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From question on page 932
Computed tomography scan demonstrated a duodenal mass intussuscepting into the jejunum causing obstruction. The scan showed a dilated duodenum and due to the retroperitoneal fixation of the duodenum, the intussusception appeared as two separate masses at the genu inferius (A in fig 1) and duodenojejunal junction (B in fig 1). The classic radiological presentation of intussusception is the coiled spring appearance; this was poorly visible in the duodenum because it was fixed to the retroperitoneum. As a result, while the leading edge of the intussusception was found at the duodenojejunal junction, part of the intussusception remained above the junction of the first and second part of the duodenum (the genu inferius) often resulting in the appearance of two separated masses.
The patient underwent surgery and the intussusception was reduced. A pancreas preserving duodenectomy (PSD) with resection of the proximal jejunum was then performed. She made an uneventful recovery. Pathology revealed a malignant change in a villous adenoma (fig 2). Despite a strong familial adenomatous polyposis (FAP) family history, no family member had undergone duodenal surveillance.
Duodenal cancer is the leading cause of cancer related death in patients with FAP who have undergone colectomy. Most patients will develop adenomatous duodenal polyps yet only 5% progress to cancer. In order to predict outcome, Spigelman stratified duodenal disease based on number, size, and histopathology. Intervention is recommended in patients with stage IV disease where 36% will develop carcinoma. Intervention for FAP patients with duodenal polyposis has ranged from endoscopic ablation to pancreaticoduodenectomy. Local therapy is associated with recurrences rates of up to 100% and does not alter disease progression. Pancreaticoduodenectomy or PSD both offer definitive therapy in preventing duodenal carcinoma. PSD has been used infrequently for the management of FAP although it offers the potential advantage of preserving the normal pancreas without additional morbidity.
Our patient demonstrated the rare finding of a duodenal intussusception—there are only a few case reports in the literature. More importantly, the case emphasises the fact that all FAP patients should be entered into an upper gastrointestinal surveillance programme.
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