Syndrome d'interruption de la tige pituitaire : à propos de 8 cas pédiatriques

Résumé

Introduction: Childhood anterior-pituitary insufficiency has many causes (malformative, genetic, traumatic, tumoral...). One particular entity can be clearly identified: pituitary stalk interruption syndrome (PSIS). The aim of our study was to evaluate clinical and paraclinical features of patients with PSIS, and to analyze their growth evolution. Patients and methods: We selected 8 children diagnosed with PSIS according to the results of MRI. We studied the perinatal characteristics of these patients, then the auxological, the endocrine features, and growth evolution after 1 year of the initiation of GH therapy. Results: The mean age at diagnosis was 5.3 years. Sex ratio of 0.6. Consanguinity was documented in 50% of cases. Perinatal findings showed: breech delivery in 2 cases and asphyxia in 3 cases. The mean height at diagnosis was -3.32 DS. A facial dysmorphia was found in 3 cases. The mean bone age retardation was 2.5 years. Hormonal assessment showed multiple hypo-pituitarism in all cases. The overall height gain after one year of beginning of GH was 0.59 DS. Conclusion: In children with PSIS, the other anterior pituitary deficiencies are often associated with GH deficiency. These functions therefore require to be carefully followed early, periodically and in the long term. Growth in these children responds particularly well to GH therapy, in particular during the first year.