Article
Cervicale intramedullary oedema plays a key role in patients with arteriovenous fistulas of the craniocervical junction
Das zervikale intramedulläre Ödem ist eine der Hauptkomplikationen in Patienten mit arterio-venöser Fistel am kraniozervikalen Übergang
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Published: | June 4, 2021 |
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Objective: Arteriovenous fistulas of the craniocervical junction (ccdAVF) are rare sorts of cranial vascular malformations. Symptoms leading to hospital admission can grow slowly or present acute. Possible symptoms are progressive myelopathy or sudden bleeding. By their low prevalence and versatile presentation diagnosis is crucial and often prolonged. Therapeutic options are endovascular embolization, microsurgical dissection or a combination of both. In this study we focused on the myelopathy and its role as symptom as well as permanent problem for affected patients.
Methods: We performed a retrospective analysis of the clinical dataset for a 12 year time period. Predefined inclusion criteria were a angiographically confirmed ccdAVF and analyzable health- and radiological records. General patient information, symptoms and neurological status on admission as well as at discharge were extracted from the health record. CcdAVF was reviewed according the Cognard classification. MRI scans were reviewed for presence and extent of myelopathy.
Results: In total 12 patients met the defined study inclusion criteria. 5 patients presented with progressive myelopathy and increasing neurological deterioration as initial symptom. In 4 cases diagnosis was made due to minor symptoms. Acute neurological detoriation due to bleeding was seen in 3 cases. For all patients long term follow-up was available. Myelopathy persisted in all patients who became symptomatic due to CcdAVF and was documented as being progressive in 3 cases during follow-up.
Conclusion: Arteriovenous fistulas of the craniocervical junction are complex malformations in diagnosis, treatment and outcome. Congestive myelopathy due to venous overload is the leading component in this disease. Prognosis of neurological deficits after obliteration of a CcDAVF seems as unpredictable as in spinal dAVF. Wether treatment modalities i.e. surgery vs. embolisation influence the disease course remains unresolved and consortional studies of collateral measures focussing on the sequelae of sudden changes of hemodynamics after treatment are recommended.