What is the best treatment approach for a patient with empty sella syndrome (ESS) and symptoms of headache, possibly due to intracranial hypertension (IH)?

Treatment of Headache in Intracranial Hypertension with Empty Sella

The primary treatment for headache in a patient with intracranial hypertension (IH) and empty sella on MRI is weight loss combined with acetazolamide, with surgical intervention reserved for visual deterioration. 1

Confirming the Diagnosis

Empty sella is a typical neuroimaging feature of raised intracranial pressure and supports the diagnosis of idiopathic intracranial hypertension (IIH) when present alongside other clinical features. 1 The empty sella develops from herniation of CSF and arachnoid membrane through an absent or rudimentary diaphragm sellae during periods of elevated intracranial pressure. 2

You must assess for papilledema and perform formal visual field testing immediately, as visual loss is the most serious complication and can be insidious and unnoticed by patients. 3

Medical Management Algorithm

First-Line Treatment

Weight loss is the cornerstone of treatment and should be initiated immediately in all obese patients with IIH. 1 This addresses the underlying disease mechanism and can lead to resolution of symptoms, including reversal of the empty sella appearance. 4

• Acetazolamide is the primary medical therapy for symptom control while weight loss is being achieved 1
• Paracetamol and/or NSAIDs can be used for symptomatic headache relief 1, 5
• NSAIDs may provide additional benefit by reducing intracranial pressure 5
• Provide gastric protection if using NSAIDs 5

Critical Medication Pitfalls to Avoid

Avoid medications that can worsen orthostatic symptoms or lower CSF pressure, including:

• Topiramate 5
• Indomethacin 5
• Beta-blockers 5
• Candesartan 5

Monitor for medication overuse headache, which occurs with simple analgesics used >15 days/month or opioids/triptans used >10 days/month. 5 This is a major risk that can complicate the clinical picture.

When Medical Management Fails

If weight loss cannot be achieved through patient effort alone, pursue professional help through structured diet programs. 1 Bariatric surgery has an increasing role for sustained weight loss in IIH, though more prospective controlled evidence is needed. 1

For non-obese patients, revisit secondary causes of intracranial hypertension, as the role of weight management is uncertain in this population. 1

Surgical Intervention

Indications for Surgery

Surgery is indicated when there is evidence of declining visual function. 1 This is the acute management to preserve vision and takes priority over medical management.

Surgical Options

Ventriculoperitoneal (VP) shunt is the preferred CSF diversion procedure due to lower reported revisions per patient compared to lumboperitoneal shunts. 1

• Use neuronavigation for VP shunt placement 1
• Consider adjustable valves with antigravity or antisiphon devices to reduce risk of low-pressure headaches 1
• A lumbar drain can be used as a temporizing measure while planning urgent surgical treatment 1

Optic nerve sheath fenestration (ONSF) is performed more frequently in Europe and the USA, with fewer complications than CSF diversion and no reported mortalities. 1 Some consider ONSF as first-line treatment in malignant fulminant cases. 1

Important Surgical Caveats

Surgical failure rates are substantial: worsening vision occurs in 34% at 1 year and 45% at 3 years, with failure to improve headache in one-third to one-half of patients. 1 This underscores the importance of continuing weight loss efforts even after successful surgery. 1

Monitoring and Follow-Up

Structure follow-up to assess:

• Peak headache severity 5
• Time to severe headache onset 5
• Time able to spend upright 5
• Serial visual field testing and visual acuity 3

Be vigilant for post-treatment complications, particularly if any intervention (such as lumbar puncture) is performed, as this can paradoxically cause CSF leak and intracranial hypotension, leading to worsening orthostatic headache. 2 The empty sella can actually reverse in this scenario. 2, 4

Special Consideration: Distinguishing IH from Intracranial Hypotension

If the headache pattern changes to worsen in upright position with relief when recumbent, suspect intracranial hypotension from CSF leak rather than persistent IH. 2 This is a dangerous complication that requires different management, including possible epidural blood patch. 2