Duplication of the Hypophysis

Abstract

The authors describe an 11-year-old girl with duplication of a pituitary gland and hypertelorism. Of the 14 cases described to date, only 2 others have lacked associated major facial or oral anomalies. Two separate glands, respective stalks and hypothalamic enlargement were incidentally found on MR imaging performed to evaluate the patient’s complaint of anosmia. A tortuous fusiform dilatation of one pericallosal artery was also discovered. A review of hypophyseal embryogenesis is presented. It is now evident that duplication of the pituitary can occur without other major dysmorphias. This may infer that notochord/prochordal plate deficits are not a prerequisite for hypophyseal duplication.

Craniovertebral anomalies associated with pituitary gland duplication

“…Like us, some authors also found a segmental dilatation of the distal anterior cerebral artery, i.e. the pericallosal vessel [7], but we also noticed a dilatation of the extradural segment of both vertebral arteries (Fig. 2).…”

“…duplication, of the basilar artery, as was noticed in some other patients [7,23,44,49]. The latter anomaly seems to be a characteristic features in patients with the pituitary duplication [44].…”

“…2). As regards the congenital cerebrovascular malformations, the arterial aneurysms were not found, but only an arteriovenous malformation was present in 1 patient [7,45].…”

“…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].…”

“…Most of the anomalies of these bony elements occur very infrequently [1-3, 6, 8-10, 12, 15, 17, 19, 24, 25, 27, 28, 35, 36, 38-40, 46, 48, 50, 51, 54, 55]. Duplication of the pituitary gland is an extremely rare event [4,5,7,11,20,23,26,30,34,37,42,44,45,49,52,53]. Because of that, we decided to examine in detail a patient with those two types of the congenital anomalies.…”