C 2004) Digestive Diseases and Sciences, Vol. 49, No. 9 (September 2004), pp. 1556–1559 (
CASE REPORT
Esophageal Intramural Pseudodiverticulosis: A Case of a Young Woman NATSUKO TATSUMI, MD, SHOJI MITSUFUJI, MD, PhD, YOICHI TATSUMI, MD, PhD, NAOKI WAKABAYASHI, MD, PhD, HIDEYUKI KONISHI, MD, PhD, KEISHO KATAOKA, MD, PhD, and TAKESHI OKANOUE, MD, PhD KEY WORDS: esophageal intramural pseudodiverticulosis; dysphagia; endoscopic balloon dilation therapy.
Esophageal intramural pseudodiverticulosis (EIPD) is an uncommon benign abnormality, characterized by a typical radiological appearance of multiple flask-shaped outpouchings in the esophageal wall, measuring a few millimeters in length. EIPD is mainly a disease of the elderly and is more frequent in males. It presents with progressive dysphagia related to esophageal stenosis or strictures in the great majority of patients. The etiology of this rare disease is unknown. Hiatal hernias, gastroesophageal reflux, esophageal strictures, candidiasis, herpes esophagitis, diabetes mellitus, and chronic alcoholism have been found to be associated with EIPD. According to the available literature, EIPD can be missed easily because of its subtle endoscopic and radiological abnormalities, but once it is diagnosed, conservative management leads to satisfactory control of the symptoms. We encountered a young female patient with EIPD. She had a severe stricture in the upper esophagus, although she had no other complications such as candidiasis or reflux esophagitis. Here we report this rare case of a young patient with EIPD, who was treated successfully with endoscopic esophageal balloon dilation therapy, and discuss this disease on review of the relevant literature. CASE REPORT A 29-year-old woman presented to our hospital with dysphagia after eating fish. Her chief complaint was the sensation of Manuscript received April 21, 2004; accepted June 3, 2004. From the Department of Molecular Gastroenterology and Hepatology, Kyoto Prefectural University of Medicine, Graduate School of Medical Science, Kyoto, Japan. Address for reprint requests: Natsuko Tatsumi, MD, Department of Molecular Gastroenterology and Hepatology, Kyoto Prefectural University of Medicine, Graduate School of Medical Science, 456 Kajii-cho, Kawaramachi Hirokoji, Kamigyo-ku, Kyoto, Kyoto 604-0911, Japan;
[email protected].
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solid food (fish) getting stuck in her throat, since the previous day, and that she was not able to swallow water. She had occasionally felt dysphagia for at least 15 years before the consultation and had experienced similar episodes in the past which had resolved spontaneously after causing discomfort for a few hours. Physical examination showed no abnormalities and laboratory findings were within normal limits. There was no history of gastroesophageal reflux diseases or of accidental ingestion of corrosives. The patient underwent an urgent esophagoscopy. After the removal of a piece of fish, esophageal stricture was evident. The stricture was so narrow that we were unable to pass the endoscope beyond it. No evidence of malignancies, but an inflammatory infiltrate consisting of lymphocytes, was found in biopsy specimens of mucosa taken from the area of the stricture. Esophagogram with water-soluble contrast revealed that the stricture was about 6 cm long in the upper thoracic esophagus. Further barium study (a week later) showed numerous fine barium-filled projections in the narrowed portion. A closer view of the esophagogram showed small outpouchings with narrow necks projecting up to a few millimeters in length from the esophageal lumen; the larger ones were flask-shaped with a flattened base (Figure 1). We went through the stricture with a fine endoscope (6.5 mm outside diameter; GIF-XQ260; Olympus, Tokyo) and the examination showed several longitudinal lines of multiple small dimples which represented small orifices of the pseudodiverticula (Figure 2). Indigo carmine spray revealed the findings more clearly (Figure 3). In addition, hiatal hernia or reflux esophagitis was not pointed out in this examination. Based on these characteristic findings, we diagnosed her illness as EIPD. In order to improve the dysphagia, the patient was subjected to endoscopic balloon dilatation. Balloon dilatation was initiated at 8 mm with a distention pressure of 3 atm, and finally, performed up to 10 mm at 5 atm. After the dilatation, the patient was free of dysphagia. Although there was residual narrowing of the esophagus, which was 8 mm in diameter on a follow-up esophagogram done a week after dilatation therapy, the patient had no complaint of dysphagia, so further treatment was not required.
DISCUSSION EIPD is an uncommon abnormality, characterized by a typical radiological appearance of multiple flask-shaped Digestive Diseases and Sciences, Vol. 49, No. 9 (September 2004)
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Fig 1. A barium esophagogram showing the stricture with characteristic multiple, small, flask-shaped, and often branching outpouchings in the upper thoracic esophagus (arrowheads).
outpouchings in the esophageal wall, measuring a few millimeters in length. Occasionally, they show branching or bridging between adjacent diverticula (1, 2). This entity was first described by Mendl et al. in 1960 as “intramural diverticulosis” (3), but the term diverticulosis did not represent this abnormality precisely, so it was renamed intramural “pseudodiverticulosis” by Boyd et al. in 1974 (4). Since then more than 220 cases have been reported. Sabanathan et al. (5) reviewed the largest number of Digestive Diseases and Sciences, Vol. 49, No. 9 (September 2004)
97 EIPD patients. According to the review, 56 cases (58%) were male, and the distribution of pseudodiverticula was segmental in 57 patients (59%) and diffuse in 40 (41%). The stricture which caused dysphagia was found in 88 cases; 39 (44%) were in the upper esophagus, 20 (23%) in the middle esophagus, and 29 (33%) in the lower esophagus. The age of the patients ranged from 8 months to 86 years and the mean was 53.5 years. EIPD can occur at any age, but its incidence has two peaks: the pediatric group and the senior group (50 to 70 years old).
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Fig 2. Endoscopy showing several longitudinal lines of multiple small dimples which represented small orifices of the pseudodiverticula (arrowheads).
The precise etiology and pathogenesis of EIPD are not yet well known. In several reports of autopsy series (4,6,7), pathological examination showed that the pseudodiverticula in EIPD corresponded to dilated excretory ducts of submucosal mucous glands, and submucosal inflammation and fibrosis resulted in the thickening of the esophagus wall. The exact reason for the dilation of excretory ducts is unknown, but most investigators have considered that the initiating event is chronic inflammation of the submu-
Fig 3. Indigo carmine spray revealing the orifices of the pseudodiverticula more clearly (arrowheads).
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cosal esophageal glands in response to a chronic irritation caused by various factors (e.g., chemical, thermal, and infective factors). On the other hand, it is open to question whether the cause in young patients is the same as that in senior patients. As stated above, there are two peaks of incidence. Although most senior patients have some complications, such as GERD, diabetes, alcoholic abuse, and candidiasis, most young patients have no evidence of chronic inflammation. It is unlikely that chronic inflammation occurs in a case of infants less than 1 year old. Increased intraluminal pressure which results from esophageal hypermotility has been suggested as another possible cause of EIPD, but several studies with manometry have not yet proved a clear association (9–11). In our case, the patient had no complications, and on endoscopy, there was no esophagitis caused by GERD or candida. The fact that the patient had experienced dysphagia since childhood proves that pseudodiverticula and stricture had existed since then. The incidence of EIPD varies among those reports. According to a report by Plavisc et al. (12), in which they analyzed retrospectively barium esophagograms of 245 patients with esophageal carcinoma and 6400 randomly selected esophagograms, EIPD was found in 11 patients with esophageal carcinoma (4.5%) and in 6 control subjects (0.09%). Levine et al. (9) reported that EIPD was diagnosed in 21 (0.15%) of 14,350 patients undergoing radiological examination. On the other hand, there was a report in 2002 that the incidence of EIPD is approximately 4 cases per 10,000 upper endoscopies (0.04%) (13). Furthermore, Medeiros et al. (7) reported that duct dilatations and cysts were found in 14 and 7% of random esophageal sections of autopsies and that more systematic examination revealed that duct dilatations were found in 55% and cyst formation in 15% of the 20 cases. These findings suggest that the earliest changes in EIPD are commonly present but can be missed easily because of their endoscopic and radiological subtlety. The majority of asymptomatic EIPD patients cannot be diagnosed precisely. Even patients with dysphagia are probably misdiagnosed. There may be more cases of asymptomatic EIPD than we have supposed. EIPD is essentially a benign disease, thus the clinical course is generally good, although several serious complications such as perforation, fatal mediastinitis, and Mallory–Weiss syndrome have been reported (5,17,18). Treatment of pseudodiverticulosis is usually directed at the underlying, associated condition rather than at the pseudodiverticulosis itself. In cases with candidiasis or reflux esophagitis, antimycotic treatment or administration of proton pump inhibitors is usually quite effective. The stricture which causes dysphagia is usually treated Digestive Diseases and Sciences, Vol. 49, No. 9 (September 2004)
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successfully with one or two dilatations, but a case of a patient who underwent an esophagotomy because of failure of endoscopic dilatation has been reported (19). There are cases complicated by life-threatening hemorrhage and retroesophageal collection after dilatation therapy (20), so careful dilatation with a guidewire or endoscopy is recommended to avoid perforation and other complications. In our case, we performed endoscopic balloon dilatation twice without complications, and her symptom was improved. REFERENCES 1. Bruhlmann WF, Zollikofer CL, Maranta E, et al.: Intramural pseudodiverticulosis of the esophagus: Report of seven cases and literature review. Gastrointest Radiol 6:199–208, 1981 2. Canon CL, Levine MS, Cherukuri R, Johnson LF, Smith JK, Koehler RE: Intramural tracking: A feature of esophageal intramural pseudodiverticulosis Am J Roentgenol 175:371–374, 2000 3. Mendl K, McKay JM, Tanner CH: Intramural diverticulosis of the oesophgus and Rokitansky-Ashoff sinuses in the gall-bladder. Br J Radiol 33:496–501, 1960 4. Boyd RM, Bogoch A, Greig JH, Trites AE: Esophageal intramural pseudodiverticulosis. Radiology 13:267–270, 1974 5. Sabanathan S, Salama FD, Morgan WE: Oesophageal intramural pseudoduverticulosis. Thorax 40:849–857, 1985 6. Umlas J, Sakhuja R: The pathology of esophageal intramural pseudodiverticulosis. Am J Clin Pathol 65:314–320, 1976 7. Medeiros LJ, Doos WG, Balogh K: Esophageal intramural pseudodiverticulosis: A report of two cases with analysis of similar, less extensive changes in “normal” autopsy esophagi. Hum Pathol 19:928–931, 1988 8. Wightman AJA, Wright EA: Intramural esophageal diverticulosis: A correlation of radiological and pathological findings. Br J Radiol 47:496–498, 1974
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9. Levine MS, Moolten DN, Herlinger H, Laufer I: Esophageal intramural pseudodiverticulosis: A reealuation. Am J Roentgenol 147:1165–1170, 1986 10. Dua KS, Stewart E, Arndorfer R, Shaker R: Esophageal intramural pseudodiverticulosis associated with achalasia. Am J Gastroenterol 91:1859–1860, 1996 11. Ritz JP, Germer CT, Zimmer T, Isbert C, Buhr HJ: Esophageal hypermotility associated with intramural pseudodiverticulosis. Primary esophageal disease or epiphenomena? Surg Endosc 14:681, 2000 12. Plavisc BM, Chen MYM, Gelfand DW: Intramulal pseudodiverculosis of the esophageal detected on barium esophagograms: Increased prevalence in patient with esophageal carcinoma. Am J Roentgenol 165:1381–1385, 1995 13. Chaudhary N, Harty RF, Mesiya SA: Clinical and endoscopic features of patients with esophageal intramural pseudodiverticulosis (EIP). Am J Gastroenterol 97:S30-S31, 2002 14. Pearlberg JL, Sandler MA, Madrazo BL: Computed tomographic features of esophageal intramural pseudodiverticulosis. Radiology 147:189–190, 1983 15. Garramone GJ, Mathur C, Weltman DI, Hadjiyane C: Endoscopic ultrasound of esophageal intramural pseudodiverticulosis. Am J Gastroenterol 96:S41, 2001 16. Fukuyama T, Kimura T, Osaki Y, et al.: A case report of esophageal intramural pseudodiverticulosis. Jpn J Med Ultrasonics 23(Suppl), 1996 17. Murakami M, Tsuchiya K, Ichikawa H, et al.: Esophageal intramural pseudodiverticulosis associated with esophageal perforation. J Gastroenterol 35:702–705, 2000 18. Yamamoto N, Nakamura M, Tachibana S, Konno H, Nakamura S, Nishino N: Esophageal intramural pseudodiverticulosis with Mallory-Weiss syndrome: Report of a case. Surg Today 32:519– 522, 2002 19. Elabsi M, Echarrab M, Oudanane M, el Ounani M, Chkoff R, Zizi A: Intramural pseudodiverticulosis of the esophagus. A case report and review of the literature. Ann Chir 127:637–640, 2002 20. Evans PR: Oesophgeal intramural pseudodiverticulosis—Always benign? Aust NZJ Med 21:58–61, 1991
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