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Empty sella syndrome: does it exist in children?
A Ammar1, A Al-Sultan, F Al Mulhim
1Department of Neurosurgery, King Fahd University Hospital, Al Khobar, Saudi Arabia. ammar99@soficom.com.eg
Insights
Empty sella syndrome (ESS) affects children, often presenting with headaches and growth issues. Early diagnosis and treatment are crucial to prevent serious neurological complications in pediatric patients.
Area of Science:
- Pediatric neurosurgery
- Endocrinology
- Radiology
Background:
- Empty sella syndrome (ESS) is a recognized condition in adults, but its occurrence and implications in children are less understood.
- This study aims to increase neurosurgical awareness of ESS in pediatric populations and contribute to existing literature.
Observation:
- Twelve children aged 2-8 years with ESS were analyzed, showing enlarged sella turcica filled with cerebrospinal fluid.
- Causes included high intracranial pressure, hydrocephalus, and arachnoid cysts; primary ESS was also observed.
- Presenting symptoms were headache, underweight status, and short stature, with normal hormone assays.
Findings:
- Neuroradiological studies confirmed cerebrospinal fluid filling the sella turcica with herniation of suprasellar and arachnoid spaces.
- Common clinical manifestations in children include headaches, abnormal body weight, and short stature.
- Hormonal evaluations were consistently normal across all pediatric cases studied.
Implications:
- Undiagnosed or untreated ESS in children can lead to severe consequences, including pituitary/hypothalamic dysfunction and optic chiasm damage.
- Raising neurosurgical awareness is vital for early diagnosis and timely intervention in pediatric ESS cases.
- A classification system for the diaphragma sellae is presented to aid in understanding and management.
Object:
The empty sella syndrome (ESS) is well documented in adults, and although the same phenomenon of herniation of the arachnoid space into the enlarged sella turcica has been noted in children, it is not widely known that children suffer from this syndrome. Therefore, the aims of this paper are to increase neurosurgeons' awareness of the existence of this phenomenon in children and to add to the scant body of literature on the subject.
Methods:
The authors treated 12 children, ranging in age between 2 and 8 years, in whom neuroradiological studies demonstrated an enlarged sella turcica filled with cerebrospinal fluid and herniation of suprasellar and arachnoid spaces. The causes of ESS in these children were high intracranial pressure, neglected or improperly treated hydrocephalus, and suprasellar arachnoid cyst. Primary ESS was found as well. Most of the children presented with headache, abnormal body weight (the majority being underweight), and short stature. The results of hormone assays were normal in all children.
Conclusions:
If undiagnosed and untreated, ESS in children may lead to serious consequences, including impairment of pituitary and hypothalamic function and damage to the optic chiasm. It is important to raise awareness in the neurosurgical community about the existence of ESS in children so that it can be diagnosed and treated at an early stage. A classification system for the diaphragma sellae is recapitulated.