GI Anomalies: Dieulafoy Lesion

Two days after a 70-year-old man was treated for suspected GI bleeding, he presented to the hospital with dyspnea, weakness, and light-headedness. He denied loss of consciousness, fall, bright red blood from rectum, diarrhea, nausea, vomiting, and pain. During his previous hospital stay, esophagogastroduodenoscopy (EGD) had shown no active bleeding. Stool was hemoccult-positive, and the patient received 2 units of packed red blood cells. He was sent home with a proton pump inhibitor.

The patient had had a GI hemorrhage with an ulcer 4 years earlier that required surgical ligation. His medical history also included myocardial infarction, chronic ischemic heart disease, implantation of a cardioverter device, hyperlipidemia, esophageal reflux, and osteoarthritis. His temperature was 36.8ºC (98.1ºF); blood pressure, 122/78 mm Hg; heart rate, 95 beats per minute; and respiration rate, 20 breaths per minute. Bowel sounds were normal; abdomen was soft, nontender, and nondistended, with no masses or hepatosplenomegaly. Rectum was grossly positive for maroon stool. Breath sounds were clear to auscultation bilaterally. Cardiac findings were normal.

The white blood cell count was 13,100/µL; hemoglobin level, 6.5 g/dL; hematocrit, 19.5%; and platelet count, 238,000/µL. An ECG indicated first-degree atrioventricular block, an old inferior infarct, and right bundle-branch block. An esophageal ultrasonogram showed a bleeding artery at the proximal fundus (A, arrow). EGD revealed a small, red, nipple-like lesion in the proximal stomach (B, arrow).

The bleeding continued despite interventional radiology coil embolization and other conservative measures, and the patient required urgent partial gastrectomy. A histological section of the resected stomach showed a tortuous, thick-walled blood vessel that extended through the gastric submucosa into the overlying luminal epithelium (C). The ruptured vessel—the origin of the hemorrhage—demonstrated necrosis, erythrocyte extravasation, and clotting with adjacent acute inflammatory exudate. These findings were consistent with a persistent-caliber vessel, or Dieulafoy lesion.

This lesion is responsible for 0.3% to 6.7% of all upper GI bleeding. It is more common in men than in women by a 2:1 ratio. There is no family association. The average age at onset is about 50 years, but it can vary from 20 months to 93 years.¹ The condition is thought to be the result of a congenital variant.² Patients often present with a massive upper GI hemorrhage and recurrent painless bleeding associated with severe life-threatening hemodynamic involvement, including shock.1 Associated comorbidities include cardiovascular disease, hypertension, chronic renal failure, diabetes mellitus, and alcohol abuse. NSAID use is also thought to incite bleeding through mucosal atrophy and ischemic injury.²

Although Dieulafoy lesions may be found in any portion of the GI tract, they usually occur in the upper portion of the lesser curvature of the stomach near the gastroesophageal junction (3 to 6 cm below the esophagogastric mucosa). The typical morphological appearance is that of a large-bore vascular stump that protrudes from the mucosa. The lesion may appear as a raised nipple or visible vessel, without an associated ulcer.2 The adherent vessel is not seen unless there is active bleeding at the site. It may range from 1 to 3 mm, which is about 10 times the normal size of mucosal capillaries.²

The diagnosis is based on results of endoscopy. Technetium-99m radionuclide scanning is used only to confirm the general area of bleeding. Endoscopic ultrasonography can identify the arterial flow and indicate the best site for sclerosing agents; however, it cannot be used during active bleeding.³

Treatment involves endoscopic hemostasis and cauterization either with bipolar or heater probe coagulation or with epinephrine and an injection of a sclerosing agent or alcohol.^4,5 Endoscopic band ligation, argon laser, injection of acrylic resins, and hemoclipping are less frequently used.^6,7 For recurrent bleeding, repeated endoscopic hemostasis is effective; a combined endoscopic and laparoscopic approach has also been used.

This patient recovered well after surgery and was transferred to a rehabilitation facility for continued follow-up. He has had no further rectal bleeding.