Clinical analysis of pituitary stalk interruption syndrome in 27 patients

Abstract

Objective To analyze the characteristics, diagnosis and treatment of patients with pituitary stalk block syndrome (PSIS). Methods The clinical manifestations, laboratory examinations and imaging data of 27 patients with PSIS admitted to the First Affiliated Hospital of Zhengzhou University from June 2014 to June 2018 were reviewed. The clinical features of PSIS were retrospectively analyzed. Results Twenty-seven patients with PSIS were diagnosed by saddle-fur magnetic resonance imaging. Among them, 2 cases (7.4%) were adult, all of whom were female; the disease developed in the adolescence period in 25 cases (92.6%), including 21 males and 4 females, and they were diagnosed from 10 to 35 years old. Two cases of adult onset patients had a history of head trauma; 15 cases (60%) of 25 adolescents had a history of dystocia, 1 case (4.0%) didn’t reach puberty, 24 cases (96.0%) with stunting, and 15 cases (60.0%) with short stature . Ten patients (40.0%) were with normal height, but endocrine tests showed growth hormone deficiency, hypogonadotrophic gonadal dysfunction, 5 cases of growth hormone 1 to 3 years in the hospital, 3 cases with chills, slow response when the thyroid function decreased, 19 cases (76.0%) with hypothyroidism and the intelligence was basically normal. Twenty-seven patients had atrophy or dysplasia of the anterior pituitary by magnetic resonance imaging, the pituitary height was 1-4 (2.48±0.94)mm; the pituitary stalk was missing in 25 cases (92.6%), and the pituitary stalk was fine in 2 cases (7.4%); there were 25 cases (92.6%) of posterior lobe ectopic, and 2 cases (7.4%) not clearly shown. No family history or midline structural abnormalities in 27 patients. Conclusions PSIS is rarely seen and it usually presents with clinical characteristics of growth etardation, partial or complete anterior hypopituitarism, but with normal posterior pituitary function.The characteristic change of MRI is the important diagnostic basis. Now, the only effective treatment is to supplement anterior pituitary hormone, but we should master the timing of the treatment and stick to it. Key words: Pituitary stalk interruption syndrome; Hypopituitarism; Anterior pituitary