Poorly Controlled Maternal Diabetes and Pregnancy Associated with Fetal Dandy Walker Malformation and Subtotal Amelia: A Case Report and Review of the Literature

Diabetes mellitus is a metabolic condition characterized by chronic hyperglycemia. Its association with pregnancy constitutes a very high-risk situation responsible for embryofetopathy. This diabetic embryofetopathy can be the cause of the occurrence of fetal congenital malformations during the first trimester of pregnancy. These are generally cardiac, digestive, skeletal, and genitourinary malformations. The role of hyperglycemia in the occurrence of congenital anomalies has been clearly established. The specific malformation of dandy walker affecting the central nervous system, grouping a Cyst of the posterior cerebral fossa in communication with the 4th ventricle and agenesis or hypoplasia of the cerebellar varnish, however, is rarely described in pregnant women with pre-existing diabetes. In this article, without confirming the direct link with diabetes mellitus, we report a rare case of Dandy-Walker malformation associated with amelia sub-total in a pregnant diabetic woman. The pregnancy occurred in the context of poor glycemic control and the absence of pre-conception diabetological preparation.