Differential Diagnosis for Headache in the Setting of Empty Sella Turcica

Single Most Likely Diagnosis

• Primary Empty Sella Syndrome: This condition is characterized by the herniation of the subarachnoid space into the sella turcica, often due to a defect in the diaphragma sellae. It is frequently asymptomatic but can cause headaches due to the traction on the surrounding structures or the increase in cerebrospinal fluid pressure.

Other Likely Diagnoses

• Pituitary Tumor or Cyst: Although the sella turcica appears empty, small tumors or cysts might not be visible on initial imaging. These can cause headaches through mass effect or by altering pituitary function.
• Idiopathic Intracranial Hypertension (IIH): This condition, also known as pseudotumor cerebri, can cause headaches and is associated with increased intracranial pressure without a detectable cause. The empty sella can be a secondary finding due to the increased pressure.
• Cerebrospinal Fluid (CSF) Leak: A defect in the dura mater can lead to a CSF leak, which might result in an empty sella appearance on imaging due to the decreased CSF volume. Headaches are a common symptom, often worsening with standing.

Do Not Miss Diagnoses

• Pituitary Apoplexy: Although less common, pituitary apoplexy is a medical emergency that can present with sudden, severe headache, often accompanied by visual disturbances and acute endocrine dysfunction. It occurs due to hemorrhage or infarction of a pituitary adenoma.
• Meningioma: These are tumors arising from the meninges, which can cause symptoms by compressing or invading nearby structures. A meningioma in the sellar region could potentially cause an empty sella appearance and headaches.
• Rathke's Cleft Cyst: A benign cystic lesion in the sellar region that can expand and cause symptoms similar to those of pituitary tumors, including headaches.

Rare Diagnoses

• Lymphocytic Hypophysitis: An autoimmune condition that causes inflammation of the pituitary gland, potentially leading to an empty sella appearance and a variety of endocrine and neurological symptoms, including headaches.
• Sarcoidosis: A systemic granulomatous disease that can affect the central nervous system, including the pituitary gland, leading to an empty sella and headaches among other symptoms.
• Histocytosis: Rare conditions like Langerhans cell histiocytosis can involve the pituitary stalk or gland, causing an empty sella and associated symptoms.