Interruption Syndrome Pituitary Stalk: About 8O Cases

Abstract

Summary We report the study of 80 patients (65 M, 15 F) with Interruption Syndrome Pituitary Stalk. They had consulted for delay stature n: 65 and polyuropolydipsic syndrome n: 2. The mean age at diagnosis was 7±2.8 years. Delivery dystocia was noted in 18.7%. Neonatal hypoxia has been reported in 5%. The average size of patients was -.3±DS/M (Sempe). Micropenis ± cryptorchidism was present in 50%. Pituitary hypoplasia represents 91.2% of abnormality. The post pituitary gland is ectopic in 37.5%: infundibular place (90%) Tubercinerum (10%). In 3.7%, it is not found. The number of ectopic and unseen post pituitary gland is significantly higher ( p <10-6) when the stalk is discontinued (n: 28). Than when pituitary gland is normally (n=3). When the post pituitary gland is ectopic or unseen (n: 37), malformations of HH are associated generally (81, 08%). Pituitary is more severely reduced when the post pituitary gland is ectopic or invisible and that the stalk is interrupted than when it is normal: 1.5mm±0.6 vs. 1.8±3 mm ( p : 0.01). Hormonal assessment revealed IGH in 100%. It was associated with others pituitary deficiencies in 68.7%. Diabetes insipidus was found in 05 cases. Multiple pituitary deficiencies are more common when there are at least two malformations anomalies. Reassessment revealed other hormonal deficiencies in 22, 5% of patients with a mean of 05 years. The genetic study performed showed no molecular defect (PROP 1 PIT1, LHX3, LHX4, HESX1).