Late-onset pituitary stalk interruption syndrome (PSIS)

Pituitary stalk interruption syndrome is a fairly common cause of the deficiency of Growth Hormone and hypopituitarism often revealed in the neonatal period and childhood. This observation illustrates the peculiarities of a late clinical onset of this syndrome. We report a case of a 17-year-old patient hospitalized for primary amenorrhoea and impuberism. She had no history of neonatal incident. Clinical examination revealed severe growth retardation Hypophysiogramme showed complete hypopituitarism without diabetes insipidus. Magnetic resonance imaging revealed pituitary stalk interruption and an ectopic posterior pituitary gland. Kidney malformation was objectified, which is in favour of a congenital malformative origin of this syndrome. Hormone replacement was administered to this patient. This late-onset form emphasizes the need for early diagnosis of impuberism and/or stunting, revealing a potentially very serious pathology.