Interruption Syndrome Pituitary Stalk: About 8O Cases  

Soumeya Nora Fedala1 , Farida Chentli2 , Djamila Meskine2 , Ali El Mahdi Haddam2
1. Department of endocrinology bab el oued hospital, Algeria
2. Department of endocrinology bologhine hospital, Algeria
Author    Correspondence author
International Journal of Clinical Case Reports, 2015, Vol. 5, No. 28   doi: 10.5376/ijccr.2015.05.0028
Received: 04 Apr., 2015    Accepted: 13 Jun., 2015    Published: 03 Jul., 2015
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Soumeya Nora Fedala, Farida Chentli, Djamila Meskine and Ali El Mahdi Haddam, 2015, Interruption Syndrome Pituitary Stalk: About 8O Cases, International Journal of Clinical Case Reports, 5(28) 1-8 (doi: 10.5376/ijccr.2015.05.0028)

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).

Keywords
SITP MRI; GH deficiency; Multiple hormone deficiencies; Malformatives abnormalities; Monitoring

The interrupt syndrome of the pituitary stalk (SITP) represents a cause of growth hormone deficiency leading to recognize. This is a rare congenital malformation defined by morphological abnormalities revealed by magnetic resonance type small or not visible pituitary stalk, hypoplasia anterior pituitary and ectopic posterior pituitary gland (Argyropoulou et al., 1992). Its diagnosis requires monitoring and regular reassessments due to a possible evolution towards a panhypopituitarism (Adamsbaum, 1993).
The purpose of this study is to analyze clinical, biological, radiological and etiological features of SITP and appreciate its evolution.
Population, Methodology
80 patients (65 male, 15 female) with SITP were followed in endocrinology. These patients had consulted for delay stature (n: 65) and polyuropolydipsic syndrome (n: 2). They received a complete clinical examination, hormonal assessment exploring the anterior and posterior pituitary functions, a magnetic resonance imaging and a genetic study:
- The exploration of somatotropic function by measurement of plasma IGF-1 and two pharmacological stimulation tests of GH: Test glucagon / propranolol and insulin.
- The corticotropic function by static assays of blood cortisol and ACTH. The reserve was studied through the insulin tolerance test. Adrenal insufficiency is evidenced by low levels of plasma cortical or low response to test insulin associated with a non high ACTH.
- Thyroid function through assays thyroxin (FT4) and TSHus. Failing the TRH TEST (not available i
International Journal of Clinical Case Reports
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