ampullary adenomas arise from the ampulla of vater , the confluence of the pancreatic and common bile ducts , located at the major duodenal papilla in the second part of the duodenum . based upon autopsy studies , ampullary adenomas
however , the vast majority of these patients will remain asymptomatic [ 1 , 2 , 3 , 4 ] . as with colonic adenocarcinoma
, ampullary adenomas also follow the adenoma - carcinoma sequence and may thus evolve from localised premalignant lesions into widespread carcinoma .
such lesions may be removed endoscopically or via surgical excision , with either a local resection or an extended procedure , for example , a pancreaticoduodenectomy .
this may occur as an idiopathic process , as is more common in the paediatric population , or it may be due to a structural pathology , such as colonic malignancy . as other gastrointestinal adenomas , ampullary adenomas assume tubular , tubulovillous or villous forms .
they can occur either sporadically or in patients with a genetic predisposition , such as familial adenomatous polyposis or gardner 's syndrome , and such lesions may therefore be identified via endoscopic surveillance programmes .
indeed , previous studies have suggested a further genetic link to other disorders , such as adult polycystic kidney disease , whilst no specific environmental factors such as diet have been identified in their development .
clinically , they are usually asymptomatic and found incidentally at endoscopy ; however , some patients may present with symptoms caused by obstruction of the main gastrointestinal tract and/or the distal biliary / pancreatic ducts .
the most common presenting symptoms are gastric outlet obstruction or gastrointestinal bleeding [ 8 , 9 ] .
however , rarer presenting complaints may include acute pancreatitis , biliary obstruction or , as in this case , intussusception .
indications for the excision of such lesions are essentially two - fold : to attempt resolution of immediate symptoms and to prevent malignant progression
. the management of such lesions may be endoscopic surveillance , endoscopic excision or surgical resection .
surgical resection has the advantage of lower recurrence rates as opposed to endoscopic resection ; however , with the obvious caveat of a much higher risk of morbidity and mortality . in this article , we present a case where a female patient developed recurrent chest infections and gastric outlet obstruction secondary to an intussuscepting ampullary adenoma . due to the size and location of the mass ,
it was surgically removed by pancreaticoduodenectomy . to the best of our knowledge , this is the first such presentation worldwide , and we hope that it may be of educational value to both physicians and surgeons alike .
a 49-year - old nigerian female presented to her local hospital with a 1-year history of recurrent chest infections .
she had no relevant past family history of any genetic disorders , in addition to no recent foreign travel or contact exposure to tuberculosis .
when she initially presented , plain chest radiographs were performed and demonstrated multiple lung lesions suggestive of an infective / inflammatory process .
she grew pseudomonas on sputum culture , but despite treatment for atypical pneumonia with multiple courses of antibiotics , she failed to adequately improve .
chest ct was consequently performed and demonstrated multiple cavitating lung lesions ( fig 1 ) . during this time
, she had intermittent vomiting with an associated 15-kg weight loss over a 12-month period .
these symptoms were initially attributed to her recurrent chest infections and were not further investigated .
a working diagnosis of aspergillus infection was established , and the patient remained under the care of the respiratory physicians . at the time of chest ct ,
however , an incidental finding was noted in the second to the fourth part of the duodenum .
concurrently , she developed subacute bowel obstruction with persistent vomiting and was unable to tolerate oral intake .
a dedicated ct scan of the abdomen and pelvis was therefore performed , demonstrating an obstructing polypoid mass at d2 ( fig 2 ) .
this lesion was confirmed on upper gastrointestinal endoscopy , and a biopsy demonstrated a tubulovillous adenoma with high - grade dysplasia .
nasojejunal tube insertion was attempted at her local hospital ; however , this failed to bypass the obstructing lesion .
her case was discussed at the regional hepatopancreaticobiliary multidisciplinary team meeting at the royal london hospital . as her biopsy demonstrated high - grade dysplasia , in addition to her symptomatic presentation , it was felt that a resection was required . based upon her cross - sectional and endoscopic imaging
, it was deemed that this would not be amenable to an endoscopic excision , and she was likely to require surgical resection and should be transferred to the hpb unit at the royal london hospital .
interestingly , despite this large lesion , she never became jaundiced and maintained a normal bilirubin level .
on arrival at the hospital , she was admitted to the surgical ward , and although she had persistent vomiting and was cachectic , she was haemodynamically stable .
g / l ( normal range 3550 ) due to the failed nasojejunal tube insertion .
consequently , a picc line was inserted by interventional radiology , and in an attempt to optimise her for major abdominal surgery , total parenteral nutrition was administered . however , due to these ongoing nutritional problems and a failure to adequately improve , she underwent a semi - urgent conventional pancreaticoduodenectomy .
intraoperatively , a large mass at d2 with a duodeno - duodenal intussusception and distal duodenal ischaemia was identified ( fig 3 ) .
histological analysis reported a polypoid tumour at the ampulla of vater , 75  28  30 mm in size , with a reduced intussusception and an ischaemic distal duodenum ( fig 3 ) .
these findings were in keeping with a duodenal ampullary low - grade tubular adenoma , with intussusception and complete resection margins .
there was no evidence of any high - grade dysplasia , in contrast to her previous diagnostic biopsy , or invasive malignancy .
the patient was admitted to the high - dependency unit postoperatively and was stepped down to the surgical ward on postoperative day 2 .
she progressed well with early mobilisation , and her nutritional status greatly improved during this time , with due care not to induce a refeeding syndrome .
she did not develop any exocrine pancreatic insufficiency or any derangement from her baseline glycaemic control .
to date , the patient has not suffered any long - term complications , and remains clinically well on follow - up .
intussusception in adults is rare and is responsible for approximately 15% of all adult intestinal obstructions
. these can be classified by location into enteroenteric , colocolonic , ileocolic and ileocaecal , respectively .
the presenting symptoms are usually non - specific and chronic , with pain and symptoms of intermittent intestinal obstruction , such as vomiting , bloating and/or diarrhoea or constipation being the major complaints .
the classic paediatric triad of cramping abdominal pain , bloody diarrhoea and a tender abdominal mass is rare in adults .
as opposed to the paediatric population , where the aetiology is normally idiopathic , in adults , the cause is usually a structural pathology , with two - thirds of colonic cases being a malignant neoplasm , as opposed to one - third of those occurring in the small intestine [ 11 , 12 , 13 ] .
abdominal ct is now regarded as the modality of choice for investigating such lesions . due to the preponderance for a structural aetiology ,
the management of such cases is almost always via laparotomy , with no role for the use of air insufflation as utilised in the paediatric population .
intraoperative reduction of the intussuscepted bowel is possible ; however , there are associated risks such as tumour seeding and anastomotic complications due to the pathological bowel tissue .
it may , however , be carried out if the lesion has been confirmed as benign in the preoperative period , and can be performed by milking the lesion in a distal to proximal direction , followed by a limited resection [ 15 , 16 ] .
a literature search revealed 13 cases of duodenal / ampullary adenomas causing duodeno - duodenal intussusception , and to the best of our knowledge , no case of an ampullary adenoma causing symptomatic intussusception with recurrent chest infections was previously reported .
incidental duodenal polyps are reported in the literature at a rate of 0.34.6% and have a variety of pathological origins , including adenomas , submucosal tumours and hamartomas amongst others .
ampullary adenomas are thought to be precursors to adenocarcinomas , as such tissue is present in 90% of the resected ampullary adenocarcinoma specimens , whilst based on autopsy studies , they may occur at a rate of 0.040.12% in the general population [ 1 , 2 , 3 ] . due to their location
, ampullary adenomas may clinically manifest via obstruction of the main gastrointestinal tract or the biliary and/or pancreatic ducts .
however , they are usually asymptomatic and found incidentally at endoscopy , whilst some patients may present with symptoms , most commonly gastric outlet obstruction or gastrointestinal bleeding [ 8 , 9 ] .
rarer presenting complaints may include acute pancreatitis , biliary obstruction or , as in this case , intussusception .
depending on the clinical urgency of definitive treatment , such lesions may be further investigated using a wide range of modalities , including ultrasonography , ct , endoscopic ultrasonography ( eus ) , magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography .
eus has proven to be a vital technique in the local assessment of periampullary tumours and has a higher sensitivity to both us and ct when detecting such lesions and assessing muscular ( suggesting invasion , and therefore malignancy ) and vascular involvement ( to assess resectability ) . however , ercp is also extremely useful and has a similar sensitivity to eus in detecting ampullary tumours .
there are three broad management strategies for ampullary adenomas : surveillance , endoscopic resection and surgery .
endoscopic resection may be possible in adenomas with low - grade dysplasia , which are < 1 cm in size and demonstrate benign characteristics at endoscopy .
however , chini and draganov   suggest that they believe endoscopic resection to be an appropriate course of action even in the presence of high - grade dysplasia if the two above criteria are met . the presence of an ampullary adenocarcinoma warrants surgical resection if the candidate is fit .
however , there have been sporadic case reports where such malignancies have been removed endoscopically [ 22 , 23 , 24 , 25 , 26 ] .
however , debate surrounding their management exists , as some groups speculate that the progression from adenoma to carcinoma is a low percentage , whilst others argue that the quality of forceps biopsy via endoscopy may miss occult foci of adenocarcinoma within the adenoma .
asymptomatic patients may be treated with endoscopic management with endoscopic surveillance as follow up ; however , there is no current consensus as to the ideal surveillance post endoscopic resection .
clinicians must ensure that patients who are previously well and present recurrently with chest infections are fully investigated and that they consider the possibility of a lesion causing gastric outlet obstruction .
clinicians should consider dedicated abdominal imaging , and if a mass is identified , undergo endoscopy to further characterise the lesion and develop an on - going management plan with appropriate specialist input .
the index of suspicion should of course be higher if the patient goes on to further develop signs of an obstructing lesion , such as persistent vomiting .
 ampullary adenomas are a rare clinical entity , occurring at a rate of 0.040.12% in the general population . 
 they are premalignant lesions which have the capability to progress to malignancy , and they should be excised if they are causing immediate symptoms and/or are likely to degenerate to carcinoma . 
 intestinal intussusception in adults is rare and , unlike in children , is often due to a structural pathology . 
 intussuscepting duodenal / ampullary adenomas have been reported in the literature on 13 previous occasions , however never before with this presentation . 
 we report the case of a woman who presented with a 1-year history of recurrent chest infections . 
 she was treated with numerous antibiotics , whilst intermittent symptoms of recurrent vomiting and weight loss were initially attributed to her lung infections . 
 a chest ct demonstrated multiple cavitating lung lesions , whilst an obstructing polypoid mass was noted at d2 on dedicated abdominal imaging . 
 due to ongoing nutritional problems , she had a semi - urgent pancreaticoduodenectomy . 
 intraoperative findings demonstrated a large mass at d2 with a duodeno - duodenal intussusception . 
 histological analysis reported a duodenal , ampullary , low - grade tubular adenoma , 75  28  30 mm in size , with intussusception and complete resection margins . 
 the patient recovered well and was discharged on postoperative day 10 , with no complications to date . 
 ampullary adenomas may present with obstruction of the main gastrointestinal tract and/or biliary / pancreatic ducts . 
 common presentations include gastric outlet obstruction , gastrointestinal bleeding or acute pancreatitis . 
 this unique presentation should remind clinicians of the need to investigate recurrent chest infections for a possible gastrointestinal cause . 