Necrotizing Pancreatitis in a Patient with Gardnerʼs Variant Familial Adenomatous Polyposis

Abstract

Purpose: To evaluate the etiology of necrotizing pancreatitis in a patient with Gardner's variant familial adenomatous polyposis (FAP). Review of patient records and literature review with OVID and PubMED. Patient was a 62-year-old male with Gardner's syndrome status-post colectomy and subsequent secondary proctectomy with ileostomy secondary to anastomatic tumor. Patient had high-grade dysplasia of papilla on prior EGD and was undergoing scheduled EGD as part of surveillance. After the endoscopy, the patient was discharged but within 10 minutes of discharge complained of severe pain and a stat CT showed no perforation but some stranding of pancreas. He was hemodynamically stable but labs were significant for WBC 21.04 (16.4 neutrophils, 0.13 bands), amylase 2,312, and lipase 4,477. Other labs were normal. Patient was admitted with pancreatitis and resuscitated aggressively. He was started on empiric antibiotic therapy and re-imaging revealed necrotizing pancreatitis. He continued to deteriorate with development of ARDS and sepsis. Patient was taken for ex-lap 29 days after the EGD with removal of 1.8 L of necrotic fluid. He succumbed to the complications and died 31 days after EGD. Review of literature revealed one other case of iatrogenic necrotizing pancreatitis in a patient with FAP.1 In a case similar to this, patient developed necrotizing pancreatitis after surveillance EGD. Even though the exact incidence of pancreatitis after biopsy is unknown, there appears to be an increased risk of pancreatitis in patients with FAP, even without endoscopic intervention. Classically, this has been attributed to development of obstructive ampullary adenomas (though the incidence of this is also rare). One retrospective review studied eight patients with Gardner's variant FAP who developed pancreatitis.2 Five of eight patients had obstructing focal or diffuse adenomatous disease involving the ampulla and the others had no identifiable cause. Finally, one case series suggests that patients with FAP are at increased risk of pancreatitis regardless of obstruction of ampulla.3 This series reviewed seven patients with FAP who experienced one or more episodes of pancreatitis. None of these patients had obstruction of the ampulla. Two of the seven patients developed pancreatitis after endoscopic treatment of ampullary adenoma, while the etiology of pancreatitis in the other five could not be determined. This suggests that pancreatitis may be a extra-colonic manifestation of FAP through an unknown mechanism. However, despite the risks of iatrogenic pancreatitis (with biopsy or with ampullary lesion) the risk of malignancy supports continued surveillance both ethically and clinically. This is the second case FAP in the literature to have fatal outcome after biopsy of papilla.