Duplication of the pituitary gland - plus syndrome

Last revised by Henry Knipe on 22 Nov 2022

Duplication of the pituitary gland (DPG) is a rare developmental anomaly that may occur in an isolated fashion or in combination with other congenital craniofacial anomalies, hence, the term DPG-plus syndrome.

Duplication of the pituitary gland is a very rare disease with a small number of cases reported in the literature.

In DPG-plus syndrome patients usually manifest with midline craniofacial anomalies such as hypertelorism, cleft lip, and palate, bifid tongue, and nasopharyngeal teratoma. Patients may also present with anosmia, and delayed or precocious puberty.

Various hypotheses have been proposed to explain the syndrome such as splitting of the rostral notochord or prechordal plate at its rostral end during blastogenesis.

  • complete duplication of the pituitary gland with two posterior pituitary bright spots

  • two separate infundibula

  • thickening of the floor of the third ventricle as a result of fusion of the mammillary bodies and tuber cinereum

  • associated findings include callosal agenesis, hypoplastic olfactory bulbs, basilar artery duplication, nasopharyngeal/orophayrngeal teratoma

The duplicated small pituitary gland should be differentiated from

Thickening of the third ventricle floor should be differentiated from hypothalamic hamartoma.

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