Abstract
Duplication of the pituitary gland (DPG)-plus syndrome is a very rare developmental disorder with few cases described in the literature and characterized by multiple midline and central nervous system malformations. The hypothalamus and hypophysis involvement may be clinically associated with endocrine abnormalities. A 5.9-year-old female child was admitted to our Clinic for premature thelarche and acceleration of growth. DPG-plus syndrome with paired infundibula and pituitary glands was diagnosed after birth, when she appeared small for gestational age and she presented with lingual hypoplasia, cleft palate, right choanal stenosis, nasopharyngeal teratoma, and facial dysmorphisms. Neuroimaging revealed a duplication of the infundibula, the pituitary gland, and the dens of the epistropheus despite surgical removal of a rhino-pharyngeal mass performed at the age of two months. An array-CGH revealed a 2p12 deletion. At our evaluation, bone age assessment resulted advanced and initial pubertal activation was confirmed by Gonadotropin-Releasing Hormone stimulation test. Hormonal suppression treatment was started with satisfactory results. This case shows that DPG-plus syndrome must be considered in presence of midline and craniofacial malformations and endocrinological evaluations should be performed for the prompt and appropriate management of pubertal anomalies.
Highlights
Duplication of the pituitary gland (DPG) is an extremely rare developmental anomaly [1]
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]
Summary
Duplication of the pituitary gland (DPG) is an extremely rare developmental anomaly [1]. A 5-year-old female was attended to the Pediatric Endocrinology Unit of Pediatric Clinic in Children’s Hospital of University of Parma due to onset of premature thelarche and acceleration of growth rate In her medical history, it was reported that she was born by emergency caesarean delivery due to placental defect after regular full-term pregnancy, the birth weight was 2,085 g (
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