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A rare cause of gastric outlet obstruction in the newborn: Pyloric ectopic pancreas
A Rare Cause of Gastric Outlet Obstruction in the Newborn: Pyloric Ectopic Pancreas ¨ zcan, Ahmet C¸elik, Cahit Gu¨c¸lu¨, and Erol Balık By Cos¸kun O I˙zmir, Turkey
This report describes a case of symptomatic pyloric ectopic pancreas simulating infantile hypertrophic pyloric stenosis (IHPS) in an 1-month-old boy. There are few cases reported in the English-language literature with the same clinical presentation during the neonatal period. Rarely is the entity symptomatic, and it should be kept in mind in differential diagnosis of nonbilious vomiting, especially in neonates. The
ectopic pancreas should be removed because of the late complications when found incidentally. J Pediatr Surg 37:119-120. Copyright © 2002 by W.B. Saunders Company.
INDEX WORDS: Ectopic pancreas, pyloric obstruction.
E
CTOPIC PANCREAS is defined as pancreatic tissue that lacks anatomic and vascular continuity with the main body of the pancreas.1-3 This is not an unusual condition in adults and occurs in 1% to 2% of autopsies.4,5 Ectopic pancreatic tissue may be found anywhere along the alimentary tract but is most common in the duodenum and in the stomach. It also may be located in the esophagus, jejunum, ileum, spleen, gallbladder, liver, mesentery, and even in the lungs, umbilicus, or fallopian tubes.1,3,6-10 However, ectopic pancreatic tissue localized in the prepyloric area rarely presents with symptoms. It usually is a silent gastrointestinal anomaly and may become evident clinically when complicated by a pathologic process such as bleeding, obstruction, or malignant transformation in advanced ages.2,5 CASE REPORT A 1-month-old boy was admitted with a 3-week history of intermittent vomiting free of bile and failure to gain weight. Physical examination and laboratory studies showed no abnormality except mild hyperbilurubinemia. With the clinical suspicion of infantile hypertrophic pyloric stenosis (IHPS), abdominal ultrasonography and upper gastrointestinal series were performed. Abdominal ultrasonography was interpreted as normal, whereas radiography results showed findings suggestive of pyloric stenosis (Fig 1). The initial diagnosis of IHPS was made, and a pyloromyotomy was planned. On exploration, IHPS was not present, and a 4- to 5-mm pyloric mass was found, and subserosal excision of the mass was performed. Histopathologic examination of the surgical material showed aberrant submucosal exocrine pancreatic tissue that had acini and ducts with no islets cells. The postoperative course of the patient was uncomplicated, and he was discharged on the postoperative seventh day. The patient is still under clinical follow-up and symptom free at second year after the operation.
DISCUSSION
Presence of ectopic pancreatic tissue rarely causes clinical symptomatology.3,5 Nevertheless, this anomaly is diagnosed mostly with the development of complicaJournal of Pediatric Surgery, Vol 37, No 1 (January), 2002: pp 119-120
Fig 1.
Upper gastrointestinal series shows pyloric stenosis.
tions such as hemorrhage, obstruction, and malignant transformation.2,5 However, these complications rarely are encountered, even in adults. In children, there has been no reported case of cancer and very few cases of gastrointestinal hemorrhage caused by ectopic pancreatic From Ege University Faculty of Medicine, Department of Pediatric Surgery, I˙zmir, Turkey. ¨ zcan, MD, Assistant Professor Address reprint requests to Cos¸kun O of Pediatric Surgery, Ege University, Faculty of Medicine, Department of Pediatric Surgery, 35100 Bornova-I˙zmir, Turkey. Copyright © 2002 by W.B. Saunders Company 0022-3468/02/3701-0026$35.00/0 doi:10.1053/jpsu.2002.29443 119
¨ ZCAN ET AL O
120
tissue in the gastric wall. However, gastric outlet obstruction caused by ectopic pancreas located in the stomach may be more frequently encountered in children, but most of them become clinically evident beyond the first year of life.13 We could find only 6 cases in the Englishlanguage literature reporting symptoms of pyloric obstruction in the neonatal period with the current case presumed to be the seventh one.11-16 The mechanism of gastric outlet obstruction can be understood easily in cases of ectopic pancreas in the prepyloric area associated with duplications of upper gastrointestinal tract or prolapse of a large submucosal polypoid lesion through the pylorus. However, the mech-
anism by which the obstruction occurs in the absence of the above-mentioned situations remains unclear. Previously, Krieg et al5 reported that the development of inflammation may be one of the responsible mechanisms leading to obstruction. Ectopic pancreas localized in the prepyloric area is a rare condition in children and occasionally produces symptomatology. However, it may be complicated by bleeding, obstruction, and malignant transformation in the long-term follow-up. Therefore, surgical removal of ectopic pancreas should be considered whenever it is found incidentally at laporotomy to prevent the potential complications seen in adults.
REFERENCES 1. Fam S, O’Briain DS, Borger JA: Ectopic pancreas with acute inflammation. J Pediatr Surg 17:86-87, 1982 2. Armstrong CP, King PM, Dixon JM, et al: The clinical significance of heterotopic pancreas in the gastrointestinal tract. Br J Surg 68:384-387, 1981 3. Margulis AR, Burhenne HJ: Alimentary Tract Roentgenology. St. Louis, MO, Mosby, 1973 4. Pearson Seibert C: Aberrant pancreas. Arch Surg 63:168-184, 1951 5. Krieg EG: Heterotopic pancreatic tissue producing pyloric obstruction: A review and case report. 113:364-370, 1941 6. Lucaya J, Boix Ochoa J: Ectopic pancreas in the stomach. J Pediatr Surg 11:101-102, 1976 7. Caberwal D, Kogan SJ, Levitt SB: Ectopic pancreas presenting as an umbilical mass. J Pediatr Surg 12:593-595, 1977 8. Mason T: Ectopic pancreas in fallopian tube. Obstet Gynecol 48:70S-75S, 1976 9. Tanaka K, Tsunoda T, Eto T, et al: Diagnosis and management of heterotopic pancreas. Int Surg 78:32-35, 1993
¨ zok G, et al: Intussusception in an 10. Erdener A, Avanog˘lu A, O infant caused by aberrant pancreas. JPMA J Pak Med Assoc 43:22-23, 1993 11. Matsumoto Y, Kwai Y, Kimura K: Aberrant pancreas causing pyloric obstruction. Surgery 76:827-829, 1974 12. Kernohan RJ, Marison JE: Symptomatic pancreatic heterotopia of pylorus associated with bilateral renal necrosis in an infant. Arch Dis Child 31:276, 1956 13. Hayes-Jordan A, Idowu O, Cohen R: Ectopic pancreas as the cause of gastric outlet obstruction in a newborn. Pediatr Radiol 28:868870, 1998 14. Dolan RV, ReMine WH, Dockerty MB: The fate of heterotopic pancreatic tissue: A study of 212 cases. Arch Surg 109:762-765, 1974 15. Visentin M, Vaysse Ph, Guitard J, et al: Pyloric obstruction with complicated pancreatic heterotopia. Eur J Surg 1:247-248, 1991 16. Gonzalez OR, Hardin WD, Isaacs H, et al: Duplication of the hepatopancreatic bud presenting as pyloric stenosis. J Pediatr Surg 23:1053-1054, 1988
This report describes a case of symptomatic pyloric ectopic pancreas simulating infantile hypertrophic pyloric stenosis (IHPS) in an 1-month-old boy. There are few cases reported in the English-language literature with the same clinical presentation during the neonatal period. Rarely is the entity symptomatic, and it should be kept in mind in differential diagnosis of nonbilious vomiting, especially in neonates. The
ectopic pancreas should be removed because of the late complications when found incidentally. J Pediatr Surg 37:119-120. Copyright © 2002 by W.B. Saunders Company.
INDEX WORDS: Ectopic pancreas, pyloric obstruction.
E
CTOPIC PANCREAS is defined as pancreatic tissue that lacks anatomic and vascular continuity with the main body of the pancreas.1-3 This is not an unusual condition in adults and occurs in 1% to 2% of autopsies.4,5 Ectopic pancreatic tissue may be found anywhere along the alimentary tract but is most common in the duodenum and in the stomach. It also may be located in the esophagus, jejunum, ileum, spleen, gallbladder, liver, mesentery, and even in the lungs, umbilicus, or fallopian tubes.1,3,6-10 However, ectopic pancreatic tissue localized in the prepyloric area rarely presents with symptoms. It usually is a silent gastrointestinal anomaly and may become evident clinically when complicated by a pathologic process such as bleeding, obstruction, or malignant transformation in advanced ages.2,5 CASE REPORT A 1-month-old boy was admitted with a 3-week history of intermittent vomiting free of bile and failure to gain weight. Physical examination and laboratory studies showed no abnormality except mild hyperbilurubinemia. With the clinical suspicion of infantile hypertrophic pyloric stenosis (IHPS), abdominal ultrasonography and upper gastrointestinal series were performed. Abdominal ultrasonography was interpreted as normal, whereas radiography results showed findings suggestive of pyloric stenosis (Fig 1). The initial diagnosis of IHPS was made, and a pyloromyotomy was planned. On exploration, IHPS was not present, and a 4- to 5-mm pyloric mass was found, and subserosal excision of the mass was performed. Histopathologic examination of the surgical material showed aberrant submucosal exocrine pancreatic tissue that had acini and ducts with no islets cells. The postoperative course of the patient was uncomplicated, and he was discharged on the postoperative seventh day. The patient is still under clinical follow-up and symptom free at second year after the operation.
DISCUSSION
Presence of ectopic pancreatic tissue rarely causes clinical symptomatology.3,5 Nevertheless, this anomaly is diagnosed mostly with the development of complicaJournal of Pediatric Surgery, Vol 37, No 1 (January), 2002: pp 119-120
Fig 1.
Upper gastrointestinal series shows pyloric stenosis.
tions such as hemorrhage, obstruction, and malignant transformation.2,5 However, these complications rarely are encountered, even in adults. In children, there has been no reported case of cancer and very few cases of gastrointestinal hemorrhage caused by ectopic pancreatic From Ege University Faculty of Medicine, Department of Pediatric Surgery, I˙zmir, Turkey. ¨ zcan, MD, Assistant Professor Address reprint requests to Cos¸kun O of Pediatric Surgery, Ege University, Faculty of Medicine, Department of Pediatric Surgery, 35100 Bornova-I˙zmir, Turkey. Copyright © 2002 by W.B. Saunders Company 0022-3468/02/3701-0026$35.00/0 doi:10.1053/jpsu.2002.29443 119
¨ ZCAN ET AL O
120
tissue in the gastric wall. However, gastric outlet obstruction caused by ectopic pancreas located in the stomach may be more frequently encountered in children, but most of them become clinically evident beyond the first year of life.13 We could find only 6 cases in the Englishlanguage literature reporting symptoms of pyloric obstruction in the neonatal period with the current case presumed to be the seventh one.11-16 The mechanism of gastric outlet obstruction can be understood easily in cases of ectopic pancreas in the prepyloric area associated with duplications of upper gastrointestinal tract or prolapse of a large submucosal polypoid lesion through the pylorus. However, the mech-
anism by which the obstruction occurs in the absence of the above-mentioned situations remains unclear. Previously, Krieg et al5 reported that the development of inflammation may be one of the responsible mechanisms leading to obstruction. Ectopic pancreas localized in the prepyloric area is a rare condition in children and occasionally produces symptomatology. However, it may be complicated by bleeding, obstruction, and malignant transformation in the long-term follow-up. Therefore, surgical removal of ectopic pancreas should be considered whenever it is found incidentally at laporotomy to prevent the potential complications seen in adults.
REFERENCES 1. Fam S, O’Briain DS, Borger JA: Ectopic pancreas with acute inflammation. J Pediatr Surg 17:86-87, 1982 2. Armstrong CP, King PM, Dixon JM, et al: The clinical significance of heterotopic pancreas in the gastrointestinal tract. Br J Surg 68:384-387, 1981 3. Margulis AR, Burhenne HJ: Alimentary Tract Roentgenology. St. Louis, MO, Mosby, 1973 4. Pearson Seibert C: Aberrant pancreas. Arch Surg 63:168-184, 1951 5. Krieg EG: Heterotopic pancreatic tissue producing pyloric obstruction: A review and case report. 113:364-370, 1941 6. Lucaya J, Boix Ochoa J: Ectopic pancreas in the stomach. J Pediatr Surg 11:101-102, 1976 7. Caberwal D, Kogan SJ, Levitt SB: Ectopic pancreas presenting as an umbilical mass. J Pediatr Surg 12:593-595, 1977 8. Mason T: Ectopic pancreas in fallopian tube. Obstet Gynecol 48:70S-75S, 1976 9. Tanaka K, Tsunoda T, Eto T, et al: Diagnosis and management of heterotopic pancreas. Int Surg 78:32-35, 1993
¨ zok G, et al: Intussusception in an 10. Erdener A, Avanog˘lu A, O infant caused by aberrant pancreas. JPMA J Pak Med Assoc 43:22-23, 1993 11. Matsumoto Y, Kwai Y, Kimura K: Aberrant pancreas causing pyloric obstruction. Surgery 76:827-829, 1974 12. Kernohan RJ, Marison JE: Symptomatic pancreatic heterotopia of pylorus associated with bilateral renal necrosis in an infant. Arch Dis Child 31:276, 1956 13. Hayes-Jordan A, Idowu O, Cohen R: Ectopic pancreas as the cause of gastric outlet obstruction in a newborn. Pediatr Radiol 28:868870, 1998 14. Dolan RV, ReMine WH, Dockerty MB: The fate of heterotopic pancreatic tissue: A study of 212 cases. Arch Surg 109:762-765, 1974 15. Visentin M, Vaysse Ph, Guitard J, et al: Pyloric obstruction with complicated pancreatic heterotopia. Eur J Surg 1:247-248, 1991 16. Gonzalez OR, Hardin WD, Isaacs H, et al: Duplication of the hepatopancreatic bud presenting as pyloric stenosis. J Pediatr Surg 23:1053-1054, 1988