Insulinoma in tuberous sclerosis: An entity not to be missed.

Pancreatic neuroendocrine tumors are rare with an incident rate of 5 cases per million individuals. Tuberous sclerosis complex is an autosomal dominant disease. This disease involves multisystem and occurs in one out of every 6,000-10,000 individuals. In this study, we describe a 47-year-old male known tuberous sclerosis patient with an insulinoma. The tumor was incidentally detected in follow-up imaging for a previous ampulla of Vater tubular adenoma. However, the patient reported symptoms of hypoglycemia. The insulinoma was enucleated successfully. Histopathology revealed a well-differentiated, grade one neuroendocrine tumor measuring around 2 cm in diameter. Seven cases were reported in the literature of tuberous sclerosis-associated insulinoma. The 7 reported cases had different hypoglycemia related symptoms. The reported tumors varied in size and location on the pancreas. This paper details the eighth case worldwide where an insulinoma occurred in a tuberous sclerosis patient.

Hepaticojejunostomy for the management of sump syndrome arising from choledochoduodenostomy in a patient who underwent bariatric Roux-en-Y gastric bypass: A case report.

INTRODUCTION: Rapid weight loss following bariatric surgery is associated with high incidence of gallstones and complications that may need bilioenteric diversion. This presents a specific challenge in the management of this group of patients. CASE PRESENTATION: A 37 years old female underwent a Roux-en-Y gastric bypass (RYGB) in 2008 for morbid obesity. In 2009 she presented with obstructive jaundice and was diagnosed with choledocholithiasis successfully managed by open cholecystectomy and choledochoduodenostomy. In the following years, she developed recurrent attacks of fever, chills, jaundice, and right upper quadrant pain and her weight loss was not satisfactory. Imaging of the liver showed multiple cholangitic abscesses. Reflux at the choledochoduodenostomy site was suggestive of sump syndrome as a cause of her recurrent cholangitis and a definitive surgical treatment was indicated. Intraoperative findings confirmed sump at the choledochoduodenostomy site and also revealed the presence of a large superficial accessory duct arising from segment four of the liver with separate drainage into the duodenum distal to the choledochoduodenostomy site. A formal hepaticojejunostomy was done after ductoplasty. The Roux limb was created by transecting the jejunum 40cm distal to the foot anastomosis of the RYGB. The gastric limb was lengthened as part of this procedure which afforded the patient the additional benefit of weight loss. CONCLUSION: Choledochoduodenostomy should be avoided in patients with RYGB due to the risk of sump syndrome which requires conversion to a formal hepaticojejunostomy.