Duodenal Dieulafoy’s Lesion: A Case Report

 T Siddiqui, C Parnaby, C MacKay

Department of General Surgery, The Southern General Hospital, Glasgow, Scotland, UK

Correspondence address: Tamim Siddiqui, Flat 6, 17 Dunaskin Street, Glasgow, G11 6PG

Email:  tamim_s@hotmail.co.uk

 

Abstract

Dieulafoy's lesion is an uncommon but important cause of recurrent upper gastrointestinal bleeding. Extragastric location of Dieulafoy's lesion is extremely rare. We report a case of upper gastrointestinal haemorrhage due to Dieulafoy's lesion of the duodenum and discuss the management of this extremely uncommon entity. 

Keywords: Duodenal Dieulafoy’s Lesion, Adrenaline Injection, Laparotomy

  

Introduction

Dieulafoy's lesion (DL) is an uncommon but important cause of gastrointestinal (GI) bleeding in which haemorrhage occurs from a pinpoint non-ulcerated arterial lesion1. While the great majority of DLs are located in the stomach within 6 cm of the gastro-oesophageal junction, the condition rarely occurs in other parts of the gastrointestinal tract1-3. We report an extremely rare  case of recurrent massive upper GI bleeding from DL in the duodenum. The endoscopic appearance, pitfalls in the diagnosis of this lesion and management strategies are discussed.

 

Case Report 

An 81 year old gentleman with a history of ischaemic heart disease and hypertension presented with a 12 hour history of repeated episodes of melaena. There was no history of alcohol abuse or non steroidal anti-inflammatory drug (NSAID) usage with the only significant findings on clinical examination being pallor, tachycardia and a haemoglobin of  8.6 gm/dl. Following a period of initial stability, he subsequently had massive fresh PR bleeding and melaena with haemodynamic compromise resistant to resuscitation . He therefore underwent emergency endoscopy, although this was abandoned due to significant concerns regarding patient instability. Following a further period of resuscitation with 8 units of packed red blood cells, 4 units fresh frozen plasma and 1 pool of platelets, his haemodynamic condition stabilised sufficiently to undergo emergency endoscopy. The main finding was of a bleeding point on the anterior wall of the first part of the duodenum with no evidence of ulceration. Endostasis was achieved via peri and intra lesional injection of 7ml 1:10,000 adrenaline. Unfortunately, despite initially improving post procedure, he had further profuse PR bleeding 4 hours later and immediately underwent repeat endoscopy. The findings were again similar to previous and satisfactory haemostasis was achieved via injection of a further 10mls of 1:10,000 adrenaline. The patient was stable post procedure and transferred to ITU in view of respiratory failure secondary to presumed aspiration pneumonia.

 

Following a 12 hour period of haemodynamic stability, he had further PR bleeding with an associated significant drop in haemoglobin and persistent tachycardia and hypotension. He was therefore again underwent endoscopy which revealed copious fresh blood in the duodenum obscuring any bleeding point. In view of his ongoing instability and lack of diagnosis and endoscopic therapeusis, laparotomy was thus performed via an upper midline incision. Following duodenotomy, an actively bleeding vessel was found at the junction of the first and second parts of the duodenum with surrounding normal mucosa. A diagnosis of duodenal DL was made and was controlled successfully via x4 2/0 vicryl sutures. The patient had no further episodes of upper GI haemorrhage and his recovery was complicated by prolonged respiratory failure and reliance on ventilatory support.

 

Discussion

In approximately 4-9% of massive upper gastrointestinal haemorrhage, no demonstrable cause can be found4-6. DL is thought to be the cause of acute and chronic upper gastrointestinal bleeding in approximately 1-2% of these cases3,8-10. The incidence, however, might vary from 0.5% to 14%, depending upon selection criteria11. It is thought to be more common in males (M:F = 2:1)10-14 with a median age of 54 years at presentation10-14. There is usually no significant NSAID or alcohol abuse15,16. The average transfusion requirement for initial resuscitation is between 3 and 8 units of packed red blood cells11,17.

 

The current consensus is that DL is caused by an abnormally large calibre persistent tortuous sub mucosal artery. This has been demonstrated by histological examination of resected specimens and by post-mortem examinations11,18,19.  The artery protrudes through a solitary, tiny mucosal defect (2-5 mm) which may rupture spontaneously and lead to massive bleeding20-22. It has been suggested that the thin mucosa overlying a pulsating artery is eroded progressively by the mechanical pressure from the abnormal vessel23,24. Histologically, the eroded artery appears normal. There is no evidence of any mucosal inflammatory process, signs of deep ulcerations, penetration of the muscularis propria, vasculitis, aneurysm formation, or atherosclerosis11,12,13,25 .

Approximately 75% to 95% of DLs are found within 6 cm of the gastro-oesophageal junction, predominantly on the lesser curve. Duodenal DL is extremely rare and was first reported in 198826. Lesions of similar morphological and histological features have been  found in the distal oesophagus, the jejunum the colon and the rectum.

 

The endoscopic criteria proposed to define DL are: 1) Active arterial spurting or micropulsatile streaming from a minute mucosal defect or through normal surrounding mucosa, 2) Visualization of a protruding vessel with or without active bleeding within a minute mucosal defect or through normal surrounding mucosa, and 3) Fresh, densely adherent clot with a narrow point of attachment to a minute mucosal defect or to normal appearing mucosa 27.

 

DL is an inherently difficult lesion to diagnose and should be considered during evaluation of any patient with unexplained, recurrent, massive GI bleeding. Upper GI Endoscopy can successfully identify the lesions in approximately 82% of patients. Approximately 49% of the lesions are identified during the initial endoscopic examination, while 33% require more than one OGD for confident identification3,9-11,16,28.The remainder of the patients with DL are identified intraoperatively or angiographically.10,29 In some recent series, however, the identification of the lesions was more accurate at the initial endoscopic examination, as DL were identified in up to 92.3%30 and 96.4%11 of cases. This has been attributed to the fact that endoscopy was performed soon after the admission of the patient, generally within the first two hours, allowing recognition of the lesion which was actively bleeding.31 It might also reflect, however, the increased awareness of the existence of a DL and the experience of the endoscopist. 

 In this case, a diagnosis was not made endoscopically, although temporary endostasis was achieved via adrenaline injection. Therapeutic OGD has evolved as the modality of choice for the initial treatment of Dieulafoy’s Lesions 1,32-34. Adrenaline injection has been used as sole therapy or in combination with other endoscopic modalities such as electrocoagulation, heater probe, laser photocoagulation, injection sclerotherapy, hemoclipping and endoscopic band ligation 1,32,33. Endoscopic management has now become the standard approach for the treatment of these lesions, with success rates reported to be as high as 95%34. Angiography with embolisation is another modality which has been reported in patients with active bleeding who are not amenable to endoscopic therapy and are poor surgical candidates 18. Laparotomy is an alternative that can be considered for failed endoscopic therapy and is required in less than 5% cases 1,26,34. In the pre endoscopic era, when patients were treated with surgery alone, the mortality rate approached 80% 13,16,18. However, in the above case where there was diagnostic dubiety, rebleeding and significant haemodynamic compromise, laparotomy was necessary and ultimately successful.

This case highlights that Duodenal DL is an uncommon but important cause of upper gastrointestinal bleeding. As portrayed by this case, endoscopic diagnosis of extragastric DL can be difficult because of the small size, obscure location of the lesion and lack of familiarity of the lesion. Increased awareness and careful and early endoscopic evaluation and adrenaline injection following the bleeding episode are the key to accurate diagnosis. Recent developments in Endoscopy have meant that fewer cases require laparotomy, although in the presence of significant haemodynamic compromise and diagnostic and therapeutic difficulty, laparotomy is essential. 

 

References

1.      Al-Mishlab T, Amin AM, Ellul JM. Dieulafoy's lesion: an obscure cause of GI bleeding. J R Coll Surg Edinb. 1999;44:222–225.

2.      Arora A, Mehrotra R, Patnaik PK, Pande G, Ahlawat S, Bhargava DK. Dieulafoy's lesion: a rare cause of massive upper gastrointestinal haemorrhage. Trop Gastroenterol. 1991;12:25–30

3.      Veldhuyzen van Zanten SJ, Bartelsman JF, Schipper ME, Tytgat GN. Recurrent massive hematemesis from Dieulafoy vascular malformation – a review of 101 cases. Gut. 1986;27:213–222

4.      Streicher HJ. Die solitare Exulceratio Simplex (Dieulafoy) als Ursache massiver Intestinasblutungen. Dtsch Med Wochenschr 1966; 91:991-5

5.      Palmer ED. The vigorous diagnostic approach to upper gastrointestinal tract haemorrhage. JAMA 1969; 207: 1477-80

6.      Cotton PB, Rosenberg MT, Waldram RPL, Axon ATR. Early endoscopy of oesophagus, stomach, and duodenal bulb in patients with haematemesis and melaena. Br Med J 1973; 2: 505-9

7.      Margreiter R, Weimann S, Reidler L, Schwamberger K. Die Exulceratio simplex Dieulafoy. Leber Magen Darm 1977; 7: 353-6

8.      Durham JD, Kumpe DA, Rothbart LJ, Van Stiegmann G. Dieulafoy disease: arteriographic finding and treatment. Radiology 1990; 174: 937-41

9.      Strong R. Dieulafoy disease: A distinct clinical entity. Aust N Z J Sur 1984; 54: 337-9

10.  Lin H, Lee F, Lee C, et al. Therapeutic endoscopy for Dieulafoy disease. Am J Gastroenterol 1989; 11: 507-10

11.  Baettig B, Haecki W, Lammer F, Jost R. Dieulafoy’s dis-ease: endoscopic treatment and follow up. Gut 1993; 34: 1418-21

12.  Fixa B, Komarca O, Dvorakova I. Submucosal arterial malformation of the stomach as a cause of gastrointestinal bleeding. Gastroenterologica 1966; 105: 357-65

13.  Jules GL, Labitzke HG, Lamb R, et al. The pathogenesis of Dieulafoy’s gastric erosion. Am J Gastroenterol 1984; 79: 195-200

14.  Rossi NP, Green EW, Pike JD. Massive bleeding of the upper gastrointestinal tract due to Dieulafoy erosion. Arch Surg 1968; 97: 797-80

15.  Dy NM, Gostout CJ, Balm RK. Bleeding from the endoscopically-identified Dieulafoy lesion of the proximal small intestine and colon. Am J Gastroenterol 1995; 90: 108-11

16.  Reilly HF, Al-Kawas FH. Dieulafoy lesion: Diagnosis and management. Dig Dis Sci 1991; 36:1702-7

17.  Sheider DM, Barthel J S, King PA, Beale G D. Dieulafoy-like lesion of the distal oesophagus. Am J Gastroenterol 1994; 89: 2080-1

18.  Katz PO, Salas L. Less frequent causes of upper gastrointestinal bleeding. Gastroenterol Clin Nor Amer 1993; 22: 875-89

19.  Miko TL, Thomazy VA. The caliber persistent artery of the stomach: a unifying approach to gastric aneurysm, Dieulafoy’s lesion and submucosal arterial malformation. Hum Path 1988; 19: 914-21

20.  Strong R. Dieulafoy disease: A distinct clinical entity. Aust N Z J Sur 1984; 54: 337-9

21.  Broberg A, Ihre T, Pyk E. Raaschou-Nielson T. Exulceration simplex as a conceivable cause of massive gastric haemorrhage. Surg Gynecol Obstet 1982; 154: 186-8

22.  Asaki S, Sato Y, et al. Endoscopic diagnosis and treat-ment of Dieulafoy’s ulcer. Tohoku J Exp Med 1988; 154: 135-41

23.  Barbier P, Luder P, Triller J, Ruchti CH, Hassler H, Stafford A. Colonic haemorrhage from a solitary minute ulcer. Report of three cases. Gastroenterology 1985; 88: 1065-8

24.  Miko TL, Thomazy VA. The caliber persistent artery of the stomach: a unifying approach to gastric aneurysm, Dieulafoy’s lesion and submucosal arterial malformation. Hum Path 1988; 19: 914-21

25.  Chapman I, Lapi N. a rare cause of gastric haemorrhage. Arch Int Med 1963; 112: 347-51

26.  McClave SA, Goldschmid S, Cunningham JT, Boyd WP Jr. Dieulafoy's cirsoid aneurysm of the duodenum. Dig Dis Sci. 1988;33:801–805.

27.  Dy NM, Gostout CJ, Balm RK. Bleeding from the endoscopically-identified Dieulafoy lesion of the proximal small intestine and colon. Am J Gastroenterol. 1995;90:108–111

28.  Hoffmann J, Beck H, Jensen HE. Dieulafoy’s lesion. Surg Gynecol Obstet 1984; 159: 537-40

29.  Helliwell M , Irving JD. Haemorrhage from gastric artery aneurysms. Br Med J 1981; 282: 460-1

30.  Parra-Blanco A, Takahashi H, Mendez-Jerez PV, et al. Endoscopic management of Dieulafoy lesions of the stomach: a case study of 26 patients. Endoscopy 1997; 29: 834-9

31.  Franko E, Chadavoyne R, Wise L. Massive rectal bleeding from a Dieulafoy’s type ulcer of the rectum: a review of this unusual disease. Am J Gastroenterol 1991; 86: 1545-7

32.  Norton ID, Petersen BT, Sorbi D, Balm RK, Alexander GL, Gostout CJ. Management and long-term prognosis of Dieulafoy lesion. Gastrointest Endosc. 1999;50:762–767

33.  Goldenberg SP, DeLuca VA Jr, Marignani P. Endoscopic treatment of Dieulafoy's lesion of the duodenum. Am J Gastroenterol. 1990;4:452–454

34.  Nikolaidis N, Zezos P, Giouleme O, Budas K, Marakis G, Paroutoglou G, Eugenidis N. Endoscopic band ligation of Dieulafoy-like lesions in the upper gastrointestinal tract. Endoscopy. 2001;33:754–760.

 

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