[Duodenal duplication revealed by acute pancreatitis: endoscopic treatment]. / Duplication duodénale révélée par une pancréatite aiguë: traitement endoscopique.

We report the case of a 17 year old man who presented with several episodes of acute pancreatitis due to a duodenal duplication. This was successfully treated by an incision by sphincterotome during interventional duodenoscopy. The patient is symptom free without recurrence 20 months after endoscopic treatment.

[A probable case of alveolar echinococcosis in Normandy, France]. / Un cas probable d'échinococcose alvéolaire en Normandie.

Echinococcus multilocularis induced liver infection was diagnosed in a Moroccan patient. Diagnosis was based on CT scan results and Western Blot test. Contamination probably occurred in France, in the Cherbourg area where the patient travelled frequently and ate wild berries. This case and other recently reported cases outside the usual endemic areas (Besançon and the Massif Central) suggest that the Echinococcus multilocularis epidemic has moved towards the west of France. French gastroenterologists should be aware of this parasitic disease.

Hepatobiliary and Pancreatic neoplasms in patients with McCune-Albright syndrome.

BACKGROUND: McCune-Albright syndrome (MAS), which includes polycystic fibrous dysplasia, precocious puberty, and café au lait spots, is a rare disorder caused by somatic activating mutations of the GNAS gene. GNAS mutations have also been implicated in various sporadic tumors, including hepatobiliary and pancreatic neoplasms. AIM: The aim of this study was to assess the prevalence of hepatobiliary and pancreatic neoplasms in patients with McCune-Albright syndrome. PATIENTS AND METHODS: Nineteen patients diagnosed between 1995 and 2012 with MAS in a tertiary referral center for rare growth disorders were screened with dedicated gadolinium-enhanced magnetic resonance imaging for hepatobiliary and pancreatic neoplasms between June 2011 and December 2012. RESULTS: Six (32%) of the 19 screened patients were found to have hepatic, pancreatic, or biliary lesions, excluding liver hemangiomas, liver cysts, and focal nodular hyperplasia. This includes pancreatic ductal lesions observed in 4 patients, including numerous branch-duct intraductal papillary mucinous neoplasms in 3 patients. Biliary lesions were observed in 1 patient, with a large choledochal cyst also involving the left biliary branch. Finally, multiple inflammatory/telangiectatic hepatic adenomas were observed in 2 patients, including 1 with proven somatic GNAS mutation. CONCLUSION: We describe the first observation of syndromic intraductal papillary mucinous neoplasms and the new association between MAS and pancreatic neoplasms, namely intraductal papillary mucinous neoplasms of the pancreas but also rare hepatobiliary neoplasms including liver adenomas and choledochal cysts. These findings strongly suggest that somatic activating GNAS mutations, possibly through cAMP pathway disorders, are involved in the tumorigenesis of hepatobiliary and pancreatic tissues originating from the foregut endoderm and have led us to use a routine screening by dedicated magnetic resonance imaging including both pancreatobiliary and liver sequences in patients with MAS.

Treatment of chronic portosystemic encephalopathy in cirrhotic patients by embolization of portosystemic shunts.

UNLABELLED: AIMS/BACKGROUNDS: Large spontaneous portal-systemic shunts can occasionally be the cause of chronic and disabling encephalopathy in cirrhotic patients. Shunt embolization has been proposed, however the clinical relevance of this technique remains uncertain. METHODS/RESULTS: We report our results in seven patients treated by shunt embolization. Although the procedure was achieved and technically successful in all patients, the clinical results were poor as long-term improvement was obtained in only one patient. Three patients died within 3 months after the procedure from cirrhoses' end stage complications. CONCLUSION: We believe that optimal management of these patients with chronic spontaneous encephalopathy is liver transplantation.

Percutaneous radiofrequency ablation for hepatocellular carcinoma before liver transplantation: a prospective study with histopathologic comparison.

OBJECTIVE: The aims of this study were to determine the feasibility and efficacy of percutaneous radiofrequency ablation in patients with hepatocellular carcinoma waiting for liver transplantation and to compare the radiologic and pathologic findings. SUBJECTS AND METHODS: Twenty-six patients with 35 hepatocellular carcinomas were addressed for transplantation. Complications of the procedures were recorded. Primary and secondary technique effectiveness and causes of exclusion from the waiting list were assessed. After transplantation, tumor recurrence was evaluated for at least 1 year in all patients. Radiologic-pathologic comparison of the explant was performed. RESULTS: Percutaneous radiofrequency ablation was performed in 21 (81%) patients for 28 tumors. Both minor and major complications occurred in three patients (10% each per session). The rates of primary technique effectiveness, secondary technique effectiveness for percutaneous radiofrequency ablation alone (seven tumors), and combined percutaneous radiofrequency ablation and transcatheter arterial chemoembolization (three tumors) were 56%, 76%, and 86%, respectively. After a mean follow-up of 11.9 months, 16 patients (76%) received transplants, whereas five patients were excluded from the waiting list because of distant tumor progression (n =3, 14%) or other causes (n = 2, 10%). After transplantation, tumor recurred in one (6%) of 16 patients. Histopathologic examinations were performed for 13 (81%) of 16 patients and showed complete necrosis and satellite nodules in, respectively, 12 (75%) and seven (44%) of 16 tumors. CONCLUSION: Percutaneous radiofrequency ablation can be performed on hepatocellular carcinoma patients waiting for transplantation, allows most patients to undergo transplantation, and does not impair posttransplantation outcomes. The procedure produces complete necrosis of the treated tumor in most cases but is associated with a high rate of satellite nodules.

Focal nodular hyperplasia inducing hepatic vein obstruction.

OBJECTIVE: The records of 10 patients with focal nodular hyperplasia inducing intrahepatic vein obstruction were reviewed. The purpose of this study was to describe and emphasize the imaging features of these findings. CONCLUSION: Focal nodular hyperplasia may be responsible for hepatic vein obstruction with hepatic vein collaterals. The relatively large size and central location of the lesions seem to play important roles in the obstruction of the hepatic veins.

Recurrence of pulmonary emphysema in an alpha-1 proteinase inhibitor-deficient lung transplant recipient.

Several types of primary disease may recur after lung transplantation, but recurrence of pulmonary emphysema has so far never been published. We report the case of a 49-year-old white male who underwent single lung transplantation for emphysema related to alpha-1 antitrypsin deficiency and to superimposed smoking. The postoperative course was complicated by several rejection episodes. Subsequently, the patient remained stable without evidence of graft dysfunction for more than 10 years, but he resumed light smoking at 8 years after transplant. At 11 years after transplant, although the patient was still asymptomatic and had a stable lung function, recurrence of emphysema on the grafted side was diagnosed on computerized tomography of the thorax. One year later, the patient began to experience a moderate decline in lung function. Two separate bronchoalveolar lavages performed after the onset of the recurrence disclosed a significant elastolytic activity related to neutrophil serine-elastase in lavage fluid. In summary, we describe a case of recurrence of pulmonary emphysema in a patient with alpha-1 antitrypsin deficiency. The resumption of smoking has probably played a central role in the presence of elastolytic activity in lavage fluid and in the recurrence of emphysema.