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Gastroentérologie Clinique et Biologique
Volume 32, n° 6-7
pages 645-648 (juin-juillet 2008)
Doi : 10.1016/j.gcb.2008.02.032
Cystic dystrophy in gastric heterotopic pancreas complicated by intracystic hemorrhage and fistulisation in the stomach – a pediatric case
Dystrophie kystique sur pancréas aberrant gastrique compliquée par une hémorragie intra kystique et une fistulisation dans l’estomac – à propos d’un cas pédiatrique
 

C. Stefanescu a, M.-P. Vullierme b, A. Couvelard c, F. Bretagnol d, P. Amouyal e, F. Maire a, V. Rebours a, P. Hammel a, P. Ruszniewski a, P. Lévy a,
a Service de gastroentérologie-pancréatologie, pôle des maladies de l’appareil digestif, hôpital Beaujon, AP–HP, 92118 Clichy cedex, France 
b Service de radiologie, pôle biologie-imagerie-pharmacie-pathologie, hôpital Beaujon, AP–HP, Clichy, France 
c Service d’anatomopathologie, pôle biologie-imagerie-pharmacie-pathologie, hôpital Beaujon, AP–HP, Clichy, France 
d Service de chirurgie colorectale, pôle des maladies de l’appareil digestif, hôpital Beaujon, AP–HP, Clichy, France 
e Clinique de Gastro-Louvre, Paris, France 

Corresponding author.
Summary
Context

Cystic dystrophy of the digestive wall, a rare but well-known complication of heterotopic pancreas when it is located in the duodenum, has been mainly described in adult series. Cystic dystrophy of the heterotopic pancreas within the gastric wall has been reported in only six adult cases. To our knowledge, no pediatric case has been described.

Case report

We report a 15-year-old boy surgically treated for cystic dystrophy located in the antrum, complicated by an intracystic hemorrhage and fistulisation into the stomach.

Conclusion

The diagnosis of heterotopic pancreas must be considered in case of submucosal cystic-gastric lesions, even in pediatric cases. Although the surgical approach is not systematic, it is recommended when cystic dystrophy is symptomatic (e.g., occlusion or hemorrhage).

The full text of this article is available in PDF format.
Résumé
Contexte

La dystrophie kystique de la paroi digestive est une complication rare, mais bien connue lorsqu’elle est située au niveau du duodénum, habituellement décrite chez les adultes. La localisation dans la paroi gastrique a été décrite seulement dans six cas. Aucun cas pédiatrique n’a pas été publié.

Cas clinique

Patient de 15 ans, traité chirurgicalement pour une dystrophie kystique antrale compliquée par hémorragie et fistulisation dans l’estomac.

Conclusion

Le diagnostic de pancréas hétérotopique doit être considéré en cas de lésion sous-muqueuse gastrique, même chez les enfants. Le traitement chirurgical est recommandé si la dystrophie kystique est symptomatique (occlusion ou hémorragie).

The full text of this article is available in PDF format.

Abbreviations : HP, CDHP, MDCT


Introduction

A heterotopic pancreas (HP) is defined as an abnormally located pancreatic tissue without any anatomical or vascular connection with the pancreas itself. HP is mainly located in the stomach and is usually small and asymptomatic [1]. Cystic dystrophy of the heterotopic pancreas (CDHP) is defined by the presence of cyst(s) surrounded by inflammation and fibrosis in the digestive wall intermingled with pancreatic ducts and lobules [2]. Mainly located in the duodenal wall in adults, CDHP may cause symptoms, such as abdominal pain, upper gastrointestinal obstruction and, more rarely, upper gastrointestinal hemorrhage [1, 3, 4]. We present the case of a child with cystic dystrophy in a gastric HP, complicated by intracystic hemorrhage and fistulisation into the stomach.

Case report

A 15-year-old boy presented with epigastralgia and vomiting in July 2006. The symptoms persisted until November 2006 and the patient lost 5kg. The patientʼs general condition was altered and the body-mass index was 15kg/m2. Clinical examination was unremarkable. Laboratory tests showed functional acute renal failure, metabolic alkalosis and hypochloremia, while the other parameters were normal, including inflammatory markers. Serum pancreatic enzymes were not measured. An upper gastrointestinal endoscopy was performed and showed alimentary stasis in the stomach, secondary to stenosis of the antrum. Duodenal examination could not be performed; surface biopsies from the stenotic area revealed edematous changes in the chorion with antral intestinal metaplasia. Abdominal helical multidetector computed tomography (MDCT) showed a circumferential polycystic and hypodense lesion that had developed in the antral wall under the mucosa, causing gastric-outlet obstruction (Figure 1). Gastric mucosa seemed to be undamaged and enhanced. The lesion measured 5cm in diameter. Besides the cystic lesion, a solid nodular lesion three-centimetre-diameter was seen in the gastric wall. No other abnormalities were found in the abdomen, in particular the pancreas itself was normal. Examination by endoscopic ultrasonography was incomplete because of the stenosis and showed only a cystic lesion with well-defined margins originating from the submucosa. Fine needle aspiration of the cystic fluid was performed under endoscopic ultrasound guidance for biochemical and cytological analysis with the following results: amylase: 100,300UI/l; lipase: 125,000UI/l. Cytological examination of the fluid showed proteic plugs and columnar epithelial duct cells. Diagnosis of cystic dystrophy of a gastric HP was suspected but other diagnoses, such as gastric duplication, stromal tumor, lymphoma, gastric tuberculosis or actinomycosis were considered. The patient was treated with intravenous fluids and nasogastric tube aspiration to decompress the stomach. Enteral nutrition was impossible because of the gastric stenosis and total parenteral nutrition had begun. Three weeks after ultrasound endoscopic guided fine needle aspiration, the patient presented with melena with a four-point hemoglobin loss. Emergency abdominal helical MDCT was performed because of the antral stenosis. A spontaneous density on unenhanced CT was seen (Figure 2a) within the cystic lesion with enhancement after contrast injection (Figure 2b). Aeric density was also found inside the upper part of the cyst. All these signs supported a diagnosis of intracystic hemorrhage and fistulisation in the digestive tract.



Figure 1


Figure 1. 

Enhanced MDCT: circumferential antral lesion with polycystic aspect. Mucosa appeared markedly enhanced and mildly thickened (arrows).

Scanner abdominopelvien avec injection de produit de contraste : lésion polykystique, circonférentielle antrale avec épaississement et prise du contraste de la muqueuse gastrique en regard (flèches).

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Figure 2


Figure 2. 

a: hemorrhagic phase of the disease – unenhanced MDCT – hyperdense image was present in the cystic lesion: hematoma (arrow); b: enhanced MDCT at the same level as 2a – the antral-wall lesion is clearly seen (arrow). Beside the gastric-wall lesion, a well-circumscribed nodule of tissue is seen (arrowhead): heterotopic pancreas.

a : scanner abdominopelvien non injecté en période hémorragique : image hyperdense spontané intrakystique correspondant à du sang frais (flèche ) ; b : scanner abdominopelvien injecté : section au même niveau que 2a : un nodule tissulaire bien circonscrit correspondant au pancréas aberrant (tête de flèche) est visible en proximité de la lésion antrale (flèche).

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Surgical treatment was proposed: antrectomy and Finstererʼs type anastomosis were performed under celioscopic guidance. Macroscopic examination of the surgical specimen showed hypertrophic gastric folds with a 1cm diameter ulceration in the centre on the great curvature of the stomach. A three-centimetre-diameter yellow mass was identified (Figure 3) in front of this ulceration, which corresponded on histology to normal pancreatic tissue containing both exocrine and endocrine structures. HP was located in the subserosa. Pancreatic ducts and acini had passed through the muscular layer to the gastric cavity under the mucosal ulceration (Figure 4). On serial sections, cysts of various dimensions were surrounded by inflammatory infiltrate containing macrophages with hemosiderin without any epithelial cells (Figure 5). However, smaller cysts corresponded to dilated pancreatic ducts, some containing proteic plugs. The postoperative course was uneventful during eight months of follow-up.



Figure 3


Figure 3. 

On the surgical specimen, the gastric mucosa was hyperplasic and ulcerated. A nodular 3-cm-diameter yellow mass was seen in the subserosa (arrow) corresponding to a heterotopic pancreas.

Sur la pièce opératoire, la muqueuse gastrique est hyperplasique et ulcérée. Le nodule jaunâtre de 3cm de diamètre visible dans la sous-séreuse correspond au pancréas abérrant.

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Figure 4


Figure 4. 

Microscopic section showing pancreatic acini (A) and ducts (D) passing through the muscular layer to ulcerated gastric mucosa. (haematoxylin-eosin staining. Original magnification×30).

Section microscopique montrant des acini (A) et canaux (D) pancréatiques passant à travers la couche musculaire vers la muqueuse gastrique ulcérée (coloration HE×30).

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Figure 5


Figure 5. 

Serial section on the surgical specimen showing hyperplasic gastric mucosa with cystic lesion (*) and hemorrhagic suffusions in the submucosa (arrow). Inflammatory cells were seen around the cysts, mainly macrophages with hemosiderin (arrowheads). (haematoxylin-eosin staining. Original magnification×25).

Section de la pièce opératoire : muqueuse gastrique hyperplasique avec lésion kystique (*) et suffusions hémorragiques dans la sous-muqueuse (flèche). Autour des kystes, il existe un infiltrat inflammatoire avec macrophages et hémosidérine (tête de flèche) (coloration HE×25).

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Postoperatively, an exhaustive research of potential causes of pancreatitis was negative (no personal history of alcohol intake, no sign suggesting an autoimmune disease, no abdominal trauma or radiotherapy, normal serum calcium and triglyceride levels, no mutation on CFT R, SPI NK1 or cationic trypsinogen genes).

Discussion

To our knowledge, this is the first pediatric case of cystic dystrophy in gastric HP. Moreover, it was complicated by an intracystic hemorrhage and fistulisation in the stomach.

HP is a pancreatic tissue located outside the pancreas itself. It is usually found in the antrum on the great curvature (24–38%) and two thirds of the cases originate from the submucosa [1]. It has been suggested that HP is a result of the separation of pancreatic tissue during the embryonic rotation of the gut and the fusion of the dorsal and ventral buds. The second hypothesis is an inappropriate expression of a pluripotent embryonic mesenchymal tissue of the gastrointestinal wall leading to pancreatic metaplasia. It is usually asymptomatic and is discovered incidentally during upper gastrointestinal endoscopy or surgery. Symptoms of gastric HP (such as epigastralgia, abdominal discomfort, nausea and eructation) have been reported, but the correlation between these symptoms and HP is hard to determine [3, 5, 6]. Symptoms seem to be related to HP greater than 1.5cm and the involvement of the mucosal surface [3].

Although clinical and morphological features of duodenal CDHP have been well described, only five papers have published findings on gastric HP [2, 5, 7, 8, 9]. Cystic changes are a result of passive dilatation of the peripheral ducts (in this case, the cysts are lined with excretory duct epithelium) or a complication of obstructive pancreatitis (pseudocysts). Duodenal CDHP is associated with chronic pancreatitis in the pancreas itself in 71% of cases, most of which are alcohol related [4]. Our case was probably a cystic dystrophy rather than a pseudocyst suggested by the presence of epithelial ducts cells lining most of the cystic cavity. In the largest cystic lesion, the epithelium was entirely eroded and surrounded by inflammatory cells, which has been previously described by Fléjou et al. [2] in cystic dystrophy developing in HP.

This is the first case of cystic dystrophy in gastric HP described in a pediatric patient. The other six published cases were described in 37- to 44-year-old patients [2, 5, 7, 8, 9]. In younger patients, the differential diagnosis is enteric duplication. In this case, the level of pancreatic enzymes should be very low or undetectable.

The size of the cysts (5cm) is another specificity (3cm maximum diameter in previously published cases [2, 5, 7, 8, 9]) that may explain the upper gastrointestinal obstruction and justified, in part, the surgical therapeutic option.

Intracystic hemorrhage is a rare complication described in 5% of duodenal CDHP [4]. The hemorrhage may be secondary to an arteriolar erosion by cystic fluid. In our case, the hemorrhage occurred simultaneously with the fistulisation in the stomach. Erosion of the cystic wall may be the mechanism of the two complications. The relation between the fine needle biopsy and these two complications was discussed, but the fistulisation and hemorrhage occurred three weeks after the endoscopic ultrasonography.

The treatment of cystic dystrophy is not well defined and is symptom-related. Rebours et al. [4] published 105 patients with duodenal CDHP: no treatment was necessary in 18% and only 27% underwent surgery. Chronic pain and duodenal obstruction were the main reasons for surgery.

In conclusion, the diagnosis of heterotopic pancreas should be considered in case of submucosal cystic-gastric lesions, even in pediatric cases. Ultrasound endoscopic fine needle aspiration with pancreatic enzyme level measurement may be helpful when images are not conclusive. As for CDHP involving the duodenum, surgical treatment should be reserved for complicated cases (e.g., obstruction or hemorrhage).

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