Esophageal Intramural Diverticulosis

Transcription

1 Esophageal Intramural Diverticulosis A Clinical and Pathological Survey John W. Hammon, Jr., M.D., Reed P. Rice, M.D., Raymond W. Postlethwait, M.D., and W. Glenn Young, Jr., M.D. ABSTRACT This paper presents 3 patients with esophageal intramural diverticulosis and discusses the roentgenographic, manometric, histological, and microbiological picture attendant to this disease. Evidence is given to support chronic infection of esophageal submucosal glands as the predominant cause. I n the patient complaining of dysphagia, an interesting condition has recently been encountered which is characterized by the appearance on barium swallow examination of numerous outpouchings in the wall of the esophagus extending a few millimeters from the margin of the lumen. Mendl, McKay, and Tanner [5] were the first to report this entity in the literature, which they termed intramural diverticulosis of the esophagus. Since 1960, 6 additional patients have been reported [l, 3, All the previous communications have appeared in the radiology literature. The purpose of this article is to describe the pathophysiology of this disease process and to link it to the patient presenting with dysphagia. Case Reports Patient 1. A 19-year-old white man presented himself to Duke University Medical Center in 1971 with a history of rapidly increasing dysphagia which caused regurgitation after practically every meal. The patient had a past history of allergy and various hypersensitivity phenomena which had required several periods of desensitization, and he said he had had difficulty swallowing since childhood. Roentgenographic examination of the esophagus demonstrated a definite 3- to 4-cm. stricture of the cervical esophagus and a generalized lack of distensibility of the esophagus that was associated with a hiatal hernia (Fig. 1A). At esophagoscopy a weblike stricture at the proximal end of the cervical esophagus was found, and this was fractured with the esophagoscope and biopsied. The esophageal mucosa appeared red and granular, and esophageal biopsy showed chronic esopha- From the Department of Surgery, Division of Thoracic Surgery, and the Department of Radiology, Duke University Medical Center, Durham, N.C. Presented at the Twentieth Annual Meeting of the Southern Thoracic Surgical Association. Louisville, Ky., Nov. 1-3, Address reprint requests to Dr. Young, Department of Surgery, Duke University School of Medicine, Durham, N.C THE ANNALS OF THORACIC SURGERY

2 Esophageal Zntramural Diverticulosis A B C FIG. 1. (A) Barium swallow examination of Patient 1 shows an irregular ceruical stricture without diverticula. (B) Repeat examination seven months later shows multiple diverticulalike spaces in a thick esophageal wall (arrows). (C) Recurrence of intramural diverticula in Patient 1 after hiatal hernia repair (arrows). gitis. Cultures of the esophagus taken at esophagoscopy grew Proteus mirubilis and anaerobic mouth flora. Seven months after the first esophagoscopy a repeat barium swallow examination showed multiple tiny projections of barium along the course of the entire esophagus (Fig. 1B). The generalized lack of distensibility of the esophagus was more prominent, and a stricture was present along with the hiatal hernia. The patient at this time was admitted to the hospital and underwent a Belsey hiatal hernia repair. Since that time he has undergone regular esophageal dilation to control strictures at both the cervical and the diaphragmatic esophagus. The immediate postoperative barium swallow examination showed no diverticula. However, a follow-up study done six months VOL. 17, NO. 3, MARCH,

3 HAMMON ET AL. after operation showed a recurrence of these structures (Fig. 1C). He has been symptomatically improved with dilation, and the most recent barium swallow roentgenogram showed no diverticula. Recently performed esophageal manometric studies showed normal peristalsis and normal resting pressures in both the upper and lower esophageal sphincters. Patient 2. A 73-year-old black man was admitted to Duke Hospital on March 30, 1973, with acute dysphagia. The patient had a history of gradually increasing dysphagia, and three days prior to his admission a piece of meat lodged in his throat and he no longer was able to swallow food or liquid. He was admitted to his regional hospital, where a barium swallow examination showed barium aspiration and complete obstruction of the upper thoracic esophagus with numerous outpouchings in the proximal esophagus and a cervical esophageal stricture (Fig. 2A). The patient was referred to Duke Hospital, where esophagoscopy was undertaken. At that time the meat bolus had passed; however, the esophagoscope was moved with great difficulty through the cervical esophagus. The esophagus showed evidence of chronic esophagitis with a rough, granular mucosal surface, and there was an area of extrinsic compression of the upper thoracic esophagus, presumably from the aortic arch. Repeat barium swallow examination showed complete resolution of the esophageal diverticula; however, the presence of the cervical esophageal stricture and compression of the midportion of the thoracic esophagus from a dilated aortic arch were noted (Fig. 2B). There were prominent tertiary contraction waves running through the esophagus with some obstruction of barium seen on the fluoroscopic study, and a generalized lack of distensibility was noted. Cultures of the esophagus taken at that time showed Candida albicuns. Diabetes mellitus was discovered on a blood chemistry study and was treated with diet and oral medication. Esophageal motility studies demonstrated a lack of normal peristalsis with a high-pressure zone in the upper esophagus. The patient was followed with regular esophageal dilations and has since done we1 1. Patient 3. A 70-year-old black woman presented herself to Duke University Medical Center on August 28, 1973, with a one-year history of dysphagia. On several occasions prior to admission swallowed food would stick in her esophagus and would gradually pass over the succeeding twelve hours. She noted that meat lodged in different places on different occasions. The patient was being treated with oral medication for diabetes she had developed as an adult. Barium swallow examination showed a very nonmotile, nondistensible esophagus with tiny pouchlike projections from the wall of the esophagus consistent with esophageal intramural diverticulosis (Fig. 2C). At esophagoscopy the lumen of the esophagus was rough and appeared to be chronically inflamed. Esophageal biopsy showed chronic esophagitis. There was a diffuse area of stricture in the upper thoracic esophagus which would barely admit 262 THE ANNALS OF THORACIC SURGERY

4 Esophageal Intramural Diverticulosis A B C FIG. 2. (A) Large outpouchings in the wall of the completely obstructed esophagus of Patient 2 with a barium tracheogram secondary to aspiration. (B) Resolution of intramural diverticula in Patient 2 with evidence of ceruical esophageal web and upper thoracic extrinsic compression. (C) Tiny esophageal diverticula in Patient 3. the esophagoscope. Cultures of the esophagus showed Pseudomonas aeruginosa and A erobacter species. Esophageal manometric studies showed a rigid esophagus with many nontransmitted swallows and an incompetent lower esophageal sphincter. The patient was treated with esophageal dilation, and a repeat barium swallow examination two weeks later showed complete resolution of the intramural diverticula. Clinical Findings ROENTGENOGRAPHY In each of the 3 patients the roentgenogram of the barium-filled esophagus showed multiple tiny outpouchings similar to diverticula along the VOI,. 17, NO. 3, MARCH,

5 HAMMON ET AL. course of the esophagus. In Patient 2 the barium projections varied from 1 to 3 mm. in size and were not very uniform in magnitude or distribution. In Patients 1 and 3 the intramural diverticula were more uniformly 0.5 to 1 mm. in size and were evenly distributed along the course of the esophagus. In addition to these diverticula there was a generalized loss of the normal distensibility of the esophagus which was conspicuous fluoroscopically in all patients. In addition, upper esophageal webs or strictures were present in all patients, and 1 patient had a hiatal hernia. ESOPHAGEAL MOTILITY STUDIES Esophageal motility in Patient 1 was normal, perhaps reflecting successful therapy for his underlying condition. Patient 2 demonstrated a highpressure zone in the upper esophagus, presumably secondary to a stenosis caused by the aortic arch. There was also a generalized loss of normal motility with numerous irregular, noncoordinated peristaltic movements. In the third patient the esophagus was stiff with many nontransmitted peristaltic movements and an incompetent lower esophageal sphincter. HISTOLOGY Biopsies in all 3 patients showed chronic esophagitis with infiltration of the submucosa of the esophagus by round cells and generalized fibrosis of the esophageal wall. In a living patient a full-thickness biopsy of the esophageal wall cannot be done, and it is necessary, therefore, to rely on other specimens. One of our group (R. W. P.) has performed serial full-thickness sections of specially prepared esophagi obtained at postmortem examination. In examining these routinely obtained specimens, the esophageal glands are observed (Fig. 3). These glands lie in the submucosa of the esophagus and are termed by FIG. 3. An example of a true, or submucosal, esophageal gland. (H&E, XIO; reduced 30% for reproduction.) 264 THE ANNALS OF THORACIC SURGERY

6 Esop hageal Intramural Diverticulosis FIG. 4. The duct of an esophageal submucosal gland. (HbE, ~10; reduced 3v0 for reproduction.) Goetsch [Z] as the submucous, deep, or true esophageal glands. These glands tend to occur in longitudinal rows parallel to the long axis of the esophagus. With reference to the axis of the esophagus, the ducts are arranged obliquely in the general direction downward toward the stomach. Each duct branches at its termination into two to five secondary ducts, each of which enters the lobule (Fig. 4). These glands, although they are found throughout the whole length of the esophagus, are more abundant in the upper half. Routine postmortem investigation has shown that the ducts of many of these glands, at the point where they open into the esophagus, can become dilated and filled with inflammatory material (Fig. 5). It is not difficult to imagine FIG. 5. The dilated, chronically inflamed duct of an esophageal submucosal gland impacted with inflammatory material. (HbE, xio; reduced 3Oy0 for reproduction.) VOL. 17, NO. 3, MARCH,

7 HAh4MON ET AL. FIG. 6. An example of monilial esophagitis with multiple superficial ulcerations on barium swallow roentgenographic examination. these tubular glands being impacted with inflammatory material secondary to stasis or distal obstruction in the esophagus and consequently becoming dilated and inflamed, producing the characteristic outpouching seen on roentgenographic examination of the esophagus. DIFFERENTIAL DIAGNOSIS The principal differential diagnosis pertinent to this discussion is monilial esophagitis. This condition presents characteristic longitudinal esophageal ulcers seen on barium swallow roentgenogram (Fig. 6) and at esophagoscopy [4]. Candidu albicuns can usually be cultured from the esophagus or oropharynx. This condition is most prevalent in elderly or debilitated patients and is not usually associated with esophageal strictures or spasm. Tuberculous esophagitis can present with esophageal obstruction and is most often associated with far-advanced pulmonary disease. Caustic esophagitis could present a similar clinical picture in a patient with an obtunded or incompetent esophagus. 266 THE ANNALS OF THORACIC SURGERY

8 Comment Esophageal Intramural Diverticulosis In this small series of patients and in the published literature, esophageal intramural diverticulosis has been seen to present with dysphagia and is often associated with cervical esophageal web [8], reflux esophagitis with or without stricture [7], and disturbances of motility. The most reasonable explanation for the disease process, however, lies not in the production of esophageal diverticula but in a severe form of chronic esophagitis with specific involvement of esophageal glands. Stasis in the esophagus as a result of distal obstruction, reflux esophagitis, or motility disturbances could lead to the overgrowth of pathogenic bacteria or fungi with secondary infection and dilatation of esophageal submucosal glands. These dilated glands would give rise to the characteristic outpouchings seen on barium swallow roentgenographic examination. In all our patients and in 1 mentioned in the literature [6], pathogenic microorganisms were cultured from the interior of the esophagus. The term esophageal adenitis might be a more accurate description of the attendant pathology. Therapy for this condition should be directed at relieving esophageal obstruction whenever possible and managing reflux by indicated medical or surgical therapy. We have not used antibiotics in our patients, although specific therapy to eradicate culture-proved organisms might be helpful in those with persistent disease. We must assume that long-term follow-up is necessary, as our first patient s lesion has recurred at least once. References 1. Culver, G. T., and Chaudhari, K. R. Intramural esophageal diverticulosis. Am. J. Roentgenol. Radium Ther. Nucl. Med. 99:210, Goetsch, E. The structure of the mammalian esophagus. Am. J. Anat. lo:l, Hodes, P. J., Atkins, J. P., and Hodes, B. L. Esophageal intramural diverticulosis. Am. J. Roentgenol. Radium Ther. Nucl. Med. 96:411, Kaufman, S. A., Scheff, S., and Levene, G. Esophageal moniliasis. Am. J. Roentgenol. Radium Ther. Nucl. Med. 75:726, Mendl, K., McKay, J:M., and Tanner, C. H. Intramural diverticulosis of the esophagus and Rokitansky-Aschoff sinuses in the gallbladder. Br. J. Radiol. 33:496, Troupin, R. H. Intramural esophageal diverticulosis and moniliasis. Am. J. Roentgenol. Radium Ther. Nucl. Med. 104:613, Weller, M. H., and Lutzker, S. A. Intramural diverticulosis of the esophagus associated with postoperative hiatal hernia, alkaline esophagitis and esophageal stricture. Radiology 98:373, Zatzkin, H. R., Green, S., and LaVine, J. J. Esophageal intramural diverticulosis. Radiology 90: 1193, VOL. 17, NO. 3, MARCH,