Clinical analysis of 5 cases of pituitary stalk interruption syndrome and literature review / 中华内分泌代谢杂志

ABSTRACT

Objective To analyze the clinical characteristics of the patients with pituitary stalk interruption syndrome (PSIS) in our hospital in the past seven years, and to achieve better comprehension of this kind of disease. Methods Five patients with PSIS (4 males, 1 female) in our hospital were retrospectively analyzed, regarding clinical manifestation, laboratory and imaging data. Results The clinical features of 5 cases of PSIS were as follows (1) growth retardation; (2) high incidence of breech delivery; (3) 159-165 cm in body height; (4) retarded bone age; (5) average intelligence; (6) adenohypophyseal dysfunction in clinical and laboratory examinations; (7) normal posterior pituitary function; (8) MRI with gadolinium contrast revealed no pituitary stalk and anterior pituitary hypoplasia with ectopic posterior pituitary. The pathogenesis of PSIS is not clear. Appropriate hormonal replacement therapy is the only effective way. Conclusion PSIS is rarely seen and its clinical feature is different from pituitary dwarfism and hypogonadotropic hypogonadism. The realization for this syndrome should be emphasized and early therapy is essential.