What is the management and treatment of Dolicoectasia?

From the Guidelines

Dolichoectasia is a condition characterized by the fusiform dilatation and elongation and tortuosity of arteries, most commonly affecting the vertebrobasilar system, and its management primarily focuses on preventing complications and treating symptoms. The condition is associated with traditional ischemic stroke risk factors such as increasing age, hypertension, and male sex, but its relationship to atherosclerotic disease is less clear 1.

Key Considerations

• The management approach must be individualized based on the location of dolichoectasia, associated symptoms, and patient comorbidities.
• Treatment typically begins with antiplatelet therapy such as aspirin (81-325 mg daily) or clopidogrel (75 mg daily) to reduce the risk of thromboembolic events, which are common complications, as recommended by the American Heart Association/American Stroke Association guideline 1.
• For patients with hypertension, blood pressure control is essential using appropriate antihypertensive medications like ACE inhibitors, ARBs, or calcium channel blockers to reduce stress on the affected vessels.
• In cases where dolichoectasia causes compression of cranial nerves, symptomatic management may include carbamazepine (starting at 100 mg twice daily, gradually increasing to 200-400 mg twice daily as needed) for trigeminal neuralgia, or gabapentin (300-1200 mg three times daily) for neuropathic pain.
• For patients with significant risk of rupture or those with recurrent symptoms despite medical therapy, surgical interventions may be considered, including vessel reconstruction, bypass procedures, or endovascular techniques, though these carry significant risks given the complex anatomy.
• Regular imaging follow-up with MRA or CTA every 1-2 years is recommended to monitor for progression of dilation or development of aneurysms.

Current Recommendations

• The use of antiplatelet or anticoagulant therapy is reasonable for the prevention of recurrent ischemic events in patients with vertebrobasilar dolichoectasia and a history of ischemic stroke or TIA without other attributable causes, according to the 2021 guideline for the prevention of stroke in patients with stroke and transient ischemic attack 1.
• However, there are insufficient data to provide a recommendation for anticoagulation over antiplatelet therapy, and the choice of therapy should be individualized based on patient-specific factors 1.

Future Research Directions

• Many important issues about the management of patients with vertebrobasilar dolichoectasia remain unclear, including the development of an agreed-on definition, accumulation of more robust natural history data, and clarification of the type, dosing regimens, and efficacy of antiplatelet therapy versus anticoagulation for primary and secondary ischemic stroke prevention 1.

From the Research

Definition and Pathophysiology of Dolicoectasia

• Dolicoectasia is a form of brain large artery disease associated with a high risk of mortality and morbidity 2.
• It is characterized by progressive enlargement of arterial size, which is a predictor of mortality 2.
• The disease is associated with dilatation and/or tortuosity of the affected vessel, and its prevalence increases with age 3.
• Multiple pathophysiological processes, including activation of metalloproteinases and irregular turbulent blood flow, may lead to the development of dolichoectatic vessels 3.

Clinical Presentations of Dolicoectasia

• Dolicoectasia commonly presents with ischemic or hemorrhagic stroke, and/or cranial neuropathies 3.
• The posterior circulation is more frequently affected by the dolichoectatic process than the anterior circulation 3.
• Compression of cranial nerves or brainstem can lead to hemifacial spasm, trigeminal neuralgia, or brainstem dysfunction 4.
• Intracranial bleeding is another possible manifestation of dolicoectasia 4.

Management and Treatment of Dolicoectasia

• There is no specific treatment for dolicoectasia, and the surgical and medical therapies that have been used to treat this condition have not been systematically evaluated 3.
• Adequate control of blood pressure may prevent ischemic or hemorrhagic stroke 4.
• Antiplatelet treatment is indicated for the prevention of recurrent stroke in case of ischemic stroke, but the risk of hemorrhagic stroke should be considered in patients with severe forms of dolicoectasia 4.
• Close observation and follow-up neuroimaging is recommended to monitor the progression of the disease and appearance of new ischemic or hemorrhagic lesions 4.
• Surgical decompression may be reserved for medically intractable patients with trigeminal neuralgia or hemifacial spasm 4.
• Off-label treatment with doxycycline and apixaban has been reported to slow down arterial dilatation and prevent thrombosis in some cases 2.