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doi:10.1111/jgh.12815
E D U C AT I O N A N D I M A G I N G
Gastrointestinal: Recurrent pancreatitis due to pancreatic arteriovenous malformation
Figure 1 Contrast-enhanced computed tomography revealing an abnormal vascular network around the pancreas head.
A 41-year-old man was admitted to our emergency room with epigastric pain lasting approximately 2 weeks. Laboratory studies revealed an elevated amylase level (145 IU/L) and an elevated white blood cell count (16 600 /uL). Plain computed tomography (CT) showed localized inflammation around the head of the pancreas. Although no etiologic factors, such as recent history of drinking, gallstone, hypertriglyceridemia, and drug history, were found, he was diagnosed with acute pancreatitis. Conservative treatment, including aggressive intravenous hydration, promptly resolved the symptom, but severe epigastric pain accompanied by serum amylase elevation relapsed immediately after oral intake resumed. Contrast-enhanced CT (CECT) revealed localized inflammation at the pancreas head and a large number of small vessels comprising an abnormal vascular network around the inflamed area with early contrast filling of the portal vein during the arterial phase, a typical feature of pancreatic arteriovenous malformation (PAVM) (Fig. 1). When he had a third recurrence of epigastric pain with elevated amylase levels on day 20, he vomited small amounts of blood. Esophagogastroduodenoscopy revealed oozing from the pancreatic duct of the major duodenal papilla (Fig. 2a). Angiography showed almost the same findings as CECT and confirmed the diagnosis of PAVM. A pancreatoduodenectomy was performed on day 65 of his hospital stay. Histologically, perforation of the vessels of PAVM into the pancreatic duct was observed, suggesting bleeding from the vessels of PAVM into the pancreatic duct (Fig. 2b). The postoperative course was good, and he was discharged on day 100. Since the first report of PAVM in 1968, approximately 90 cases have been reported. PAVM is usually classified as congenital or acquired. The congenital type includes congenital anomalies arising from a remnant of the rudimentary plexus of primordial blood vessels and is complicated by hereditary hemorrhagic telangiectasia, also known as Osler–Weber–Rendu syndrome. The
2
Figure 2 (a) Esophagogastroduodenoscopy showing oozing from the pancreatic duct of the major duodenal papilla. (b) Histological finding revealing the perforation of the vessels of pancreatic arteriovenous malformation (PAVM) into the pancreatic duct.
acquired type occurs secondarily to inflammation, trauma, or tumor. Our case was thought to be a congenital anomaly because findings suggestive of hereditary hemorrhagic telangiectasia and acquired factors were absent. PAVM shows various clinical presentations, including gastrointestinal bleeding mainly due to the portal hypertension state, abdominal pain, and jaundice, while some cases progress asymptomatically. Pancreatitis due to PAVM is very rare, with only nine previously reported cases. The mechanism of pancreatitis caused by PAVM is considered to be bleeding from the vessels of the AVM into the pancreatic duct that stimulates the pancreas or ischemia of the pancreas tissue around the PAVM. In our case, pooling of blood in the pancreatic duct was confirmed by both endoscopic and histological examinations. Treatment of symptomatic PAVM usually requires a radical approach, and surgical resection is the most effective approach, although it is highly invasive. Transarterial embolization is an alternative method, but it can be difficult to perform successfully because PAVM usually has multiple feeding arteries, which makes it difficult to attain effective embolization. These findings emphasize that although PAVM is a rare disease, it should be considered one of the causative factors for acute pancreatitis that sometimes require radical surgery. Contributed by M Lee,* H Marusawa* and Y Yamashita† *Department of Gastroenterology and Hepatology Graduate School of Medicine, Kyoto University, Kyoto and † Department of Gastroenterology and Hepatology Wakayama Red Cross Hospital, Wakayama, Japan
Journal of Gastroenterology and Hepatology 30 (2015) 2 © 2014 Journal of Gastroenterology and Hepatology Foundation and Wiley Publishing Asia Pty Ltd
doi:10.1111/jgh.12815
E D U C AT I O N A N D I M A G I N G
Gastrointestinal: Recurrent pancreatitis due to pancreatic arteriovenous malformation
Figure 1 Contrast-enhanced computed tomography revealing an abnormal vascular network around the pancreas head.
A 41-year-old man was admitted to our emergency room with epigastric pain lasting approximately 2 weeks. Laboratory studies revealed an elevated amylase level (145 IU/L) and an elevated white blood cell count (16 600 /uL). Plain computed tomography (CT) showed localized inflammation around the head of the pancreas. Although no etiologic factors, such as recent history of drinking, gallstone, hypertriglyceridemia, and drug history, were found, he was diagnosed with acute pancreatitis. Conservative treatment, including aggressive intravenous hydration, promptly resolved the symptom, but severe epigastric pain accompanied by serum amylase elevation relapsed immediately after oral intake resumed. Contrast-enhanced CT (CECT) revealed localized inflammation at the pancreas head and a large number of small vessels comprising an abnormal vascular network around the inflamed area with early contrast filling of the portal vein during the arterial phase, a typical feature of pancreatic arteriovenous malformation (PAVM) (Fig. 1). When he had a third recurrence of epigastric pain with elevated amylase levels on day 20, he vomited small amounts of blood. Esophagogastroduodenoscopy revealed oozing from the pancreatic duct of the major duodenal papilla (Fig. 2a). Angiography showed almost the same findings as CECT and confirmed the diagnosis of PAVM. A pancreatoduodenectomy was performed on day 65 of his hospital stay. Histologically, perforation of the vessels of PAVM into the pancreatic duct was observed, suggesting bleeding from the vessels of PAVM into the pancreatic duct (Fig. 2b). The postoperative course was good, and he was discharged on day 100. Since the first report of PAVM in 1968, approximately 90 cases have been reported. PAVM is usually classified as congenital or acquired. The congenital type includes congenital anomalies arising from a remnant of the rudimentary plexus of primordial blood vessels and is complicated by hereditary hemorrhagic telangiectasia, also known as Osler–Weber–Rendu syndrome. The
2
Figure 2 (a) Esophagogastroduodenoscopy showing oozing from the pancreatic duct of the major duodenal papilla. (b) Histological finding revealing the perforation of the vessels of pancreatic arteriovenous malformation (PAVM) into the pancreatic duct.
acquired type occurs secondarily to inflammation, trauma, or tumor. Our case was thought to be a congenital anomaly because findings suggestive of hereditary hemorrhagic telangiectasia and acquired factors were absent. PAVM shows various clinical presentations, including gastrointestinal bleeding mainly due to the portal hypertension state, abdominal pain, and jaundice, while some cases progress asymptomatically. Pancreatitis due to PAVM is very rare, with only nine previously reported cases. The mechanism of pancreatitis caused by PAVM is considered to be bleeding from the vessels of the AVM into the pancreatic duct that stimulates the pancreas or ischemia of the pancreas tissue around the PAVM. In our case, pooling of blood in the pancreatic duct was confirmed by both endoscopic and histological examinations. Treatment of symptomatic PAVM usually requires a radical approach, and surgical resection is the most effective approach, although it is highly invasive. Transarterial embolization is an alternative method, but it can be difficult to perform successfully because PAVM usually has multiple feeding arteries, which makes it difficult to attain effective embolization. These findings emphasize that although PAVM is a rare disease, it should be considered one of the causative factors for acute pancreatitis that sometimes require radical surgery. Contributed by M Lee,* H Marusawa* and Y Yamashita† *Department of Gastroenterology and Hepatology Graduate School of Medicine, Kyoto University, Kyoto and † Department of Gastroenterology and Hepatology Wakayama Red Cross Hospital, Wakayama, Japan
Journal of Gastroenterology and Hepatology 30 (2015) 2 © 2014 Journal of Gastroenterology and Hepatology Foundation and Wiley Publishing Asia Pty Ltd
