Case report on Mauriac syndrome-revisiting a rare complication of a common condition

Abstract

Mauriac syndrome is a rare complication of poorly controlled type 1 diabetes mellitus, characterized by growth retardation, hepatomegaly, delayed puberty, and cushingoid features. We describe a 9-year-old girl with long-standing diabetes and poor glycemic control who presented with short stature, abdominal distension and dyslipidemia, with imaging findings suggestive of glycogen accumulation. These features established the diagnosis of Mauriac syndrome; a condition infrequently encountered today due to advances in insulin therapy. However, the case underscores that this syndrome still occurs in settings with limited resources and suboptimal treatment adherence. Early recognition is crucial, as improved glycemic control can reverse most clinical and biochemical abnormalities. This case report emphasizes the need for vigilance in diabetic children with growth failure or delayed puberty, and highlights the importance of education, monitoring, and multidisciplinary care in prevention of complications.