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A galling disease? Dieulafoy’s lesion of the gallbladder

Highlights • Dieulafoy’s lesion is characterized by an abnormally large and tortuous submucosal arteriole leading to an area of mucosal defect with minimal inflammation.• It is most often seen in the stomach but could also occur along the gastrointestinal tract.• Only four cases of gallbladder Dieulafoy’s lesion have been published so far.• The clinical, radiologic and histologic findings are discussed in light of the existing literature on this unusual entity.


Introduction
The term "Dieulafoy's lesion" (DL) is attributed to Dr Paul Georges Dieulafoy, who in 1898, described a series of 10 patients who had massive hematemesis due to a lesion in the stomach that he called "exulceratio simplex" [1]. DL classically presents acutely as massive hemorrhage which is often recurrent and can manifest as hematemesis, melena, or hematochezia [2]. The lesions are more common in men (M:F = 2:1) and the elderly (50-70 years) [1]. Patients often have multiple co-morbidities though not necessarily previous history of gastrointestinal pathology [2]. Histologically, it is characterized by a normal vessel with an abnormally large caliber of 1-3 mm that runs a tortuous course within the submucosa [2]. Typically the vessel protrudes through a small mucosal defect with fibrinoid necrosis at its base.
The commonest location for DL is on the lesser curvature of the stomach [2]. In one case series involving 101 cases, 82% of the gastric DLs were found within 6 cm of the gastroesophageal junction [3]. In 1978, two "Dieulafoy-like erosions, " previously thought to be unique to the stomach, were reported in the jejunum [4].
Subsequently, examples of extragastric DL have been described throughout the gastrointestinal tract including the esophagus, duodenum, jejunum, ileum, cecum, colon, rectum and anal canal [2]. Of the gastrointestinal bleed caused by DLs, stomach accounts for 71% of the cases, followed by duodenum with 15% and esophagus with 8% [2]. Bronchial DL, the first case of non-gastrointestinal DL, was not reported until 1995 [1].
Gallbladder DLs have rarely been reported in literature with only five previously published cases [5][6][7][8][9][10]. This report details another case of gallbladder DL in line with the SCARE criteria [11] and reviews the existing literature on the clinical presentation, investigations and treatment options for gallbladder DL.

Case presentation
A 44-year-old Caucasian man with known Crohn's disease, hypertension, ankylosing spondylitis and left hip replacement presented to the emergency department with a 5-h history of chest and epigastric achy pain. He had similar pains intermittently for a few months and was diagnosed with atypical non-cardiac chest pain.
On examination he had crepitation in the right lung base and mild palpation pain in the epigastrium. His blood tests showed normal troponin I and elevated white blood cell count (WBC) ( Table 1). The liver function tests (LFTs) showed elevated alanine aminotransferase (ALT) only. The chest X-ray showed right-sided basal atelectasis. Despite oral amoxicillin treatment for possible community-acquired pneumonia, his epigastric pain was still not resolved and he remained pyrexial with worsening WBC and raised C-reactive protein. An abdominal ultrasound arranged to check for gallstone disease failed due to the patient's ankylosing spondylitis and body habitus. Instead, a computed tomography (CT) scan was performed and showed features in keeping with calculous cholecystitis. The patient was started on oral co-amoxiclav. Two weeks after the initial presentation, the patient had an elective laparoscopic cholecystectomy and uneventful recovery.

Reference range
On

Histologic findings
Macroscopic inspection of the 80 × 40 mm gallbladder showed the presence of stones and roughened, hemorrhagic serosa. The mucosal surface was mostly velvety with some areas having a smoother surface and brownish discoloration. The wall was thickened and measured up to 8 mm (Fig. 1). On microscopic examination, focal severe ulceration was found along with chronic mucosal inflammation, submucosal fibrosis, muscle hyperplasia and Rokitansky-Aschoff sinus formation. In addition to these features of chronic cholecystitis, a large tortuous muscular arteriole measuring 1.2 mm in diameter was noted running through the pericholecystic fat and penetrating into the muscular wall and submucosa of the gallbladder (Fig. 2). Ulceration was seen where the vessel approached the mucosa with very mild surrounding inflammation and extensive fibrosis. Organizing thrombus was present at the site of vessel rupture at the ulcer base. The appearances are those of a DL (Fig. 3).

Discussion
Paraf first raised the possibility of Dieulafoy's disease of the gallbladder in a correspondence in 1996 [12]. Truong et al. had reported a case of hemoperitoneum caused by rupture of the gallbladder secondary to arterial ulceration by a calculus [5]. Paraf observed that the microscopic description and the figure in the report showed an artery with abnormal caliber deep to the ulcerated area of gallbladder with no mention of inflammatory infiltrate, similar to those   Table 2. The 6 patients with gallbladder DL were aged 45-84 years with a  Four of the patients had pain focused in the epigastrium and right upper quadrant. One patient had a rigid abdomen on presentation [6]. Some blood test results were available for 5 of the patients (Table 3). Of those, 83% had decreased hemoglobin. All showed a degree of liver function derangement. Both ultrasound and CT were utilized to investigate the cause of abdominal pain. Only one case revealed a hypervascular lesion in the gallbladder on CT which might raise suspicion for DL [8]. Four patients required emergency cholecystectomy. This review illustrates the difficulty of diagnosing gallbladder DL in the acute setting. Physical examination can localize pain and reveal acute abdomen. However, there are a multitude of causes for right upper quadrant and epigastric pain with overlapping signs and symptoms. Elevation of WBC and derangements in LFTs might help focus attention on hepatobiliary causes abdominal pain. Decreased hemoglobin level could point to a bleed but this is not seen in all patients. None of the patients reviewed had overt signs of bleeding such as hematemesis or melena.
Ultrasound is an excellent screening tool for suspected acute cholecystitis. Bleeding and perforation of the gallbladder can also be detected with ultrasound [13]. Of the three patients who had an abdominal ultrasound, all showed either a ruptured gallbladder or hyperechogenic material within the gallbladder to indicate bleeding. However, the underlying cause of hemorrhage into the gallbladder was not identified. CT can detect high attenuation blood products and has the added advantage of being able to evaluate other intra-abdominal pathologies at the same time [13]. In one case the CT scan demonstrated a 8 mm hypervascular lesion leading to immediate surgery [8]. However, radiology did not contribute to the diagnosis of gallbladder DL for the remaining cases. CT angiography is the modality of choice for suspected hemobilia because of its ability identify the source of bleeding [14]. But unlike other DLs in the gastrointestinal tract, the treatment of choice for gallbladder DL is cholecystectomy rather than endoscopic clipping, sclerotherapy or embolization, rendering the angiography moot in an emergency situation.
Potential complications associated with gallbladder DL include hemobilia, ruptured gallbladder and hemoperitoneum. Sixty-seven percent of the patients reviewed required emergency surgery. Whilst cholecystectomy is in general a safe procedure, it is well recognized that morbidity and mortality rates are significantly higher for emergent cholecystectomy compared to elective cholecystectomy [15]. In this review, all six patients had uneventful recovery after their cholecystectomy.

Conclusion
This case provides further evidence of the existence of DL in the gallbladder. Given the rarity of the entity, the diagnosis is often not considered. However, gallbladder DL has potentially serious complications and emergency surgery is often required. Clinicians should be aware of this potentially lethal but easily curable disease. A major limitation of this study is its small patient population. Further case reporting of gallbladder DL could help better characterization of the disease.

Conflicts of interest
None.

Funding
None.

Ethical approval
Ethical approval is exempt by our institution for case reports.

Consent
Patient consent has been obtained and is available for review by the Editor-in-Chief.