DPG-plus syndrome: new report of a rare entity

Abstract

Pituitary gland duplication is a particularly rare finding. Different theories have been proposed to explain its pathogenesis, however, this phenomenon is not yet totally understood. Recently, duplication of the pituitary gland (DPG)-plus syndrome has been described, associating DPG with other blastogenic defects. We present the clinical and imaging findings of a newborn girl with DPG, associated with multiple other midline anomalies, including a nasopharyngeal teratoma, palate cleft deformity, bifid nasal bridge, tongue and uvula, hypoplasia of the basis pontis and corpus callosum, duplication of the basilar artery and hypothalamic hamartoma. We describe our patient's multidisciplinary team approach and emphasise the importance of reporting upcoming cases, in order to give more insight into the understanding of this complex entity.

Figure 1

Coronal CT image demonstrating a nasopharyngeal teratoma (arrow) with a tooth-like morphology, extending through a large defect in the hard palate.

Figure 2

Sagittal 3-D FIESTA MR image showing a pituitary gland and stalk (box) on the right and left of the midline. Note that these structures are absent in the midline.

Figure 3

Coronal T2-weighted MR image demonstrating a thickening of the floor of the third ventricle, suggestive of a sessile hypothalamic hamartoma (left), and basilar artery duplication (right).