What is the treatment for adult craniostenosis?

Treatment of Adult Craniostenosis

Critical Clarification

The term "craniostenosis" in adults requires immediate clarification, as it refers to two completely different conditions with entirely different treatments:

If Referring to Craniosynostosis (Premature Skull Suture Fusion)

Surgical correction via frontocranial remodeling with an intracranial approach is the definitive treatment for adults with unoperated craniosynostosis causing significant deformity. 1

Surgical Approach

• Frontocranial remodeling with intracranial approach is required in most cases to achieve adequate correction of the frontal and upper cranial vault deformities 1
• Limited approaches or frontal implants alone are only appropriate for mild cases, as they avoid intracranial surgery but provide inferior results 1
• The operation is technically more complex in adults compared to infants, but successful morphologic correction is achievable 1

Key Considerations

• The psychological impact of facial disfigurement is substantial, making most young adults willing to accept the risks of radical surgical correction 1
• No significant perioperative complications occurred in a series of 13 adult patients (mean age 24 years) undergoing correction 1
• Late revisions for bony irregularities may be needed in some cases, performed through limited approaches 1

Important Caveat

• Early surgical correction in infancy is strongly preferable (optimal age 3-6 months), but adult correction remains feasible when early intervention was not performed 1, 2

---

If Referring to Intracranial Arterial Stenosis (Atherosclerotic Disease)

Aggressive medical management with antiplatelet therapy is superior to endovascular stenting for symptomatic intracranial atherosclerotic stenosis. 3

First-Line Medical Management

• Dual antiplatelet therapy (clopidogrel 75 mg + aspirin 75-81 mg daily) for 21-30 days is recommended for symptomatic patients 3
• Loading dose of aspirin 160 mg should be given immediately after brain imaging excludes hemorrhage 3
• After 21-30 days, transition to single antiplatelet agent (aspirin or clopidogrel alone) 3

Why Medical Management Over Stenting

• The SAMMPRIS trial demonstrated that aggressive medical management is superior to intracranial stenting for reducing morbidity and mortality 3
• Endovascular stenting carries substantial procedural risks: 7.9% stroke rate, 3.4% death rate, and 9.5% combined stroke or death rate perioperatively 4
• High restenosis rates (up to 30-32%) occur with stenting, with 39% of restenoses being symptomatic 4

Endovascular Intervention Considerations

• Stenting should only be considered for highly selected patients with symptomatic stenosis ≥50% refractory to medical therapy 4
• The Wingspan stent system has FDA humanitarian device exemption for this narrow indication 4
• Procedures should only be performed at high-volume academic centers with significant neurovascular expertise 4
• A 14% rate of stroke, hemorrhage, or death at 30 days or ipsilateral stroke at 6 months was observed in NIH registry data 4

Risk Stratification

• Severe stenosis ≥70% carries highest stroke risk (hazard ratio 2.00) in the territory of the stenotic artery 4
• Patients enrolled early (≤17 days) after qualifying ischemic event have higher stroke risk (hazard ratio 1.72) 4

Alternative Diagnoses to Consider

• If diffuse intracranial stenosis is present, consider rare causes including moyamoya disease/syndrome, primary CNS vasculitis, connective tissue disorders (Ehlers-Danlos, Marfan), or radiation-induced arteriopathy 5
• MRI with MRA is the recommended initial diagnostic test for suspected arteriopathy 5