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Case report: Vascular anomaly in dieulafoy's disease
ClinicalRadiology (1991) 44, 201-202
Case Report: Vascular Anomaly in Dieulafoy's Disease p. A . G R A C E ,
R. O'SULLIVAN*,
A. N, ADAM*
a n d R . C. N . W I L L I A M S O N
Departments of Surgery and * Radiology, Royal Postgraduate Medical School, Hammersmith Hospital, London We present a case of a vascular anomaly with Dieulafoy's disease in a young man. The diagnosis was made by coeliac and selective left gastric angiography after endoscopy, laparotomy and angiography had failed to detect the lesion. The patient was treated successfully by laparotomy and resection of the bleeding area in the stomach. This is the second report of a vascular anomaly with Dieulafoy's disease. Grace, P.A., O'Sullivan, R., Adam, A.N. & W i l l i a m s o n , R . C . N . (1991). Clinical Dieulafoy's Disease
Radiology 44, 201 202. C a s e R e p o r t : V a s c u l a r A n o m a l y
in
D i e u l a f o y ' s d i s e a s e is a r a r e c a u s e o f m a s s i v e r e c u r r e n t u p p e r g a s t r o i n t e s t i n a l h a e m o r r h a g e w h i c h c a n b e ext r e m e l y difficult t o d i a g n o s e ( V e l d h u y z e n v a n Z a n t e n et al., 1986). T h e c o n d i t i o n is c h a r a c t e r i z e d b y n o r m a l gastric mucosa with a shallow defect of up to 2 mm in diameter usually sited along the lesser curve of the stomach. Through this small defect heavy bleeding can o c c u r i n t e r m i t t e n t l y f r o m a b n o r m a l s u b m u c o s a l vessels. T h e d i a g n o s i s is m a d e e i t h e r b y g a s t r o s c o p y , s e l e c t i v e angiography or during emergency laparotomy. We present a case in which the diagnosis was made angiographically a n d w a s a s s o c i a t e d w i t h a v a s c u l a r a n o m a l y o f t h e splenic artery.
CASE R E P O R T A 25-year-old man was admitted to another hospital in March 1989 with a 6 h history of haematemesis and melaena. In 1985 he had been investigated for iron-deficiency anaemia but no cause was found. Upper GI endoscopy performed 24 h after admission was normal; no blood was seen in the stomach or duodenum. Emergency iaparotomy was undertaken because of continued haemorrhage. There was intralurninal blood in the jejunum and distal gut but the source of bleeding was not detected. Further haemorrbage post-operatively was investigated by selective visceral angiography. The coeliac axis injection demonstrated abnormal arteries around the body and tail of the pancreas but with no obvious source of bleeding. The bleeding stopped spontaneously and he was transferred to our hospital for further investigation. On admission, he was stable with a haemoglobin of 8.7 g/dl. He rebled 24 h later and underwent immediate angiography via a right femoral puncture. A combination of cut film and digital subtraction images was used. Superior and inferior mesenteric arteriograms were normal. The coeliac axis injection showed occlusion of the splenic artery just beyond its origin with distal reconstitution via collaterals (Fig. 1). Trauma or hypercoagulation were considered as causes for this finding, although specific questioning did not elicit a history of either. Selective left gastric injection was then performed. A serpiginous vessel was noted posteroinferior to the gastro-oesophageal junction (Fig. 2), which was associated with a poorly defined blush (1.5 cm diameter) in the capillary phase that persisted into the venous phase. The stomach was markedly distended with blood rather than the CO2 that is often used during coeliac angiography. Injections into the gastroduodenai artery showed that the .vascular abnormality was also supplied via the right gastroepiploic artery. During the procedure extravasation was noted indicating active bleeding. Embolization was considered but not attempted because of the dual arterial supply to the lesion, together with the concern that left gastric artery occlusion might compromise the lower oesophagus. Immediate laparotomy was performed instead. Through a gastrotomy the bleeding point was identified on the posterior wall of the stomach adjacent to the gastro-oesophageal junction (as indicated by angiography). There was Correspondence to: Prof R. C. N. Williamson, Department of Surgery-, Royal Postgraduate,Medical School, Hammersmith Hospital, London W12 0NN.
Fig. 1 Anteroposterior view showing occlusion of the splenic artery just distal to its origin (arrow). The distal splenic artery is filled via extensive collateral channels.
Fig. 2 - A serpiginous vessel (arrow) is seen posteroinferior to the gastro-oesophageat junction associated with a 1.5 × 1,5 cm poorly defined blush. Note also the marked distension of the stomach with blood.
202
CLINICAL RADIOLOGY
no gastric ulcer, no tumour and no other abnormality in the abdominal cavity. A wedge resection of the bleeding area was performed, and the patient made an uneventful recovery. Histological examination of the specimen showed ectatic, thin-walled vessels in the submucosa and lamina propria as well as intramucosal haemorrhage. DISCUSSION A l t h o u g h G a l l a r d was p r o b a b l y the first to describe this lesion in 1884, its d e s c r i p t i o n has been a t t r i b u t e d to D i e u l a f o y (1898). H e believed the lesion to be a p r e c u r s o r o f gastric ulcer a n d called it ' E x u l c e r a t i o simplex'. Since then over 100 p a t i e n t s with D i e u l a f o y ' s disease have been r e p o r t e d (Veldhuyzen v a n Z a n t e n et al., 1986). T h e typical lesion in D i e u l a f o y ' s disease is a small m u c o s a l defect, 2 - 5 m m in d i a m e t e r , with m i n i m a l i n f l a m m a t o r y r e a c t i o n a n d sited on the lesser curve o f the s t o m a c h within 6 c m o f the g a s t r o - o e s o p h a g e a l j u n c t i o n (Veldhuyzen van Z a n t e n et al., 1986). U n d e r l y i n g this defect is a large a n d t o r t u o u s a r t e r y which bleeds intermittently, The artery is neither vasculitic, a t h e r o m a tous n o r a n e u r y s m a l ( G o l d m a n , 1964). A s s o c i a t e d vascular a b n o r m a l i t i e s are unusual. O u r p a t i e n t h a d occlusion o f the splenic artery j u s t distal to its origin. T h e distal splenic a r t e r y filled via collaterals. It is possible t h a t increased b l o o d flow to the spleen via the s h o r t gastric vessels m a y have c o n t r i b u t e d to the p a t h o g e n e s i s o f the D i e u l a f o y ' s erosion. I n one o t h e r r e p o r t o f D i e u l a f o y ' s disease, the coeliac a n d s u p e r i o r mesenteric arteries were o c c l u d e d at their origins a n d there was an extensive collateral circulation to the small bowel a n d s t o m a c h via a huge m a r g i n a l a r t e r y o f D r u m m o n d a n d left phrenic artery ( L o u w e r e n s et al., 1988). T h e clinical features o f D i e u l a f o y ' s disease have been well described ( G o l d m a n , 1964; V e l d h u y z e n van Z a n t e n
et al., 1986). It can occur at a n y age a n d there is a male p r e d o m i n a n c e . T h e r e is no familial tendency a n d t h e most frequent p r e s e n t a t i o n is massive haematemesis. D i a g n o sis is difficult a n d r e p e a t e d e n d o s c o p y m a y be required. O n l y six p r e v i o u s cases have been recognized b y visceral a n g i o g r a p h y ( M o r t e n s e n et al., 1983; V e l d h u y z e n van Z a n t e n et al., 1986). T h e a n g i o g r a m will only be o f benefit if p e r f o r m e d while the p a t i e n t is actively bleeding. O p e r a t i o n is the t r e a t m e n t o f choice; a local wedge resection is all t h a t is required. T h e m o r t a l i t y rate o f this c o n d i t i o n ranges f r o m 23% to 35%. Before the a d v e n t of e n d o s c o p y the p r o g n o s i s o f D i e u l a f o y ' s disease was p o o r because o f failure to m a k e the diagnosis.
REFERENCES
Dieulafoy, G (1898). Exculceratio simplex. L'intervention chirurgicale darts les hematemeses foudroyantes conseeutives a l'exulceration simple de l'estomac. Bull. Acad. Med., 49, 49-84. Gallard, T (1884). Aneurysmes miliaires de l'estomac, dormant lieu a des hematemeses mortelles. Bulletin of the Society of Medicine, Paris, l, 84 91. Goldman, RL (1964). Submucosal arterial malformation ('aneurysm') of the stomach with fatal haemorrhage. Gastroenterology, 46, 589594. Louwerens, JWK, Gratama, S, Zwaan, A & van der Schaar, H (1988). Dieulafoy's erosion in the stomach as a result of an intra-abdominal vascular anomaly. British Journal of Surgery, 7S, 489-490. Mortensen, NJMcC, Mountford, RA, Davies, JD & Jeans, WD (1983). Dieulafoy's disease: a distinctive arteriovenous malformation eaus; ing massive gastric haemorrhage. British Journal of Surgery, 70, 7678. Veldhuyzen van Zanten, bJO, ~artelsman, Jl~WiVl,~cnlpp~r, lvs~zJc~ Tytgat, GNJ (1986). Recurrent massive haematemesis from Dieulafoy vascular malformations- a review of 101 cases. Gut, 27, 213 222.
Case Report: Vascular Anomaly in Dieulafoy's Disease p. A . G R A C E ,
R. O'SULLIVAN*,
A. N, ADAM*
a n d R . C. N . W I L L I A M S O N
Departments of Surgery and * Radiology, Royal Postgraduate Medical School, Hammersmith Hospital, London We present a case of a vascular anomaly with Dieulafoy's disease in a young man. The diagnosis was made by coeliac and selective left gastric angiography after endoscopy, laparotomy and angiography had failed to detect the lesion. The patient was treated successfully by laparotomy and resection of the bleeding area in the stomach. This is the second report of a vascular anomaly with Dieulafoy's disease. Grace, P.A., O'Sullivan, R., Adam, A.N. & W i l l i a m s o n , R . C . N . (1991). Clinical Dieulafoy's Disease
Radiology 44, 201 202. C a s e R e p o r t : V a s c u l a r A n o m a l y
in
D i e u l a f o y ' s d i s e a s e is a r a r e c a u s e o f m a s s i v e r e c u r r e n t u p p e r g a s t r o i n t e s t i n a l h a e m o r r h a g e w h i c h c a n b e ext r e m e l y difficult t o d i a g n o s e ( V e l d h u y z e n v a n Z a n t e n et al., 1986). T h e c o n d i t i o n is c h a r a c t e r i z e d b y n o r m a l gastric mucosa with a shallow defect of up to 2 mm in diameter usually sited along the lesser curve of the stomach. Through this small defect heavy bleeding can o c c u r i n t e r m i t t e n t l y f r o m a b n o r m a l s u b m u c o s a l vessels. T h e d i a g n o s i s is m a d e e i t h e r b y g a s t r o s c o p y , s e l e c t i v e angiography or during emergency laparotomy. We present a case in which the diagnosis was made angiographically a n d w a s a s s o c i a t e d w i t h a v a s c u l a r a n o m a l y o f t h e splenic artery.
CASE R E P O R T A 25-year-old man was admitted to another hospital in March 1989 with a 6 h history of haematemesis and melaena. In 1985 he had been investigated for iron-deficiency anaemia but no cause was found. Upper GI endoscopy performed 24 h after admission was normal; no blood was seen in the stomach or duodenum. Emergency iaparotomy was undertaken because of continued haemorrhage. There was intralurninal blood in the jejunum and distal gut but the source of bleeding was not detected. Further haemorrbage post-operatively was investigated by selective visceral angiography. The coeliac axis injection demonstrated abnormal arteries around the body and tail of the pancreas but with no obvious source of bleeding. The bleeding stopped spontaneously and he was transferred to our hospital for further investigation. On admission, he was stable with a haemoglobin of 8.7 g/dl. He rebled 24 h later and underwent immediate angiography via a right femoral puncture. A combination of cut film and digital subtraction images was used. Superior and inferior mesenteric arteriograms were normal. The coeliac axis injection showed occlusion of the splenic artery just beyond its origin with distal reconstitution via collaterals (Fig. 1). Trauma or hypercoagulation were considered as causes for this finding, although specific questioning did not elicit a history of either. Selective left gastric injection was then performed. A serpiginous vessel was noted posteroinferior to the gastro-oesophageal junction (Fig. 2), which was associated with a poorly defined blush (1.5 cm diameter) in the capillary phase that persisted into the venous phase. The stomach was markedly distended with blood rather than the CO2 that is often used during coeliac angiography. Injections into the gastroduodenai artery showed that the .vascular abnormality was also supplied via the right gastroepiploic artery. During the procedure extravasation was noted indicating active bleeding. Embolization was considered but not attempted because of the dual arterial supply to the lesion, together with the concern that left gastric artery occlusion might compromise the lower oesophagus. Immediate laparotomy was performed instead. Through a gastrotomy the bleeding point was identified on the posterior wall of the stomach adjacent to the gastro-oesophageal junction (as indicated by angiography). There was Correspondence to: Prof R. C. N. Williamson, Department of Surgery-, Royal Postgraduate,Medical School, Hammersmith Hospital, London W12 0NN.
Fig. 1 Anteroposterior view showing occlusion of the splenic artery just distal to its origin (arrow). The distal splenic artery is filled via extensive collateral channels.
Fig. 2 - A serpiginous vessel (arrow) is seen posteroinferior to the gastro-oesophageat junction associated with a 1.5 × 1,5 cm poorly defined blush. Note also the marked distension of the stomach with blood.
202
CLINICAL RADIOLOGY
no gastric ulcer, no tumour and no other abnormality in the abdominal cavity. A wedge resection of the bleeding area was performed, and the patient made an uneventful recovery. Histological examination of the specimen showed ectatic, thin-walled vessels in the submucosa and lamina propria as well as intramucosal haemorrhage. DISCUSSION A l t h o u g h G a l l a r d was p r o b a b l y the first to describe this lesion in 1884, its d e s c r i p t i o n has been a t t r i b u t e d to D i e u l a f o y (1898). H e believed the lesion to be a p r e c u r s o r o f gastric ulcer a n d called it ' E x u l c e r a t i o simplex'. Since then over 100 p a t i e n t s with D i e u l a f o y ' s disease have been r e p o r t e d (Veldhuyzen v a n Z a n t e n et al., 1986). T h e typical lesion in D i e u l a f o y ' s disease is a small m u c o s a l defect, 2 - 5 m m in d i a m e t e r , with m i n i m a l i n f l a m m a t o r y r e a c t i o n a n d sited on the lesser curve o f the s t o m a c h within 6 c m o f the g a s t r o - o e s o p h a g e a l j u n c t i o n (Veldhuyzen van Z a n t e n et al., 1986). U n d e r l y i n g this defect is a large a n d t o r t u o u s a r t e r y which bleeds intermittently, The artery is neither vasculitic, a t h e r o m a tous n o r a n e u r y s m a l ( G o l d m a n , 1964). A s s o c i a t e d vascular a b n o r m a l i t i e s are unusual. O u r p a t i e n t h a d occlusion o f the splenic artery j u s t distal to its origin. T h e distal splenic a r t e r y filled via collaterals. It is possible t h a t increased b l o o d flow to the spleen via the s h o r t gastric vessels m a y have c o n t r i b u t e d to the p a t h o g e n e s i s o f the D i e u l a f o y ' s erosion. I n one o t h e r r e p o r t o f D i e u l a f o y ' s disease, the coeliac a n d s u p e r i o r mesenteric arteries were o c c l u d e d at their origins a n d there was an extensive collateral circulation to the small bowel a n d s t o m a c h via a huge m a r g i n a l a r t e r y o f D r u m m o n d a n d left phrenic artery ( L o u w e r e n s et al., 1988). T h e clinical features o f D i e u l a f o y ' s disease have been well described ( G o l d m a n , 1964; V e l d h u y z e n van Z a n t e n
et al., 1986). It can occur at a n y age a n d there is a male p r e d o m i n a n c e . T h e r e is no familial tendency a n d t h e most frequent p r e s e n t a t i o n is massive haematemesis. D i a g n o sis is difficult a n d r e p e a t e d e n d o s c o p y m a y be required. O n l y six p r e v i o u s cases have been recognized b y visceral a n g i o g r a p h y ( M o r t e n s e n et al., 1983; V e l d h u y z e n van Z a n t e n et al., 1986). T h e a n g i o g r a m will only be o f benefit if p e r f o r m e d while the p a t i e n t is actively bleeding. O p e r a t i o n is the t r e a t m e n t o f choice; a local wedge resection is all t h a t is required. T h e m o r t a l i t y rate o f this c o n d i t i o n ranges f r o m 23% to 35%. Before the a d v e n t of e n d o s c o p y the p r o g n o s i s o f D i e u l a f o y ' s disease was p o o r because o f failure to m a k e the diagnosis.
REFERENCES
Dieulafoy, G (1898). Exculceratio simplex. L'intervention chirurgicale darts les hematemeses foudroyantes conseeutives a l'exulceration simple de l'estomac. Bull. Acad. Med., 49, 49-84. Gallard, T (1884). Aneurysmes miliaires de l'estomac, dormant lieu a des hematemeses mortelles. Bulletin of the Society of Medicine, Paris, l, 84 91. Goldman, RL (1964). Submucosal arterial malformation ('aneurysm') of the stomach with fatal haemorrhage. Gastroenterology, 46, 589594. Louwerens, JWK, Gratama, S, Zwaan, A & van der Schaar, H (1988). Dieulafoy's erosion in the stomach as a result of an intra-abdominal vascular anomaly. British Journal of Surgery, 7S, 489-490. Mortensen, NJMcC, Mountford, RA, Davies, JD & Jeans, WD (1983). Dieulafoy's disease: a distinctive arteriovenous malformation eaus; ing massive gastric haemorrhage. British Journal of Surgery, 70, 7678. Veldhuyzen van Zanten, bJO, ~artelsman, Jl~WiVl,~cnlpp~r, lvs~zJc~ Tytgat, GNJ (1986). Recurrent massive haematemesis from Dieulafoy vascular malformations- a review of 101 cases. Gut, 27, 213 222.