Child short stature is defined as a height that is 2 or more standard deviations below the mean for age and gender. Many pathogeneses of short stature were well known, including skeletal dysplasia and hormonal disorders. Such endocrinal pathogenesis due to pituitary growth hormone deficiency may also be familial, syndromic or isolated idiopathic as a result of developmental anomaly, malnutrition and chronic debilitating disease. The pituitary gland (hypophysis) is the magistrate endocrine gland, as it the source of the organizing hormones of the whole endocrine glands in the body. Normally, the gland is located at the sella turcica, below the hypothalamus, which is the overnor of the pituitary gland [1, 2].
The pituitary gland is composed of 2 lobes: the anterior lobe (the adenohypophysis) and the posterior lobe (the neurohypophysis). The adenohypophysis has hypothalamic regulation through a portal system (circulatory), while the neurohypophysis activity has direct neural hypothalamic control. Each lobe has a specific group of different hormones, so pituitary gland deficiency can lead to a wide range of clinical conditions, e.g. hypothyroidism, central diabetes insipidus, or growth retardation. Proportional growth retardation usually has a hormonal cause, such as growth hormone deficiency, called pituitary dwarfism. Unlike skeletal dysplasia which is a disproportionate dwarfism, the hormonal dwarfism may be associated with some mental disability [3, 4].
One of the rare aetiologies of the hormonal dwarfism is the pituitary stalk interruption syndrome (PSIS), which is also known as pituitary stalk transection syndrome. This is a syndrome characterized by a specific triad, an absent or hypoplastic anterior pituitary gland, thin or absent infundibulum, and ectopic posterior pituitary location. This type of pituitary dwarfism is usually presented in the first decade of life and may be first isolated with normal other pituitary hormones. However, deficiencies of other hormones present afterwards, with end stage anterior pan-hypopituitarism, with preservation of posterior pituitary function, regardless it is usually ectopic in location [5, 6].
The aim of this study was to detect the incidence of pituitary stalk interruption syndrome in cases of pituitary dwarfism in our institute and to highlight the magnetic resonance imaging (MRI) findings in such syndrome.