Hypophyseal Triplication: Case Report and Embryologic Considerations

Abstract

SUMMARY:Hypophyseal triplication is malformation that has not been described previously. We present a child with midline abnormalities who underwent epignathus excision at birth. Brain MR imaging revealed 2 paired lateral pituitary glands and an oval midline gland, each with an independent stalk, connected to a thickened third ventricle floor. Because malformations represent a failure in embryogenesis, this case may provide interesting clues on the normal development of the hypophysis. Duplication of the pituitary gland is an extremely rare malformation; most patients do not survive beyond infancy because of the severity of associated abnormalities.1 Several cases present epipharyngeal teratoma, midline palatal cleft, or spinal malformations, thus revealing the failure of development of multiple midline structures. [2][3][4] Besides the classic form with 2 complete pituitary glands located in the lateral recesses of the pituitary fossa, the spectrum of hypophyseal malformation includes incomplete duplication affecting the anterior pituitary gland or the pituitary stalk. 5 The floor of the third ventricle usually appears thickened, and this feature has been differently interpreted as a hamartoma or as the result of either the duplication of the hypothalamic nuclei, or the fusion of the mammillary bodies and tuber cinereum into a single midline mass. 1 We report the MR imaging findings of an hypophyseal triplication in a 7-year-old girl with concomitant craniofacial and spinal abnormalities. Because the embryogenesis of the pituitary gland is still controversial, 1,3,4 morphologic and signal intensity characteristics of the 3 pituitary glands have been considered to gain insight into the normal development of the hypophyseal-hypothalamic axis. Case ReportA 7-year-old first child of nonconsanguineous parents, with a normal female karyotype and a known occult spinal dysraphism, was referred to our center for worsening of scoliosis and the onset of micturition.According to her clinical history, she was born at 36 weeks' gestation by cesarean delivery because of a massive oropharyngeal epignathus detected by sonography examination at the fifth month of gestation. At birth, to safely manage airway obstruction, an ex utero intrapartum treatment (EXIT) procedure was performed, which consisted of maintaining placental support until the airway was secured. Fifteen days later, the protruding epignathus teratoma was removed, while a concomitant midline palate and lip cleft was repaired by serial maxillofacial surgery.In our center, the patient underwent an MR imaging examination of the lumbosacral spine and the brain. The spinal MR imaging confirmed sacral spina bifida with butterfly vertebra at S1 and hemispondyls in the inferior part of the sacrum. Moreover, an intradural lipoma tethering the spinal cord to its anterior-superior surface was detected.Brain MR imaging confirmed the midline palatal cleft and disclosed concomitant multiple pituitary glands (Fig 1C). Two clearly showed adenohypophyseal and neurohypophyseal comp...

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

“…This finding has alternately been interpreted as hypothalamic hamartoma or duplication of hypothalamic nuclei. [ 21 ] The association with DPG and precocious puberty has been reported previously and hypothesized to be affected by the accompanying hypothalamic malformations. [ 9 ] In cases where autopsy has been possible, there have been reports of hypothalamic hamartoma and pseudohamartoma.…”

“…One previous report of triplication of the hypophysis exists; the patient displayed a third midline gland with separate stalk that was incompletely developed. [ 21 ]…”

Craniovertebral anomalies associated with pituitary gland duplication

“…In 1 case even a triplication of the pituitary gland was revealed [29]. Some patients with duplication present precocious puberty [11,52,53].…”

“…As regards the brain structures, hypothalamic enlargement was noticed occasionally, as well as tuberomamillary fusion [7,29,34,45,53]. In some patients, hypoplasia, or a partial or total agenesis of the corpus callosum were revealed, then agenesis of the anterior commissure or the septum pellucidum, absence of the olfactory bulb and/or tract, broad optic nerves or chiasm, hydrocephalus or ventricular enlargement, then duplication of the anterior 3rd ventricle, Sylvian aqueduct or mammillary bodies, as well as third cerebral peduncle, hypoplastic or broad pons, hypoplastic cerebellum, clival encephalocoele, Dandy--Walker syndrome, and cleft or partial duplication of the spinal cord [4,5,7,20,23,30,34,37,45,49].…”