Dieulafoy lesion: two pediatric case reports

Dieulafoy lesion (DL) is an extremely rare cause of massive and repeated upper gastrointestinal (GI) bleeding in children, which may lead to consequences ranging from anemia to hypovolemic shock [1]. On microscopic examination, the lesion consists of the submucosal, abnormally large arterial vessel that has protruded through a 2-to-5 mm long mucosal defect. Bleeding starts from the small mucosal vessels and is followed by erosion of the vein. The artery tends to rupture soon afterward, resulting in massive bleeding [2]. The pathogenesis of vessel rupture and hemorrhage is uncertain but may be due to necrosis of the vessel wall induced by chronic gastritis [3].

DL is typically located in the fundus and in the small curvature of the stomach and only rarely in the distal part, with 80 to 95% of lesions occurring within 6 cm from the gastroesophageal junction. However, DL can be found along all the GI tract as well as in extra-GI sites such as the bronchial tree [4].

The incidence of DL in children remains undefined. DL has a male predominance (M:F 1.5:1) and it can occur at any age of life, starting from birth, which supports the proposed congenital origin of the disease [5].

Clinical features depend on the location of the lesions. Specifically, massive upper GI bleeding occurs with gastric and duodenal lesions. Small intestinal lesions lead to upper GI bleeding and hematochezia, while colonic lesions can cause fresh blood per rectum [6].

Currently, therapeutic endoscopy is the treatment method of choice for patients with acute active bleeding and it has substantially improved patients’ chances of survival, with the mortality rate decreasing from approximately 30–60% in the 1970s to 9-13% to date [7].

In this study, two cases of gastric DL successfully treated with two different endoscopic approaches are reported.

Diagnosis of the rare but potentially life-threatening DL are often delayed, even when recurrent GI bleeding has become clinically evident. Before the use of endoscopy as the first-line of choice for diagnosing DL, the diagnosis were basically made during autopsies or surgeries [8]. Currently, there is no consensus about the optimum treatment of DL; the best choice depends on clinical presentation, the site of the lesions and the physician’s own expertise in treating gastrointestinal bleedings.

Bleeding from DL was treated surgically before 1990 [9, 10]. Since then, endoscopic treatment has been recommended as first-line management for patients who are actively bleeding or for patients at risk of emergency surgery, recurrent bleeding, or mortality [11]. However, in a systematic revision of pediatric literature of the last 10 years, based on a total of five cases reports on pediatric gastric DL, one case was treated with surgery [12] and four cases received an endoscopic treatment. Details on gastric DL treated with endoscopic therapy are shown in Table 1 [13‐16].

Several advanced endoscopic techniques, including injections of epinephrine or sclerotherapy, mechanical interventions such as hemostatic clips and banding, and thermal treatments have been effective overall, even in the pediatric population [3, 17, 18].

Each technique has advantages and disadvantages in the treatment of DL. In particular, epinephrine is a safe and inexpensive method, but it is not recommended as monotherapy in DL due to the high re-bleeding rate [19]. Sclerotherapy using ethanol or polidocanol (sclerosing substances) can also be used to treat DL with good bleeding control [20]. Thermal endoscopic methods (i.e. bipolar and heater probes or Argon Plasma Coagulator) are not recommended because they carry a risk of transmural injury and could cause inadequate coagulation of the lesion [18].

To date, mechanical devices such as hemoclips and ligation bands are new choices for the treatment of GI bleedings. Hemostatic clipping has been found to be significantly more effective in preventing re-bleeding of gastric body DL than other methods. However, it is not easy to apply a hemoclip when the lesion is found in difficult sites such as the gastric fundus, the lower curvature of the stomach or the posterior wall of the duodenal bulb, or when the lesion has a fibrotic base. It is very important to have good expertise in this technique because a correct first application of hemoclips could prevent future needs of these [21, 22]. Band ligation is another mechanical hemostatic technique with very low perforation risk. It allows accessibility to difficult sites such as the esophagogastric junction and the posterior wall of the proximal body of the stomach. Occasionally, such as in the case of a residual vessel within a necrotic ulcer, band ligation may be associated with rare complications such as delayed bleeding [23]. Chung et al., compared the efficacy of hemostatic methods in patients with DL, and showed that mechanical methods for hemostasis, such as hemoclipping and band ligation, were superior to injection methods for controlling bleeding and preventing recurrent bleeding [24].

In this article, we confirm the efficacy of hemoclipping in the treatment of gastric DL and describe, for the first time, the use of banding in child. The choice between two different devices (banding or clipping) was performed according to the location of the DL, the diameter of visible vessel and on the endoscopist’s experience.

In conclusion, in our cases, we showed that, similar to DL in adults, gastric DLin children can be successfully treated with endoscopic therapy, and both hemoclipping and band ligation are suitable techniques.