Objective: To describe six cases of possible partial ectopic posterior pituitary gland (PEPP) seen on head magnetic resonance imaging (MRI) and their associated clinical and endocrinological manifestations.
Methods: This is a single-center case series, from a tertiary public university health center in Montreal, Canada. Cases of children with possible PEPP were selected prospectively from 2005 to 2017, based on head MRI findings. Medical history, exam findings and hormonal evaluation were extracted from the medical record, and images were reviewed and interpreted by an experienced pediatric neuro-radiologist.
Results: All the cases, two boys and four girls between 8 days and 14 years old, were characterized by the presence of two midline bright spots on the thin focused T1 weighted sequences obtained with fat suppression technique. While one bright spot was located at the normal expected site of the neurohypophysis in the posterior sella, another was in the midline median eminence or along the normal appearing pituitary stalk above the sella, most likely corresponding to a partial presentation of an ectopic posterior pituitary gland. The possible PEEP was associated with different clinical phenotypes. One patient had isolated growth hormone deficiency, another had combined thyroid stimulating hormone and growth hormone deficiency, while the others had intact pituitary function at their last follow up. Of the remaining four patients, one had CHARGE syndrome, another one had motor developmental delay and one had septo-optic dysplasia without evidence of endocrinopathies to date.
Conclusions: Evaluation of pituitary function may be needed when PEPP is possibly found in the MRI. Long-term follow-up may provide additional information on others pituitary hormone deficiencies.
27 - 29 Sep 2018
European Society for Paediatric Endocrinology