Duodenopancreatectomy for cystic dystrophy in heterotopic pancreas of the duodenal wall

© Masson, Paris, 2006.

ORIGINAL ARTICLE

Gastroenterol Clin Biol 2006;30:24-28

Duodenopancreatectomy for cystic dystrophy in heterotopic pancreas of the duodenal wall

Patrick PESSAUX (1), Paul LADA (1), Sandrine ETIENNE (1), Jean-Jacques TUECH (1), Emilie LERMITE (1), Olivier BREHANT (1), Stéphane TRIAU (2), Jean-Pierre ARNAUD (1) (1) Service de Chirurgie Digestive,(2) Service d’Anatomo-Pathologie, CHU Angers.

SUMMARY

RÉSUMÉ

Aim of the study — Cystic dystrophy in heterotopic pancreas (CDHP) is rare. The aim of this study was to evaluate the diagnosis, management, and follow-up of the CDHP.

Duodénopancréatectomie pour dystrophie kystique sur pancréas aberrant de la paroi duodénale Patrick PESSAUX, Paul LADA, Sandrine ETIENNE, Jean-Jacques TUECH, Emilie LERMITE, Olivier BREHANT, Stéphane TRIAU, Jean-Pierre ARNAUD

Patients and methods — Between August 1990 and March 2004, 12 patients with CDHP underwent a duodenopancreatectomy. The patients were retrospectively reviewed.

(Gastroenterol Clin Biol 2006;30:24-28)

But — La dystrophie kystique sur pancréas aberrant (DKPA) est une pathologie rare. Le but de notre étude était d’analyser les aspects cliniques et diagnostiques ainsi que la prise en charge chirurgicale des DKPA.

Results — There were 11 men and 1 woman with a mean age of 42.4 years (range : 34-54 years). Nine patients (75%) were alcoholic and 8 patients had chronic pancreatitis. The diagnosis of CDHP was performed in 8 patients (66.6%) after the preoperative workup. Seven patient had a medical treatment with octreotid and endoscopic cystic ponction (N = 3) or cystic fenestration (N = 1). Recurrence of pain was noted after a mean period of 5 months. Three patients had recurrent acute pancreatitis. Duodenopancreatectomy was performed in all cases. The mortality and morbidity rate were respectively 8.3% (N = 1) and 25% (N = 3). Mean follow-up was 64 months (ranges: 6 - 158 months). One patient was seen 70 months later with epigastric pain and features of acute pancreatitis of the pancreatic stump due to anastomotic stenosis. The other patients were asymptomatic.

Malades et méthodes — D’août 1990 à mars 2004, 12 malades présentant une DKPA ont été opérés d’une duodénopancréatectomie céphalique (DPC). Ces dossiers ont été analysés de façon rétrospective. Résultats — Il y avait 11 hommes et une femme d’un âge moyen de 42,4 ans (extrêmes : 34-54 ans). Neuf malades (75%) avaient une intoxication alcoolique chronique et huit malades des lésions de pancréatite chronique. Le bilan d’imagerie a permis de faire le diagnostic de DKPA chez 8 malades (66,6%). Sept malades ont eu un traitement médical initial qui comprenait toujours un analogue de la somatostatine, associé à une ponction endoscopique des kystes chez 3 malades ou à une fenestration des kystes chez un malade. Trois malades ont présenté des récidives de poussées de pancréatite aiguë. Tous ont été opérés d’une duodénopancréatectomie céphalique. Les taux de mortalité et de morbidité étaient respectivement de 8,3% (N = 1) et de 25% (N = 3). Le suivi moyen était de 64 mois (extrêmes : 6-158 mois). Un seul malade a présenté une récidive douloureuse après 70 mois en rapport avec des poussées de pancréatite aiguë du moignon sur sténose de l’anastomose pancréatico-gastrique. Tous les autres malades étaient asymptomatiques.

Conclusions — Diagnosis of CDHP is difficult. After failure of medical treatment, duodenopancreatectomy can be proposed.

Conclusions — Le diagnostic de DKPA n’est pas toujours posé en pré-opératoire. Après un échec du traitement médical, la résection chirurgicale par une DPC nous semble indiquée.

C

clinical and diagnostic features of CDHP and optimal surgical management.

ystic dystrophy in heterotopic pancreas (CDHP) located in the duodenal wall is rare. Described for the first time by Potet and Duclert in 1970 [1] CDHP is characterized by the presence of cysts within an ectopic pancreas situated in the muscular layer of the duodenal wall. Modern imaging techniques afford easy diagnosis but the clinical expression is quite variable and treatment remains a subject of debate. The efficacy and exact indications of different therapeutic options (surgical, endscopic, medical treatment) remain to be elucidated. The purpose of our study was to analyze the

Patients and method From August 1990 to March 2004, twelve patients with CDHP underwent duodenopancreatectomy. These twelve patients accounted for 9.3% of such procedures during this time (12/ 129). Patient files were analyzed retrospectively. Demographic data, patient history, diagnostic circumstances (symptoms, physical examination, time to diagnosis), laboratory results, and therapeutic modalities were noted as well as patient follow-up. Firstintention medical treatment was systematic when CDHP was suspected. The efficacy of this treatment was assessed basically on

Reprints: P. PESSAUX, Service de Chirurgie Digestive, CHU Angers, 4 rue Larrey, 49033 Angers Cedex 01. E-mail : [email protected]

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Cystic dystrophy in heterotopic pancreas

or nervous connection to the main pancreatic gland. Frequency is estimated at 0.5 to 14% in different series [3-5]. The main localizations include the duodenum (30-35%), the stomach (3035%) as well as other levels of the digestive tract [6]. Cystic dystrophy is an almost exclusive complication of heterotopic pancreas situated in the superomedial border of the second duodenum (suprapapillary localization). Ectopic exocrine lobules may have excretion channels which are generally too small for function. Accumulation of pancreatic secretions can then lead to repeated episodes of obstructive acute pancreatitis and the formation of retention cysts [7]. CDHP can be associated with a healthy pancreas, an isolated pathological entity, or more generally with a pancreas suffering from chronic pancreatitis (67% in our series). For Léger et al. [8], CDHP is an extension of existing chronic pancreatitis in an ectopic localization. For Potet et al. [1], the chronic pancreatitis evolves independently in the ectopic gland, leading to upstream compression of the pancreatic duct.

the clinical response in terms of pain relief, use of antalgesics, and recurrence of acute pancreatitis.

Results The series included eleven men and one woman, mean age 42.4 years (range 34-54 years). Time from symptom onset to diagnosis was 17 months on average (range 4-41 months). Nine patients (75%) had chronic alcoholism and eight presented associated chronic pancreatitis lesions. Epigastric pain was noted in 92% of patients (N = 11) with posterior irradiation in ten. This pain became constant in four patients who required major antalgesic drugs (morphine). Vomiting was noted in eight patients (67%) and progressed to complete intolerance of oral food intake in two. Vomiting was associated with 5.7 kg weight loss on average (range 2-10 kg). One patient presented a sudden episode of hematemesis and developed a state of shock. Six patients (50%) developed recurrent episodes of acute pancreatitis (3 to 7 acute episodes). Jaundice was noted in two patients (17%) (table I). Preoperative imaging included transparietal ultrasonography in eleven patients (92%), computed tomography (CT) in all patients, magnetic resonance imaging (MRI) in four patients (33%) and endoscopic ultrasonography in nine (75%) (table II). The diagnosis of CDHP was established in eight patients (67%) on the basis of imaging findings. Diagnosis was established in the presence of: pancreatic duct damage by erosion of gastroduodenal artery into a pancreatic pseudocyst subsequent to chronic pancreatitis, mucinous cystadenoma, cyst-like neuroendocrine tumor, and a duodenal tumor. Seven patients were given first-intention medical treatment using octreotid in all seven (1 to 3 months) associated with endoscopy-guided cyst puncture in three and cyst fenestration in one. Complete abstention from alcohol was achieved in six of the nine alcoholic patients. Recurrent pain developed after five months on average (range 3-10 months). Three patients presented recurrent episodes of acute pancreatitis (including two who had not abstained from alcohol). Duodenopancreatectomy was performed 25 months on average (range 1-54 months) after symptom onset in eleven patients. The twelveth patient underwent emergency duodenopancreatectomy for hemostasis subsequent to rupture of an aneurysm of the gastroduodenal artery into a cyst fistulized in the duodenum. Arterial embolization was attempted first but was unsuccessful because the celiac trunk could not be catheterized. This patient died from multiple organ failure three days after surgery. Morbidity was 25%: two pancreatic fistulae treated medically and one central catheter infection. Mean hospital stay was 22 days and mean follow-up 64 months (range 6-158). One abstinent patient developed painful recurrence 36 months after surgery related to acute pancreatitis of the pancreatic stump subsequent to stenosis of the pancreatogastric anastomosis. This stenosis was treated by deviating the pancreatic duct into the jejunum with a Y loop [2]. All patients remained asymptomatic, eight are living, one was lost to follow-up 26 months after surgery and one died 88 months after surgery due to lung cancer. Among the patients who had a CT scan postoperatively, two presented pancreatic calcifications. Pathology examination confirmed the diagnosis of CDHP in all patients. The cysts were situated along the inner border of the second duodenum, in the submucosa in nine patients, in the duodenal wall in two and in the musculosis in one (figures 1 and 2).

CDHP is generally observed in men (93%) in their fifth decade [9], as was found in our series. In the literature, about 75% of patients present chronic alcoholism [6, 9]. The diagnosis is often missed and established late (17 months on average in our series) due to the non-specific clinical manifestations or attribution of symptoms to chronic pancreatitis. Symptoms of CDHP can be epigastric pain, intolerance of oral food intake due to duodenal compression, or jaundice by biliary compression. Major weight loss can be attributed to pain which can lead to restriction of food intake and vomiting (duodenal stenosis). The diagnosis of CDHP is sometimes established during an etiological search in patients with recurrent acute pancreatitis. Endoscopic ultrasonography is the most useful diagnostic examination [10-12]. It can confirm the diagnosis of CDHP in the presence of three specific components in the duodenal wall: thickening, presence of cysts, and a network of channels around the cysts. This examination also allows the differential diagnosis with other diseases including duodenal duplication, necrotic duodenal tumor, or pancreatic pseudocysts. It is sometimes operator-dependent and in some patients with CDHK cannot be performed due to the duodenal stenosis. A miniprobe may be useful [13, 14]. Transparietal ultrasonography and CT [15] are much less discriminative, but do enable visualization of the duodenal wall thickening and sometimes the presence of cyst-like lesions. Ultrasonography can also assess the impact on biliary and pancreatic ducts. MRI with cholangiopancreatography remains to be evaluated in this disease [16, 17]. It can demonstrate the presence of cysts in the duodenal wall (high intensity signal on the T2 sequences) and interposition of a tissue between the duodenum and the head of the pancreas which appears as a low intensity signal on all sequences. Treatment of CDHP remains a subject of debate and the role of the different therapeutic options (medical, endoscopic, surgical treatment) is not clearly established. Symptoms can be improved with fasting and if needed gastric aspiration associated with abstention from alcohol intake, which is the best way of avoiding painful relapse. Administration of octreotid is proposed but has a variable effect on pain, reduction of cyst size, and weight gain [18, 19]. The delay of efficacy and duration of action of octreotid remains variable and its long-term effect is unknown. Endoscopic fenestration has been proposed [20, 21] for selected patients: voluminous cysts, few cysts, rather superficial cysts. However, CDHP cysts are generally small, numerous, and developed within a thickened duodenal wall, explaining why endoscopic fenestration is usually incomplete, providing only temporary relief. In our series, all patients treated medically experienced relapse. Surgery is reserved for cases with symptoms resisting treatment or relapsing after medical treatment. Certain authors report digestive deviations in the event of an

Discussion Heterotopic pancreas is defined as the presence of pancreatic tissue in an ectopic localization without anatomic, vascular, 25

P. Pessaux et al.

Table I. – Characteristic features of the patients. Caractéristiques des malades. Age (ans)

Sex

Chronic alcoholism

Chronic pancreatitis

Symptoms

Weig ht loss

Complementary explorations

Preoperative diagnosis

35

M

YES

YES

Pain

10 Kg

Endoscopy, US, CT, ERCP, EUS

CDHP

6 Kg

Endoscopy, US, CT

Arterial erosion into a pancreatic cyst

Vomiting 54

M

YES

YES

Pain

Vomiting Jaundice Hematemesis 50

M

YES

NO

Acute pancreatitis

8 Kg

Endoscopy, US, CT, ERCP, EUS

CDHP

34

F

NO

NO

Pain

2Kg

Endoscopy, US, CT, ERCP,

Mucinous cystadenoma

3 Kg

Endoscopy, US, CT, ERCP,

CDHP

Vomiting 37

M

YES

YES

Pain Vomiting

Jaundice 40

M

YES

YES

Pain

EUS 5 Kg

Vomiting

Endoscopy, US, CT, ERCP,

CDHP

EUS

Acute pancreatitis 39

M

NO

NO

Hypoglycemia

0

US, CT, MRI, EUS

Cystic neuroendocri ne tumor

44

M

YES

YES

Pain

5 Kg

Endoscopy, US, CT

CDHP

Vomiting

EUS

Acute pancreatitis 40

M

YES

YES

Acute pancreatitis

0

Endoscopy,CT, ERCP

CDHP

EUS 45

M

YES

YES

Pain

4 Kg

Endoscopy, US, CT, MRI

Duodenal tumor

8 Kg

US, CT, MRI, EUS

CDHP

0

Endoscopy, US, CT, MRI

CDHP

Vomiting Jaundice Acute pancreatitis 39

M

YES

NO

Pain Vomiting Acute pancreatitis

52

M

NO

YES

Pain

Acute pancreatitis

26

EUS

Cystic dystrophy in heterotopic pancreas

obstructive lesion without painful episodes [7, 22]. The advantages are the low morbidity and sparing of the pancreatic parenchyma, but the lesions are left in place and can become painful with episodes of acute pancreatitis. Some patients thus undergo secondary duodenopancreatectomy. This procedure removes the risk of recurrence and the potential impact on the pancreas. Furthermore, duodenopancreatectomy is indicated when there is a diagnostic doubt about a malignant tumor. There is however a high mortality and mortality associated with duodenopancreatectomy. Certain authors have thus proposed limited resection of the second duodenum without pancreatectomy, a procedure which depends on the supra-papillary localization of the cystic lesions [23]. In conclusion, symptoms of CDHP are variable and non-specific, explaining the late diagnosis. Endoscopic ultrasonography and MRI are currently the examinations of choice for diagnosis. Management remains a topic of discussion but surgical resection can be reserved for patients who fail to respond sufficiently to medical treatment.

Table II. – Results of preoperative workup. Résultats des examens complémentaires préopératoires. Ultrasound CT N = 12 N = 11

MRI N=4

EUS N=9

Thickening of the duodenal wall

5 (45.5%)

6 (50%)

3 (75%)

8 (88.9%)

Cysts of the duodenal wall

2 (18.2%)

5 (41.7%)

4 (100%)

8 (88.9%)

Atrophy of pancreas head and body

1 (91%)

3 (25%)

2 (50%)

2 (22.2%)

Dilatation of the main bile duct

2 (18.2%)

3 (25%)

3 (75%)

3 (33.3%)

0

2 (16.7%)

0

0

3 (27.3%)

3 (25%)

3 (75%)

3 (33.3%)

Calcifications Dilatation of the main pancreatic duct

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Fig. 1 – Surgical resection specimen (Papilla-Duodenal mucosa — CDHP — Pancreas). Aspect macroscopique de la pièce opératoire.

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Fig. 2 – Microscopic examination (HPS stain x 120 — Ectopic pancreas — Cyst). Coupe microscopique.

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