Duplication of the pituitary gland associated with multiple blastogenesis defects: Duplication of the pituitary gland (DPG)-plus syndrome. Case report and review of literature

Abstract

Background:Duplication of the pituitary gland (DPG) is a rare craniofacial developmental anomaly occurring during blastogenesis with postulated etiology such as incomplete twinning, teratogens, median cleft face syndrome or splitting of the notochord. The complex craniocaudal spectrum of blastogenesis defects associated with DPG is examined with an illustrative case.Case Description:We report for the first time in the medical literature some unique associations with DPG, such as a clival encephalocele, third cerebral peduncle, duplicate odontoid process and a double tongue with independent volitional control. This patient also has the previously reported common associations such as duplicated sella, cleft palate, hypertelorism, callosal agenesis, hypothalamic enlargement, nasopharyngeal teratoma, fenestrated basilar artery and supernumerary teeth. This study also reviews 37 cases of DPG identified through MEDLINE literature search from 1880 to 2011. It provides a detailed analysis of the current case through physical examination and imaging.Conclusion:The authors propose that the developmental deformities associated with duplication of pituitary gland (DPG) occur as part of a developmental continuum, not as chance associations. Considering the fact that DPG is uniquely and certainly present throughout the spectrum of these blastogenesis defects, we suggest the term DPG-plus syndrome.

Duplication of the pituitary gland - plus syndrome

“…DPG is a very rare developmental anomaly with only 40 cases reported till 2012. [ 1 2 3 ] It has been reported in both the pediatric and adult populations. Most patients are females.…”

“…Most patients are females. [ 1 2 3 ] DPG is usually detected in unsuspected patients on imaging due to the associated midline craniofacial anomalies. [ 4 ] The rarity of this entity may hence be partially attributable to incidental detection on imaging and early mortality in some patients.…”

“…[ 1 2 3 ] With DPG as the common denominator, a large number of associated anomalies have been reported - the DPG-plus syndrome. [ 1 ] In this article, we report a case of pituitary duplication that was associated with duplication of the sella, hypertelorism, cleft palate, a large craniopharyngeal canal and oropharyngeal teratoma, hypoplastic olfactory bulbs and tracts, duplication of the basilar artery, and cervical anterior cleft vertebrae. DPG with this constellation of associated anomalies in the same patient has, to the best of our knowledge, not been reported previously.…”

Endoscopic Resection of Skull Base Teratoma in Klippel-Feil Syndrome through Use of Combined Ultrasonic and Bipolar Diathermy Platforms

“…We identified approximately 41 documented cases of pituitary duplication in our literature analysis. Of these reports, less than half of cases are associated with the presence of a skull base teratoma [14–16]. Common anomalies associated with pituitary gland duplication include vertebral anomalies and cleft palate [16], both of which are present in this patient.…”

Magnetic resonance imaging of sellar and juxtasellar abnormalities in the paediatric population: an imaging review

“…MRI findings include duplicated pituitary stalks and glands within separate pituitary fossa in the sphenoid bone. Abnormal thickening, “pseudohamartoma”, of the hypothalamus with duplication of the infundibular recess of the third ventricle is often seen [ 16 , 18 ]. Pituitary duplication can also be associated with duplication of the basilar artery [ 19 ].…”