Clinical manifestations of hypothalamic-pituitary Langerhans’cell histiocyto sis are commonly, diabetes insipidus and sometimes growth hormone deficiency. Th eir morphologic characteristics on magnetic resonance imag...Clinical manifestations of hypothalamic-pituitary Langerhans’cell histiocyto sis are commonly, diabetes insipidus and sometimes growth hormone deficiency. Th eir morphologic characteristics on magnetic resonance imaging are absence of posterior pituitary hyperintensity and thickening of the pituitary stalk. Pituitary stalk transection is characterized on magnetic resonance imagin g by the absence of pituitary stalk visibility, hypoplasia of the anterior hypop hysis and ectopic posterior pituitary hyperintense signal. This syndrome has bee n shown to be associated with either isolated growth hormone deficiency or multi ple anterior pituitary hormone deficiency, but normal posterior pituitary functi on. Case report.We report our experience with a six-year-old boy who had been treated for three years for a multisystem Langerhans’cell histiocytosis with di abetes insipidus and who was admitted because of short stature. Endocrinological examinations demonstrated a profound growth hormone deficiency and a partial ce ntral hypocorticism. Magnetic resonance imaging showed pituitary stalk transecti on and a midline anomaly of the brain (Arnold Chiari type I malformation). Concl usion. Although, some events of his perinatal history lead to the hypothesis of a malformative origin, the progression of Langerhans’cell histiocytosis affecte d tissues to fibrosis, suggest that this disease is the cause of the patient’s hypothalamohypophyseal lesions.展开更多
文摘Clinical manifestations of hypothalamic-pituitary Langerhans’cell histiocyto sis are commonly, diabetes insipidus and sometimes growth hormone deficiency. Th eir morphologic characteristics on magnetic resonance imaging are absence of posterior pituitary hyperintensity and thickening of the pituitary stalk. Pituitary stalk transection is characterized on magnetic resonance imagin g by the absence of pituitary stalk visibility, hypoplasia of the anterior hypop hysis and ectopic posterior pituitary hyperintense signal. This syndrome has bee n shown to be associated with either isolated growth hormone deficiency or multi ple anterior pituitary hormone deficiency, but normal posterior pituitary functi on. Case report.We report our experience with a six-year-old boy who had been treated for three years for a multisystem Langerhans’cell histiocytosis with di abetes insipidus and who was admitted because of short stature. Endocrinological examinations demonstrated a profound growth hormone deficiency and a partial ce ntral hypocorticism. Magnetic resonance imaging showed pituitary stalk transecti on and a midline anomaly of the brain (Arnold Chiari type I malformation). Concl usion. Although, some events of his perinatal history lead to the hypothesis of a malformative origin, the progression of Langerhans’cell histiocytosis affecte d tissues to fibrosis, suggest that this disease is the cause of the patient’s hypothalamohypophyseal lesions.
