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Posterior pituitary ectopy in children with idiopathic growth hormone deficiency
F Mészáros1, K Vergesslich, S Riedl
1Paediatric Department, University of Vienna, Austria.
Insights
Pituitary hormone deficiency in children often involves a characteristic anomaly detected by brain MRI, including a small pituitary gland and ectopic posterior lobe. This finding is linked to multiple pituitary hormone deficiency.
Area of Science:
- Pediatric Endocrinology
- Neuroimaging
- Developmental Biology
Background:
- Pituitary hormone deficiency in children presents diagnostic challenges.
- Understanding the underlying pathogenesis is crucial for effective management.
- High-resolution brain MRI offers detailed anatomical insights into the hypothalamo-pituitary region.
Purpose of the Study:
- To investigate the anatomical basis of pituitary hormone deficiency in children using high-resolution brain MRI.
- To correlate specific MRI findings with clinical presentations of isolated GH deficiency and multiple pituitary hormone deficiency.
Main Methods:
- MRI scans of the brain were performed on 37 children (aged 1.0-17.3 years) with short stature and diagnosed pituitary hormone deficiency.
- The hypothalamo-pituitary area was analyzed for anatomical abnormalities.
- Pituitary gland height was measured and compared to age-matched healthy controls.
Main Results:
- A characteristic pituitary anomaly was observed in 70% of patients, including hypoplastic adenohypophysis, absent infundibulum, and ectopic posterior pituitary lobe.
- Patients with the anomaly showed significantly reduced anterior pituitary height (1.9 mm) compared to controls (4.1 mm).
- Multiple pituitary hormone deficiency was more prevalent in children with the pituitary anomaly (62%) than in those with normal MRI findings (27%).
Conclusions:
- The study highlights a common anatomical malformation in children with pituitary hormone deficiency, likely originating during embryonic development or perinatal events.
- Brain MRI is essential for diagnosing pituitary anomalies in hypopituitary children.
- Diagnostic terminology should reflect the identified anatomical malformations.
Aims:
To evaluate the underlying pathogenesis in children with pituitary hormone deficiency by means of high resolution MRI of the brain.
Patients/Methods:
Thirty-seven children with short stature and isolated GH deficiency (IGHD, n = 17) or multiple pituitary hormone deficiency (MPHD, n = 20) were subjected to an MRI of the brain at the age of 1.0-17.3 years. The anatomic condition of the hypothalamo-pituitary area was analyzed and the height of the pituitary gland was measured and compared to the data of age-matched healthy subjects.
Results:
Seventy percent of the patients had a characteristic anomaly: the adenohypophysis was hypoplastic, the infundibulum was absent and the posterior pituitary lobe was ectopic at the bottom of the median eminence. The height of the anterior pituitary was significantly reduced in these patients (1.9 +/- 0.1 mm; mean +/- SD) when compared to age-matched healthy controls (4.1 +/- 0.8 mm, p<0.001) or hypopituitary patients with a normal MRI (4.3 +/- 0.8 mm). MPHD was found in 62% of patients with the pituitary anomaly whereas only 27% of children with a normal MRI had MPHD (p<0.05).
Conclusions:
The pathogenesis of the pituitary anomaly is unknown; a disorder during embryonal development or perinatal events have been discussed as causal factors. MRI should have a prominent position in the work-up of hypopituitary children. When an anatomical malformation is visualized by MRI, the diagnostic terminology should be adapted accordingly.