Agenesis of the Left Internal Carotid Artery and Congenital Pituitary Aplasia

Less than 0.01% of the overall society worldwide has been recorded to have internal carotid artery (ICA) agenesis, making it a remarkably uncommon embryonic vascular defect. Pituitary aplasia is another uncommon congenital disorder. In this article, we describe an incredibly rare pediatric case that had the development of one internal carotid artery and aplasia of the pituitary. The patient is a 2-and-a-half-year-old boy with dysmorphic features, such as frontal bossing, deep-seated eyes, low-set ears, depressed nasal bridge, head lags, and micropenis. He suffered generalized severe hypoglycemia and hypothyroidism on day 1 of life. He was hospitalized at our hospital for a workup and an MRI (Magnetic resonance image) of the brain to check the pituitary gland. His left internal carotid artery and carotid canal were missing, and the pituitary and Sella turcica revealed aplasia on MRI. A diagnosis of congenital panhypopituitarism coupled with agenesis of ICA was made. Here we present the first incidence of a verified complete pituitary aplasia in a patient with ICA agenesis. In the case of Congenital pituitary aplasia/hypoplasia, our experience indicates any vascular anomalies should be thoroughly evaluated. The formation of collateral circulation in unilateral ICA agenesis cases to complement the circulation and the presence of hormonal replacement therapy help such patients massively. It is not uncommon for unilateral ICA agenesis to remain asymptomatic because of the formation of collateral circulation that obtains the proper blood supply.