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Endoscopic Endonasal Trans-sphenoidal Approach: Minimally Invasive Surgery for Pituitary Adenomas
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[Empty sella syndrome in childhood]

K Unsinn1, J Glatzl

  • 1Universitäts-Kinderklinik, Innsbruck.

Padiatrie Und Padologie
|January 1, 1991
PubMed

Insights

Primary empty sella syndrome in children often presents with pituitary hormone deficiencies, most commonly growth hormone deficiency. Early diagnosis through cranial CT is crucial for managing growth and hormonal imbalances.

Area of Science:

  • Pediatric Endocrinology
  • Neuroendocrinology
  • Radiology

Context:

  • Primary empty sella syndrome (PESS) is a rare condition affecting the pituitary gland.
  • Diagnosis in pediatric patients often relies on identifying hormonal deficiencies and imaging findings.

Purpose:

  • To report on the clinical presentation and diagnostic findings of PESS in a cohort of pediatric patients.
  • To highlight the utility of cranial computed tomography (CT) in diagnosing PESS.

Summary:

  • This study details 6 pediatric cases of PESS (5 boys, 1 girl, ages 1-22).
  • Commonly observed pituitary hormone deficiencies included growth hormone (GH) deficiency (all 6 patients), secondary hypothyroidism (2 patients), and hypogonadotropic hypogonadism (2 patients).
  • Skeletal maturation was retarded in all cases. Skull X-rays showed an enlarged sella turcica in only one patient, while cranial CT confirmed the diagnosis in all.

Impact:

  • PESS diagnosis in children requires a high index of suspicion for pituitary hormone deficiencies.
  • Cranial CT is essential for definitive diagnosis, as conventional radiography may not reveal characteristic sella turcica abnormalities.
  • Understanding PESS presentation aids in timely intervention and management of endocrine dysfunction in affected children.

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