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Ectopic pancreas with pseudocyst and pseudoaneurysm formation
Clinical Radiology (2009) 64, 734e737
CASE REPORT
Ectopic pancreas with pseudocyst and pseudoaneurysm formation A. Surova,*, M. Hainzb, L. Hinzc, H.-J. Holzhausenb, R. Finkec, R.-P. Spielmanna, C. Kunzea Departments of aRadiology, bPathology, cSurgery, Martin-Luther-University Halle-Wittenberg, Halle, Germany Received 12 November 2008; received in revised form 25 January 2009; accepted 29 January 2009
Ectopic pancreas is a rare congenital anomaly. It is usually asymptomatic, or presents with non specific gastrointestinal symptoms. We describe here a case of ectopic pancreas in the gastric antrum, with pseudocyst and pseudoaneurysm formation. This entity has not been reported previously in the literature. ª 2009 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.
Introduction Ectopic pancreas is a congenital anomaly defined as aberrant pancreatic tissue without direct or vascular connections with the true pancreas.1 Its prevalence varies from 0.55% to 13.7%.1,2,3 The most common sites for ectopic pancreatic tissue are the stomach, the duodenum, and the jejunum.2,4 Ectopic pancreas is usually asymptomatic, or presents with non-specific gastrointestinal symptoms.2 However, acute complications have also been described in the literature.5,6 We present here a case of ectopic pancreas in the gastric antrum, with pseudocyst and pseudoaneurysm formation. This entity has not been reported previously in the literature.
Case report A 12-day-old female infant presented to our emergency room with recurrent episodes of
* Guarantor and correspondent: A. Surov, Department of Radiology, Martin-Luther-University Halle-Wittenberg, Ernst-GrubeStr. 40, 06097 Halle, Germany. Tel.: þ49 345 557 32 66; fax: þ49 345 557 21 67. E-mail address: [email protected] (A. Surov).
haematemesis and melaena. Physical examination revealed no abnormalities. Vital signs, temperature, pulse, and blood pressure were normal. Laboratory investigations showed a haemoglobin level of 15.1 g/dl (normal range 15.5e18.8 g/dl), a red cell count of 4.3 109/l (normal range 4.5e6.4 109/l), and a platelet count of 694 109/l (normal range 140e440 109/l). Other routine blood and serum tests were normal. Transabdominal sonography showed a homogeneous mass in the right gastrohepatic region, measuring approximately 1.3 1.6 1.5 cm3. It was hypoechogenic to the liver, but it contained several internal echoes (Fig. 1a). On colour Doppler a communication between the mass and a vessel was seen (Fig. 1b, arrow). Bidirectional spectral Doppler of the mass documented a pulsatile turbulent flow (Fig. 1c). A non-contrast-enhanced computed tomography (CT) examination revealed a hypodense, oval, submucosal lesion with a hyperdense margin at the wall of the prepyloric antrum, correlating with the mass noted before (Fig. 2a). After administering intravenous contrast medium (10 ml Solutrast 300, Altana Berlin), marked homogeneous enhancement was seen (Fig. 2b). No oral contrast medium was given prior to the CT scans. In view of the clinical presentation and radiological findings, showing a well-vascularized lesion
0009-9260/$ - see front matter ª 2009 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.crad.2009.01.013
Ectopic pancreas
735
Figure 2 (a) CT image of the abdomen without intravenous or oral contrast medium showing a hypodense oval submucosal lesion with well-defined margins in the wall of prepyloric antrum (arrow). (b) CT scan of the abdomen with administration of intravenous contrast revealed a marked homogenous enhancement of the lesion.
Figure 1 (a) Transverse ultrasound image of the upper abdomen showing a hypoechogenic mass in the right gastrohepatic region. (b) Colour Doppler flow image demonstrating a communication between the mass and a vessel (arrow). (c) Pulse spectral Doppler flow documented a pulsatile turbulent flow within the mass.
at the gastric wall, a bleeding arteriovenous malformation was suspected. The lesion was surgically resected. Because of the presumably ample vascularization of the mass, the surrounding vessels in the operation field were electrocoagulated. There were no intraoperative complications. At surgery the mass was red in colour, encapsulated, and confined to the gastric wall at the lesser curvature. It was elastic and contained fresh blood. Further, coagulated blood was seen within the wall of the mass. This finding may correspond to the
736
Figure 3 Histologic section of the mass ectopic pancreatic tissue (arrows) within the normal mucosa (hematoxylin and eosin, 100).
finding at unenhanced CT of the very dense margin of the mass. Gross pathology did not show any direct contact of the mass with the surrounding vessels. There was no evidence of arteriovenous malformation. Histological examination of the mass revealed ectopic pancreatic tissue with a pseudocyst, containing fresh and partially coagulated blood (Fig. 3a,b). The infant tolerated the operation well and was discharged in good general condition from our hospital. Thereafter no further episode of gastrointestinal bleeding occurred over the following 2 months.
Discussion According to the literature, pancreatic heterotopia is rarely diagnosed before the age of 40.1,2 Findings of this entity are very rare in children and infants. Clinically, ectopic pancreas can manifest with a broad spectrum of symptoms from dyspepsia and epigastric fullness to acute gastrointestinal obstruction, severe haemorrhage, and intestinal perforation.2,3,5e7 Most patients with ectopic pancreas are asymptomatic.2 In our case a pseudocyst and pseudoaneurysm within the ectopic pancreatic tissue was detected, complicated by gastrointestinal bleeding. Upper gastrointestinal bleeding in children and infants can be caused by several disorders, such as arteriovenous malformations, peptic ulcer disease, gastroduodenal malignancies, foreign body ingestion, haemorrhagic gastroduodenopathy, and gastric varices.8e13 Gastrointestinal endoscopy has established itself as a useful method of diagnosis, reassessment, and therapy of gastrointestinal
A. Surov et al.
bleeding.13,14 In our case abdominal sonography and computer tomography as non-invasive approaches were performed after admission. In view of the clinical presentation and radiological findings a bleeding arteriovenous malformation was suspected and no endoscopy was performed. As the mass, which finally proved to be an ectopic pancreatic pseudo cyst, demonstrated pulsatile flow by ultrasound,it obviously formed a pseudoaneurysm. Although histopathological examination did not show any direct communication of the diagnosed ectopic tissue to the surrounding vessels, the finding of fresh blood within the pseudocyst supports this suggestion. Further, intraoperative electrocoagulation of the vessels around the mass may also explain this discrepancy between radiological and pathological findings. According to Mulholland et al., cystic formation in ectopic pancreas could result from retention of exocrine secretions in the absence of a communication between the glandular epithelium and the gastric lumen.7 Furthermore, the accumulation of pancreatic secretions can lead to recurrent episodes of acute obstructive pancreatitis and to the formation of retention cysts.15 True pseudocyst formation, as in our case, is extremely rare.7 This complication can occur after pancreatitis in ectopic pancreatic tissue and has been described in only a few reports.16e18 It is well known that pancreatic pseudocysts may form pseudoaneurysms due to enzymatic erosion of blood vessels.19,20 However, a pseudoaneurysm resulting from ectopic pancreatic tissue has not been reported previously. A definitive diagnosis of ectopic pancreas in the stomach can only be made by histological examination. The clinical signs are non-specific. At CT, ectopic pancreas manifests as an oval or round mass in the gastric wall with contrast enhancement similar to that of the normal pancreas.4,21 Endoscopically, pancreatic heterotopia may present as a broad-based submucosal nodule.7 However, the differential diagnosis from other submucosal tumours, such as leiomyoma or carcinoid, is not possible by endoscopy.4,7,21,22 Ectopic pancreatic tissue can also develop in gastric wall duplication.23 In the present case there was no evidence of gastric duplication. The treatment of complicated ectopic pancreatic tissue is surgical resection or endoscopical excision.2 According to the literature, an asymptomatic pancreatic heterotopia should not be managed operatively, as the risk of malignant change is low.24 In conclusion, ectopic pancreas should be included in the differential diagnosis of intramural masses of the stomach. The unique features of the
Ectopic pancreas
presented case are the development of a pseudocyst and subsequently of a pseudoaneurysm.
References 1. Thoeni RF, Gedgaudas RK. Ectopic pancreas: usual and unusual features. Gastrointest Radiol 1980;5(1):37e42. 2. Pang LC. Pancreatic heterotopia: a reappraisal and clinicopathologic analysis of 32 cases. South Med J 1988;81: 1264e75. 3. Marshall SF, Curtiss FM. Aberrant pancreas in stomach wall. Surg Clin North Am 1952;32:867e75. 4. Cho JS, Shin KS, Kwon ST, et al. Heterotopic pancreas in the stomach: CT findings. Radiology 2000;217:139e44. 5. Fragoso AC, Correia-Pinto J, Carvalho JL, et al. Ectopic pancreas and foveolar hyperplasia in a newborn: a unifying etiopathogenesis for gastric outlet obstruction. J Pediatr Gastroenterol Nutr 2003;39:92e4. 6. Joo YE, Kim HS, Choi SK, et al. Massive gastrointestinal bleeding caused by ectopic pancreas mimicking jejunal tumor. Digestion 2001;64:133e6. 7. Mulholland K, Wallace W, Epanomeritakis E, et al. Pseudocyst in gastric ectopic pancreas. JOP 2004;5:498e501. 8. Millar AJ, Brown RA, Hill ID, et al. The fundal pile: bleeding gastric varices. J Pediatr Surg 1991;6:707e9. 9. Henderson CT, Engel J, Schlesinger P. Foreign body ingestion: review and suggested guidelines for management. Endoscopy 1987;19:68e71. 10. Berezin SH, Bostwick HE, Halata MS, et al. Gastrointestinal bleeding in children following ingestion of low-dose ibuprofen. J Pediatr Gastroenterol Nutr 2007;4:506e8. 11. Odell JM, Haas JE, Tapper D, et al. Infantile hemorrhagic angiodysplasia. Pediatr Pathol 1987;7:629e36. 12. Libson E, Mapp E, Dachman AH. Hodgkin’s disease of the gastrointestinal tract. Clin Radiol 1994;49:166e9.
737
13. Tam PK, Saing H. Pediatric upper gastrointestinal endoscopy: a 13-year experience. J Pediatr Surg 1989;5:443e7. 14. Ching YA, Modi BP, Duggan C. High diagnostic yield of gastrointestinal endoscopy in children with intestinal failure. J Pediatr Surg 2008;43:906e10. 15. Galloro G, Napolitano V, Magno L, et al. Diagnosis and therapeutic management of cystic dystrophy of the duodenal wall in heterotopic pancreas. A case report and revision of the literature. JOP 2008;9:725e32. 16. Tillig B, Gereinm V, Coerdt W, et al. Large supraumbilical pseudocystic tumor due to ectopic pancreatic tissue located in a rest of the omphaloenteric duct. Eur J Pediatr Surg 2004;14:126e9. 17. Claudon M, Verain AL, Bigard MA, et al. Cyst formation in gastric heterotopic pancreas: report of two cases. Radiology 1988;169:659e60. 18. Chung JP, lee SI, Kim KW, et al. Duodenal ectopic pancreas complicated by chronic pancreatitis and pseudocyst formationda case report. J Korean Med Sci 1994;9:353e6. 19. Carr JA, Cho JS, Shepard A, et al. Visceral pseudoaneurysm due to pancreatic pseudocyst: rare but lethal complication of pancreatitis. J Vasc Surg 2000;32:722e30. 20. Dasqupta R, Davies NJ, Williamson RC, et al. Haemosuccus pancreaticus: treatment by arterial embolization. Clin Radiol 2002;57:1021e7. 21. Wang CK, Kuo YT, Yeung KW, et al. CT appearance of ectopic pancreas: a case report. Abdom Imaging 1998;23: 332e3. 22. Shah A, Gordon AR, Ginsberg GG, et al. Case report: ectopic pancreatic rest in the proximal stomach mimicking gastric neoplasms. Clin Radiol 2007;62:600e2. 23. Camoglio FS, Forestieri C, Zanatta C, et al. Complete pancreatic ectopia in a gastric duplication cyst: a case report and review of the literature. Eur J Pediatr Surg 2004;14:60e2. 24. Ura H, Denno R, Hirata K, et al. Carcinoma arising from ectopic pancreas in the stomach: endosonographic detection of malignant change. J Clin Ultrasound 1998;26:265e8.
CASE REPORT
Ectopic pancreas with pseudocyst and pseudoaneurysm formation A. Surova,*, M. Hainzb, L. Hinzc, H.-J. Holzhausenb, R. Finkec, R.-P. Spielmanna, C. Kunzea Departments of aRadiology, bPathology, cSurgery, Martin-Luther-University Halle-Wittenberg, Halle, Germany Received 12 November 2008; received in revised form 25 January 2009; accepted 29 January 2009
Ectopic pancreas is a rare congenital anomaly. It is usually asymptomatic, or presents with non specific gastrointestinal symptoms. We describe here a case of ectopic pancreas in the gastric antrum, with pseudocyst and pseudoaneurysm formation. This entity has not been reported previously in the literature. ª 2009 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.
Introduction Ectopic pancreas is a congenital anomaly defined as aberrant pancreatic tissue without direct or vascular connections with the true pancreas.1 Its prevalence varies from 0.55% to 13.7%.1,2,3 The most common sites for ectopic pancreatic tissue are the stomach, the duodenum, and the jejunum.2,4 Ectopic pancreas is usually asymptomatic, or presents with non-specific gastrointestinal symptoms.2 However, acute complications have also been described in the literature.5,6 We present here a case of ectopic pancreas in the gastric antrum, with pseudocyst and pseudoaneurysm formation. This entity has not been reported previously in the literature.
Case report A 12-day-old female infant presented to our emergency room with recurrent episodes of
* Guarantor and correspondent: A. Surov, Department of Radiology, Martin-Luther-University Halle-Wittenberg, Ernst-GrubeStr. 40, 06097 Halle, Germany. Tel.: þ49 345 557 32 66; fax: þ49 345 557 21 67. E-mail address: [email protected] (A. Surov).
haematemesis and melaena. Physical examination revealed no abnormalities. Vital signs, temperature, pulse, and blood pressure were normal. Laboratory investigations showed a haemoglobin level of 15.1 g/dl (normal range 15.5e18.8 g/dl), a red cell count of 4.3 109/l (normal range 4.5e6.4 109/l), and a platelet count of 694 109/l (normal range 140e440 109/l). Other routine blood and serum tests were normal. Transabdominal sonography showed a homogeneous mass in the right gastrohepatic region, measuring approximately 1.3 1.6 1.5 cm3. It was hypoechogenic to the liver, but it contained several internal echoes (Fig. 1a). On colour Doppler a communication between the mass and a vessel was seen (Fig. 1b, arrow). Bidirectional spectral Doppler of the mass documented a pulsatile turbulent flow (Fig. 1c). A non-contrast-enhanced computed tomography (CT) examination revealed a hypodense, oval, submucosal lesion with a hyperdense margin at the wall of the prepyloric antrum, correlating with the mass noted before (Fig. 2a). After administering intravenous contrast medium (10 ml Solutrast 300, Altana Berlin), marked homogeneous enhancement was seen (Fig. 2b). No oral contrast medium was given prior to the CT scans. In view of the clinical presentation and radiological findings, showing a well-vascularized lesion
0009-9260/$ - see front matter ª 2009 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.crad.2009.01.013
Ectopic pancreas
735
Figure 2 (a) CT image of the abdomen without intravenous or oral contrast medium showing a hypodense oval submucosal lesion with well-defined margins in the wall of prepyloric antrum (arrow). (b) CT scan of the abdomen with administration of intravenous contrast revealed a marked homogenous enhancement of the lesion.
Figure 1 (a) Transverse ultrasound image of the upper abdomen showing a hypoechogenic mass in the right gastrohepatic region. (b) Colour Doppler flow image demonstrating a communication between the mass and a vessel (arrow). (c) Pulse spectral Doppler flow documented a pulsatile turbulent flow within the mass.
at the gastric wall, a bleeding arteriovenous malformation was suspected. The lesion was surgically resected. Because of the presumably ample vascularization of the mass, the surrounding vessels in the operation field were electrocoagulated. There were no intraoperative complications. At surgery the mass was red in colour, encapsulated, and confined to the gastric wall at the lesser curvature. It was elastic and contained fresh blood. Further, coagulated blood was seen within the wall of the mass. This finding may correspond to the
736
Figure 3 Histologic section of the mass ectopic pancreatic tissue (arrows) within the normal mucosa (hematoxylin and eosin, 100).
finding at unenhanced CT of the very dense margin of the mass. Gross pathology did not show any direct contact of the mass with the surrounding vessels. There was no evidence of arteriovenous malformation. Histological examination of the mass revealed ectopic pancreatic tissue with a pseudocyst, containing fresh and partially coagulated blood (Fig. 3a,b). The infant tolerated the operation well and was discharged in good general condition from our hospital. Thereafter no further episode of gastrointestinal bleeding occurred over the following 2 months.
Discussion According to the literature, pancreatic heterotopia is rarely diagnosed before the age of 40.1,2 Findings of this entity are very rare in children and infants. Clinically, ectopic pancreas can manifest with a broad spectrum of symptoms from dyspepsia and epigastric fullness to acute gastrointestinal obstruction, severe haemorrhage, and intestinal perforation.2,3,5e7 Most patients with ectopic pancreas are asymptomatic.2 In our case a pseudocyst and pseudoaneurysm within the ectopic pancreatic tissue was detected, complicated by gastrointestinal bleeding. Upper gastrointestinal bleeding in children and infants can be caused by several disorders, such as arteriovenous malformations, peptic ulcer disease, gastroduodenal malignancies, foreign body ingestion, haemorrhagic gastroduodenopathy, and gastric varices.8e13 Gastrointestinal endoscopy has established itself as a useful method of diagnosis, reassessment, and therapy of gastrointestinal
A. Surov et al.
bleeding.13,14 In our case abdominal sonography and computer tomography as non-invasive approaches were performed after admission. In view of the clinical presentation and radiological findings a bleeding arteriovenous malformation was suspected and no endoscopy was performed. As the mass, which finally proved to be an ectopic pancreatic pseudo cyst, demonstrated pulsatile flow by ultrasound,it obviously formed a pseudoaneurysm. Although histopathological examination did not show any direct communication of the diagnosed ectopic tissue to the surrounding vessels, the finding of fresh blood within the pseudocyst supports this suggestion. Further, intraoperative electrocoagulation of the vessels around the mass may also explain this discrepancy between radiological and pathological findings. According to Mulholland et al., cystic formation in ectopic pancreas could result from retention of exocrine secretions in the absence of a communication between the glandular epithelium and the gastric lumen.7 Furthermore, the accumulation of pancreatic secretions can lead to recurrent episodes of acute obstructive pancreatitis and to the formation of retention cysts.15 True pseudocyst formation, as in our case, is extremely rare.7 This complication can occur after pancreatitis in ectopic pancreatic tissue and has been described in only a few reports.16e18 It is well known that pancreatic pseudocysts may form pseudoaneurysms due to enzymatic erosion of blood vessels.19,20 However, a pseudoaneurysm resulting from ectopic pancreatic tissue has not been reported previously. A definitive diagnosis of ectopic pancreas in the stomach can only be made by histological examination. The clinical signs are non-specific. At CT, ectopic pancreas manifests as an oval or round mass in the gastric wall with contrast enhancement similar to that of the normal pancreas.4,21 Endoscopically, pancreatic heterotopia may present as a broad-based submucosal nodule.7 However, the differential diagnosis from other submucosal tumours, such as leiomyoma or carcinoid, is not possible by endoscopy.4,7,21,22 Ectopic pancreatic tissue can also develop in gastric wall duplication.23 In the present case there was no evidence of gastric duplication. The treatment of complicated ectopic pancreatic tissue is surgical resection or endoscopical excision.2 According to the literature, an asymptomatic pancreatic heterotopia should not be managed operatively, as the risk of malignant change is low.24 In conclusion, ectopic pancreas should be included in the differential diagnosis of intramural masses of the stomach. The unique features of the
Ectopic pancreas
presented case are the development of a pseudocyst and subsequently of a pseudoaneurysm.
References 1. Thoeni RF, Gedgaudas RK. Ectopic pancreas: usual and unusual features. Gastrointest Radiol 1980;5(1):37e42. 2. Pang LC. Pancreatic heterotopia: a reappraisal and clinicopathologic analysis of 32 cases. South Med J 1988;81: 1264e75. 3. Marshall SF, Curtiss FM. Aberrant pancreas in stomach wall. Surg Clin North Am 1952;32:867e75. 4. Cho JS, Shin KS, Kwon ST, et al. Heterotopic pancreas in the stomach: CT findings. Radiology 2000;217:139e44. 5. Fragoso AC, Correia-Pinto J, Carvalho JL, et al. Ectopic pancreas and foveolar hyperplasia in a newborn: a unifying etiopathogenesis for gastric outlet obstruction. J Pediatr Gastroenterol Nutr 2003;39:92e4. 6. Joo YE, Kim HS, Choi SK, et al. Massive gastrointestinal bleeding caused by ectopic pancreas mimicking jejunal tumor. Digestion 2001;64:133e6. 7. Mulholland K, Wallace W, Epanomeritakis E, et al. Pseudocyst in gastric ectopic pancreas. JOP 2004;5:498e501. 8. Millar AJ, Brown RA, Hill ID, et al. The fundal pile: bleeding gastric varices. J Pediatr Surg 1991;6:707e9. 9. Henderson CT, Engel J, Schlesinger P. Foreign body ingestion: review and suggested guidelines for management. Endoscopy 1987;19:68e71. 10. Berezin SH, Bostwick HE, Halata MS, et al. Gastrointestinal bleeding in children following ingestion of low-dose ibuprofen. J Pediatr Gastroenterol Nutr 2007;4:506e8. 11. Odell JM, Haas JE, Tapper D, et al. Infantile hemorrhagic angiodysplasia. Pediatr Pathol 1987;7:629e36. 12. Libson E, Mapp E, Dachman AH. Hodgkin’s disease of the gastrointestinal tract. Clin Radiol 1994;49:166e9.
737
13. Tam PK, Saing H. Pediatric upper gastrointestinal endoscopy: a 13-year experience. J Pediatr Surg 1989;5:443e7. 14. Ching YA, Modi BP, Duggan C. High diagnostic yield of gastrointestinal endoscopy in children with intestinal failure. J Pediatr Surg 2008;43:906e10. 15. Galloro G, Napolitano V, Magno L, et al. Diagnosis and therapeutic management of cystic dystrophy of the duodenal wall in heterotopic pancreas. A case report and revision of the literature. JOP 2008;9:725e32. 16. Tillig B, Gereinm V, Coerdt W, et al. Large supraumbilical pseudocystic tumor due to ectopic pancreatic tissue located in a rest of the omphaloenteric duct. Eur J Pediatr Surg 2004;14:126e9. 17. Claudon M, Verain AL, Bigard MA, et al. Cyst formation in gastric heterotopic pancreas: report of two cases. Radiology 1988;169:659e60. 18. Chung JP, lee SI, Kim KW, et al. Duodenal ectopic pancreas complicated by chronic pancreatitis and pseudocyst formationda case report. J Korean Med Sci 1994;9:353e6. 19. Carr JA, Cho JS, Shepard A, et al. Visceral pseudoaneurysm due to pancreatic pseudocyst: rare but lethal complication of pancreatitis. J Vasc Surg 2000;32:722e30. 20. Dasqupta R, Davies NJ, Williamson RC, et al. Haemosuccus pancreaticus: treatment by arterial embolization. Clin Radiol 2002;57:1021e7. 21. Wang CK, Kuo YT, Yeung KW, et al. CT appearance of ectopic pancreas: a case report. Abdom Imaging 1998;23: 332e3. 22. Shah A, Gordon AR, Ginsberg GG, et al. Case report: ectopic pancreatic rest in the proximal stomach mimicking gastric neoplasms. Clin Radiol 2007;62:600e2. 23. Camoglio FS, Forestieri C, Zanatta C, et al. Complete pancreatic ectopia in a gastric duplication cyst: a case report and review of the literature. Eur J Pediatr Surg 2004;14:60e2. 24. Ura H, Denno R, Hirata K, et al. Carcinoma arising from ectopic pancreas in the stomach: endosonographic detection of malignant change. J Clin Ultrasound 1998;26:265e8.