Duplication of the pituitary gland-plus syndrome

Case contributed by Jeffrey B Peeke

Presentation

Preoperative planning evaluation for surgical correction of a persistent craniopharyngeal canal. History of cleft palate repaired in early childhood.

Patient Data

Age: 25 years
Gender: Female

CASE OF THE MONTH: This case was selected as the Case of the Month for October 2022.

CT images demonstrating duplication of the dens and sella, as well as fusion of the C2-C4 vertebrae. The known cleft palate is also visible with a widened midface and hypertelorism. A persistent craniopharyngeal canal is seen.

MR images showing duplication of the pituitary gland and infundibulum. In addition, the septum pellucidum is absent and there is splaying of the cerebral peduncles, as well as widening of the optic chiasm.

Case Discussion

Duplication of the pituitary gland-plus syndrome (DPG-plus syndrome) is a very rare syndrome thought to occur as a result of blastogenic defects. DPG-plus syndrome presents with several midline anomalies, such as duplications of the dens, sella turcica, infundibulum, basilar artery, and pituitary gland. Other notable features include hypoplasia of the corpus callosum, cleft palate, nasopharyngeal teratomas, widening of the optic chiasm, and hypertelorism. 

This case highlights the principle of "the face predicting the brain," reminding the reader to evaluate the intracranial structures when encountering a midline facial abnormality.

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