Empty sella syndrome: does it exist in children?

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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.

Article Information

Address reprint requests to: Ahmed Ammar, M.D., Department of Neurosurgery, El Salam Hospital, First Post Office, El Salam City, Cairo, Egypt. email: ammar99@soficom.com.eg.

© AANS, except where prohibited by US copyright law.

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Figures

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    Case 1. Sagittal MR image revealing a case of primary ESS in an 8-year-old girl.

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    Case 2. Sagittal MR image revealing a case of ESS as a consequence of longstanding high ICP due to hydrocephalus caused by a suprasellar cyst in a 4-year-old boy who presented with bubbling sign.

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    Case 3. Sagittal MR image obtained in a 6-year-old girl, demonstrating a case of ESS as a complication from longstanding hydrocephalus caused by a posterior fossa astrocytoma.

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