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Intrapancreatic gastric duplication cyst presenting as lower gastrointestinal bleeding
CASE REPORTS
Intrapancreatic Gastric Duplication Cyst Presenting as Lower Gastrointestinal Bleeding By K.L.N. Rao, Indira Sunil, Ashwin Pimpalwar, Kim Vaiphei, and Sujit Chowdhary Chandigarh, India
This report describes a rare foregut malformation of a noncontiguous gastric duplication cyst embedded within the body and tail of the pancreas. An 8-month-old boy presented with massive malena of 3 days’ duration. The laparotomy showed an intrapancreatic cyst with bleeding and ulcer perforation into a loop of adherent jejunum. Histologically, the cyst contained gastric mucosa. This is believed to be the first
reported instance of an intrapancreatic gastric duplication causing massive lower gastrointestinal bleeding in an infant. J Pediatr Surg 38:243-244. Copyright 2003, Elsevier Science (USA). All rights reserved.
G
placed appropriately in the wall within the pancreatic parenchyma (Fig 2). The jejunum showed ulceration and inflammation of the mucosa and reactive fibroblastic proliferation. The pancreas essentially was normal except in the vicinity of the cystic mass where fibroblastic proliferation was seen. The overall morphology was consistent with an intrapancreatic gastric duplication cyst. The child had an uneventful postoperative recovery. Initial hyperglycemic blood glucose levels returned to normal by the third postoperative day. The child was administered pneumococcal vaccine. At 4-year postoperative follow-up, the child was well, thriving, free of abdominal symptoms, and has normal milestones.
ASTRIC DUPLICATON CYSTS, noncontiguous with the stomach, are rare. They may present with bizarre but life-threatening complications. An intrapancreatic gastric duplication cyst eroding into the jejunum and presenting with massive lower gastrointestinal bleeding is reported herein. CASE REPORT A previously well and healthy 8-month-old boy was brought in with the history of refusal of feedings, irritability, and excessive crying of one week’s duration and bleeding per rectum for 3 days in the form of frank blood, clots, and hematochezia. At admission, the child was found to be grossly anemic and in hypovolemic shock. The abdominal and other physical examinations showed no abnormality, whereas the rectal examination showed hematochezia and blood clots. The hematologic tests found a hematocrit level of 35%, normal bleeding, and clotting parameters. After adequate resuscitation and blood transfusion, the child underwent investigation. An abdominal ultrasound scan was normal, and upper and lower gastrointestinal endoscopy showed no mucosal lesion. Results of a Meckel’s scan and a red blood cell pool scan were negative. After 96 hours of admission, resuscitation, and investigations, the child was taken up for laparotomy. A loop of jejunum 30 cm from the duodeno-jejunal flexure was found to be adherent to the pancreatic mass with an ulcer perforation at the site of contact. The encapsulated mass was found to be intrapancreatic, embedded in the body and tail of the pancreas, with the splenic vein inseparably adherent anterosuperiorly. Excision of the body and tail of the pancreas along with the embedded mass was performed in continuity with the spleen and the adherent ulcerated segment of jejunum (Fig 1). An end-to-end anastomosis of the jejunal ends restored the intestinal continuity. Examination of the specimen found a 3-cm segment of jejunum with an ulcer perforation at the site of adhesion to the cyst. The resected pancreas showed a 5-cm cystic cavity, the cut section of which showed velvety and congested mucosa thrown into papillary folds. The spleen itself was unremarkable. The microscopic examination of the cystic mass showed hyperplastic gastric mucosa thrown into villiform structures with an intact lining epithelium along with glands having mucous and parietal cells. The submucosa and the muscularis propria were Journal of Pediatric Surgery, Vol 38, No 2 (February), 2003: pp 243-244
INDEX WORDS: Gastric duplication cysts, pancreas, gastrointestinal bleeding.
DISCUSSION
Duplications of the intestinal tract can occur anywhere from the mouth to the anus. They can be either tubular or spherical, and the wall reproduces normal bowel structure. The duplication cysts either are entirely separate from the adjacent bowel or share a common wall.1 By far, the gastric duplications are the least common and are described as contiguous or noncontiguous with the stomach.2 Gastric duplications communicating with the pancreatic duct and as a cause of recurrent pancreatitis have been described in 8 cases.3 The most common symptoms described in these cases were recurrent abdominal pain either caused by recurrent pancreatitis or peptic ulceration.4 One of the 8 patients described presented with From the Departments of Pediatric Surgery and Pathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India. Address reprint requests to K.L.N. Rao, Professor & Head of the Department of Pediatric Surgery, Post Graduate Institute of Medical Education & Research, Chandigarh 160012, India. Copyright 2003, Elsevier Science (USA). All rights reserved. 0022-3468/03/3802-0021$35.00/0 doi:10.1053/jpsu.2003.50053 243
244
RAO ET AL
life-threatening upper gastrointestinal bleeding through the pancreatic duct originating in an ulcerated area of the pancreas at its junction with the duplication.4 The current patient could also possibly be considered a case of jejunal duplication; lined with gastric epithelium, and adherent to the pancreas. But the fact that the cyst is nearly encircled by the pancreatic tissue negates this concept. The duplication cyst must be excised because it is nonfunctional and often has been the cause of morbidity; even adenocarcinoma has been reported in foregut duplications.5 The pancreatic resections should be as lim-
Fig 2. Photomicrograph of the cyst within the pancreatic parenchyma to show the lining mucosa (gastric type), which were thrown into mucosal folds. The submucosa and muscularis propria, which formed the wall of the cyst, also could be seen (H&E original magnification ⴛ55).
Fig 1. Gross photograph of organ complex consisting of cut open pancreas and small bowel segment and a cystic lesion within the pancreatic parenchyma, which was in continuity with the bowel lumen and contained blood clot. The straight arrows point to the cyst, and the curved arrow indicates the point of erosion into the jejunum.
ited as possible to prevent the development of pancreatic insufficiency in the young patients. Duplication cysts in the pancreatic head have been treated with local resection avoiding more radical procedures.6 The current case appears to be the youngest child reported with an intrapancreatic gastric duplication cyst causing massive lower gastrointestinal bleeding resulting from a peptic ulceration of the adherent jejunum. All preoperative investigations were inconclusive, and the diagnosis was established at laparotomy and histopathologic examination of the resected specimen.
REFERENCES 1. Lawrence M, John AR, Richard AK, et al: Pancreatitis caused by a gastic duplication communicating with an aberrant pancreatic lobe. J Pediatr Surg 31:733-736, 1996 2. Johnstone DW, Forde KA, Markowitz D, et al: Gastric duplication cyst communicating with the pancreatic duct: A rare cause of recurrent abdominal pain. Surgery 109:97-100, 1991 3. Traverso LW, Damus PS, Longmire WP: Pancreatitis of unusual origin. Surg Gynecol Obstet 141:383-386, 1975
4. Longmire WP, Rose AS: Haemoductal pancreatitis. Surg Gynecol Obstet 136:246-250, 1973 5. Oslen JB, Lemmensen O, Anderson K: Adenocarcinoma arising in a foregut cyst of the mediastinum. Ann Thorac Surg 51:497-499, 1991 6. Siddiqui AM, Shamberger RC, Filler RM, et al: Enteric duplications of the pancreatic head: definitive management by local resection. J Pediatr Surg 33:1117-1120, 1998
Intrapancreatic Gastric Duplication Cyst Presenting as Lower Gastrointestinal Bleeding By K.L.N. Rao, Indira Sunil, Ashwin Pimpalwar, Kim Vaiphei, and Sujit Chowdhary Chandigarh, India
This report describes a rare foregut malformation of a noncontiguous gastric duplication cyst embedded within the body and tail of the pancreas. An 8-month-old boy presented with massive malena of 3 days’ duration. The laparotomy showed an intrapancreatic cyst with bleeding and ulcer perforation into a loop of adherent jejunum. Histologically, the cyst contained gastric mucosa. This is believed to be the first
reported instance of an intrapancreatic gastric duplication causing massive lower gastrointestinal bleeding in an infant. J Pediatr Surg 38:243-244. Copyright 2003, Elsevier Science (USA). All rights reserved.
G
placed appropriately in the wall within the pancreatic parenchyma (Fig 2). The jejunum showed ulceration and inflammation of the mucosa and reactive fibroblastic proliferation. The pancreas essentially was normal except in the vicinity of the cystic mass where fibroblastic proliferation was seen. The overall morphology was consistent with an intrapancreatic gastric duplication cyst. The child had an uneventful postoperative recovery. Initial hyperglycemic blood glucose levels returned to normal by the third postoperative day. The child was administered pneumococcal vaccine. At 4-year postoperative follow-up, the child was well, thriving, free of abdominal symptoms, and has normal milestones.
ASTRIC DUPLICATON CYSTS, noncontiguous with the stomach, are rare. They may present with bizarre but life-threatening complications. An intrapancreatic gastric duplication cyst eroding into the jejunum and presenting with massive lower gastrointestinal bleeding is reported herein. CASE REPORT A previously well and healthy 8-month-old boy was brought in with the history of refusal of feedings, irritability, and excessive crying of one week’s duration and bleeding per rectum for 3 days in the form of frank blood, clots, and hematochezia. At admission, the child was found to be grossly anemic and in hypovolemic shock. The abdominal and other physical examinations showed no abnormality, whereas the rectal examination showed hematochezia and blood clots. The hematologic tests found a hematocrit level of 35%, normal bleeding, and clotting parameters. After adequate resuscitation and blood transfusion, the child underwent investigation. An abdominal ultrasound scan was normal, and upper and lower gastrointestinal endoscopy showed no mucosal lesion. Results of a Meckel’s scan and a red blood cell pool scan were negative. After 96 hours of admission, resuscitation, and investigations, the child was taken up for laparotomy. A loop of jejunum 30 cm from the duodeno-jejunal flexure was found to be adherent to the pancreatic mass with an ulcer perforation at the site of contact. The encapsulated mass was found to be intrapancreatic, embedded in the body and tail of the pancreas, with the splenic vein inseparably adherent anterosuperiorly. Excision of the body and tail of the pancreas along with the embedded mass was performed in continuity with the spleen and the adherent ulcerated segment of jejunum (Fig 1). An end-to-end anastomosis of the jejunal ends restored the intestinal continuity. Examination of the specimen found a 3-cm segment of jejunum with an ulcer perforation at the site of adhesion to the cyst. The resected pancreas showed a 5-cm cystic cavity, the cut section of which showed velvety and congested mucosa thrown into papillary folds. The spleen itself was unremarkable. The microscopic examination of the cystic mass showed hyperplastic gastric mucosa thrown into villiform structures with an intact lining epithelium along with glands having mucous and parietal cells. The submucosa and the muscularis propria were Journal of Pediatric Surgery, Vol 38, No 2 (February), 2003: pp 243-244
INDEX WORDS: Gastric duplication cysts, pancreas, gastrointestinal bleeding.
DISCUSSION
Duplications of the intestinal tract can occur anywhere from the mouth to the anus. They can be either tubular or spherical, and the wall reproduces normal bowel structure. The duplication cysts either are entirely separate from the adjacent bowel or share a common wall.1 By far, the gastric duplications are the least common and are described as contiguous or noncontiguous with the stomach.2 Gastric duplications communicating with the pancreatic duct and as a cause of recurrent pancreatitis have been described in 8 cases.3 The most common symptoms described in these cases were recurrent abdominal pain either caused by recurrent pancreatitis or peptic ulceration.4 One of the 8 patients described presented with From the Departments of Pediatric Surgery and Pathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India. Address reprint requests to K.L.N. Rao, Professor & Head of the Department of Pediatric Surgery, Post Graduate Institute of Medical Education & Research, Chandigarh 160012, India. Copyright 2003, Elsevier Science (USA). All rights reserved. 0022-3468/03/3802-0021$35.00/0 doi:10.1053/jpsu.2003.50053 243
244
RAO ET AL
life-threatening upper gastrointestinal bleeding through the pancreatic duct originating in an ulcerated area of the pancreas at its junction with the duplication.4 The current patient could also possibly be considered a case of jejunal duplication; lined with gastric epithelium, and adherent to the pancreas. But the fact that the cyst is nearly encircled by the pancreatic tissue negates this concept. The duplication cyst must be excised because it is nonfunctional and often has been the cause of morbidity; even adenocarcinoma has been reported in foregut duplications.5 The pancreatic resections should be as lim-
Fig 2. Photomicrograph of the cyst within the pancreatic parenchyma to show the lining mucosa (gastric type), which were thrown into mucosal folds. The submucosa and muscularis propria, which formed the wall of the cyst, also could be seen (H&E original magnification ⴛ55).
Fig 1. Gross photograph of organ complex consisting of cut open pancreas and small bowel segment and a cystic lesion within the pancreatic parenchyma, which was in continuity with the bowel lumen and contained blood clot. The straight arrows point to the cyst, and the curved arrow indicates the point of erosion into the jejunum.
ited as possible to prevent the development of pancreatic insufficiency in the young patients. Duplication cysts in the pancreatic head have been treated with local resection avoiding more radical procedures.6 The current case appears to be the youngest child reported with an intrapancreatic gastric duplication cyst causing massive lower gastrointestinal bleeding resulting from a peptic ulceration of the adherent jejunum. All preoperative investigations were inconclusive, and the diagnosis was established at laparotomy and histopathologic examination of the resected specimen.
REFERENCES 1. Lawrence M, John AR, Richard AK, et al: Pancreatitis caused by a gastic duplication communicating with an aberrant pancreatic lobe. J Pediatr Surg 31:733-736, 1996 2. Johnstone DW, Forde KA, Markowitz D, et al: Gastric duplication cyst communicating with the pancreatic duct: A rare cause of recurrent abdominal pain. Surgery 109:97-100, 1991 3. Traverso LW, Damus PS, Longmire WP: Pancreatitis of unusual origin. Surg Gynecol Obstet 141:383-386, 1975
4. Longmire WP, Rose AS: Haemoductal pancreatitis. Surg Gynecol Obstet 136:246-250, 1973 5. Oslen JB, Lemmensen O, Anderson K: Adenocarcinoma arising in a foregut cyst of the mediastinum. Ann Thorac Surg 51:497-499, 1991 6. Siddiqui AM, Shamberger RC, Filler RM, et al: Enteric duplications of the pancreatic head: definitive management by local resection. J Pediatr Surg 33:1117-1120, 1998