Primary amenorrhea due to empty sella: an underestimated entity

Area of Science:

• Neuroendocrinology
• Pediatric Neurology
• Neurosurgery

Background:

• Posterior fossa dermoid cysts are rare congenital tumors that can cause significant neurological and endocrine dysfunction in adolescents.
• Hydrocephalus and empty sella are common associated findings, impacting pituitary function and overall development.

Observation:

• A 16-year-old female presented with stunted growth, primary amenorrhea, headache, and vomiting.
• Brain MRI revealed a posterior fossa dermoid cyst with hydrocephalus and empty sella, accompanied by reduced levels of Growth Hormone (GH), Leutinizing Hormone (LH), and Follicular Stimulating Hormone (FSH).

Findings:

• Surgical excision of the dermoid cyst via suboccipital craniectomy resulted in no new neurological deficits.
• Post-operatively, the patient achieved menarche and developed secondary sexual characteristics, indicating partial recovery of endocrine function.
• Despite hormonal improvement, the patient's height remained subnormal two years after surgery compared to age-matched controls.

Implications:

• Surgical intervention for posterior fossa dermoid cysts can restore pituitary function but may not fully reverse pre-existing growth deficits.
• Early diagnosis and management are crucial for optimizing outcomes in pediatric patients with brain tumors affecting the endocrine system.
• This case highlights the importance of considering neuroendocrine evaluation in adolescents presenting with symptoms suggestive of pituitary dysfunction and intracranial masses.