RESUMO
ABSTRACT: We report a 16-year-old boy who presented with abdominal pain and severe hyperglycemia. His random blood glucose on admission was above 300 mg/dL, without ketosis. Urine examination revealed mild ketonuria. Renal function tests were normal. Antibodies to glutamate acid decarboxylase (anti-GAD) and islet tyrosine phosphatase 2 (anti-IA2) were negative. Findings on enhanced abdominal computed tomography scan were compatible with complete dorsal pancreatic agenesis. The body and tail of the pancreas were not visualized. Additionally, ectopic and malrotated left kidney was detected. Magnetic resonance cholangiopancreatography confirmed the absence of the dorsal pancreatic duct. The patient was treated with multiple subcutaneous insulin injections and attained good glycemic control. He is currently doing well on follow-up. The present case is a rare combination of complete agenesis of the dorsal pancreas with an ectopic, malrotated kidney. Clinical awareness of this rare association will help improve patient management.