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Journal of Indian Association of Pediatric Surgeons
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Year : 2012  |  Volume : 17  |  Issue : 1  |  Page : 40-42

Heterotopic pancreas causing duodenal obstruction in a patient previously treated for choledochal cyst

Department of Paediatric Surgery, Maulana Azad Medical College and Associated Lok Nayak Hospital, New Delhi, India

Date of Web Publication22-Dec-2011

Correspondence Address:
Vidyanand P Deshpande
Department of Paediatric Surgery, Maulana Azad Medical College and Associated Lok Nayak Hospital, New Delhi - 110002
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-9261.91088

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A 9-year-old boy presented with duodenal pancreatic rest causing obstruction and required surgical intervention. He had been treated at the age of 4 months for a choledochal cyst. Both choledochal cyst and heterotopic pancreas are entities that are commonly encountered in children, but the incidental presence of both the entities in the same child, albeit presenting metachronously, is extremely rare.

Keywords: Duodenal obstruction, gastric outlet obstruction, heterotopic pancreas

How to cite this article:
Deshpande VP, Raghunath B V, Sarin YK, Sinha S. Heterotopic pancreas causing duodenal obstruction in a patient previously treated for choledochal cyst. J Indian Assoc Pediatr Surg 2012;17:40-2

How to cite this URL:
Deshpande VP, Raghunath B V, Sarin YK, Sinha S. Heterotopic pancreas causing duodenal obstruction in a patient previously treated for choledochal cyst. J Indian Assoc Pediatr Surg [serial online] 2012 [cited 2023 Jun 8];17:40-2. Available from: https://www.jiaps.com/text.asp?2012/17/1/40/91088

   Introduction Top

The presence of pancreatic tissue outside its normal localization and without anatomic and vascular continuity with the normal pancreas is termed heterotopic pancreas (HP); pancreatic rests, ectopic, aberrant, or accessory pancreas are the other terms to describe the entity. HP may occur throughout the gastrointestinal tract, but has a propensity to affect the stomach, duodenum, jejunum, Meckel's diverticulum, and ileum. Less frequently, it is observed in the liver, gallbladder, bile duct system, and papilla of Vater. [1] HP is known to be associated with various associated anomalies like malrotation, small intestinal atresias, biliary atresias, congenital diaphragmatic hernias, Meckel's diverticuli, annular pancreas and esophageal atresias. [2],[3] There have been isolated case reports of the presence of HP within the choledochal cyst (CC). [4,5] Association of jejunal HP with CC has also been reported once. [6]

   Case Report Top

A 9-year-old boy presented with features of gastric outlet obstruction for 1 month. He was operated for CC type 1 at the age of 4 months at the same center; complete excision of cyst with Roux-en-Y hepaticojejunostomy was done. Magnetic resonance cholangio-pancreaticography (MRCP) done prior to first surgery had shown pancreaticobiliary malunion (PBMU), CC and dilated pancreatic duct, suggestive of chronic pancreatitis [Figure 1]. No annular pancreas or obvious pancreatic rests were noted at the MRCP or subsequent surgery. Abdominal examination revealed fullness in epigastric region; reverse gastric peristalsis was noted. Upper gastrointestinal (GI) contrast study revealed partial obstruction of the first part of duodenum [Figure 2]. Upper GI endoscopy revealed the lumen of first part of the duodenum completely obliterated by a large polypoidal mass; the endoscope could not be negotiated beyond the first part of duodenum.
Figure 1: Magnetic resonance cholangio-pancreaticography showing pancreaticobiliary malunion, type I CC and dilated pancreatic duct

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Figure 2: Upper gastrointestinal contrast study showing near-total obstruction of first part of duodenum

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On exploration, the first part of duodenum was found to be severely inflamed and thickened with near complete obliteration of its lumen. The Roux-en-Y loop of the previous hepaticojejunostomy was 5 cm away from the duodenal lesion. Excision of 2.5 cm of distal stomach, pylorus and first part of duodenum with gastroduodenostomy akin to Billroth I procedure was done. Histopathologic examination of the entire excised specimen revealed transmural acute on chronic inflammation and diffuse fibrosis. There were submucosal heterotopic pancreatic rests in the excised duodenum, though there were no abnormal cells in the excised pylorus and the stomach [Figure 3]. The postoperative period was uneventful. The child is doing well at 10 months follow-up.
Figure 3: Heterotopic pancreatic rests in the submucosa of the duodenal partition (H and E, × 40)

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   Discussion Top

The etiology of HP is unknown. [7] The proposed theory is that during rotation of foregut in a fetus and fusion of dorsal and ventral parts of pancreas, small islands of pancreatic rests are carried away and continue to develop at this aberrant location. [8] HP is seen in 1-2% of patients as autopsy findings and 1 in 500 of upper abdominal surgeries. [9] Macroscopically, it may appear as 1-4 cm yellow nodular structures. Our case was considered to be a type 2 ectopic pancreas, based on Heinrich classification. [10] Majority of the patients with HP are asymptomatic. However, they may present with obstruction, lump, GI bleeding, etc., depending on the size and location of HP.

Correct preoperative diagnosis is usually not possible and the eventual diagnosis is made on surgical exploration and pathological examination. Clinically significant lesions are greater than 1.5 cm in maximum diameter and are adjacent to or directly involve the mucosa. [11] Ulceration may give a crater-like or volcanic appearance on endoscopy. [7] Pancreatitis, malignant transformation and pseudocyst are amongst the less common but documented complications. [12] Upper GI obstruction as seen in our patient is common with HP. But most of the cases of HP causing gastric outlet syndrome have been described in adults. Such an occurrence has been occasionally reported in children and rarely reported in infants and neonates too. [13],[14]

Radiological investigations like computed tomogram (CT) scans, endoscopic ultrasonograms and nuclear scan studies assist in preoperative diagnosis, but with difficulty. [15] Upper GI endoscopy, though helpful in certain patients presenting with submucosal nodules, was not helpful in the preoperative diagnosis in our case. Hence, the diagnosis is prominently intraoperative or based on histology. [15] Therefore, surgery is frequently needed to make a definitive diagnosis and plan further management and also because differential diagnosis includes leiomyomas, lymphomas, carcinoid tumors, gastrointestinal stromal tumors (GIST) and other malignancies. On review of available literature on HP, we found that all the pathologies known to affect the pancreas can also be seen in HP. Pre-operative diagnosis of the exact cause can also be extremely difficult. Hence, we feel that whenever HP is encountered incidentally or because of complication, it warrants excision. Keeping this in mind, we performed the excision of the lesion rather than a simple bypass such as posterior gastrojejunostomy.

   References Top

1.Heer C, Pförtner M, Hamberger U, Raute-Kreinsen U, Hanraths M, Bartsch DK. Heterotopic pancreatic tissue in the bifurcation of the bile duct. Rare diagnosis mimicking a Klatskintumour. Chirurg 2010;81:151-4.  Back to cited text no. 1
2.Ogata H, Oshio T, Ishibashi H, Takano S, Yagi M. Heterotopic pancreas in children: Review of the literature and report of 12 cases. Pediatr Surg Int 2008;24:271-5.  Back to cited text no. 2
3.Moreau B, Lévesque D, Faure C. Association of gastric heterotopic pancreas and esophageal atresia in children. J Pediatr Gastroenterol Nutr 2010;50:394-6.  Back to cited text no. 3
4.Suzuki K, Uchida T, Nakayama H, Ugajin W, Inaniwa Y, Sugitani M, et al. Heterotopic pancreatic tissue associated with intra-and extrahepaticcholedochal cysts. Pathol Int 1999;49:759-62.  Back to cited text no. 4
5.Kattepura S, Nanjegowda NB, Babu MK, Das K. Macroscopic pancreatic heterotopia on a congenital biliary dilatation. Pediatr Surg Int 2010;26:847-9.  Back to cited text no. 5
6.Prasad TR, Gupta D, Bhatnagar V. Ectopic pancreas associated with a choledochal cyst and extrahepatic biliary atresia. Pediatr Surg Int 2001;17:552-4.  Back to cited text no. 6
7.Shi HQ, Zhang QY, Teng HL, Chen JC. Heterotopic pancreas: Report of 7 patients. Hepatobiliary Pancreat Dis Int 2002;1:299-301.   Back to cited text no. 7
8.Yuan Z, Chen J, Zheng Q, Huang XY, Yang Z, Tang J. Heterotopic pancreas in the gastrointestinal tract. World J Gastroenterol 2009;15:3701-3.  Back to cited text no. 8
9.Gokhale UA, Nanda A, Pillai R, Al-Layla D. Heterotopic pancreas in the stomach: A case report and a brief review of the Literature. J Pediatr 2010;11:255-7.  Back to cited text no. 9
10.Von Heinrich H. EinBeitragzurHistologie des Sogen: Akzessorischen Pankreas. Virchows Arch A Pathol Anat Histopathol 1909;198:392-401.  Back to cited text no. 10
11.Armstrong CP, King PM, Dixon JM, Macleod IB. The clinical significance of heterotopic pancreas in the gastrointestinal tract. Br J Surg 1981;68:384-7.  Back to cited text no. 11
12.Sadeghi NR, Godambe A, Shienbaum AJ, Alloy A. Premalignant gastric heterotopic pancreas. Gastroenterol Hepatol (N Y) 2008;4:218-21.  Back to cited text no. 12
13.Sharma SB, Gupta V. Ectopic pancreas as a cause of gastric outlet obstruction in an infant. Indian J Gastroenterol 2004;23:219.  Back to cited text no. 13
14.Ozcan C, Celik A, Güçlü C, Balik E. A rare cause of gastric outlet obstruction in the newborn: Pyloric ectopic pancreas. J Pediatr Surg 2002;37:119-20.  Back to cited text no. 14
15.Mönig SP, Selzner M, Raab M, Eidt S. Heterotopic pancreas: A difficult diagnosis. Dig Dis Sci 1996;41:1238-40.  Back to cited text no. 15


  [Figure 1], [Figure 2], [Figure 3]

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