Abstract

Congenital hypopituitarism (CH) occurs in 1 in 100 000 live births.1 Genetic factors, hypoperfusion, and environmental changes in utero may play causative roles.2 Maternal diabetes mellitus (DM) in relation to other congenital central nervous system abnormalities such as neural tube defects (NTDs) and hydrocephalus have been described, but an association between hyperglycemia in utero and pituitary formation has, to our knowledge, not been reported.3 We present 2 infants with CH, both born to mothers with hyperglycemia in early pregnancy. Both infants' pituitary glands are structurally identical; each has a hypoplastic anterior pituitary, an ectopic posterior pituitary located at the base of the third ventricle, and an absent pituitary stalk.

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