Dieulafoy's lesion of duodenum: a case report

Dieulafoy's lesion (DL) is an uncommon but important cause of gastrointestinal (GI) bleeding in which hemorrhage occurs from a pinpoint nonulcerated arterial lesion [1]. While the great majority of DL are located in the stomach within 6 cm of the gastro-esophageal junction the same occuring in other parts of the GI tract is rare [1‐3]. We report two cases of recurrent massive upper GI bleeding from DL in duodenum. The endoscopic appearance, pitfalls in the diagnosis of this lesion and its management have been discussed in this report.

Approximately 75 – 95 % DLs are found within 6 cms of the gastro-esophageal junction predominantly on the lesser curvature which is possibly related to the peculiar vascular anatomy of the stomach in this region [1]. In one large series from a tertiary care centre in India, of 900 cases of upper GI bleeding, DL was the cause in only six (0.67%) cases with the lesion being located within 6 cms of the GE junction in all cases [2]. Extragastric DLs are uncommon. In a review of over 100 cases of DLs, Veldhuyzen found no lesion of the duodenum [3]. Duodenal DL is rare and was reported for the first time in 1988 [4]. Similar lesions have also been described in the esophagus [5, 6], jejunum, colon and rectum [6‐8]. Extragastric DLs have been identified more frequently in recent years because of increased awareness of the condition [6, 7]. In a large series of 89 patients with DLs, in a third of cases the lesions were extragastric. Duodenum was the commonest location(18%) of extragastric DLs followed by colon(10%) and jejunum(2%) and esophagus(2%) [6]. The pathology of the lesion is essentially the same throughout the gastrointestinal tract and it is caused by an abnormally large calibre persistent tortuous submucosal artery [7].

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 [8]. The lesion in the first patient was missed initially. This was attributed to the tiny clot obscuring the lesion that had stopped bleeding. The surrounding normal mucosa and the deformed duodenal bulb misled us further. The correct diagnosis could be made only when endoscopy was carried out 24 hrs later during another episode of active bleeding. The endoscopic finding in the second patient i.e. punctate oozing from an otherwise normal mucosa is also quite characteristic of duodenal DL. We presume it would have been difficult to diagnose the lesion if the patient were not actively bleeding during the procedure.

DL is an inherently difficult lesion to diagnose and should be considered during evaluation of any patient with unexplained, recurrent, massive GI bleeding. History of NSAID intake, acid peptic disease or alcohol abuse is usually absent [1, 4]. The diagnosis at initial endoscopy in earlier reports was in only half the cases. A third of lesions were diagnosed at repeat endoscopy, the remainder identified either intraoperatively or on angiography. In recent series the identification at initial endoscopic examination has been in more than 90% of cases. This is not only due to early endoscopy which helps in identification but also due to increased awareness of the pathology and careful endoscopy which are the key to accurate diagnosis [1].

The lesions in both the patients were amenable to endoscopic adrenaline injection. Therapeutic endoscopy has evolved as the modality of choice for the initial treatment of DLs [1, 6, 9, 10]. Adrenaline injection has been used as sole therapy or in combination with other endoscopic modalities. The other endoscopic hemostatic techniques apart from adrenaline injection include bipolar and monopolar electrocoagulation, heater probe, laser photocoagulation, injection sclerotherapy, hemoclipping and endoscopic band ligation (EBL)[1, 6, 9]. The electro and thermocoagulation methods carry a risk of transmural injury in thin walled organs like duodenum and are better suited for gastric DL [1]. Some Japanese groups have also suggested the use of vascular clips as an effective and safe method of hemostasis in DLs in thin walled organs like duodenum. The normal surrounding mucosa in a DL makes it an adequate target for hemoclipping [11, 12]. Recently EBL has also been reported as an effective, safe, simple and inexpensive method for the treatment of DL [10, 13]. The bleeding vessel with the surrounding mucosa is sucked into the lumen of the ligator and the preloaded band is fired which strangulates the bleeding vessel and stops bleeding. Nikolaidis et al have reported successful control of bleeding in 96% (n = 22/23) patients with Dieulafoy like lesions who were treated by EBL [10]. Chung et al in a comparative study of efficacy of hemostatic methods in patients with DLs reported that mechanical methods for hemostasis like EBL and hemoclipping were more superior to injection methods in control of bleeding as well as preventing recurrent bleeding [14]. Surgical ligation is an alternative that can be considered for failed endoscopic therapy and in the present day scenario is required in less than 5% cases [1, 4, 10]. Angiography and 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 [15].

In conclusion, duodenal DL is an uncommon but important cause of recurrent and significant upper GI bleeding. Increased awareness and early endoscopy during a bleeding episode are essential for accurate diagnosis. Endoscopic adrenaline injection is an important technique to control the bleeding.