Pancreas- and Pylorus-Preserving Duodenectomy for Advanced Familial Duodenal Polyposis Open Access

Familial adenomatous polyposis (FAP) is characterized by the development of multiple adenomas along the gastrointestinal tract. The most frequent genetic mutations responsible for adenomatous polyposis are the APC mutation, associated with FAP, and the MYH mutations, the MUTYH-associated polyposis [1, 2].

Prophylactic proctocolectomy decreased the incidence of colorectal cancer in these patients, leaving desmoid tumors and duodenal cancers as the leading causes of death in FAP patients [3]. In contrast to colonic polyps, no more than 5% of duodenal polyps are likely to progress to duodenal malignancy [4]. The Spigelman classification [5] segregates patients based on the number, size, histology, and grade of dysplasia of the duodenal lesions (Table 1). The stages range from I to IV, with IV representing the most advanced stage, and 36% developed cancer at 10 years in a prospective study of 114 patients from St Mark’s Hospital [6]. Local endoscopic or surgical therapy may be appropriate for early disease, but with a high recurrence rate [7, 8]. Now, with lower mortality following pancreatic surgery and better understanding of duodenal disease in FAP as a result of surveillance, prophylactic resectional duodenal surgery has become a reasonable option. Pancreaticoduodenectomy or pancreas-sparing duodenectomy offer definitive therapy in preventing duodenal carcinoma. Although pancreas-sparing duodenectomy has been used less frequently, it offers the potential advantage of preserving a normal pancreas and some preliminary results are promising [9]. This surgical option was performed at our hospital, and as far as we know also in Portugal, for the first time.

A 45-year-old female with FAP with no mutation found, but with a family history in her father and brothers, was submitted to prophylactic restorative proctocolectomy at 24 years. After 1 year she presented with abdominal pain and a 5-cm desmoid of the mesentery was diagnosed by CT scan. Partial regression was observed with sulindac. She had two pregnancies.

A duodenal polyposis stage III carpeting the periampullary region was observed when the patient was 37 years old. Endoscopic papillectomy and piecemeal mucosectomy was performed in one session; bleeding occurred during resection and was managed by coagulation with the tip of the snare and argon plasma. After the operation, no obvious bleeding or perforation was seen and the procedure was considered complete with no apparent residual adenoma. However, the early postprocedure period, about 12 h later, was complicated by hemorrhage originating from the resection scar. A decision to intervene surgically was made but a duodenotomy showed that the bleeding had stopped spontaneously and no hemostasis was needed. Unfortunately, endoscopic re-evaluation several months after surgery showed recurrent adenoma; due to the perceived risk of perforation in the context of fibrosis due to previous extensive mucosectomy, no further endoscopic resection was tried and a few attempts of ablation with argon plasma were also unsuccessful. Thereafter, regular endoscopic surveillance was maintained with the presence of a tubulovillous adenoma with low-grade dysplasia over several years but, eventually, high-grade dysplasia was diagnosed (Fig. 1a, b). Some diminutive polyps were also seen in the small-bowel capsule endoscopy (Fig. 2). The CT scan and the endoscopic ultrasonography did not demonstrate parietal or nodal involvement and the MRCP showed normal biliary and pancreatic ducts without visualization of the Santorini duct (Fig. 3). A pancreas- and pylorus-preserving duodenectomy was performed in 3 main steps (Fig. 4):

1.A cholecystectomy was carried out with a catheter introduced through the cystic duct to identify the papilla. The carpeting periampullary lesion was confirmed by duodenotomy without evidence of degeneration or other polyps in the proximal or distal duodenum and the transition to the jejunum was divided. The distal duodenum was progressively separated from the pancreas to the ampulla using Ligasure. The pylorus and the proximal duodenum were identified, separated from the pancreas without evidence of the Santorini duct. The duodenum was transected 2 cm distal to the pylorus. The dissection was completed leaving only the ampullary complex attached to the duodenum (Fig. 5).

2.The reconstruction was then performed using an advanced jejunal limb. The stapled duodenum was sutured with the stapled jejunum and a latero-lateral duodenojejunostomy was performed with separated stitches.

3.Afterwards, the ampullary complex was transected with scissors: the pancreatic duct was cut and a stent introduced. Next, the biliary duct was transected with preservation of the transcystic catheter and the duodenum removed. No induration or suspicious degenerescence was observed. Both ducts were joined with a suture. After a small enterotomy, the anastomosis of the biliary and pancreatic duct was performed using interrupted 5/0 Prolene sutures with an internal pancreatic stent and the transcystic catheter (Fig. 6). The pancreaticojejunal anastomosis was reinforced with Blumgart mattress sutures. The omentum was placed around the anastomosis with a closed suction drain. A nasojejunal feeding tube was placed and secured.

The specimen showed the carpeting periampullary lesion, a tubulovillous adenoma with low-grade dysplasia (Fig. 7). The histopathology of the surgical specimen did not confirm the previous diagnosis of high-grade dysplasia.

The patient was discharged on the 11th postoperative day without complications and the transcystic tube was removed at 2 months. Follow-up endoscopy during the first postoperative year showed a wide and regular duodenojejunostomy (Fig. 8) and a discrete biliary anastomosis (neo-ampulla) in the jejunum loop (Fig. 9).

With enhanced survival as a consequence of screening and prophylactic colectomy, the significance of duodenal disease has increased [10]. The aim of endoscopic surveillance was to identify patients before the transition from adenoma to carcinoma or when only small foci of carcinomatous change were present. Once the advanced malignant change is established the prognosis after surgery is poor [11].

The selection of patients for the prophylactic surgical procedure is not easy. In a recent cohort study of 437 patients with endoscopic surveillance according to the Spigelman criteria, 52 underwent radical surgical treatment and 11 (21%) were found to have adenocarcinoma, with a further 2 cases of conservative transduodenal ampullectomy [12]. Of these 13 patients with carcinoma, only 5 were classified as Spigelman IV. The authors [12] concluded that the Spigelman score must dictate the rhythm of endoscopic surveillance whereas the presence of high-grade dysplasia or of a large unresectable ampullary lesion is an indication for prophylactic radical surgery. These risk factors were present in our patient, supporting the radical surgical option.

Pancreatic surgery may be more technically demanding in patients with FAP because of previous colectomy, the adhesions and desmoplastic changes in the mesentery with more difficult mobilizations, anastomosis of non-dilated ducts, and prolonged postoperative ileus with additional stresses on the surgical anastomosis. The St. Mark’s group presented the operative outcome of the pylorus-preserving pancreatoduodenectomy in 16 FAP patients with Spigelman IV, including mortality in 2 (12%) and morbidity in 8 (50%), and a median hospital stay of 36 days [13]. The experience of the surgeons at the Cleveland Foundation with pancreas- and pylorus-sparing duodenectomy [9] is, in 21 cases with no mortality, a 38% rate of morbidity, 19% leakage rate, and a length of stay of 14 days. These results are comparable with the experience of the same group in performing pylorus-preserving pancreatoduodenectomy in 238 cases, with 1.3% mortality, 43% morbidity, a 9% leakage rate, and a length of postoperative stay of 14 days. The published experience of this group with pancreas-sparing duodenectomy now totals 42 cases [14]. Another Asian group has reported good outcomes in 21 patients who underwent pancreas-sparing duodenectomy in comparison with 44 cases with pancreaticoduodenectomy associated with a soft pancreas [15].

The morbidity of the pancreas- and pylorus-sparing duodenectomy is principally related with delayed gastric emptying and a leak at the pancreaticobiliary anastomosis. The delayed gastric emptying is multifactorial and can also occur in the classic Whipple procedure or in the pylorus-preserving pancreatoduodenectomy. For this reason, a nasojejunal tube for postoperative enteral feeding is recommended for this procedure. Our patient did not tolerate clamping of the transcystic biliary decompression catheter in the first postoperative week and it was only removed in the second postoperative month.

The anastomotic leak is the most dangerous complication and is more frequent with a soft pancreas and a small pancreatic duct [16]. The incidence of pancreatic leak is decreasing with experience and attention to technical details such as a wide duct to mucosa anastomosis and the dunking effect of the Blumgart stitches, which was the anastomotic technique used in this patient. However, until now, no differences have been found in several meta-analyses in the postoperative pancreatic fistulas related to the comparison of different techniques of pancreaticojejunostomy [17].

The pancreas-preserving duodenectomy has the potential disadvantage of mixing the bile and pancreatic secretions at one anastomotic site [9]. However, by using an open transcystic tube, we believe this risk can be decreased, as was shown in our case. Another issue relates to patients with pancreas divisum or with a large Santorini duct. To identify these cases, it is necessary to carry out a preoperative MRCP to define the pancreatic ductal anatomy with the additional advantage of identifying eventual mesenteric desmoid tumors. In these cases it is recommended to reimplant the minor papilla through a separate anastomosis to the jejunum [9].

Pancreas- and pylorus-preserving duodenectomy has an important advantage related to the possibility of complete endoscopic postoperative assessment of the small duodenal cuff, the neo-ampulla, and the proximal jejunum, in which recurrence of adenomatous tissue or carcinoma can occur. The long-term outcome of this procedure, at a mean follow-up of 79 months, has proven that it is effective in preventing duodenal carcinoma, even at the ampullary complex [9]. The resection of the duodenum shifts the carcinoma risk to the remaining small bowel and the duodenal cuff, if it is preserved. The requirement for complete surveillance would be more difficult with a Whipple procedure.

In conclusion, pancreas- and pylorus-preserving duodenectomy is a promising alternative to pancreaticoduodenectomy for advanced duodenal polyposis that enables complete endoscopic surveillance.

This study did not require informed consent or review approval by the appropriate ethics committee.

The authors report no potential conflicts of interest.

All the authors read and approved the manuscript being submitted. All authors listed contributed significantly to the work.