Dieulafoy’s lesion is named after the French surgeon Paul Georges Dieulafoy (1839 – 1911). The bleeding is thought to emanate from an abnormally dilated submucosal arteriole of 1 – 3 mm (1). The aetiology is unknown. One hypothesis is that the blood vessel defect is congenital and that the blood vessel is unable to be provided with calibre-reducing bifurcations (2). Bleeding occurs secondary to a macroscopically invisible erosion of the overlying mucous membrane. The diagnosis is made endoscopically by the presence of active arterial bleeding, visible blood vessel or a fresh adherent thrombus in macroscopically normal mucous membrane, or a small defect in the mucous membrane of a maximum of 3 mm (3).
Our patient had signs of chronic occult gastrointestinal bleeding, with acute deterioration. Only with the second gastroscopy was bleeding detected from an area of the duodenum which is not normally viewed properly with a gastroscope. With the aid of an ERCP scope a focus of bleeding was then detected that was consistent with Dieulafoy’s lesion. This is a rare but important condition that must be considered when faced with unexplained chronic or acute gastrointestinal bleeding. In up to 70 % of cases the lesion is discovered after the first gastroscopy (1). In the remainder of cases, as with our patient, repeat gastroscopies will normally be successful. Endoscopic ultrasound examination may be a diagnostic tool (2). The reason why the focus of bleeding is not found with the first gastroscopy may either be that the picture is impaired by a large quantity of blood (44 %) or because the lesion is not found (56 %) (1). Based on suspicion and local expertise a choice can then be made to continue with a repeat gastroscopy, balloon-assisted enteroscopy, capsule enteroscopy, angiography or technetium-labelled erythrocyte scintigraphy (2). Aside from this, there is little to be gained from radiological diagnostics (CT, MRI etc.).
Studies from hospital material show that Dieulafoy’s lesion is the cause of between 1 % and 5.8 % of acute gastrointestinal bleedings (4, 5). It is also assumed that the condition is underdiagnosed. Bleeding is often intermittent and can thus be difficult to detect. It presents mainly as haematemesis and/or melena, and in a minority of cases there may be fresh rectal bleeding (haematochezia). The defect most frequently occurs in the stomach sac, and usually on the lesser curvature within 6 cm of the junction of the oesophagus and stomach sac (1, 4). One third of the lesions are outside the stomach sac (1, 6). Although the duodenum is the second most frequent location, a focus on the descending part of the duodenum is very unusual (7). However, the lesion can occur in any area of the intestine and has also been described as occurring outside the intestine (1).
The patient group with Dieulafoy’s lesion closely resembles the group with peptic ulcer. In one study the average age of patients with Dieulafoy’s lesion was 67, and there was a preponderance of men (1, 4). In up to 90 % of the cases there are comorbid diseases such as ischaemic heart disease, renal failure, diabetes, hypertension and liver failure (6). Stomach pains with Dieulafoy’s lesion are, however, unusual (2), and no clear causal association with the use of NSAID drugs, acetylsalicylic acid or warfarin has been found (1, 4).
The main differential diagnoses for upper gastronintestinal bleeding are mentioned above. When an endoscopic diagnosis of Dieulafoy’s lesion is made, a bleeding peptic ulcer has in practice already been ruled out. Bleeding close to the papilla must be distinguished from bleeding emanating from the biliary tree (haemobilia), when blood will leak from the papilla. The latter type of bleeding is less usual, often resulting from trauma, and may be accompanied by stomach pain and jaundice (8).
Surgery was previously the only curative treatment for this condition and historical material describes a mortality rate of 80 % (1). Endoscopic treatment has now become the preferred treatment modality, and progress in the treatment has resulted in a fall in mortality to 8.6 % (1). Available endoscopic treatment alternatives can either be thermal therapy (electrocoagulation, argon plasma coagulation etc.), local injection therapy (epinephrine and sclerotherapy) or mechanical therapy (band ligation and haemoclipping). Injection and argon plasma coagulation were used in combination in our patient. Endoscopic haemostasis is described as occurring in over 90 % of the cases (4). Mechanical therapy is considered to be most effective (1). Use of at least two treatment methods has proven to reduce the rate of rebleeding (1). In one study, rebleeding occurred in seven of 39 patients within three days of successful primary haemostasis (9). In one review article the rate of rebleeding is considered to be between 9 % and 40 % (1). A repeat endoscopic treatment is preferred for rebleeding.
Endovascular detection and treatment of bleeding lesions is an alternative to endoscopic treatment (6). Surgical treatment is reserved for the 5 % of cases which fail to respond to endoscopic and/or endovascular treatment (1). Surgical treatment consists of either ligation or wedge resection of the lesion. Encouraging progress has been described involving laparascopic treatment and methods combining perioperative endoscopy and laparascopy, or laparoscopy following prior endoscopic tattooing of the lesion (1).
Bleeding from Dieulafoy’s lesion will often present diagnostic and therapeutic challenges. In the vast majority of cases, gastrointestinal endoscopy will provide the correct diagnosis and effective treatment. A study with a 28-month follow-up period has shown that the prognosis for those discharged from hospital following endoscopic haemostasis is excellent. (5). Mortality is primarily associated with comorbid conditions (4, 5).