Duplication of the pituitary gland-plus syndrome associated with precocious puberty: Case report

Abstract

Duplication of the pituitary gland (DPG) is an extremely rare embryonic developmental anomaly that can occur in association with midline malformations and hypothalamic-pituitary axis disorders, such as central precocious puberty (CPP). The aim of this report is to describe the management and long-term clinical course of a patient with this condition. This is a female patient with a neonatal history of complex maxillofacial malformation (mandibular duplication, cleft palate, and palatal teratoma). She was referred at 7 years and 6 months of age due to thelarche, showing signs of puberty on physical examination. Hormonal studies and gynecological ultrasound confirmed activation of the gonadotropic axis, leading to diagnosis of CPP. A Central Nervous System magnetic resonance imaging (MRI) scan was performed, revealing complete duplication of the pituitary gland, hypoplasia of the corpus callosum, and a hypothalamic hamartoma. She received treatment with GnRH analogs between the ages of 8 and 12 with a good clinical outcome. Menarche occurred at age 17, and she reached a final height of 155 cm (-0.9 SDS). Other pituitary axis remained normal during follow-up. DPG is a marker of complex midline anomalies. In cases of maxillofacial malformations and midline abnormalities, neuroimaging is essential to detect pituitary anatomical variants. Timely diagnosis and treatment of CPP in these cases allows for adequate pubertal development and preservation of final height potential.