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Ectopic pancreas with acute inflammation
E c t o p i c P a n c r e a s with Acute Inflammation By S. Fam, D. S. O'Briain, J. A. Borger Boston, Massachusetts 9 This case r e p o r t m a k e s the eighth r e p o r t e d case of an a c u t e inflammation of the pancreas occurring at an ectopic site in t h e English l i t e r a t u r e and the first, to our k n o w l e d g e , occurring in the small bowel mesentary. I N D E X W O R D S : Ectopic pancreas.
C T O P I C pancreas is defined as pancreatic tissue that lacks anatomic and vascular continuity with the main body of the pancreas. This is no longer an unusual condition. There have been several reports in the literature of ectopia at various locations, ~ 3 however, cases of acute pancreatitis at an ectopic site are rare and often anecdotal in the English literature. 2~~,~2
E
CASE REPORT U. C. is a 12-yr-old who presented with a 24-hr history of periumbilica[ abdominal pain, nausea and vomiting. On admission he had a temperature of 100~ Physical examination revealed a rigid abdomen with absent bowel sounds. No mass was palpable. Rectal examination was negative. Laboratory results showed that the WBC was 14,000. The preoperative diagnosis was probable perforated appendicitis although the history was somewhat atypical. On exploration purulent looking material was found in the abdomen. The appendix was normal. A purulent node with fibrinous exudate was found at the base of the midjejunal mesentary and a diagnosis of acute suppurative adenitis was made at this time. This node was excised along with a smaller firm node in the same area of the mesentary. The main pancreas was not explored. The patient's postoperative course was uncomplicated and he was discharged on the fifth postoperative day. Amylase was done on postoperative day 2 and was within normal limits at 138 U. Cultures from the peritoneal cavity at operation were negative. Microscopic examination revealed that the larger of the two nodes was acutely inflamed ectopic pancreas. Later, questioning of the patient revealed no history of mumps, alcohol consumption, family history of hyperlipidemia or pancreatitis.
PATHOLOGY The appendix was histologically normal. The smaller mesenteric nodule was a lymph node with reactive sinusoidal
From the Department of Pediatric Surgery, Boston City Hospital, 30 Warren St., Brighton, Mass. 02135 Address reprint requests to James A. Borger, M.D., 818 Harrison Avenue, Boston, Mass. 02118. 9 1982 by Grune & Stratton, Inc. 0022-3468/82/170 1-0024501.00/0 86
hyperplasia. The larger mesenteric nodule measuring 1.5 • 1 • 0.7 cm consisted on ectopic pancreatic tissue with islets of Langerhans, abundant acini and well-formed ducts. A well developed acute pancreatitis involved about one-third of the tissue with necrosis of parenchyma, in some lobules involving only the central area and in others extending to the perilobular connective tissue. An abundant exudate of polymorphonuclear cells surrounded the necrosis. There was a mild amount of red blood cell extravasation and several foci of fat necrosis. A large vessel showed recent thrombus formation, probably secondary to the surrounding inflammation.
DISCUSSION Ectopic
pancreas
o c c u r s in 1 % - 2 %
s i e s a n d in 1 : 5 0 0 l a p a r o t o m i e s
of autop-
with a male ratio
of 3:1. According to Dolan 3 who reviewed 212 cases at the Mayo Clinic 45% of the cases occur in the stomach and duodenum, 35% in the jejunum, 15% in the Meckel's diverticulum and 6% in the ileum. Ectopia has also been reported in the biliary tract, umbilicus, appendix, liver, omenturn, mesentary and fallopian t u b e s Y Dolan critically examined the association of ectopic pancreas with symptoms. He found that 66% of the cases were incidental findings at laparotomy. In 17% of the cases, ectopic pancreas was the only abnormality to account for the patient's symptoms and was, therefore, surgically excised. Only 35% of this group showed postoperative improvement. The remainder of the group either showed no improvement, or had symptoms that were felt in retrospect to be functional. In the remaining 17% of the cases, the ectopic pancreas was diagnosed by x-rays but not surgically removed. Follow-up of this group showed that the disease was not attributed to ectopic pancreas. This makes it apparent that most cases of ectopic pancreas are indeed asymptomatic. Almost all GI symptoms have been attributed to ectopic pancreas. The most common symptoms fall into the following groups: (1) upper G I bleeding; (2) abdominal pain, discomfort and bloating. There are two reported cases of cancer occurring in an ectopic site. 4 The mechanism by which symptoms are produced is not always clear. When the presenting symptom is an upper GI hemorrhage, one may postulate that the alkaline Journal of Pediatric Surgery, Vol. 17, No. 1 (February), 1982
ECTOPIC PANCREAS
87
e n z y m e s p r o d u c e d by the ectopic p a n c r e a s stimulate gastric secretion and increase a c i d i t y a n d lead to ulcer f o r m a t i o n and bleeding. 5 T h e pathogenesis of the other G I s y m p t o m s of pain a n d discomfort is less clear. O c c a s i o n a l cases of m e c h a n i c a l obstruction of the c o m m o n bile d u c t or pylorus by ectopic p a n c r e a s have been reported, 2'69 but most cases show no obvious m e c h a n i s m of disease production. O n e t h e o r y suggested t h a t ectopic p a n c r e a s without connection to the bowel l u m e n is somehow s t i m u l a t e d to secrete and so p r o d u c e inflammation, spasm and h y p e r m o t i l i t y . I~ T h e present case revealed a c u t e p a n c r e a t i c i n f l a m m a t i o n . This is very unusual in p a n c r e a t i c heterotopia. Q u i z i l b a s h ~ described a case of a c u t e p a n c r e a t i tis within the g a l l b l a d d e r wall. T h e r e have been
two cases of m u m p s occurring in both ectopic a n d isotopic pancreas, as well as one case r e p o r t of a c u t e l y inflamed ectopic p a n c r e a s l e a d i n g to c o m m o n bile d u c t obstruction. 2 T h e r e have been a n o t h e r t h r e e cases w h e r e the i n f l a m m a tion of ectopic p a n c r e a s was felt to be d u e to p r o x i m i t y to a n o t h e r ongoing i n f l a m m a t o r y process.12 T h e cause for a c u t e i n f l a m m a t i o n in this p a t i e n t is obscure. T h e r e is no f a m i l y history of h y p e r l i p i d e m i a or p a n c r e a t i t i s , no c l i n i c a l evidence of m u m p s or other infectious processes. T h e p a t i e n t gave no history of recent t r a u m a , or steroid or alcohol ingestion. Histologic e x a m i n a t i o n revealed no evidence of d u c t obstruction, a l t h o u g h a recent a c u t e obstruction could not be ruled out.
REFERENCES
1. Dolan RF: The fate of heterotopic pancreatic tissue. Arch Surg 109:767, 1974 2. Barbosa J: Pancreatic heterotopia: Review of the literature and report of 41 authenticated surgical cases of which 25 were clinically significant. Surg Gynecol Obstet 87:527, 1946 3. Mason T: Ectopic pancreas in fallopian tube. Obstet Gynecol 48:705, 1976 4. Goldfarb WB: Carcinoma in heterotopic gastric pancreas. Ann Surg 37:77, 1971 6. Lucaya J: Ectopic pancreas in the stomach. J Pediatr Surg 11:101, 1976 7. Dietz MW: Pancreatic heterotopia. Miss Med 65:665, 1968
8. Pearson Seibert C: Aberrant pancreas. Arch Surg 63:168, 1951 9. Weber CM, Zito PF, Becket SM: Heterotopic pancreas and common bile duct obstruction and review of the literature. Am J Gastroenterol 49:153, 1968 10. Feldman W: Aberrant pancreas: A cause of duodenal syndrome. JAMA 168:893, 1952 11. Quizilbash A: Acute pancreatitis occurring in heterotopic pancreatic tissue within the gallbladder. Canad J Surg 19:413, 1976 12. Longmeyer W, in Ackerman (ed): Surgical pathology. St. Louis, Mosby, 1974 pp 566
C T O P I C pancreas is defined as pancreatic tissue that lacks anatomic and vascular continuity with the main body of the pancreas. This is no longer an unusual condition. There have been several reports in the literature of ectopia at various locations, ~ 3 however, cases of acute pancreatitis at an ectopic site are rare and often anecdotal in the English literature. 2~~,~2
E
CASE REPORT U. C. is a 12-yr-old who presented with a 24-hr history of periumbilica[ abdominal pain, nausea and vomiting. On admission he had a temperature of 100~ Physical examination revealed a rigid abdomen with absent bowel sounds. No mass was palpable. Rectal examination was negative. Laboratory results showed that the WBC was 14,000. The preoperative diagnosis was probable perforated appendicitis although the history was somewhat atypical. On exploration purulent looking material was found in the abdomen. The appendix was normal. A purulent node with fibrinous exudate was found at the base of the midjejunal mesentary and a diagnosis of acute suppurative adenitis was made at this time. This node was excised along with a smaller firm node in the same area of the mesentary. The main pancreas was not explored. The patient's postoperative course was uncomplicated and he was discharged on the fifth postoperative day. Amylase was done on postoperative day 2 and was within normal limits at 138 U. Cultures from the peritoneal cavity at operation were negative. Microscopic examination revealed that the larger of the two nodes was acutely inflamed ectopic pancreas. Later, questioning of the patient revealed no history of mumps, alcohol consumption, family history of hyperlipidemia or pancreatitis.
PATHOLOGY The appendix was histologically normal. The smaller mesenteric nodule was a lymph node with reactive sinusoidal
From the Department of Pediatric Surgery, Boston City Hospital, 30 Warren St., Brighton, Mass. 02135 Address reprint requests to James A. Borger, M.D., 818 Harrison Avenue, Boston, Mass. 02118. 9 1982 by Grune & Stratton, Inc. 0022-3468/82/170 1-0024501.00/0 86
hyperplasia. The larger mesenteric nodule measuring 1.5 • 1 • 0.7 cm consisted on ectopic pancreatic tissue with islets of Langerhans, abundant acini and well-formed ducts. A well developed acute pancreatitis involved about one-third of the tissue with necrosis of parenchyma, in some lobules involving only the central area and in others extending to the perilobular connective tissue. An abundant exudate of polymorphonuclear cells surrounded the necrosis. There was a mild amount of red blood cell extravasation and several foci of fat necrosis. A large vessel showed recent thrombus formation, probably secondary to the surrounding inflammation.
DISCUSSION Ectopic
pancreas
o c c u r s in 1 % - 2 %
s i e s a n d in 1 : 5 0 0 l a p a r o t o m i e s
of autop-
with a male ratio
of 3:1. According to Dolan 3 who reviewed 212 cases at the Mayo Clinic 45% of the cases occur in the stomach and duodenum, 35% in the jejunum, 15% in the Meckel's diverticulum and 6% in the ileum. Ectopia has also been reported in the biliary tract, umbilicus, appendix, liver, omenturn, mesentary and fallopian t u b e s Y Dolan critically examined the association of ectopic pancreas with symptoms. He found that 66% of the cases were incidental findings at laparotomy. In 17% of the cases, ectopic pancreas was the only abnormality to account for the patient's symptoms and was, therefore, surgically excised. Only 35% of this group showed postoperative improvement. The remainder of the group either showed no improvement, or had symptoms that were felt in retrospect to be functional. In the remaining 17% of the cases, the ectopic pancreas was diagnosed by x-rays but not surgically removed. Follow-up of this group showed that the disease was not attributed to ectopic pancreas. This makes it apparent that most cases of ectopic pancreas are indeed asymptomatic. Almost all GI symptoms have been attributed to ectopic pancreas. The most common symptoms fall into the following groups: (1) upper G I bleeding; (2) abdominal pain, discomfort and bloating. There are two reported cases of cancer occurring in an ectopic site. 4 The mechanism by which symptoms are produced is not always clear. When the presenting symptom is an upper GI hemorrhage, one may postulate that the alkaline Journal of Pediatric Surgery, Vol. 17, No. 1 (February), 1982
ECTOPIC PANCREAS
87
e n z y m e s p r o d u c e d by the ectopic p a n c r e a s stimulate gastric secretion and increase a c i d i t y a n d lead to ulcer f o r m a t i o n and bleeding. 5 T h e pathogenesis of the other G I s y m p t o m s of pain a n d discomfort is less clear. O c c a s i o n a l cases of m e c h a n i c a l obstruction of the c o m m o n bile d u c t or pylorus by ectopic p a n c r e a s have been reported, 2'69 but most cases show no obvious m e c h a n i s m of disease production. O n e t h e o r y suggested t h a t ectopic p a n c r e a s without connection to the bowel l u m e n is somehow s t i m u l a t e d to secrete and so p r o d u c e inflammation, spasm and h y p e r m o t i l i t y . I~ T h e present case revealed a c u t e p a n c r e a t i c i n f l a m m a t i o n . This is very unusual in p a n c r e a t i c heterotopia. Q u i z i l b a s h ~ described a case of a c u t e p a n c r e a t i tis within the g a l l b l a d d e r wall. T h e r e have been
two cases of m u m p s occurring in both ectopic a n d isotopic pancreas, as well as one case r e p o r t of a c u t e l y inflamed ectopic p a n c r e a s l e a d i n g to c o m m o n bile d u c t obstruction. 2 T h e r e have been a n o t h e r t h r e e cases w h e r e the i n f l a m m a tion of ectopic p a n c r e a s was felt to be d u e to p r o x i m i t y to a n o t h e r ongoing i n f l a m m a t o r y process.12 T h e cause for a c u t e i n f l a m m a t i o n in this p a t i e n t is obscure. T h e r e is no f a m i l y history of h y p e r l i p i d e m i a or p a n c r e a t i t i s , no c l i n i c a l evidence of m u m p s or other infectious processes. T h e p a t i e n t gave no history of recent t r a u m a , or steroid or alcohol ingestion. Histologic e x a m i n a t i o n revealed no evidence of d u c t obstruction, a l t h o u g h a recent a c u t e obstruction could not be ruled out.
REFERENCES
1. Dolan RF: The fate of heterotopic pancreatic tissue. Arch Surg 109:767, 1974 2. Barbosa J: Pancreatic heterotopia: Review of the literature and report of 41 authenticated surgical cases of which 25 were clinically significant. Surg Gynecol Obstet 87:527, 1946 3. Mason T: Ectopic pancreas in fallopian tube. Obstet Gynecol 48:705, 1976 4. Goldfarb WB: Carcinoma in heterotopic gastric pancreas. Ann Surg 37:77, 1971 6. Lucaya J: Ectopic pancreas in the stomach. J Pediatr Surg 11:101, 1976 7. Dietz MW: Pancreatic heterotopia. Miss Med 65:665, 1968
8. Pearson Seibert C: Aberrant pancreas. Arch Surg 63:168, 1951 9. Weber CM, Zito PF, Becket SM: Heterotopic pancreas and common bile duct obstruction and review of the literature. Am J Gastroenterol 49:153, 1968 10. Feldman W: Aberrant pancreas: A cause of duodenal syndrome. JAMA 168:893, 1952 11. Quizilbash A: Acute pancreatitis occurring in heterotopic pancreatic tissue within the gallbladder. Canad J Surg 19:413, 1976 12. Longmeyer W, in Ackerman (ed): Surgical pathology. St. Louis, Mosby, 1974 pp 566