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Primary empty sella syndrome in childhood: association with precocious puberty
1UMD-New Jersey Medical School, Newark.
Insights
Primary empty sella syndrome is rare in children, often causing endocrine issues like growth hormone deficiency. This study highlights a rare case of precocious puberty in a child with this condition, suggesting pubertal abnormalities should be considered.
Area of Science:
- Pediatric Endocrinology
- Neuroendocrinology
- Radiology
Background:
- Primary empty sella syndrome (PESS) is an infrequent finding in childhood.
- Previous reports document PESS in 112 children (ages 0.7-18 years) with a reported frequency of 1-58%.
- Endocrine abnormalities are common in pediatric PESS, while visual deficits are rare (6%).
Abstract:
Primary empty sella syndrome has been considered an infrequent finding in childhood. One hundred and twelve cases have been reported in children ages 0.7 to 18 years. The frequency of primary empty sella ranged from 1% to 58%. Endocrine abnormalities were described in nearly all children while visual abnormalities were noted in only 6%. Growth hormone deficiency was the single most common hormonal dysfunction noted in children with primary empty sella. Precocious puberty has been rarely reported in association with primary empty sella. We report the case of a 7-1/2 year old girl with gonadotropin dependent central precocious puberty and a partially empty sella who had no other hypothalamic-pituitary dysfunction. We suggest that pubertal abnormalities be included among the endocrine disorders potentially associated with the primary empty sella syndrome.