reproduction can be arranged at cost to the author. References should be indicated by consecutive numbers in the text and enumerated at the end of the paper in the same order. Each reference should conform to the following example: Scope GE, Optic F: What we saw. Gastroenterology 50: 100 I, 1962. All authors should be listed (not "et al."). Abbreviations of journal names are to be used as in Index Medicus. Helpful information pertaining to the preparation of scientific articles will be found in Style Manual for Biological Journals, Ed. 2, published by American Institute of Biological Sciences, 3900 Wisconsin Ave., N.W., Washington, D.C. 20016. Galley proofs will be sent to the author for necessary corrections with the understanding that they are to be promptly returned. Reprints should be ordered when galley proof is returned. A purchase form will be furnished. Authors are reminded to retain in their own files a copy of the typescript and illustrations submitted.