Duplication of the pituitary gland (DPG-plus) syndrome

Case contributed by Abbas M. Mahmood
Diagnosis certain

Presentation

Precocious puberty

Patient Data

Age: 7 years
Gender: Female

Complete duplication of the pituitary gland with two posterior pituitary bright spots and paired infundibula, located in two separated infundibular recesses. There is thickening of the floor of the third ventricle as a result of the fusion of the mammillary bodies and the tuber cinereum associated with early division/duplication of the upper basilar artery (best seen on axial T2). 

Duplication of the dens is also suggested on the sagittal images. 

Features are of DPG-plus syndrome (duplication of the pituitary gland).

Case Discussion

A consultant pediatrician referred this case to our department as the child suffered from signs of precocious puberty

Imaging demonstrated features of duplication of the pituitary gland (DPG plus syndrome), an extremely rare developmental anomaly that may be associated with other midline or CNS malformations, in this case, duplication of the basilar artery and probably also of the dens.

It is more prevalent in females and it is probably due to the splitting of the rostral notochord and prechordal plate during blastogenesis & can lead to endocrine abnormalities, mainly affecting the gonadotropic axis.

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