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A Akbar, N Reading, PD Fairclough, A McLean and RRSH Greaves Esophageal intramucosal pseudodiverticulosis an unusual cause of upper GI bleeding
A Akbar, N Reading, PD Fairclough, A McLean and RRSH Greaves, 03 October 2006
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Summary

A case of esophageal intramural pseudodiverticulosis (EIPD) in a 50-year-old diabetic presenting with an upper GI bleed is reported. Initial EGD was suggestive of an esophageal tumor, but biopsies were non-diagnostic. CT scan showed esophageal thickening suggestive of malignancy. Subsequent endoscopic ultrasound demonstrated the unusual condition of esophageal intramural pseudodiverticulosis, which was later confirmed by barium swallow examination.

EIPD is often accompanied by esophageal stricturing resulting in dysphagia, or candida esophagitis, but esophageal bleeding as a presentation is rare with 2 cases previously reported in the literature.

Case report

History
A 50-year-old man presented via casualty with a 2-day history of hematemesis and melena. He reported that he felt faint and dizzy, and had suffered from a previous duodenal ulcer 5 years ago. He had a history of type II diabetes mellitus and hypertension. He was a smoker of 20 cigarettes per day, and consumed 30 units of alcohol per week. He denied use of NSAIDs, history of abdominal pain, dyspepsia, dysphagia, anorexia or weight loss.

Examination
Examination revealed a fit-looking African-Caribbean gentleman who was cardiovascularly stable. Rectal examination revealed melena. No other abnormalities were found on physical examination. Laboratory studies revealed anemia with a hemoglobin level of 7.9 g/dl with a MCV of 79fL. Other laboratory results gave normal results.

Investigation and management
The patient was treated with oral omeprazole and was given a 2-unit blood transfusion. EGD performed the following morning was suggestive of an esophageal tumor extending from 33 to 43cm (Fig. 1). Biopsies taken at the time revealed active chronic inflammation.


Figure 1
EGD showing appearances suggestive of an esophageal carcinoma

A subsequent CT scan was also suspicious of an esophageal malignancy, with esophageal wall thickening extending from below the level of the carina to the gastroesophageal junction (Fig. 2). Repeat endoscopic biopsies again revealed squamous epithelium fragments with acute and chronic inflammatory infiltrate. No evidence of atypia or malignancy was evident.


Figure 2
CT Scan revealing esophageal wall thickening

An endoscopic ultrasound scan (EUS) was performed for further assessment. This demonstrated the appearance of esophageal intramural pseudodiverticulosis (EIPD), with very marked thickening of the mucosa and submucosa in the distal esophagus (Fig. 3). Biopsies taken at the time showed no evidence of malignancy, with inflammatory infiltration by lymphocytes, eosinophils and neutrophils, and the presence of candida infection. A subsequent barium swallow examination was consistent with the radiological appearance of EIPD (Figs. 4a and 4b).

The patient was treated with a 2-week course of oral fluconazole 100mg daily.


Figure 3 EUS with appearances suggestive of EIPD - marked thickening of the distal esophageal mucosa and submucosa.


Figure 4a Barium swallow 1.


Figure 4b Barium swallow 2 - multiple "flask-shaped" outpouchings typical of EIPD

Outcome
Following fluconazole treatment, the patient has remained well with no further episodes of GI bleeding. He reports very occasional mild intermittent dyshagia to solids.

Discussion

Mendl et al. [1] first described EIPD as a rare benign disease which results in the dilatation of esophageal submucosal glands. A total of 191 cases have been reported in the literature to date [2], and the condition has a slight male predilection (M: F 1.4:1). EIPD was picked up in 21 (0.15%) of 14350 patients undergoing radiological esophageal examinations [3]. At least 80% of patients are symptomatic, with the predominant symptom being intermittent or progressive dysphagia, as EIPD is often complicated by an esophageal stricture.

The etiology and pathogenesis of EIPD remains unknown. There is speculation that obstruction of submucosal gland ducts by inflammatory cells, desquamated epithelium and mucous, or stenosis of the ducts due to surrounding fibrosis from chronic esophageal inflammation, results in subsequent dilatation of the glands [4,5]. EIPD has been associated with reflux esophagitis, diabetes mellitus, and esophageal candidiasis. It is unclear whether candida is a contributing factor in the pathogenesis, or as is more likely, a secondary finding due to stasis. Motility disorders may predispose to EIPD; Sabanathan et al. [6] noted abnormal esophageal body motility in 30% of his series of patients with EIPD.

The imaging modality of choice is a barium swallow. This characteristically reveals flask-shaped out-pouchings in the esophageal wall, ranging from 1-4mm in length, and communicating with the lumen through narrow necks. In a review of 97 patients with EIPD by Sabanathan et al. [6], the pseudodiverticulae were found to be segmentally distributed in 59% patients and diffuse in 41%. There was radiological narrowing observed in 91% of the patients.

Endoscopic visualisation of the pseudodiverticulae is often difficult, and seen in approximately 25% of cases. Other endoscopic findings include stricturing, coexisting reflux esophagitis and candidiasis.

Esophageal biopsies tend to be unhelpful in the diagnosis, as the pseudodiverticulae are intramural in location and not usually present in the specimens taken endoscopically.

Esophageal manometry is often abnormal, but the findings are non-specific.

Management includes dilatation of the esophageal stricture, and treatment of any associated reflux or candida. The prognosis is good.

The typical presentation of EIPD is with dysphagia. However, in this case the patient presented with an upper GI bleed. According to the literature, there have only been 2 previous reports of EIPD presenting with bleeding [7,8].

In summary, EIPD, although rare, should be considered as a differential diagnosis in cases of dysphagia or esophageal stricture after other causes are excluded. A barium swallow is an important but perhaps under-used diagnostic tool.

This article was first published on GastroHep.com on 3 October 2006.

Authors

A Akbar1, N Reading1, PD Fairclough2, A McLean3 and RRSH Greaves1

1 Whipps Cross University Hospital, Department of Gastroenterology.
2 Bart's and the Royal London NHS Trust, Department of Gastroenterology.
3 Bart's and the Royal London NHS Trust, Department of Radiology.

Corresponding author

Dr A Akbar, Whipps Cross University Hospital, Department of Gastroenterology, London, United Kingdom. a.akbar@imperial.ac.uk

References

  1. Mendl K, McKay JM, Tanner CH. Intramural diverticulosis of the oesophagus and Rokitansky-Aschoff sinuses in the gallbladder. Br J Radiol 1960; 33: 496-501.
  2. Herter B, Dittler HJ, Wuttge-Hannig A, et al. Intramural pseudodiverticulosis of the esophagus: a case series. Endoscopy 1997; 29(2): 109-13.
  3. Levine MS, Moolten DN, Herlinger H, et al. Esophageal intramural pseudodiverticulosis: a re-valuation. AJR Am J Roentgenol 1986; 147(6): 1165-70.
  4. Boyd RM, Bogoch AB, Grei JH, et al. Esophageal intramural pseudodiverticulosis. Radiology 1974; 113(2): 267-70.
  5. Castillo S, Aburashed A, Kimmelman J, et al. Diffuse intramural pseudodiverticulosis. New cases and review. Gastroenterol 1977; 72(3): 541-5.
  6. Sabanathan S, Salama FD, Morgan WE. Oesophageal intramural pseudodiverticulosis. Thorax 1985; 40(11): 849-57.
  7. Hahne M, Schilling D, Arnold JC, et al. Esophageal intramural pseudodiverticulosis: review of symptoms including upper gastrointestinal bleeding. J Clin Gastroenterol 2001; 33(5): 378-82.
  8. Yamamoto N, Nakamura M, Tachibana S, et al. Esophageal intramural pseudodiverticulosis with Mallory-Weiss syndrome: report of a case. Surg Today 2002; 32(6): 519-22.

 
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