RESUMO
@#Prader Willi Syndrome (PWS) includes complex endocrinological issues because of the hypothalamic and pituitary dysfunction which include obesity and diabetes, as well as behavioural issues. Other important aspects of PWS, such as hepatosplenomegaly are sometimes neglected. We present a case of diabetic ketoacidosis precipitated by torsion of a wandering spleen in a 22-year-old woman with PWS and type 2 diabetes mellitus. The pancreatic tail was involved in the torsion leading to hyperamylasaemia and pancreatitis. The splenic torsion and pancreatitis were initially treated conservatively with resolution of symptoms. A year later, she had another 2 episodes of severe abdominal pain due to worsening splenic torsion which subsided with conservative management. She subsequently underwent an elective splenectomy which revealed an enlarged and wandering spleen, with 720 degrees torsion of the long splenic pedicle.