Celiac disease (CD) is a common genetic autoimmune disease, triggered by gluten sensitivity. CD can present at any age in both children and adults after consuming gluten containing diet. Presentation of CD varies widely. There is paucity of data regarding emergency presentation of CD in the literature. Severe abdominal pain and gastric obstructions have been reported, but there are no reports of spontaneous duodenal perforation as of CD presentation. We searched the Mayo Clinic electronic medical records, between 1997-2011 for any cases of association between celiac disease and duodenal perforation. We identified two patients (7 yo M, 31 yo F). First patient presented with gastric obstruction due to duodenal perforation. Gastroduodenostomy was performed and elevated celiac serology few years after his initial presentation lead to the diagnosis of CD. Second patient had sudden acute RUQ abdominal pain and was found to have sign of visceral perforation on abdominal CT. Evaluation for anemia and rectal bleeding few years later showed positive CD markers that was confirmed by histology. Spontaneous duodenal perforation can be the presenting sign of CD. Celiac serology testing may be considered in patients presenting with spontaneous duodenal perforation.

A 7-year-old boy presents with a history of recurrent pancreatitis. His abdominal pain started in 2007, but his first episode of pancreatitis was in January 2010. Since that time has had at least five known episodes of pancreatitis with mild elevation of his amylase and lipase (range: 200 -300). There is no history of abdominal trauma and he had negative genetic testing for the hereditary pancreatitis (CFTR, PRSS1, and SPINK1 gene mutations). He had numerous imaging studies revealing a persistent cystic lesion near the pancreatic head, since his first episode of pancreatitis. Computed tomography of the abdomen showed thick-walled U-shaped cystic structure lying between the head of the pancreas, the inferior gastric antrum and the proximal descending duodenum. An abdominal US demonstrated a bi-lobed thick-walled cystic mass with a positive rim sign. Recurrent pancreatitis is uncommon in pediatric patients. Causes of recurrent pancreatitis in pediatrics are different than those in adults. While hereditary pancreatitis is one of the causes, the genetic testing was negative and there was no sign of pancreatic injury or inflammation in any of the images. Pancreatic pseudocyst is a complication of acute pancreatitis and usually has a thin wall instead of a thick wall, and it's unlikely to be present during the first episode of pancreatitis. Foregut duplication cyst is a rare (about 1 in 4,500) congenital anomaly that can happen anywhere throughout the GI tract. The US finding of a rim sign is a typical appearance of a duplication cyst and usually is managed by surgical resection. Even though foregut duplication is a rare congenital anomaly, it should always be considered in the differential diagnosis of cases of chronic pancreatis, especially with the presence of thick walled cystic formation on imaging studies.