Objective To analyze the clinical characteristics and pathogenesis of the patients with pituitary stalk interruption(block) syndrome (PSIS), there by better comprehending this disease. Methods A new case of PSIS was reported, and a literature review was conducted of Chinese reports on PSIS retrieved in CHKD and WANFANG Medical database. Results Since 2004, 311 cases of PSIS were reported in 33 papers from 14 provinces and cities, including this case. PSIS had a male predominance (257 male, 54 female). 183 of 311 had an abnormal labor or cerebral anoxia history. Magnetic resonance imaging (MRI) showed pituitary stalk rupture in 239 cases and significant thin pituitary stalk in 62 cases. Multiple hormone deficits were observed in 190 patients and single growth hormone deficiency in 75 cases. The patients with pituitary stalk rupture have a predominance of multiple hormone deficits, and the patients with pituitary stalk necking have a predominance of growth hormone deficiencies (P＜0.01). The major clinical manifestations in adolescent patients are growth retardation and secondary sexual characters missing or delay, and the babies with PSIS are characterized by repeated attack of hypoglycemia and convulsions. Generally, the treatment for PSIS patients is insufficient and delayed. Only 12 cases (3.86%) received the growth hormone treatment. Conclusion Abnormal labor or cerebral anoxia history is probably one of the most important causes of PSIS; MRI is an important method for diagnosis of PSIS. For suspected PSIS patients, cerebral MRI and endocrinological evaluation can be helpful for early diagnosis and treatment; strengthening protection for the perinatal fetus may be an important means for prevention and control of PSIS.
Key words
pituitary stalk interruption syndrome /
MRI /
meta-analysis
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