Unique Presentation of Duplication of the Pituitary Gland-Plus Syndrome

Abstract

Objective: To shed light on a unique presentation of duplication of the pituitary gland (DPG)-plus syndrome. Methods: We present the case report gathered from the patient. Results: This patient uniquely had DPG-plus syndrome with a concurrent dermoid cyst. Conclusion: DPG is a very rare developmental anomaly and there are not many case reports published in the literature. The presence of DPG with dermoid cyst in our patient has not been reported previously. This case report discusses the various presentations of the syndrome and also provides an overview of investigations and management of these patients.

Duplication of the Pituitary Gland (DPG)-Plus Syndrome Associated With Midline Anomalies and Precocious Puberty: A Case Report and Review of the Literature

“…Therefore, the characteristic thickening of the floor of the third ventricle extending from the median eminence to the mammillary bodies, which is easily visible on midline sagittal MR image of our patient, might be related to duplication of that hypothalamic nuclei. This occurrence could explain the appearance of the endocrine alterations later in life ( 2 ).…”

“…DPG-plus syndrome is a rare condition with only 52 cases described worldwide and few MRI studies reported ( 1 , 2 ). The rarity of this entity may hence be partially attributable to incidental detection on imaging and early mortality in some patients.…”

“…reported an adult male with DPG-plus syndrome presenting with hypogonadotropic hypogonadism and delayed sexual development who underwent hormonal therapy with testosterone. Baseline values revealed a normal thyroid function ( 2 ). Although a CT of the central nervous system is the standard of care when a neonate is born with midline and craniofacial abnormalities, DPG-plus syndrome is rare and our case report is useful for future medical treatment.…”

“…It may be associated with other midline malformations such as facial anomalies (median cleft lip, median cleft face syndromes, and hypertelorism), vertebral malformations, nasopharyngeal teratoma, and other central nervous system abnormalities, such as the agenesis of the corpus callosum, posterior fossa abnormalities, the absence of the olfactory bulbs and tracts, the absence of the anterior commissure, and anatomic variations of the circle of Willis. When all these anomalies are present, they are called DPG-plus syndrome ( 1 , 2 ). DPG-plus syndrome is more prevalent in females and it is probably due to the splitting of the rostral notochord and prechordal plate during blastogenesis ( 2 ).…”

“…When all these anomalies are present, they are called DPG-plus syndrome ( 1 , 2 ). DPG-plus syndrome is more prevalent in females and it is probably due to the splitting of the rostral notochord and prechordal plate during blastogenesis ( 2 ). DPG-plus syndrome can lead to endocrine abnormalities, mainly affecting the gonadotropic axis ( 3 , 4 ).…”