Hepatomegaly in Poorly Controlled Type 1 Diabetes Mellitus: A Case Series of Mauriac Syndrome with Review of Literature

Background: Mauriac syndrome is an uncommon complication of poorly controlled type 1 diabetes mellitus (T1DM), characterized by hepatomegaly, growth retardation, delayed puberty, and Cushingoid features. We report 3 children with Mauriac syndrome and a review of 9 additional Indian cases from the literature. Clinical Description: Case 1: A 12 year-old-boy, diagnosed with type 1 Diabetes Mellitus (T1DM) two years ago, presented with short stature, and was found to be in Tanner Stage 1 with huge hepatomegaly and mild splenomegaly. elevated transaminases, uncontrolled blood sugar and glycosuria without ketonuria. Case 2: A 14-year old boy, diagnosed with T1DM at 8 years of age, having diabetic ketoacidosis a week prior, was referred for poor growth. He had short stature but no pallor, was in Tanner stage 1 with hepatosplenomegaly. Blood sugar was elevated, though liver enzymes and routine work-up were normal. Case 3: A 12 year-old-boy diagnosed with T1DM at 9 years with no significant past illness, but poor adherence to insulin and diet therapy, presented with abdominal distension and growth failure. Examination showed short stature, hepatomegaly, elevated transaminases. Management and Outcome: While Mauriac syndrome was confirmed by liver biopsy in Case 1, the diagnosis was in Case 2 and 3 were made based on the constellation of hepatomegaly, poor glycemic control, short stature, and delayed puberty. All three cases were strictly reinforced on intensified basal-bolus insulin therapy with titration and medical nutrition therapy. Some improvement in HbA1C and growth velocity were seen at 6months, 2 and 3 years, for Case1, 2, 3, respectively. Case 2 and 3 had achieved complete pubertal development at last follow-up. Conclusion: Mauriac syndrome, though rare, continues to be encountered in Indian children with poorly controlled T1DM. Diabetes education and optimized insulin therapy can reverse many features, including hepatomegaly, growth delay, and pubertal arrest. Awareness of this condition avoids misdiagnosis and ensures timely intervention.