Endovascular Treatment of Vein of Galen Malformation in Kenya, East Africa
Edwin Mogere , Cecilia Munguti
Department of Neurosurgery, Aga Khan University Hospital, Nairobi, Kenya
Correspondences to: Dr. Edwin Mogere. Email: firstname.lastname@example.org
Received: 31 Mar 2021; Revised: 24 Sep 2021; Accepted: 05 Oct 2021; Available online: 10 Nov 2021
Vein of Galen aneurysmal malformations (VGAM) are arteriovenous fistulas between the choroidal or quadrigeminal arteries and the embryonic precursor of the vein of Galen – the median prosencephalic vein. VGAM represents 30% of arteriovenous malformations in the paediatric age group. They are associated with a high morbidity and 90% mortality if untreated but respond well to intervention with mortality reduced to below 40%. There are few documented cases of VGAM worldwide and scarce data about these lesions in sub-Saharan Africa with challenges in diagnosis and management. We present two cases of infants diagnosed with VGAM and treated with endovascular embolisation. We examine the clinical and radiological data at presentation, intervention and follow-up at one year. We report on some of the technical neuro-interventional details. Both patients had satisfactory radiological results and the interventions resulted notable clinical improvements from baseline. Physicians should maintain a high index of suspicion treating infants and toddlers presenting with macrocephaly, neurocognitive decline and cardiopulmonary symptoms. Upon diagnosis endovascular interventions are feasible and available.
Keywords: Endovascular, Vein of Galen, Coiling, Embolization, Arteriovenous Fistula
Conflicts of Interest: None
© 2021 Author. This work is licensed under the Creative Commons Attribution 4.0 International License.
Vein of Galen aneurysmal malformations (VGAM) are arteriovenous fistulas between the choroidal and quadrigeminal arteries and the embryonic precursor of the vein of Galen – the median prosencephalic vein (1, 2). VGAM represents 30% of arteriovenous malformations in the paediatric age group. VGAM is associated with 90% mortality if left untreated, and an associated high morbidity rate(3). In sub-Saharan Africa there are very few documented cases of VGAM, with diagnosis and management being cited as a challenge(4). There is paucity of data as to the treatment and outcomes of VGAM in this region. The lesion requires intensive medical management and timely endovascular treatment to arrest brain parenchymal injury and severe neurodevelopmental compromise. We present two cases of infants diagnosed with VGAM and treated with endovascular embolization with satisfactory outcome.
A 1 year 6-month-old male infant was referred because of macrocephaly and progressive neurological decline noted with worsening speech, unsteady gait and spasticity over 6 months. There was no history of convulsions. The child was appropriate for age developmental milestones and had unremarkable past medical history in infancy. On examination, he was in fair general condition, alert, pupils were bilaterally equal and reactive to light. On CNS examination, of note was the macrocranium with a head circumference of 58.5 cm but had no sunset eyes. (Normal head circumference at birth is 35cm and 40-45 cm at 18 months of age) (5). Gaze was appropriate without nystagmus or any cranial nerve deficit. He had an ataxic gait, raised muscle tone with hyperreflexia and power grade 5 on all muscle groups. Magnetic Resonance Imaging (MRI) as depicted in image 1 demonstrated a VAGM with ventriculomegaly. Full hemogram, blood biochemistry and liver function assessments were within normal parameters. An echocardiogram was also unremarkable. The child was managed with trans arterial coil embolization in a single session. The Right femoral artery was accessed, the Left Vertebral artery cannulated with 5Fr Multi- Purpose D Envoy guide catheter. DSA runs noted a large fistulous connection of the left Posterior Cerebral artery, PCA, P2 segment with another feeder of the Right PVA as well. It was consistent with a Lasjunias- Mural type of VAGM/ Yasargil Type 2. A Vasco-10MP microcatheter was navigated into the Left P2 and then into the dilated Vein of Galen. Six large coils were deployed with reduced flow of approximately 80%. Magnetic Resonance Angiography (MRA) at five months follow up revealed resolution of venous hypertension and ventriculomegaly. He had much improved speech and gait had become an active young man.
Click to view Figure 1: Sagittal (A) and axial (B) T1 MRI of the brain demonstrating aneurysmal dilatation of the vein of Galen with thrombus causing compression of aqueduct of Sylvius with subsequent hydrocephalus
Click to view Figure 2: Digital subtraction imaging pre (A) and post coiling of the venous sac (B). Demonstration of fistulas connection from branches of posterior cerebral artery to Vein of Galen
Click to view Figure 3: MRI T2 pre-endovascular embolization (A) and 5 months post treatment (B) demonstrating resolution of the dilated vein of Galen and tributaries, reduced ventriculomegaly
A 1-year female infant who was previously well with normal developmental milestones presented with 3-month history of progressive loss of previously achieved motor milestones, with associated three week history of right lower limb focal seizures, headache and vomiting. At presentation the child was unable to sit up with loss of neck support, unable to stand or walk. On assessment, the child was in poor general condition, with macrocrania with a head circumference of 43 cm, closed fontanelles and generalized hypotonia. An MRI done demonstrated an aneurysmal dilatation of the vein of Galen with associated ventriculomegaly as noted in Image 3. She was managed with trans-arterial coiling embolization over 2 sessions at 3-month intervals. The right femoral artery was accessed with 4 Fr Sheath. A 4Fr C2 guide catheter was used to access bilateral Middle Cerebral Arteries (MCA) and the left Vertebral artery. DSA runs noted a large fistulous connection of the left MCA Cerebral artery with multiple other feeders from both the right MCA and bilateral PCAs with both choroidal and quadrigeminal supply. She had the persistent abnormal sinus draining high into the lambdoid area of the superior sagittal sinus. The VGAM was consistent with Lasjaunias Choroidal type/ Yasargil Type 1. A Vasco-10MP microcatheter was navigated into the Left P2 and then into the dilated Vein of Galen with single 6mm/20cm standard coil for embolization of the left MCA/PCA feeders at the first session with 60% reduction in flow. Twelve weeks later, we repeated the procedure and coiled the right MCA/PCA with further flow reductions totalling to about 90% of the original flow rates.
At 1 year post procedure patient has had impressive neurological improvement with resumption of sitting, truncal stability and standing. We also have resumption of monosyllabic speech.