Dig Dis Sci (2014) 59:2653–2655 DOI 10.1007/s10620-014-3388-8
UNM CLINICAL CASE CONFERENCES
A 50-Year-Old Man with Postprandial Epigastric Pain Dino Beduya • Itzhak Nir • Gulshan Parasher
Received: 1 October 2014 / Accepted: 7 October 2014 / Published online: 15 October 2014 Ó Springer Science+Business Media New York 2014
Case Presentation and Evolution A 50-year-old otherwise healthy man, with moderate hypertension, had suffered from intermittent postprandial epigastric pain and bloating for over 3 years. The pain was moderate in intensity, lasted for about 1 h, did not radiate, and had no obvious other precipitating or relieving factors. Although occasional belching was noted, the pain was not accompanied by nausea, vomiting, weight loss, or disturbance of bowel habits. Physical examination revealed an obese man with normal vital signs. His abdomen was soft, non-tender, and non-distended, and bowel sounds were normal. There were no masses palpable in the abdomen. Results of routine blood tests, including serum liver enzymes, bilirubin, amylase, and lipase, carcinoembryonic antigen and CA19-9, were all normal. Solid gastric emptying, delayed 36 % at 2 h, was initially treated empirically with domperidone 10 mg postoperatively three times a day, with partial relief of symptoms. A 0.5-cm duodenal bulb carcinoid was removed via endoscopic mucosal resection, with considerable improvement in his symptoms. Nevertheless, 2 years later during routine follow-up, an elevated serum concentration of chromogranin A, without elevation of concentration of any other gut hormone, was noted as part of an investigation for symptomatic relapse. A computerized tomographic (CT) scan of his abdomen and D. Beduya (&) G. Parasher Division of Gastroenterology and Hepatology, Department of Internal Medicine, MSC10-5550, 1 University of New Mexico, Albuquerque, NM 87131, USA e-mail: [email protected] I. Nir Department of Surgery, University of New Mexico Health Sciences Center, Albuquerque, NM, USA
pelvis was unremarkable. He was then referred to an endoscopic ultrasound examination to evaluate the possibility of having residual or recurrent carcinoid tumor. Radial endosonography (EUS), although revealing no evidence of residual tumor, identified circumferential pancreatic tissue around the second portion of the duodenum, immediately distal to the duodenal sweep, with associated luminal narrowing (Figs. 1, 2). A magnetic resonance imaging (MRI) scan, with MR cholangiopancreatography (MRCP), confirmed the existence of an annular pancreas (Fig. 3a, b). A subsequent upper gastrointestinal barium study displayed narrowing of the second portion of the duodenum, but failed to reveal the presence of a dilated duodenal bulb, characteristic of the condition.
Management The patient underwent exploratory laparotomy, confirming the diagnosis of annular pancreas. A Roux-en-Y gastrojejunostomy with a short duodenojejunal loop was performed, bypassing the duodenal obstruction. His diet was advanced to allow consumption of clear liquids on the fifth postoperative day, with discharge a week after surgery. His symptoms, which had resolved completely, remained absent at his 2-month postoperative follow-up visit.
Discussion Annular pancreas is a rare congenital anomaly in which pancreatic tissue partially or completely encircles the duodenum. Pancreatic tissue can be intramural, interspersed with the muscle fibers of the duodenal wall, or extramural. The reported incidence in adults varies from
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Dig Dis Sci (2014) 59:2653–2655
Fig. 1 Luminal narrowing in the duodenal sweep
Fig. 2 Radial echoendoscope in the duodenal sweep showing circumferential band of tissue with ‘‘salt and pepper’’ echo pattern consistent with pancreatic tissue. Dotted arrow pancreatic tissue; R radial endoscope probe
0.005 to 0.015 % [1]. Although most cases are asymptomatic, symptoms can manifest at any age [2, 3], with half of the cases identified during the neonatal period, often evident as duodenal obstruction [4]. Adult patients are usually diagnosed in the third–sixth decade of life with abdominal pain, the initial symptom in 70–90 %, although other gastrointestinal symptoms may also be present, including vomiting and obstructive jaundice [1]. Pancreatitis as the initial disease expression is reported more commonly among adult patients than it is in children. Annular pancreas is believed to result from an aberration in the embryological development of the pancreas. During the seventh week of gestation, the duodenum rotates,
123
Fig. 3 a T1-weighted coronal MRI shows pancreatic tissue (dotted arrow), encircling the duodenum (arrow). b T1-weighted axial MRI showing descending duodenum encircled by pancreatic tissue. Dotted arrow pancreatic tissue; arrow duodenum
fusing the ventral and dorsal anlages of the pancreas. Lecco theorizes that the free end of a sole ventral bud adheres to the duodenal wall, which during normal embryonic rotation is stretched to encircle the duodenum [5]. Baldwin proposes that the ventral anlage is initially bilobed with the left lobe persisting in the wrong location after duodenal rotation. A modification of Baldwin’s hypothesis proposes that the tip of the left lobe of the ventral anlage adheres to the duodenal wall [3, 4]. More than one embryologic abnormality may be involved. Annular pancreas is often associated with other congenital anomalies, most frequently apparent in children but also occasionally becoming evident only in adulthood. Down syndrome is present in up to 25 % of all cases, with cardiac anomalies present in 25 % of the pediatric age group [6, 7]. Associated pancreas divisum is diagnosed
Dig Dis Sci (2014) 59:2653–2655
mainly in adults. Enteric malrotation, duodenal atresia, and tracheoesophageal fistula can be diagnosed at any age. Adults are particularly susceptible to the development of pancreatic cancer, ampullary adenoma, cholangiocarcinoma, insulinoma, and intraductal mucinous neoplasm, reported in 4 % of cases [7, 8]. Diagnostic modalities useful for the diagnosis of annular pancreas include ultrasound or plain abdominal radiographs, which usually show the classic ‘‘double bubble’’ sign [the presence of two air-filled structures in the upper abdomen (the stomach and proximal duodenum) with little or no air distally] in infants with duodenal obstruction. Surgical management is usually required to relieve the obstruction when the diagnosis of annular pancreas is confirmed operatively. Although surgery has been considered the ‘‘gold standard’’ for the diagnosis of annular pancreas [1, 3], non-operative modalities (CT scanning, MRI, MRCP, ERCP, and EUS) may all suggest the presence of pancreatic tissue or pancreatic ducts encircling the duodenum [9]. MRI and CT scanning have the benefit of being noninvasive, although the correct diagnosis may be overlooked by either scan if the pancreatic tissue is present as a thin band incorporated in the duodenal wall [10]. Since CT findings can be nonspecific, EUS may be a superior diagnostic modality, as it was in the described case [1, 11]. Although radial is superior to linear endosonography at delineating annular pancreas, findings are usually not definitive. If duodenal obstruction prevents passage of the endoscope, endosonography can be completed from the duodenal bulb [12]. In contrast, ERCP, when significant duodenal stenosis precludes cannulation of the ampulla, is minimally successful. Furthermore, when the annular pancreas arises from the duct of Santorini, or the duct drains directly into the duodenum independently of major or minor papillae, a pancreatogram performed through the major papilla at ERCP is not able to demonstrate an annular pancreas [11, 12]. The risk of ERCP-related pancreatitis is reported as 1.6–15.1 % [13]. Annular pancreas complicated by clinical duodenal obstruction is surgically bypassed with a duodenoduodenostomy, duodenojejunostomy, or gastrojejunostomy with excellent outcomes reported in most cases, e.g., 83 % resolution of symptoms in one study of adult patients [14]. In cases with biliary obstruction, a biliary bypass operation (e.g., hepaticojejunostomy) is also performed.
Summary Annular pancreas is a rare congenital anomaly in which pancreatic tissue partially or completely encircles the
2655
duodenum, variably compressing the enteric lumen. Asymptomatic in most patients, it may be initially diagnosed at any age when evaluating the etiology of duodenal obstruction, vomiting, and abdominal pain. Several imaging modalities can strongly suggest the presence of pancreatic tissue or ducts surrounding the duodenum, with MRI, MRCP, and EUS being most effective in this regard. Nonetheless, definitive diagnosis and treatment for complete or partial duodenal obstruction are surgical, with duodenal bypass (enteroenterostomy or gastroenterostomy), and additional biliary bypass when biliary obstruction is also present. The prognosis is excellent in most cases. Conflict of interest
None
References 1. Maker V, Gerzenshtein J, Lerner T. Annular pancreas in the adult: two case reports and review of more than a century of literature. Am Surg. 2003;69:404–410. 2. Ravitch MM, Woods AC Jr. Annular pancreas. Ann Surg. 1950;132:1116–1127. 3. Etienne D, John A, Menias CO, Ward R, et al. Annular pancreas: a review of its molecular embryology, genetic basis and clinical considerations. Annu Anat. 2012;194:422–428. 4. Mortele´ KJ, Rocha TC, Streeter JL, Taylor AJ. Multimodality imaging of pancreatic and biliary congenital anomalies. Radiographics. 2006;26:715–731. 5. Nobukawa B, Otaka M, Suda K, Fujii H, et al. Annular pancreas derived from the paired ventral pancreata, supporting Baldwin’s hypothesis. Pancreas. 2000;20:408–410. 6. Jimenez JC, Emil S, Podnos Y, Nguyen N. Annular pancreas in children: a recent decade’s experience. J Pediatr Surg. 2004;39:1654–1657. 7. Zyromski NJ, Sandoval JA, Ladd AP, et al. Annular pancreas: dramatic differences between children and adults. J Am Coll Surg. 2008;206:1019–1025 (discussion ibid: 1025–1027). 8. Wang K, Wu W, Cong L, Xing X, Zhao Y. Laparoscopic enucleation of insulinoma in a patient with annular pancreas: a rare case. J Gastrointest Surg. 2014;18:878–879. 9. Choi JY, Kim MJ, Kim JH, Lim JS, et al. Annular pancreas: emphasis on magnetic resonance cholangiopancreatography findings. J Comput Assist Tomogr. 2004;28:528–532. 10. Sandrasegaran K, Patel A, Fogel EL, Zyromski NJ, Pitt HA. Annular pancreas in adults. Am J Roentgenol. 2009;193:455–460. 11. Papachristou GI, Topazian MD, Gleeson FC, Levy MJ. EUS features of annular pancreas (with video). Gastrointest Endosc. 2007;65:340–344. 12. Gress F, Yiengpruksawan A, Sherman S, et al. Diagnosis of annular pancreas by endoscopic ultrasound. Gastrointest Endosc. 1996;44:485–489. 13. ASGE Standards of Practice Committee, Anderson MA, Fisher L, et al. Complications of ERCP. Gastrointest Endosc. 2012;75:467–473. 14. Thomford NR, Knight PR, Pace WG, Madura JA. Annular pancreas in the adult: selection of operation. Ann Surg. 1972;176:159–162.
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UNM CLINICAL CASE CONFERENCES
A 50-Year-Old Man with Postprandial Epigastric Pain Dino Beduya • Itzhak Nir • Gulshan Parasher
Received: 1 October 2014 / Accepted: 7 October 2014 / Published online: 15 October 2014 Ó Springer Science+Business Media New York 2014
Case Presentation and Evolution A 50-year-old otherwise healthy man, with moderate hypertension, had suffered from intermittent postprandial epigastric pain and bloating for over 3 years. The pain was moderate in intensity, lasted for about 1 h, did not radiate, and had no obvious other precipitating or relieving factors. Although occasional belching was noted, the pain was not accompanied by nausea, vomiting, weight loss, or disturbance of bowel habits. Physical examination revealed an obese man with normal vital signs. His abdomen was soft, non-tender, and non-distended, and bowel sounds were normal. There were no masses palpable in the abdomen. Results of routine blood tests, including serum liver enzymes, bilirubin, amylase, and lipase, carcinoembryonic antigen and CA19-9, were all normal. Solid gastric emptying, delayed 36 % at 2 h, was initially treated empirically with domperidone 10 mg postoperatively three times a day, with partial relief of symptoms. A 0.5-cm duodenal bulb carcinoid was removed via endoscopic mucosal resection, with considerable improvement in his symptoms. Nevertheless, 2 years later during routine follow-up, an elevated serum concentration of chromogranin A, without elevation of concentration of any other gut hormone, was noted as part of an investigation for symptomatic relapse. A computerized tomographic (CT) scan of his abdomen and D. Beduya (&) G. Parasher Division of Gastroenterology and Hepatology, Department of Internal Medicine, MSC10-5550, 1 University of New Mexico, Albuquerque, NM 87131, USA e-mail: [email protected] I. Nir Department of Surgery, University of New Mexico Health Sciences Center, Albuquerque, NM, USA
pelvis was unremarkable. He was then referred to an endoscopic ultrasound examination to evaluate the possibility of having residual or recurrent carcinoid tumor. Radial endosonography (EUS), although revealing no evidence of residual tumor, identified circumferential pancreatic tissue around the second portion of the duodenum, immediately distal to the duodenal sweep, with associated luminal narrowing (Figs. 1, 2). A magnetic resonance imaging (MRI) scan, with MR cholangiopancreatography (MRCP), confirmed the existence of an annular pancreas (Fig. 3a, b). A subsequent upper gastrointestinal barium study displayed narrowing of the second portion of the duodenum, but failed to reveal the presence of a dilated duodenal bulb, characteristic of the condition.
Management The patient underwent exploratory laparotomy, confirming the diagnosis of annular pancreas. A Roux-en-Y gastrojejunostomy with a short duodenojejunal loop was performed, bypassing the duodenal obstruction. His diet was advanced to allow consumption of clear liquids on the fifth postoperative day, with discharge a week after surgery. His symptoms, which had resolved completely, remained absent at his 2-month postoperative follow-up visit.
Discussion Annular pancreas is a rare congenital anomaly in which pancreatic tissue partially or completely encircles the duodenum. Pancreatic tissue can be intramural, interspersed with the muscle fibers of the duodenal wall, or extramural. The reported incidence in adults varies from
123
2654
Dig Dis Sci (2014) 59:2653–2655
Fig. 1 Luminal narrowing in the duodenal sweep
Fig. 2 Radial echoendoscope in the duodenal sweep showing circumferential band of tissue with ‘‘salt and pepper’’ echo pattern consistent with pancreatic tissue. Dotted arrow pancreatic tissue; R radial endoscope probe
0.005 to 0.015 % [1]. Although most cases are asymptomatic, symptoms can manifest at any age [2, 3], with half of the cases identified during the neonatal period, often evident as duodenal obstruction [4]. Adult patients are usually diagnosed in the third–sixth decade of life with abdominal pain, the initial symptom in 70–90 %, although other gastrointestinal symptoms may also be present, including vomiting and obstructive jaundice [1]. Pancreatitis as the initial disease expression is reported more commonly among adult patients than it is in children. Annular pancreas is believed to result from an aberration in the embryological development of the pancreas. During the seventh week of gestation, the duodenum rotates,
123
Fig. 3 a T1-weighted coronal MRI shows pancreatic tissue (dotted arrow), encircling the duodenum (arrow). b T1-weighted axial MRI showing descending duodenum encircled by pancreatic tissue. Dotted arrow pancreatic tissue; arrow duodenum
fusing the ventral and dorsal anlages of the pancreas. Lecco theorizes that the free end of a sole ventral bud adheres to the duodenal wall, which during normal embryonic rotation is stretched to encircle the duodenum [5]. Baldwin proposes that the ventral anlage is initially bilobed with the left lobe persisting in the wrong location after duodenal rotation. A modification of Baldwin’s hypothesis proposes that the tip of the left lobe of the ventral anlage adheres to the duodenal wall [3, 4]. More than one embryologic abnormality may be involved. Annular pancreas is often associated with other congenital anomalies, most frequently apparent in children but also occasionally becoming evident only in adulthood. Down syndrome is present in up to 25 % of all cases, with cardiac anomalies present in 25 % of the pediatric age group [6, 7]. Associated pancreas divisum is diagnosed
Dig Dis Sci (2014) 59:2653–2655
mainly in adults. Enteric malrotation, duodenal atresia, and tracheoesophageal fistula can be diagnosed at any age. Adults are particularly susceptible to the development of pancreatic cancer, ampullary adenoma, cholangiocarcinoma, insulinoma, and intraductal mucinous neoplasm, reported in 4 % of cases [7, 8]. Diagnostic modalities useful for the diagnosis of annular pancreas include ultrasound or plain abdominal radiographs, which usually show the classic ‘‘double bubble’’ sign [the presence of two air-filled structures in the upper abdomen (the stomach and proximal duodenum) with little or no air distally] in infants with duodenal obstruction. Surgical management is usually required to relieve the obstruction when the diagnosis of annular pancreas is confirmed operatively. Although surgery has been considered the ‘‘gold standard’’ for the diagnosis of annular pancreas [1, 3], non-operative modalities (CT scanning, MRI, MRCP, ERCP, and EUS) may all suggest the presence of pancreatic tissue or pancreatic ducts encircling the duodenum [9]. MRI and CT scanning have the benefit of being noninvasive, although the correct diagnosis may be overlooked by either scan if the pancreatic tissue is present as a thin band incorporated in the duodenal wall [10]. Since CT findings can be nonspecific, EUS may be a superior diagnostic modality, as it was in the described case [1, 11]. Although radial is superior to linear endosonography at delineating annular pancreas, findings are usually not definitive. If duodenal obstruction prevents passage of the endoscope, endosonography can be completed from the duodenal bulb [12]. In contrast, ERCP, when significant duodenal stenosis precludes cannulation of the ampulla, is minimally successful. Furthermore, when the annular pancreas arises from the duct of Santorini, or the duct drains directly into the duodenum independently of major or minor papillae, a pancreatogram performed through the major papilla at ERCP is not able to demonstrate an annular pancreas [11, 12]. The risk of ERCP-related pancreatitis is reported as 1.6–15.1 % [13]. Annular pancreas complicated by clinical duodenal obstruction is surgically bypassed with a duodenoduodenostomy, duodenojejunostomy, or gastrojejunostomy with excellent outcomes reported in most cases, e.g., 83 % resolution of symptoms in one study of adult patients [14]. In cases with biliary obstruction, a biliary bypass operation (e.g., hepaticojejunostomy) is also performed.
Summary Annular pancreas is a rare congenital anomaly in which pancreatic tissue partially or completely encircles the
2655
duodenum, variably compressing the enteric lumen. Asymptomatic in most patients, it may be initially diagnosed at any age when evaluating the etiology of duodenal obstruction, vomiting, and abdominal pain. Several imaging modalities can strongly suggest the presence of pancreatic tissue or ducts surrounding the duodenum, with MRI, MRCP, and EUS being most effective in this regard. Nonetheless, definitive diagnosis and treatment for complete or partial duodenal obstruction are surgical, with duodenal bypass (enteroenterostomy or gastroenterostomy), and additional biliary bypass when biliary obstruction is also present. The prognosis is excellent in most cases. Conflict of interest
None
References 1. Maker V, Gerzenshtein J, Lerner T. Annular pancreas in the adult: two case reports and review of more than a century of literature. Am Surg. 2003;69:404–410. 2. Ravitch MM, Woods AC Jr. Annular pancreas. Ann Surg. 1950;132:1116–1127. 3. Etienne D, John A, Menias CO, Ward R, et al. Annular pancreas: a review of its molecular embryology, genetic basis and clinical considerations. Annu Anat. 2012;194:422–428. 4. Mortele´ KJ, Rocha TC, Streeter JL, Taylor AJ. Multimodality imaging of pancreatic and biliary congenital anomalies. Radiographics. 2006;26:715–731. 5. Nobukawa B, Otaka M, Suda K, Fujii H, et al. Annular pancreas derived from the paired ventral pancreata, supporting Baldwin’s hypothesis. Pancreas. 2000;20:408–410. 6. Jimenez JC, Emil S, Podnos Y, Nguyen N. Annular pancreas in children: a recent decade’s experience. J Pediatr Surg. 2004;39:1654–1657. 7. Zyromski NJ, Sandoval JA, Ladd AP, et al. Annular pancreas: dramatic differences between children and adults. J Am Coll Surg. 2008;206:1019–1025 (discussion ibid: 1025–1027). 8. Wang K, Wu W, Cong L, Xing X, Zhao Y. Laparoscopic enucleation of insulinoma in a patient with annular pancreas: a rare case. J Gastrointest Surg. 2014;18:878–879. 9. Choi JY, Kim MJ, Kim JH, Lim JS, et al. Annular pancreas: emphasis on magnetic resonance cholangiopancreatography findings. J Comput Assist Tomogr. 2004;28:528–532. 10. Sandrasegaran K, Patel A, Fogel EL, Zyromski NJ, Pitt HA. Annular pancreas in adults. Am J Roentgenol. 2009;193:455–460. 11. Papachristou GI, Topazian MD, Gleeson FC, Levy MJ. EUS features of annular pancreas (with video). Gastrointest Endosc. 2007;65:340–344. 12. Gress F, Yiengpruksawan A, Sherman S, et al. Diagnosis of annular pancreas by endoscopic ultrasound. Gastrointest Endosc. 1996;44:485–489. 13. ASGE Standards of Practice Committee, Anderson MA, Fisher L, et al. Complications of ERCP. Gastrointest Endosc. 2012;75:467–473. 14. Thomford NR, Knight PR, Pace WG, Madura JA. Annular pancreas in the adult: selection of operation. Ann Surg. 1972;176:159–162.
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