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Arterial malformation of stomach: A cause of massive bleeding
Pathology
ISSN: 0031-3025 (Print) 1465-3931 (Online) Journal homepage: http://www.tandfonline.com/loi/ipat20
Arterial Malformation of Stomach: A Cause of Massive Bleeding Tak-Min Kung & J. Wong To cite this article: Tak-Min Kung & J. Wong (1982) Arterial Malformation of Stomach: A Cause of Massive Bleeding, Pathology, 14:1, 81-84 To link to this article: http://dx.doi.org/10.3109/00313028209069046
Published online: 06 Jul 2009.
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Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=ipat20 Download by: [FU Berlin]
Date: 27 December 2016, At: 14:13
Pathology (1982). 14, pp. 81-4
ARTERIAL MALFORMATION OF STOMACH: A CAUSE OF MASSIVE BLEEDING TAK-MINKUNG*AND J. W O N G ~ Departments o j *Pathology and ?Surgery, University of Hong Kong
Summary Fatal gastrointestinal bleeding is rarely caused by a ruptured abnormal intramural gastric artery, and in such cases the nature of the abnormality in the ruptured artery has not been clearly defined. In this report, a case of fatal haemorrhage from a ruptured artery in the stomach of a 34-yr-old male is presented. The arterial lesion was demonstrated and appeared similar to the berry aneurysms of the Circle of Willis-which are probably the result of developmental defects in the media of the arterial wall. Phlebosclerosis due to portal hypertension was a coincidental finding. Previously reported cases are considered and compared with the present case.
Key words: arterial malformation of stomach, gastrointestinal bleeding
Ruptured intramural gastric artery has been recognized as a rare cause of massive, often fatal gastrointestinal bleeding. The pathology in the affected artery has been thought to be either arteriosclerosis or congenital malformation' the nature of which has not been demonstrated clearly. In the following report, fatal gastrointestinal bleeding was shown t o arise from an abnormal gastric artery, the histological features of which were studied in detail. The aetiology and pathogenesis of the abnormality are discussed.
CASE HISTORY A 34-yr-old Chinese male was first admitted to hospital in 1968 because of bleeding from oesophageal varices as a result of portal hypertension due to macronodular post-hepatitic cirrhosis. Repeated upper gastrointestinal bleeding necessitated emergency transabdominal transgastric plication of varices, and subsequently a splenorenal shunt. He remained well for 9 yr until 1979 when severe upper gastrointestinal bleeding recurred. Endoscopy failed to define the site of bleeding, though duodenal ulceration was suspected. Oesophageal or gastric varices were not noted. Laparotomy was carried out and a Polya gastrectomy was performed in view of the endoscopic findings. Further bleeding occurred. Coeliac and superior mesenteric arteriography was done which demonstrated an angiomatous malformation of the left gastric artery and also varices in the fundus of the stomach (Fig. I). A total gastrectomy was performed and the abdominal oesophagus
anastomosed to a roux-en-Y jejunal loop. Two wk later he suddenly developed catastrophic gastrointestinal bleeding and died. Consent for an autopsy was not granted.
PATHOLOGICAL FINDINGS The 1st operative specimen, consisting of the pylorus and antrum of the stomach, showed only dilated and thickened veins in the submucosa. The 2nd operative specimen included the fundus and body of the stomach. A small mucosal breach plugged by blood clot was found in the anterior gastric wall of the body of the stomach (Fig. 2). Sectioning revealed a ruptured blood vessel beneath the clot. Tortuous thick-walled blood vessels traversing the submucosa were found in the fundus and upper stomach, but they were absent from the distal areas. Histology The specimens were fixed in 10% buffered formalin. Blocks embedded in paraffin were cut at 5 p intervals and stained with haematoxylin and eosin, elastic and Masson's trichrome stains. In the submucosa of the proximal stomach. there were multiple tortuous muscular arteries of a calibre larger than normally expected in this region. They had the 3 well defined layers of a muscular artery, but there were multiple abrupt interruptions of the media and of the internal elastic lamina, resulting in thinning and small saccular aneurysmal dilatations of their walls. In these areas, the intima was directly apposed to the adventitia. The wall of these aneurysms was thus composed of only a few collagenous fibres. Focal intimal thickening was also seen in these arteries. These changes are shown in Fig. 3 which includes also the ruptured artery. The submucosal veins were dilated, with mild focal intimal fibrosis, but were otherwise unremarkable. The covering gastric mucosa was not inflamed and showed no features of peptic ulceration. No abnormal arteries were found in the pylorus and antrum of the stomach.
DISCUSSION An abnormal artery in the submucosa of the stomach as a cause of massive, often fatal haemorrhage has been
82
Pathology (1982), 14, January
KUNG & WONG
FIG. 1 Coeliac arteriogram in the arterial phase demonstrating angiomatous malformation affecting the branches of the left gastric artery. Tortuous and dilated vessels are seen at the fundus of the stomach lateral t o the opacified Ryle’s tube
FIG. 2 A close-up picture of the gastric mucosa. There is a small mucosal ulceration (arrow) beneath which is a ruptured artery. Focal petechiae are also seen
recorded in about 30 cases in the literature. The earlier cases were reviewed by Mandelbaum’ and later by Goldman.’ In his review, Goldman noted that the reported lesions showed a remarkable uniformity of both gross and microscopic characteristics: namely a small
mucosal erosion capped by thrombotic material overlying a ruptured submucosal vessel.’ The involved vessel invariably was unusually large and tortuous and had the typical structure of larger muscular arteries containing 3 well defined layers with a prominent muscular media.
ARTERIAL MALFORMATION OF STOMACH
83
FIG.3 Unusually large tortuous muscular arteries in the submucosa of the stomach. The ruptured artery seen in the gross specimen in Fig. 2 is shown on the left side of the figure. The blood clot covering the denuded area has been dislodged during processing. The artery is found just beneath the mucosa, with compression of the intervening submucosal tissue. The precise rupture site is not seen here, but 2 markedly attenuated areas in the arterial wall can be noticed towards the mucosal side. These probably represent aneurysms. More obvious small saccular aneurysms are seen in the artery on the right side of the figure. The arrow points to one aneurysm, the high power picture of which is shown in Fig. 4. (Masson’s trichrome stain x 9)
Intimal thickening interpreted as localized arteriosclerosis was seen in some but not all cases. He concluded that the vascular abnormality was primary, arteriosclerotic changes being secondary. The affected vessel was thought to represent a localized congenital malformation, but the nature of this could not be demonstrated, though absence of the media in the vicinity of rupture was seen in the case reported by Bailey.3 Several additional cases of arterial malformation were reported in recent years.46 Richter presented another case of rupture of a single tortuous large submucosal artery which displayed no atherosclerosis, aneurysmal dilatation or elastic tissue a b n ~ r m a l i t y No . ~ histological description of the affected vessel was available in Gough’s case,’ though the angiographic studies were consistent with a malformed artery. In the case of Sherman et nl., multiple vessel involvement was found in the stomach, in contrast to the single vessel involvement in previous reports.’-5 Individual vessels in this case were difficult to categorize as arteries or veins. The authors considered that the abnormal structure of these vascular channels was consistent with arterio-venous malformation. In the present case, the gross morphology of the gastric mucosa was similar to that of the cases reviewed by Goldman,’ i.e. a small mucosal defect plugged by blood clot. However, although only one vessel had ruptured, multiple vessels were affected. The affected vessels were intramural branches of the left gastric artery. The lesion
in the arteries was unlikely to be inflammatory in nature, as there was no histological evidence of fibrinoid necrosis or other inflammatory reaction such as those in polyarteritis nodosa.’ The internal elastic lamina was abruptly interrupted rather than fragmented as in giant cell arteritis7 (Fig. 4). The few reactive changes in and around these arteries did not suggest that the abnormalities resulted from previous surgical intervention; of course the plicated vessels were veins, not arteries. The focal interruption of both the media and the internal elastic lamina was reminiscent of berry aneurysms of the cerebral arteries. Berry aneurysms are results of developmental faults in the media and the internal elastic lamina whose absence impairs the ability of the arteries to withstand blood pressure: rupture is therefore a frequent occurrence.’. Berry aneurysms are usually found at the bifurcation ofcerebral arteries. In the present case, though the microaneurysms were not found at bifurcations, the histological features were similar to those of berry aneurysms. It was unfortunate that an autopsy could not be done in this case, but even without it, it could be established that only the branches of a single artery of the stomach were involved. Thus. while the aneurysm in the case reported by Mandelbaum2 appeared to be due to arteriosclerosis, and while the cause of the arterial abnormality in most of the cases reported in the literature was controversial, developmental faults appeared to be the cause in this case.
84
Pathology (1982). 14, January
KUNG & W O N G
FIG. 4 A higher power view of a small aneurysm. In addition to the medial defect, the internal elastic membrane also stops abruptly at the entrance of the aneurysm. The thin wall of the aneurysm is composed of only collagen fibres. (Elastic stain. x 72)
The uniformly dilated veins with intimal fibrosis were features of phlebosclerosis, probably related to the portosystemic shunting of blood as a result of portal hypertension’ in cirrhosis. They were thus not congenitally malformed, and their pathological changes were not related to those in the arteries. This case therefore differed from that of Sherman et aL6 in that arterial malformation rather than combined arterial and venous malformation was its principal feature. It is probable that congenital developmental defects are the cause of multiple vessel involvement while a single artery abnormality is more likely to be due to acquired causes. This case also suggests that arterial malformation should be looked for as a cause of gastrointestinal bleeding when commoner causes have been excluded. The authors are grateful to Miss ACKNOWLEDGEMENT Debbie Lo for typing the manuscript. Address for correspondence: T.M.K., Department of Pathology, University of Hong Kong. Queen Mary Hospital, Hong Kong
References 1. GOLDMAN,R. L. (1964): Submucosal arterial malformation
(‘aneurysm’) of the stomach with fatal hemorrhage. Gastroenterology 46, 589-594. I., KAISER,G. C. & LEMPKE,R. E. (1962): Gastric 2. MANDELBAUM, intramural aneurysm as a cause for massive gastrointestinal hemorrhage. Ann. Surg. 155, 199-203. 3. BAILEY, M. E. (1956): Rupture of a gastric artery aneurysm. Can. Med. Assoc. J . 75, 131-133. 4. RICHTER,R. M. (1975): Massive gastric hemorrhage from submucosal arterial malformation. Am. J. Gastroenterol. 64, 324326. 5. GOUGH,M. (1977): Submucosal arterial malformation of the stomach as the probable cause of recurrent severe haematemesis in a 16-year-old girl. Br. J . Surg. 64, 522-524. S. S., SATCHIDANAND, S. K. et al. (1979): 6. SHERMAN, L., SHENOY, Arteriovenous malformation of the stomach. Am. J . Gastroenterol. 72, 160-164. W. , St C. (1976): Systemic Pathology, Vol. I , 2nd ed. ~ .SY MMERS Churchill Livingstone, London. pp. 140-146. 8. GLYNN, L. E. (1940): Medical defects in the circle of Willis and their relation to aneurysm formation. J. Pathol. Bacteriol. 51, 213-222. E. (1959): Pathogenesis of phlebosclerosis (I. 9. MOSCHCOWITZ, Phlebosclerosis of the portal vein). A M A Arch. Pathol. 68,180-184.
ISSN: 0031-3025 (Print) 1465-3931 (Online) Journal homepage: http://www.tandfonline.com/loi/ipat20
Arterial Malformation of Stomach: A Cause of Massive Bleeding Tak-Min Kung & J. Wong To cite this article: Tak-Min Kung & J. Wong (1982) Arterial Malformation of Stomach: A Cause of Massive Bleeding, Pathology, 14:1, 81-84 To link to this article: http://dx.doi.org/10.3109/00313028209069046
Published online: 06 Jul 2009.
Submit your article to this journal
Article views: 1
View related articles
Citing articles: 2 View citing articles
Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=ipat20 Download by: [FU Berlin]
Date: 27 December 2016, At: 14:13
Pathology (1982). 14, pp. 81-4
ARTERIAL MALFORMATION OF STOMACH: A CAUSE OF MASSIVE BLEEDING TAK-MINKUNG*AND J. W O N G ~ Departments o j *Pathology and ?Surgery, University of Hong Kong
Summary Fatal gastrointestinal bleeding is rarely caused by a ruptured abnormal intramural gastric artery, and in such cases the nature of the abnormality in the ruptured artery has not been clearly defined. In this report, a case of fatal haemorrhage from a ruptured artery in the stomach of a 34-yr-old male is presented. The arterial lesion was demonstrated and appeared similar to the berry aneurysms of the Circle of Willis-which are probably the result of developmental defects in the media of the arterial wall. Phlebosclerosis due to portal hypertension was a coincidental finding. Previously reported cases are considered and compared with the present case.
Key words: arterial malformation of stomach, gastrointestinal bleeding
Ruptured intramural gastric artery has been recognized as a rare cause of massive, often fatal gastrointestinal bleeding. The pathology in the affected artery has been thought to be either arteriosclerosis or congenital malformation' the nature of which has not been demonstrated clearly. In the following report, fatal gastrointestinal bleeding was shown t o arise from an abnormal gastric artery, the histological features of which were studied in detail. The aetiology and pathogenesis of the abnormality are discussed.
CASE HISTORY A 34-yr-old Chinese male was first admitted to hospital in 1968 because of bleeding from oesophageal varices as a result of portal hypertension due to macronodular post-hepatitic cirrhosis. Repeated upper gastrointestinal bleeding necessitated emergency transabdominal transgastric plication of varices, and subsequently a splenorenal shunt. He remained well for 9 yr until 1979 when severe upper gastrointestinal bleeding recurred. Endoscopy failed to define the site of bleeding, though duodenal ulceration was suspected. Oesophageal or gastric varices were not noted. Laparotomy was carried out and a Polya gastrectomy was performed in view of the endoscopic findings. Further bleeding occurred. Coeliac and superior mesenteric arteriography was done which demonstrated an angiomatous malformation of the left gastric artery and also varices in the fundus of the stomach (Fig. I). A total gastrectomy was performed and the abdominal oesophagus
anastomosed to a roux-en-Y jejunal loop. Two wk later he suddenly developed catastrophic gastrointestinal bleeding and died. Consent for an autopsy was not granted.
PATHOLOGICAL FINDINGS The 1st operative specimen, consisting of the pylorus and antrum of the stomach, showed only dilated and thickened veins in the submucosa. The 2nd operative specimen included the fundus and body of the stomach. A small mucosal breach plugged by blood clot was found in the anterior gastric wall of the body of the stomach (Fig. 2). Sectioning revealed a ruptured blood vessel beneath the clot. Tortuous thick-walled blood vessels traversing the submucosa were found in the fundus and upper stomach, but they were absent from the distal areas. Histology The specimens were fixed in 10% buffered formalin. Blocks embedded in paraffin were cut at 5 p intervals and stained with haematoxylin and eosin, elastic and Masson's trichrome stains. In the submucosa of the proximal stomach. there were multiple tortuous muscular arteries of a calibre larger than normally expected in this region. They had the 3 well defined layers of a muscular artery, but there were multiple abrupt interruptions of the media and of the internal elastic lamina, resulting in thinning and small saccular aneurysmal dilatations of their walls. In these areas, the intima was directly apposed to the adventitia. The wall of these aneurysms was thus composed of only a few collagenous fibres. Focal intimal thickening was also seen in these arteries. These changes are shown in Fig. 3 which includes also the ruptured artery. The submucosal veins were dilated, with mild focal intimal fibrosis, but were otherwise unremarkable. The covering gastric mucosa was not inflamed and showed no features of peptic ulceration. No abnormal arteries were found in the pylorus and antrum of the stomach.
DISCUSSION An abnormal artery in the submucosa of the stomach as a cause of massive, often fatal haemorrhage has been
82
Pathology (1982), 14, January
KUNG & WONG
FIG. 1 Coeliac arteriogram in the arterial phase demonstrating angiomatous malformation affecting the branches of the left gastric artery. Tortuous and dilated vessels are seen at the fundus of the stomach lateral t o the opacified Ryle’s tube
FIG. 2 A close-up picture of the gastric mucosa. There is a small mucosal ulceration (arrow) beneath which is a ruptured artery. Focal petechiae are also seen
recorded in about 30 cases in the literature. The earlier cases were reviewed by Mandelbaum’ and later by Goldman.’ In his review, Goldman noted that the reported lesions showed a remarkable uniformity of both gross and microscopic characteristics: namely a small
mucosal erosion capped by thrombotic material overlying a ruptured submucosal vessel.’ The involved vessel invariably was unusually large and tortuous and had the typical structure of larger muscular arteries containing 3 well defined layers with a prominent muscular media.
ARTERIAL MALFORMATION OF STOMACH
83
FIG.3 Unusually large tortuous muscular arteries in the submucosa of the stomach. The ruptured artery seen in the gross specimen in Fig. 2 is shown on the left side of the figure. The blood clot covering the denuded area has been dislodged during processing. The artery is found just beneath the mucosa, with compression of the intervening submucosal tissue. The precise rupture site is not seen here, but 2 markedly attenuated areas in the arterial wall can be noticed towards the mucosal side. These probably represent aneurysms. More obvious small saccular aneurysms are seen in the artery on the right side of the figure. The arrow points to one aneurysm, the high power picture of which is shown in Fig. 4. (Masson’s trichrome stain x 9)
Intimal thickening interpreted as localized arteriosclerosis was seen in some but not all cases. He concluded that the vascular abnormality was primary, arteriosclerotic changes being secondary. The affected vessel was thought to represent a localized congenital malformation, but the nature of this could not be demonstrated, though absence of the media in the vicinity of rupture was seen in the case reported by Bailey.3 Several additional cases of arterial malformation were reported in recent years.46 Richter presented another case of rupture of a single tortuous large submucosal artery which displayed no atherosclerosis, aneurysmal dilatation or elastic tissue a b n ~ r m a l i t y No . ~ histological description of the affected vessel was available in Gough’s case,’ though the angiographic studies were consistent with a malformed artery. In the case of Sherman et nl., multiple vessel involvement was found in the stomach, in contrast to the single vessel involvement in previous reports.’-5 Individual vessels in this case were difficult to categorize as arteries or veins. The authors considered that the abnormal structure of these vascular channels was consistent with arterio-venous malformation. In the present case, the gross morphology of the gastric mucosa was similar to that of the cases reviewed by Goldman,’ i.e. a small mucosal defect plugged by blood clot. However, although only one vessel had ruptured, multiple vessels were affected. The affected vessels were intramural branches of the left gastric artery. The lesion
in the arteries was unlikely to be inflammatory in nature, as there was no histological evidence of fibrinoid necrosis or other inflammatory reaction such as those in polyarteritis nodosa.’ The internal elastic lamina was abruptly interrupted rather than fragmented as in giant cell arteritis7 (Fig. 4). The few reactive changes in and around these arteries did not suggest that the abnormalities resulted from previous surgical intervention; of course the plicated vessels were veins, not arteries. The focal interruption of both the media and the internal elastic lamina was reminiscent of berry aneurysms of the cerebral arteries. Berry aneurysms are results of developmental faults in the media and the internal elastic lamina whose absence impairs the ability of the arteries to withstand blood pressure: rupture is therefore a frequent occurrence.’. Berry aneurysms are usually found at the bifurcation ofcerebral arteries. In the present case, though the microaneurysms were not found at bifurcations, the histological features were similar to those of berry aneurysms. It was unfortunate that an autopsy could not be done in this case, but even without it, it could be established that only the branches of a single artery of the stomach were involved. Thus. while the aneurysm in the case reported by Mandelbaum2 appeared to be due to arteriosclerosis, and while the cause of the arterial abnormality in most of the cases reported in the literature was controversial, developmental faults appeared to be the cause in this case.
84
Pathology (1982). 14, January
KUNG & W O N G
FIG. 4 A higher power view of a small aneurysm. In addition to the medial defect, the internal elastic membrane also stops abruptly at the entrance of the aneurysm. The thin wall of the aneurysm is composed of only collagen fibres. (Elastic stain. x 72)
The uniformly dilated veins with intimal fibrosis were features of phlebosclerosis, probably related to the portosystemic shunting of blood as a result of portal hypertension’ in cirrhosis. They were thus not congenitally malformed, and their pathological changes were not related to those in the arteries. This case therefore differed from that of Sherman et aL6 in that arterial malformation rather than combined arterial and venous malformation was its principal feature. It is probable that congenital developmental defects are the cause of multiple vessel involvement while a single artery abnormality is more likely to be due to acquired causes. This case also suggests that arterial malformation should be looked for as a cause of gastrointestinal bleeding when commoner causes have been excluded. The authors are grateful to Miss ACKNOWLEDGEMENT Debbie Lo for typing the manuscript. Address for correspondence: T.M.K., Department of Pathology, University of Hong Kong. Queen Mary Hospital, Hong Kong
References 1. GOLDMAN,R. L. (1964): Submucosal arterial malformation
(‘aneurysm’) of the stomach with fatal hemorrhage. Gastroenterology 46, 589-594. I., KAISER,G. C. & LEMPKE,R. E. (1962): Gastric 2. MANDELBAUM, intramural aneurysm as a cause for massive gastrointestinal hemorrhage. Ann. Surg. 155, 199-203. 3. BAILEY, M. E. (1956): Rupture of a gastric artery aneurysm. Can. Med. Assoc. J . 75, 131-133. 4. RICHTER,R. M. (1975): Massive gastric hemorrhage from submucosal arterial malformation. Am. J. Gastroenterol. 64, 324326. 5. GOUGH,M. (1977): Submucosal arterial malformation of the stomach as the probable cause of recurrent severe haematemesis in a 16-year-old girl. Br. J . Surg. 64, 522-524. S. S., SATCHIDANAND, S. K. et al. (1979): 6. SHERMAN, L., SHENOY, Arteriovenous malformation of the stomach. Am. J . Gastroenterol. 72, 160-164. W. , St C. (1976): Systemic Pathology, Vol. I , 2nd ed. ~ .SY MMERS Churchill Livingstone, London. pp. 140-146. 8. GLYNN, L. E. (1940): Medical defects in the circle of Willis and their relation to aneurysm formation. J. Pathol. Bacteriol. 51, 213-222. E. (1959): Pathogenesis of phlebosclerosis (I. 9. MOSCHCOWITZ, Phlebosclerosis of the portal vein). A M A Arch. Pathol. 68,180-184.