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Foundations of the clinical research project:
Vein of Galen vascular malformations are a group of rare cerebral vascular malformations with complex anatomy and severe clinical consequences. The pathology consists of direct communications between small intracerebral arteries and veins without the presence of capillaries. The result is a vascular malformation with low resistance and high flow, a lesion that will evolve during fetal development into aneurysmal dilatation.
These lesions evolve towards rupture, but due to the high flow rate through these malformations, children very quickly develop cardiac decompensation (high-output failure). Treatment must be rapid, often on the first day of life. The technique currently used is iterative embolization. General anaesthesia is required for this procedure. However, neonatal anesthesia in children with cardiac decompensation and pulmonary hypertension poses major challenges for the anesthesia team. A high percentage of these patients require a short stabilization period with management of cardiac decompensation and pulmonary hypertension, most often by continuous milrinone infusion.
Hôpital Erasme is a reference center for the management of vein of Galen malformations. In this work, we propose to analyze all cases of children with veins of Galen malformations managed by the interventional neuroradiology department.
Objectives of the clinical research project:
2.1 Main objective:
Description of cerebral lesions as described by angiography. Details of anesthetic management with all anesthetic drugs, details of cardiac management. Details of ventilatory management, intubation duration required.
Description of embolization outcome. Description of the neurological and cardiac outcome.
2.2 Secondary objectives:
Possibly, depending on the number of patients, attempt to identify factors predictive of poor outcome.
Methodology of the clinical research project:
We will carry out a retrospective analysis of the records of all patients who have been managed at Erasme for vein of Galen-type malformation. Activity began ± in 2011, and we will produce a listing of all patients from 2010 to August 2024. From this listing, obtained by the radiology department, we will collect data from the patients computer files.
Design of the clinical research project:
This is a retrospective study based on file analysis.
Participants:
All patients treated at Erasme for a vein of Galen malformation. We will check the file to ensure that the parents have not expressed any opposition to the use of the patient medical data.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Vein of Galen malformation | All patients treated at Erasme hospital for a vein of Galen malformation. |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| embolization therapy | Procedure | Embolization in the neuroradiological department is the Golden Standard for the treatment of vein of Galen malformations. |
|
| Measure | Description | Time Frame |
|---|---|---|
| Neurological outcome | Neurological outcome after each embolization therapy will be recorded (%): No neurological deficit, minor neurological deficit, major neurological deficit | 28 days |
| Measure | Description | Time Frame |
|---|---|---|
| Cardiac failure | Presence of cardiac failure before, during and after each embolization therapy will be recorded. (%) | 28 days |
| Inotropic support | Inotropic support for heart failure will be recorded (%), as well as the type of inotropic agents used for treatment and maximum dosage. |
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Inclusion Criteria:
- All patients treated at Erasme for a vein of Galen malformation
Exclusion Criteria:
- Opposition of parents to the use of medical data
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All patients treated at Erasme for a vein of Galen malformation up to august 2024
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| Name | Affiliation | Role |
|---|---|---|
| Denis Schmartz, MD | Université Libre de Bruxelles | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| H.U.B - Hôpital Erasme | Brussels | 1070 | Belgium |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 15910357 | Background | Ashida Y, Miyahara H, Sawada H, Mitani Y, Maruyama K. Anesthetic management of a neonate with vein of Galen aneurysmal malformations and severe pulmonary hypertension. Paediatr Anaesth. 2005 Jun;15(6):525-8. doi: 10.1111/j.1460-9592.2005.01471.x. | |
| 15269462 | Background | Sinha PK, Neema PK, Rathod RC. Anesthesia and intracranial arteriovenous malformation. Neurol India. 2004 Jun;52(2):163-70. |
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| ID | Term |
|---|---|
| D054080 | Vein of Galen Malformations |
| C536535 | Vein of Galen aneurysm |
| ID | Term |
|---|---|
| D002538 | Intracranial Arteriovenous Malformations |
| D002561 | Cerebrovascular Disorders |
| D001927 | Brain Diseases |
| D002493 | Central Nervous System Diseases |
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| ID | Term |
|---|---|
| D004621 | Embolization, Therapeutic |
| ID | Term |
|---|---|
| D006489 | Hemostatic Techniques |
| D013812 | Therapeutics |
| D060205 | Therapeutic Occlusion |
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| 28 days |
| 23889354 | Background | Mortazavi MM, Griessenauer CJ, Foreman P, Bavarsad Shahripour R, Shoja MM, Rozzelle CJ, Tubbs RS, Fisher WS 3rd, Fukushima T. Vein of Galen aneurysmal malformations: critical analysis of the literature with proposal of a new classification system. J Neurosurg Pediatr. 2013 Sep;12(3):293-306. doi: 10.3171/2013.5.PEDS12587. Epub 2013 Jul 26. |
| D009422 | Nervous System Diseases |
| D020785 | Central Nervous System Vascular Malformations |
| D009421 | Nervous System Malformations |
| D001165 | Arteriovenous Malformations |
| D054079 | Vascular Malformations |
| D018376 | Cardiovascular Abnormalities |
| D002318 | Cardiovascular Diseases |
| D014652 | Vascular Diseases |
| D020765 | Intracranial Arterial Diseases |
| D000013 | Congenital Abnormalities |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |