Endovascular treatment of vein of Galen aneurysmal malformation: hospital-based case series in two tertiary centers

VGAM, constituting less than 1% of all brain vascular malformations, is indeed a rare brain arteriovenous shunt in the pediatric population [1].

During the neonatal period, a significant portion of brain arteriovenous shunts (BAVS) present as VGAM, with many cases being associated with congestive heart failure or hydrodynamic disorders [12, 13].

An important meta-analysis on VGAM embolization, encompassing 35 studies with 307 participants, evaluated the outcomes post-endovascular embolization for VGAM. The follow-up duration averaged around 42 months, with the analysis reflecting a distribution of 42% neonates (less than 1 month), 45% infants (1 month to 2 years), and 13% children (above 2 years) among the participants [14].

The findings from this additional meta-analysis are equally insightful. This study incorporated 27 series with a larger cohort of 578 patients, emphasizing the wide age distribution within the VGAM patient population. The median age of patients was notably low at 0.1 month. Among the patients with available age data (547 patients), the distribution was as follows: 229 neonates (41.9%), 246 infants (45.0%), and 72 children (13.2%).

Furthermore, sex data, available for 252 patients, showcased a notable male predominance, with 173 patients (68.7%) being male. These statistics further emphasize the prevalence of VGAM in early infancy and the male predominance in this specific patient group. Understanding these demographics is crucial in tailoring treatments and management strategies for VGAM patients [15].

The study conducted by Rajeev et al. offers a comprehensive view of 26 patients with VGAM. Among these patients, there were 17 males (65.3%) and 9 females (33.3%), indicating a slightly higher male predominance. The age range spanned from 1 day to 18 years, encompassing a diverse group of patients.

The age distribution within this cohort was as follows: one neonate (3.7%), 16 in the infantile age group (59.2%), eight children (29.6%), and one adult (more than 16 years) (3.7%). This study highlights the occurrence of VGAM across a wide age spectrum, emphasizing its presence not just in infants and children, but also in adults, albeit less frequently. Understanding its occurrence across age groups aids in developing nuanced treatment approaches and care strategies for patients with VGAM [16].

Kartik et al., in their study over 48 patients, 31patients were boys [64.6%] and 17 patients were girls [35.4%] [17].

In our study, we had 19 patients with VGAM 3 patients of them were neonates (15.8%) and 10 patients were infants (52.6%) while 6 patients were in early childhood (31.6%) with no patients presented with age above 10 years.

Regarding sex distribution, 12 patients were males (63.2%) while 7 patients were females (36.8%) from our 19 patients with VGAM.

This is in line with the previous studies that VGAM is mainly presented in neonatal and infantile pediatric age group except that neonatal age group is much more than infantile one in Hale et al., and Brinjikji et al., studies, while in Rajeev et al. study it was as our study.

All the studies demonstrated that male sex is more predominant than females in cases of VGM which was also demonstrated in our study.

The studies by Rajeev et al. and Brinjikji et al. shed light on distinct clinical presentations of VGAM across different age groups.

Rajeev et al.’s study revealed that among neonates, congestive heart failure (CHF) was the sole presenting symptom. In the infantile age group, the most common presentation was macrocrania in 11 out of 16 patients (68.75%), followed by developmental delay in 5 out of 16 patients (31.25%) 0.16.

Similarly, Brinjikji et al. found that in neonates, CHF was the most frequent presentation, observed in 88.2% of cases. In contrast, in infants and children, increased head circumference was more prevalent, occurring in 53.3% and 37.5% of cases, respectively. Understanding these age-specific clinical manifestations is crucial in diagnosing and managing VGAM in pediatric patients, allowing for tailored treatment approaches based on the presenting symptoms in different age groups [15].

The meta-analysis conducted by Jun Yan et al. revealed that within the 34 studies evaluated, neonates represented 44% of the sample. The predominant presentation in this neonatal group was congestive heart failure (CHF). This aligns with previous findings emphasizing CHF as a primary presentation among neonates with VGAM [18].

Additionally, Vignesh et al.’s study focusing on 31 patients older than one month highlighted that macrocrania is the most common presentation among the infantile age group. These consistent findings across studies underscore the age-specific symptomatology of VGAM, aiding in the identification and treatment of this condition within different pediatric age brackets [19].

Savage et al., in their study explained that the most common three symptoms were heart failure (68%; 142/210), hydrocephalus (15%; 31/210), and increasing head circumference (6%; 13/210) [20].

All these studies are in line with our study, as in our study we had 19 patients with VGAM 3 patients of them were neonates and 2 of them presented with CHF and the other one had brain melting syndrome (hydrodynamic disorder), we had 10 patients in infantile age group with 8 out of these 10 patients presented with macrocrania.

In our study in patients with VGAM 8 patients (42.1%) had macrocrania, 3 patients (15.8%) had CHF, another 3 patients (15.8%) presented with seizures and 2 patients (10.5%) presented with developmental delay.

As mentioned before, high-flow AV shunts and the physiological condition of the neonates and infants induce a specific symptoms according to the shunt type, there are other characteristic features of BAVS in young children (2–10 years), where new presentation of VGAM is rare and PAVF and AVM are seen more frequently. VGAM is rarely seen in older children with headache, seizures and ICH or incidentally [21].

Currently, endovascular therapy remains the preferred first-line treatment for VGAM, with the lowest mortality and complication rates and favorable clinical outcomes.

A meta-analysis conducted by Jun Yan et al. revealed that the mortality rate without treatment reaches 47%, whereas endovascular intervention reduces it to 12% [22].

Berenstein in his study on 45 patients with VGM, had 4.4% mortality rate, while Kartik in his study on 48 patients with VOGM had mortality rate reaching 31% [23].

The study done by Chingiz Nurimanov declared a mortality rate of 9.1% (two cases): one patient died the day postoperatively due to severe cardiac decompensation, and the other patient passed away after one month from pneumonia related to pulmonary hypertension [24].

In a study done by FB Nuñez on 30 cases with newly diagnosed VGM, 30 percent of all patients (n = 30) were discharged without any further complications. 95% of all patients with endovascular treatment survived until discharge, but only 41% of these patients were discharged home without any complications [23].

Chingiz’s study reported total occlusion rate (36.3%) and partial occlusion rate (63.7%) with good outcome in 72.7% and poor outcome in 27.3%. in comparison to Berenstein study who had total of 66.6% cases are neurologically and developmentally intact with outcome score 4, 20% had outcome score of 3, and 8.9% had outcome score of 2. He also reported reaching total occlusion in 82% of his cases. [24].

Arthur Hosmann had an experience on 18 consecutive patients 10 with choroidal and 8 with mural VGMs. Total occlusion was achieved in 87.5% of mural VGMs and 11.1% of choroidal VGMs. Good outcome was achieved in 53.8% and poor outcome in 46.2%, with an overall mortality of 16.7% [25].

In comparison to our study, we had 13 cases of mural type and 6 cases of the choroidal type. We could reach total occlusion in 15 patients (78.9%) and only 4 patients had residual after occlusion (21.1%).

From the 15 cases who reached total occlusion, we accomplished that in one setting in 12 patients and only 3 patients needed further sessions.

We had improvement in 11 patients (57.9%) in our study, while 4 patients discharged on score 3 (21%), and two patients discharged on score 2.

In our study, we had mortality rate 10.5% (two cases). One died during the embolization procedure, the other from glue embolization to the lung and the other had intraprocedural arterial perforation.

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