Case Report. Duplication of Pituitary Gland

Abstract

We present a case of duplication of the pituitary gland with associated clefts of the hard palate and body of the sphenoid and a midline palatine dermoid. This is an extremely rare malformation with only six previous case reports in the literature. Imaging findings are discussed and literature reviewed for possible morphogenesis, including its association with partial twinning and midline cleft face syndrome.

Craniovertebral anomalies associated with pituitary gland duplication

“…Within the skull, duplication of the sella was noticed, or a broad sella with 2 hypophyseal fossae (like in our patient), or cleft of the body of the sphenoid or basi-sphenoid [5,41,42,45]. We also noticed in our patient a very expressed inner relief of the skull bones, which is like "gyral impressions" in shape.…”

“…According to some reports, from 1880 until 2011 only 38 such patients were reported [30]. The pituitary duplication can be associated with certain face abnormalities: a bifid or double tongue, cleft lips or palate, macrostomia, supernumerary teeth, micrognathia or retrognathia, palatine dermoid, epignathus, pharyngeal teratoma, hypertelorism, broad forehead, and an altered shape of the auricle [5,20,23,26,42,45,49,52].…”

“…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.…”

Duplication of the Hypophysis

“…In 2 other instances [6, 11], there was a hormonal therapy involved in the first trimester of pregnancy to induce menstruation. Finally, in 1 case [12], the mother was a smoker who received cortisone injections and naproxen for psoriatic arthritis during the first trimester. All of the exposures documented in the authors’ reports occurred later than one would expect to have an effect on early embryologic differentiation of the pituitary gland.…”

Duplication of the hypophysis associated with precocious puberty: presentation of two cases and review of pituitary embryogenesis

“…ITUITARY DUPLICATION IS A rare malformation, reported previously in approximately 23 patients (1)(2)(3)(4)(5)(6)(7)(8), three of them presenting with precocious or delayed puberty (2,6,8). Most of the cases are associated with other major neural/craniofacial anomalies which are easily shown by magnetic resonance imaging (MRI) and the majority of patients do not survive beyond infancy.…”