Langenbecks Arch Surg (2005) 390:39–41 DOI 10.1007/s00423-004-0516-5
Aljamir D. Chedid Cleber R. P. Kruel Marcio F. Chedid Ronaldo J. S. Torresini Geraldo R. Geyer
Received: 11 May 2004 Accepted: 14 August 2004 Published online: 24 September 2004 Springer-Verlag 2004
A. D. Chedid ()) · C. R. P. Kruel · M. F. Chedid · R. J. S. Torresini · G. R. Geyer Service of General Surgery, Hospital de Clnicas de Porto Alegre, Federal University of Rio Grande do Sul–College of Medicine, Porto Alegre, Brazil e-mail:
[email protected] Tel.: +55-51-33282472 Fax: +55-51-33282472
ORIGINAL ARTICLE
Development of clinical celiac disease after pancreatoduodenectomy: a potential complication of major upper abdominal surgery
Abstract Background: Celiac disease is a gluten-induced disease of global malabsorption. There is a subset of patients with celiac disease who are free of major symptoms but who have typical damage to the intestinal mucosa (silent disease). We present the case of a 50-year-old white woman with no clinical symptoms of celiac disease who developed diarrhea and weight loss 12 weeks after a pancreatoduodenectomy for ampullary cancer. Methods: Microbiological and biochemical examination of the feces did not provide clues useful to diagnosis, and diarrhea was not affected by pancreatic enzyme
Introduction Celiac disease is a syndrome of malabsorption characterized by intolerance to enteral gluten, a protein predominantly found in wheat, barley and rye [1–3]. The prevalence of celiac disease is 1/300 to 1/2,000, depending on the geographic region studied [2, 3]. Nevertheless, a recent study showed that the latent (subclinical) form of the disease might affect as much as 1% of the population, with middle-aged women making up the largest patient group [4]. Celiac disease is associated with human leukocyte antigen DQ2 (HLA-DQ2) in approximately 90% of the patients, with human leukocyte antigen DQ8 (HLA-DQ8) accounting for most of the other patients [1–5]. Diarrhea, abdominal pain, steatorrhea, and weight loss are generally present in the clinical presentation, although in most patients celiac disease appears to be a silent disease [4]. We report on a patient with asymptomatic celiac disease that became clinically manifest after a pylorus-preserving proximal pancreatoduo-
replacement or administration of antiperistaltic drugs. Results: Review of the pathologic specimen and blood tests were compatible with celiac disease. Conclusion: This clinical scenario illustrates that subclinical celiac disease may be an underdiagnosed cause of malabsorption after major upper gastrointestinal surgery and should be considered in the differential diagnosis of diarrhea after pancreatoduodenectomy. Keywords Celiac disease · Malabsorption syndrome · Pancreatoduodenectomy · Ampullary adenocarcinoma
denectomy (PPPD) for adenocarcinoma of the ampulla of Vater.
Case report A 50-year-old woman of Italian origin with an unremarkable previous past medical history underwent a PPPD for a node-negative 2.5 cm ampullary carcinoma. Postoperatively, a pancreatic fistula and gastric stasis were resolved by treatment with octreotide and intravenous erythromycin, respectively, and the patient was discharged on the 31st postoperative day, eating regular food. Twelve weeks postoperatively she returned, complaining of 5–6 bowel movements per day and a 15 kg weight loss; although she was eating regular food, her serum albumin was only 2.8 g/dl. Abdominal computed tomography did not demonstrate tumor recurrence, and the fecal fat excretion was normal. An empirical trial of oral antibiotics for 2 weeks gave no improvement. At the time of an otherwise normal upper gastrointestinal endoscopy, jejunal biopsies revealed severe mucosal atrophy with moderate lymphoid infiltration (Fig. 1a). Serum antiendomysial and antigliadin antibodies were positive for celiac disease. Review of the surgical
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specimen identified atrophy of the duodenal mucosa compatible with celiac disease (Fig. 1b). After 2 months on a gluten-free diet, the patient gained 5 kg and the diarrhea had fully abated. After 18 months the patient had regained all her weight and was in excellent nutritional state (albumin = 4 g/dl). Serum antiendomysial and antigliadin antibodies were negative. Endoscopic jejunal biopsies were normal and showed no evidence of mucosal atrophy or lymphatic infiltration in the intestinal mucosa (Fig. 1c).
Discussion
Fig. 1 Intestinal histology. a Mucosal biopsy 12 weeks after PPPD. Note severe mucosal atrophy with lymphocytic infiltration. Hematoxylin–eosin (100). b Duodenal mucosa in the pancreatoduodenectomy specimen showing villous atrophy with flattening of the mucosal surface. Hematoxylin–eosin (40). c Jejunal biopsy 12 months after PPPD on a gluten-free diet. The mucosal histology is normal. Hematoxylin–eosin (100)
Celiac disease is characterized by intestinal mucosal atrophy, which results in intestinal malabsorption, diarrhea, and weight loss as a consequence of gluten “intolerance.” During the course of the disease, chronic irritation of the intestinal mucosa and duodenal papilla possibly augment the permeability to carcinogen agents, which has been hypothesized to favor the onset of neoplasms [6]. Both Tcell lymphomas and adenocarcinomas of the proximal digestive tract are much more frequent in patients with celiac disease than in the general population [4, 5]. Our patient had no previous clinical evidence of celiac disease but developed diarrhea after undergoing a PPPD for a periampullary neoplasm. We have found only six previous patients that developed clinical and laboratory evidence of celiac disease after pancreatoduodenectomy as treatment for digestive tract carcinomas [7–11]. Only two of those patients presented with ampullary cancers and obstructive jaundice [10, 11]. It is not rare for patients that are undergoing pancreatoduodenectomy to have malabsorption, diarrhea, and malnutrition. Diarrhea occurs commonly in the postoperative period after pancreatoduodenectomy secondary to disruption of normal gastrointestinal motility, absorption, and secretion. Pancreatic insufficiency that results in steatorrhea or recurrence of the neoplastic lesion is a clinical event that leads to diarrhea, malnutrition and weight loss more frequently than celiac disease [10]. Within the setting of diarrhea after PPPD or other major upper abdominal surgery that alters the physiologic processing of ingested food, celiac disease is rarely entertained as a possibility, which may delay or prevent the correct diagnosis [12, 13]. We conclude that celiac disease should be at least considered in the differential diagnosis in patients who, in the postoperative period, present with an intestinal malabsorption syndrome without a definite etiology. Acknowledgment The authors thank Dr Michael G. Sarr from Mayo Clinic Department of Surgery for the review of the manuscript and suggestions.
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