We report the case of a 69-year-old man in a regular hemodialysis program for autosomal dominant polycystic kidney disease (ADPKD) with no residual diuresis admitted to the Nephrology service for the 5th time in one year period with fever (axillary temperature: 39 °C), with onset during hemodialysis session, and altered state of consciousness. The patient was on regular HD program for 3 years with a functioning left umerocephalic arteriovenous native fistula and suffered from hypertension, obesity (BMI 34Kg/m2) and preserved ejection fraction heart failure. The patient was on losartan 100mg once daily, bisoprolol 2.5mg once daily, 100mg acetylsalicylic acid once daily and omeprazol 20mg once daily. 

The patient had a relevant history of 4 previous hospitalizations for fever of unknown etiology, with no further sign or symptom, secondary to broad-spectrum beta-lactamase producer E.Coli, only sensitive to carbapenems and colistin. In previous hospitalizations pulmonary, kidney and liver cysts infection were excluded through computer tomography. Native AV fistula infection had also been excluded in every previous hospitalization through physical examination and ecographic evaluation. The patient had colonic diverticulosis as part of his ADPKD and had performed colonoscopy twice on previous hospitalizations for exclusion of colonic diverticulitis. As no evident source of infection was found, the patient had been treated with an empiric course of meropenem (load dose of 1gr, followed by 500 mg per day) for 10 to 14 days on the previous hospitalizations. On every previous hospitalization the patient showed marked improvement after directed antibiotic therapy was initiated with resolution of both fever and inflammatory markers on blood work. 

On his 5th hospitalization the patient started complaining of abdominal pain, of sudden onset, tightening type and moderate intensity 6/10 on the first 24 hours after hospitalization. In association, he described chills, feeling of postprandial fullness and nausea. The patient denied fatigue, vomiting or other changes in intestinal transit, such as diarrhea, recent trips, contact with animals, consumption of untreated water or pasteurized products.

On physical examination, the patient was hypotensive, tachycardic and presented a globose abdomen, painful on deep palpation of the right hypochondrium and epigastrium, with slight defense and absent Murphy's sign. Full skin, no wounds and no jaundice. On blood work he had leukocytosis (16 x 10³ /μL) with neutrophilia (11.51 x 10³ /μL), marked elevation of C-reactive protein (414.5 mg/L). Hepatic laboratory tests showed a slight rise of aspartate transaminase (52U/L), alanine transaminase (71 U/L), Alkaline phosphatase (300 U/L), gamma-Glutamyl Transferase (255 U/L) and mild hyperbilirubinemia (2.1 mg/dL) mostly due to direct bilirubin (1.3 mg/dL).

Abdominal tomography at this time showed dilatation and ectasia of the main biliary tract (MBT). Blood cultures (BC) were positive again for broad-spectrum beta-lactamase producer E.coli with the same pattern of antibiotic resistances as previously reported. It was also found that the tumor marker CA19.9 and chromogranin A were altered, the latter being markedly elevated. 

The case was discussed with Gastroenterology and General Surgery departments. We chose to perform an upper digestive endoscopic examination, in which a formation that prolapsed from the lumen into the MBT, which corresponded to Vater's papilla (Figure 1), was seen. This was followed by duodenoscopy, in which progression to DII was possible, where initially the papilla could not be seen by mobilization into the insides of an apparent ampullary IDD (Figure 2A). Using a biopsy forceps, it was possible to pull and exteriorize the papilla, which had increased dimensions (approximately 15mm) and villous morphology, findings suggestive of ampuloma (Figure 2B and 2C). The papilla was biopsied and the anatomopathological report revealed a tubular adenoma with low-grade dysplasia.

The patient completed antibiotic therapy cycle with meropenem, rifaximin and ursodeoxycholic acid for 3 weeks with complete resolution of all complaints. Repeated blood cultures were negative. After discharge, he was referred to hepatobiliary team consultation.

In this case, endoscopic resection would have been ideal, and was the first approach, however on the second duodenoscopy (2 months after initial diagnosis) the ampulloma had grown and could not be mobilized from the main biliary duct. The surgical team proposed a transduodenal surgical ampullectomy, even though the dimensions of the tumour and expected scarring due to recurrent inflammation, would make it technically difficult for the biliary and pancreatic duct reconstruction, and the risk of intra-operative complications would be very high. Meanwhile, the patient-maintained episodes of bacteremia with sepsis every 2 months and his clinical status had worsened and he had been losing weight. A multidisciplinary clinical decision-making consultation with the patient was held 8 months after ampulloma diagnosis was held. The anesthesiology and surgical team considered the patient very polymorbid (end stage renal disease, obesity, recent weight loss, heart failure with preserved ejection fraction) for such major surgery, especially as the biliary tract would be colonized with a multiresistant bacteria which would leave the patient at an extreme risk for intra operative and post operative complications. The patient refused the surgery and opted for a palliative option.  Gastroenterology department performed an endoscopic sphincterotomy and biliary plastic stent placement with good drainage after the procedure (Figure 3).

Despite the success of the treatment, the patient returned to the hospital 4 more times with sepsis and without signs of biliary obstruction. The same pathogenic bacteria was isolated on blood cultures.  Patient eventually died from septic shock 9 months after stent placement. After his death, blood cultures revealed E. Coli resistant to carbapenems, which explains why on his last hospitalization the patient did not respond to antibiotic therapy and progressed to septic shock.