Cystic dystrophy of the duodenal wall is not always associated with chronic pancreatitis

Abstract

Cystic dystrophy of the duodenal wall is a rare form of the disease which was described in 1970 by French authors who reported the presence of focal pancreatic disease localized in an area comprising the C-loop of the duodenum and the head of the pancreas. German authors have defined this area as a "groove". We report our recent experience on cystic dystrophy of the paraduodenal space and systematically review the data in the literature regarding the alterations of this space. A MEDLINE search of papers published between 1966 and 2010 was carried out and 59 papers were considered for the present study; there were 19 cohort studies and 40 case reports. The majority of patients having groove pancreatitis were middle aged. Mean age was significantly higher in patients having groove carcinoma. The diagnosis of cystic dystrophy of the duodenal wall can now be assessed by multidetector computer tomography, magnetic resonance imaging and endoscopic ultrasonography. These latter two techniques may also add more information on the involvement of the remaining pancreatic gland not involved by the duodenal malformation and they may help in differentiating "groove pancreatitis" from "groove adenocarcinoma". In conclusion, chronic pancreatitis involving the entire pancreatic gland was present in half of the patients with cystic dystrophy of the duodenal wall and, in the majority of them, the pancreatitis had calcifications.

Keywords: Cystic dystrophy of duodenal wall; Outcome; Pancreatitis; Therapy.

Figure 1

Classification of the various types of groove pancreatitis. A: Typical finding of groove pancreatitis (purple area); B: Segmental head pancreatitis: the scar tissue (dark blue) expands towards the duodenum; C: Pancreatitis of the head: the scar tissue (dark blue) expands to the duodenal area, determining duodenal stenosis and displacement of the common bile duct.

Figure 2

Case No. 1 computer tomography liver evaluation. Liver multiple hypodense lesions compatible with abscesses.

Figure 3

Case No. 1 computer tomography duodenal and pancreatic gland evaluation. A: Presence of duodenal bulging; B: Normal appearance of the pancreatic gland.

Figure 4

Case No. 1 computer tomography duodenal and pancreatic gland evaluation. A: Presence of duodenal bulging; B: Presence of cysts in the duodenal wall.

Figure 5

Case No. 2 computer tomography duodenal and pancreatic gland evaluation. A: Enlarged pancreatic head and the presence of multiple cysts between the enlarged pancreatic head and the duodenum (multidetector computer tomography); B: The remaining pancreas was normal as demonstrated by the magnetic resonance imaging.

Figure 6

Case No. 2 pancreatic and duodenal surgical specimens. A: Resected specimen showing cystic dystrophy of the duodenal wall with hypertrophy of the Brunner glands and the presence of an ectopic pancreas (arrows); B: Chronic pancreatitis in the remaining pancreas together with cystic dystrophy of the duodenal wall.

Figure 7

Case No. 3 pancreatic and duodenal pathological specimens. A: Cystic dystrophy of the duodenal wall with aspects of chronic pancreatitis in the heterotopic pancreas; B: Aspects of autoimmune pancreatitis (arrow); C: Groove adenocarcinoma extending to the pancreatic head (arrow).

Figure 8

New surgical option for patients having cystic dystrophy of duodenal wall. A: Scheme of the pancreas-preserving resection of the second portion of the duodenum. The second part of the duodenum, including the main papilla, is removed and the segment of the proximal jejunum supplied by the artery and vein is cut out and prepared for transposition between the 1st and 3rd portions of the duodenum; B: The shifted segment is interposed between the 1st and the 3rd parts of the duodenum. Jejuno-jejuno- and duodeno-jejuno-anastomoses are performed. The bile and the pancreatic ducts were implanted in the neodudenum 4 cm below the proximal duodeno-jejuno-anastomosis (from Egorov et al[70] with the kind permission of the authors).