Abstract

The calibre persistent artery or Dieulafoy’s disease of stomach is the most fatal form of upper gastrointestinal tract bleeding with mortality of around 60.5%. We are reporting a fatal case of a 37-year-old male who complained of haematemesis and was admitted in hypovolaemic shock. Endoscopy revealed stomach filled with fresh blood. On autopsy, a gaping blood vessel was found near the cardiac end and section of which showed a histologically normal, dilated and tortuous artery in the submucosa. Diagnosis of calibre persistent artery of stomach was made. It can occur in gastrointestinal as well as respiratory tract. Awareness of this condition by the endoscopist could have prevented mortality and hence it should be considered in the differential diagnosis of unexplained gastrointestinal bleeding.

Introduction

The calibre persistent artery or Dieulafoy’s disease of stomach is a vascular lesion with histologically normal artery but because of failure of normal development of artery below the muscularis mucosa, the calibre of the artery remains persistent throughout the gastric wall and can lead to profuse upper gastrointestinal tract bleeding.1,2 This condition is often missed on endoscopy due to technical difficulties as well as lack of awareness regarding this condition. We are hereby reporting such an unusual case.

Case Report

A 37 year old male, non-alcoholic came with complaints of haemoptysis, haematemesis and melaena since 4 days. He also gave history of multiple episodes of jaundice in last two years. He was hypotensive on admission with pallor but no icterus. His haemoglobin was 6.6 g/dl. In view of his haematemesis, endoscopy was performed which revealed stomach filled with fresh blood but no cause could be identified. There were no varices. The patient subsequently deteriorated and expired within 24 hours. On autopsy all organs were markedly pale. Stomach was filled with around 1 litre of fresh blood. Serosa was unremarkable and mucosa at the fundus near the cardiac end at the greater curvature showed a gaping blood vessel (Fig. 1a). Rest of the intestine contained altered blood. Other organs were unremarkable. Sections from the stomach near the blood vessel showed normal mucosa. Submucosa was expanded with thickened, dilated, tortuous artery, which was histologically normal (Fig. 1b). There was no accompanying thrombus or inflammation. Kidney section showed changes of acute necrosis. Hence the final cause of death was given as hypovolaemic shock due to erosion of calibre persistent artery of stomach or Dieulafoy’s disease.

Fig. 1 : A-left hand gross photograph showing a gaping artery in the fundic region of the stomach. B-Microphotograph shows a large tortuous artery in the submucosa of the stomach. H and E-40X.

Discussion

‘Caliber persistent artery’ or ‘Dieulafoy’s lesion is one of the causes of massive gastrointestinal tract bleeding with mortality of 60.5%. The exact incidence is not cited in the literature.1 It was first described by Gallard in 1884 and has many synonyms like caliber persistent artery, Dieulafoy’s lesion, exulceratio simplex, but ‘calibre persistent artery’ is the most appropriate. It is due to failure of normal development of arteries below the muscularis mucosa and can lead to profuse bleeding in case of rupture.1,2 But the final cause of rupture of this calibre persistent artery is not clear.

It is seen more commonly in males between 50-70 years.1 Usually the patients present with unexplained haematemesis and melaena as in our case.1,2 It is often missed on endoscopy due to massive bleed in stomach obscuring the vision.2 It is located usually in the subcardiac region next to either curvature as a mucosal defect. Majority of cases are reported in stomach, though it can occur in the rest of the gastrointestinal tract also.2 There are few reports involving the respiratory tract also and patients having died of massive haemoptysis.3 Other causes of gastric bleeding were ruled out in this case, like there were no varices or features of gastric antral vascular ectasia.

As the treatment of this condition is available in form of sclerosant agents, heat probe coagulation, hemoclip device, etc; it is important to diagnose this condition.2 In our case though the endoscopy was performed, but the diagnosis was missed. Hence it is important for the physician to consider this differential in cases of unexplained gastrointestinal tract bleeding as it could be much more common than the literature indicates.

References

1. Tivadar LM, Thomázy VA. The caliber persistent artery of the stomach: A unifying approach to gastric aneurysms, Dieulafoy’s lesion, and submucosal arterial malformation. Hum Pathol 1988; 19 : 914-21.
2. Scudiere JR, Cimbaluk D, Jakate S. A 74-year-old man with fatal gastrointestinal bleeding. Arch of Path and Lab Med 2005; 130 (2) : 223-4.
3. Kuzuc A, Gurses I, Soysal O, Kutlu R, Ozgel M. Dieulafoy’s disease: a cause of massive hemoptysis that is probably underdiagnosed. Ann Thorac Surg 2005; 80 (3) : 1126-8.
   

*Registrar; **Associate Professor; ***HOD; +Observer; LTMGH Sion Hospital, Mumbai 400 022