Vein of Galen malformations in the newborn: case series

Abstract

BACKGROUND

Vein of Galen malformations (VoGMs) in newborns often represent life-threatening emergencies. Outcome is difficult to predict. The authors review 50 VoGM cases to correlate anatomical types with treatment and outcome.

OBSERVATIONS

Four distinct types of VoGMs are identified: mural simple (type I), mural complex (type II), choroidal (type III), and choroidal with deep venous drainage (type IV). Seven patients presented with mural simple VoGMs with a “single hole” fistula supplied by only one large feeder. These patients were treated electively at >6 months; development was normal. Fifteen patients presented with complex mural VoGMs. Multiple large feeders joined a single fistulous point within the wall of the varix. Patients typically presented with congestive heart failure (CHF) and required emergent transarterial intervention. Mortality was 7.7% with less than two-thirds developing normally. Twenty-five patients presented with choroidal VoGMs. Multiple large arterial feeders joined at multiple fistulous sites. Severe CHF in most patients required emergent transarterial and sometimes transvenous intervention. Mortality was 9.5%; two-thirds of the patients had a normal development. Three babies presented with choroidal VoGMs with deep intraventricular venous drainage. This phenomenon caused fatal “melting brain syndrome” in all three patients.

LESSONS

Recognition of the specific VoGM type determines treatment options and sets outcome expectations.

Aneurysms associated with arteriovenous fistulae have been recognized as a pathological entity at least since 1757.¹ William Hunter described “arteriovenous aneurysms” originating at the “anastomotic” site, the fistulous connection between artery and vein.¹ Similarly, vein of Galen aneurysmal malformations (VoGMs) are known to represent fistulous arterial connections to a persistent prosencephalic median vein of Markowski, the precursor to the vein of Galen.^2–7 Consistent with the fistulous nature of the lesion, a (venous) aneurysmal malformation is associated.

The first “aneurysmal varix” involving the vein of Galen was reported by Steinheil in 1895.⁸ Later, the fistulous characteristics of VoGMs were defined by Yaşargil in the 1970s and 1980s. Yaşargil differentiated fistulous VoGMs (types I–III) from parenchymal arteriovenous malformations (AVMs) with drainage to the vein of Galen (type IV).^6,7,9 Yaşargil type I VoGMs presented larger feeding arteries in the absence of a nidus-like network of smaller vessels and was surgically accessible. Feeding arteries originated off the pericallosal and P3 arteries. Yaşargil type II VoGMs were characterized by nidus-like, small feeding arteries (thalamic perforators) connecting to the draining vein in the absence of larger feeders—hazardous with surgery. Type III was a combination of type I and type II.

With the evolution of endovascular procedures, VoGMs became more amenable to treatment, including newborns. Pierre Lasjaunias treated 216 children with VoGMs via a transarterial approach.³ Reminiscent of Yaşargil’s observations, Lasjaunias described two types of VoGMs: “mural” and “choroidal.” The mural type had larger fistulous connections in the wall of the venous aneurysm, similar to Yaşargil’s type I. The choroidal type had a nidus-like network of mixed feeding vessels, similar to a Yaşargil’s type III.

Both, the Yaşargil and the Lasjaunias classifications have been used much to describe VoGMs. Both classifications have limitations regarding current practice. Yaşargil’s classification was designed to help select surgical candidates, an approach rarely chosen today secondary to advances in the endovascular field. The Lasjaunias types³ (mural versus choroidal) overlap and are not directly linked to specific outcomes or treatment modalities. As a result, criteria regarding patient selection, outcome expectations, treatment, and timing are still not well defined. On the basis of our clinical experience and review of the literature, we identified substantial differences in prognosis based on anatomical features of the malformations. To further investigate the nature of VoGMs, this article aims to identify VoGM subtypes, correlating with specific treatment and outcome expectations.

Study Description

Fifty patients with VoGMs were reviewed. Six patients were evaluated and treated by the first author at our institution during the last 4 years. Besides the known mural and choroidal variants of VoGMs, we recognized four distinct VoGM subtypes, defined by their anatomy. To explore associations of treatment variations and outcomes for these VoGM subtypes, we randomly identified 44 additional patients with VoGMs in the recent literature. Only cases with sufficient information were included. Anatomical details of the malformation, type of treatment, site of treatment, and demographics needed to be disclosed in the reports. Larger studies merely summarizing data without presenting case specifics were excluded. Data collection and evaluation were approved by the local institutional review board. Descriptive statistics were performed. Data are presented as median and interquartile range, 25th to 75th percentiles (Q1–Q3). On the basis of anatomical characteristics, we identified four distinct VoGM types. An illustrative case is reported for each type. Subgroup analysis is performed for the four different types of VoGMs.

Case Series

Clinical data of the 50 patients are displayed in Table 1. Median age at treatment was 5 weeks (interquartile range 1–24 weeks). Twenty-two lesions were found to be mural, or Yaşargil type I. The majority of the lesions (28 cases) were of the choroidal type, or Yaşargil type III. None were Yaşargil type II. Two patients did not receive an intervention and only underwent catheter angiography. Twenty-seven were boys, and 19 were girls. In four patients, the gender was not disclosed (i.e., “the twin” or “the child”). Twenty-eight patients (56%) presented with congestive heart failure (CHF; right heart failure/pulmonary hypertension). Twenty-five patients (50%) were treated emergently. Three patients presented with deep intraventricular venous drainage, a feature of VoGMs that has not been recognized previously, to our knowledge. Long-term follow-up was available in 43 patients. Of those, 28 patients (65%) had normal neurological development at last follow-up. Nine patients were delayed developmentally (21%). Six patients died (14%) within 1 month after the procedure. In those patients, there was no obvious procedure-related complication. The procedures simply were insufficient to resolve the patients’ heart failure. Overall follow-up was 19 months (range 9–36 months). Treatment options included embolization via an arterial or venous approach, surgical clipping, and stereotactic access to the vein.

TABLE 1.

Summary of the results of our case series and reference cases from the literature

Case No.	Yaşargil3	Lasjaunias et al.7	Present Study	Age (wks)	Sex		CHF	Target	Outcome	FU (mos)	Authors & Year
1	1	Mural	I	12	M	No		Feeder	Normal	36	Yoon et al., 201815
2	1	Mural	I	20	M	No		Feeder			Hockley et al., 201811
3	1	Mural	I	28	F	No		Feeder	Normal	9	Joo et al., 201712
4	1	Mural	I	32	M	No		Feeder	Normal	12
5	1	Mural	I	36	M	No		Feeder	Normal		Lomachinsky et al., 202213
6	1	Mural	I	40	M	No		Feeder	Normal	9	Runck et al., 201914
7	1	Mural	I	64	F	No		NA	Normal	60	Pulido et al., 202110
8	1	Mural	II	0	F	Yes		Feeder		1	De Rosa et al., 201919
9	1	Mural	II	0	M	Yes		Feeder	Delayed	24
10	1	Mural	II	1	F	Yes		Feeder	Normal	36	Deepti et al., 201818
11	1	Mural	II	1	F	Yes		Feeder	Normal	72	Alexander & Spetzler, 200616
12	1	Mural	II	1	M	Yes		Feeder	Dead	1	Hockley et al., 201811
13	1	Mural	II	1	M	Yes		Feeder	Normal	48
14	1	Mural	II	2	F	Yes		Feeder	Normal	60	Okcesiz & Donmez, 202122
15	1	Mural	II	6	F	No		Feeder	Delayed	54	Grillner et al., 201620
16	1	Mural	II	8		No		Feeder	Normal	24	Berenstein et al., 202217
17	1	Mural	II	11	M	No		Feeder	Normal	23	Pop et al., 201525
18	1	Mural	II	14	M	Yes		Feeder	Normal	20	Ramgren et al., 201723
19	1	Mural	II	18	M	No		Feeder	Delayed	12	Castro-Afonso & Abud, 202126
20	1	Mural	II	20	M	Yes		Feeder			Krings et al., 201121
21	1	Mural	II	28	F	No		Feeder	Normal	6	White et al., 202124
22	1	Mural	II	208	M	Yes		Feeder	Delayed	24	Todnem et al., 201827
23	3	Choroidal	III	0	F	Yes		Feeder	Dead	1	Bohiltea et al., 201639
24	3	Choroidal	III	0	F	Yes		Feeder	Normal	6	Puvabanditsin et al., 201740
25	3	Choroidal	III	0	F	Yes		Vein	Normal	60	Hoda et al., 202329
26	3	Choroidal	III	0	F	Yes		Feeder	Delayed	36	Wagner et al., 202241
27	3	Choroidal	III	0	M	Yes		Vein	Normal	36	Spazzapan et al., 201930
28	3	Choroidal	III	0	M	Yes		Feeder	Dead		Puvabanditsin et al., 201740
29	3	Choroidal	III	0		Yes		Feeder	Normal	6	Puvabanditsin et al., 201740
30	3	Choroidal	III	1	F	Yes		Feeder			Ullman et al., 201942
31	3	Choroidal	III	1	F	No		NA	Normal	108
32	3	Choroidal	III	1	M	Yes		Feeder		22	Hockley et al., 201811
33	3	Choroidal	III	1	M	Yes		Combined	Delayed	48	Demartini et al., 201731
34	3	Choroidal	III	2	M			Feeder		12	Spazzapan et al., 201930
35	3	Choroidal	III	2	M	Yes		Feeder	Normal	48	Spazzapan et al., 201930
36	3	Choroidal	III	3	F	Yes		Feeder	Delayed	9	Gupta et al., 202143
37	3	Choroidal	III	5	M	Yes		Feeder	Normal		Uwaezuoke & Wahba, 202144
38	3	Choroidal	III	5	M	No		Combined	Normal	18	Jagadeesan et al., 201632
39	3	Choroidal	III	16	F			Vein	Normal	108	Hoda et al., 202329
40	3	Choroidal	III	20	F	Yes		Feeder	Normal		Rousslang et al., 202245
41	3	Choroidal	III	24	F	No		Vein	Delayed	6	Zenteno et al., 201433
42	3	Choroidal	III	24		Yes		Feeder	Normal	18	Komiyama et al., 201646
43	3	Choroidal	III	28	M	No		Feeder	Delayed	16	Elmahrouk et al., 201847
44	3	Choroidal	III	32	F	No		Feeder	Normal	8	Chang et al., 202148
45	3	Choroidal	III	46	M	No		Feeder		25	Jones & Kane, 202249
46	3	Choroidal	III	96	M	No		Feeder	Normal	12	Sarica et al., 202050
47	3	Choroidal	III	104	M	No		Feeder	Normal		Abend et al., 200851
48	3	Choroidal	IV	0	M	Yes		Vein	Dead	1
49	3	Choroidal	IV	1	M	Yes		Feeder	Dead	1
50	3	Choroidal	IV	2		Yes		Feeder	Dead	1	Stone et al., 202034

NA = not applicable.

Cases are organized by a running case number and lesion types as characterized by Yaşargil,⁷ Lasjaunias,³ and the present study. Clinical data are presented as patient age (weeks), sex (male versus female), presence of CHF either “yes” or “no,” the targeted vessel during embolization (either an arterial feeder or the vein/venous aneurysm), outcome at last follow-up, and follow-up duration (months).

Type I: Mural Simple

Seven patients presented with a “single-hole fistula” with only one major feeding artery (Yaşargil type I, Lasjaunias type mural). Feeders included either a pericallosal or choroidal branch (see Fig. 1). None of those patients required emergent intervention at birth. All were discharged during the first week of life. One patient had spontaneous thrombosis of his fistula confirmed by angiography at 2 years of age and required no embolization.¹⁰ The other six patients underwent elective embolization several months after birth.^11–15 Median age at treatment was 32 weeks (range 1–24 weeks). Typical reason for treatment was growth of the lesion during the first 6 months of life. Median size of the varix was 4.4 cm (range 3.6–5 cm). Treatment included embolization of the large, single feeding artery, preferably with high-concentration n-butyl cyanoacrylate (NBCA).¹¹ Other options included coils,¹⁵ WEB (Microvention),¹⁴ and the MVP Amplatzer device (Medtronic). All patients survived (100%), and no adverse outcomes (0%) were reported.^10–15

FIG. 1.

A: Artistic sketch depicting a “mural simple” (type I) VoGM. Note the single main arterial feeder joining the venous aneurysm at a single fistulous point (“single hole” fistula). B: Left internal carotid angiogram, lateral view. A large posterior communicating artery feeds into mainly a single, large posterior choroidal feeder. C: Axial T2 MRI. Smaller VoGM with one fistulous pouch in the wall of the aneurysm.

Illustrative Case

Three weeks prior to delivery, a VoGM had been diagnosed in a 2.5-kg full-term boy who was delivered at our institution. His echocardiogram and brain magnetic resonance imaging (MRI) were normal, except for the VoGM. The lesion was fed by only one main, large posterior choroidal branch toward a single fistulous site (Fig. 1). He did not require any medical treatment and was discharged on day 5. Follow-up at 7 months demonstrated growth of the VoGM. He then underwent catheter angiography and embolization with 90% NBCA via a transfemoral approach. Embolization of the sole feeder resulted in near obliteration of the lesion. He is growing up normally, and, at age 1 year, he was walking and exceeding his developmental milestones. He is now 15 months old.

Type II: Mural Complex

Fifteen patients presented with a “single-hole” fistula fed by multiple larger arteries (Fig. 2). Ten (67%) of 15 patients presented emergently with right heart failure, CHF, and pulmonary hypertension. Emergent treatment was typically required, with median age at treatment being 6 weeks (range 1–16 weeks). Multiple larger feeders originated from pericallosal and choroidal branches. The median size of the venous varix was 3.25 cm (range 3–4 cm). Treatment was closure of arterial feeders in all patients. In one patient, after failed attempts at endovascular treatment, numerous feeding arteries were clipped via craniotomy in two stages.¹⁶ All other patients were successfully treated via transarterial endovascular embolization. NCBA was the preferred agent in most cases (73%), including ours.^11,18–24 One patient was treated with Onyx via Scepter miniballoon catheter (Microvention).²⁵ Two patients were treated with coils.^26,27 Long-term outcome was not available in two children. One patient failed to improve and died of refractory heart failure (7.7% mortality).¹¹ Eight children had a normal development (61.5%). Four children were developmentally delayed (30.8%). Median follow-up was 24 months.

FIG. 2.

A: Artistic sketch depicting a “mural complex” (type II) VoGM. Multiple arterial feeders join the venous aneurysm at a single fistulous point. B: Right internal carotid angiogram, lateral view. A large pericallosal artery and several enlarged choroidal arteries feed into a single, large pouch in the anteromedial wall of the aneurysm. C: Axial T2 MRI. Note the fistulous pouch in the wall of the aneurysm, where all the feeders connect.

Illustrative Case

This case has been reported previously.²⁸ A 3.1-kg boy was delivered via cesarean section at 36/4 gestation. Shortly after birth, he developed acute respiratory failure secondary to CHF and required intubation. Α VoGM was diagnosed by MRI showing multiple large choroidal feeding arteries converging on a single fistulous connector in the anteromedial wall of the giant venous aneurysm (Fig. 2). The patient was emergently treated with two stages of 90% NBCA embolization. Postoperatively, his echo series demonstrated progressive reversal of his suprasystemic pulmonary hypertension. He was discharged home after 2 months. Additional embolizations at months 6 and 9 resulted in near occlusion of the VoGM. At 2 years of age, he is growing and thriving with minimal delay.

Type III: Choroidal

This type has been well described previously.^3,6,7 Twenty-five patients (the majority) were identified as matching the choroidal type III, which is consistent with a Yaşargil type III or the standard Lasjaunias choroidal type. The choroidal type has multiple fistulous connections, including larger and smaller feeders (Fig. 3). The smaller feeders create a nidus-like appearance. Because there is often a myriad of smaller feeding arteries, curative embolization is less likely from a purely transarterial approach. Similar to type II mural complex, almost two-thirds (64%) of the patients required emergent intervention secondary to acute heart failure, CHF, and pulmonary hypertension of the newborn. One patient seen at our institution did not require intervention and has grown up to be a healthy 9-year-old girl (Fig. 3). Median age at treatment was 2 weeks (range 0–24 weeks). Unlike with type II, the vein was targeted directly in 6 (24%) of 25 patients.^29–33 In four patients (16%), the vein was the initial target.^29,30,33 In two patients, the procedure involved both arterial and venous embolizations.³² Zenteno et al.³³ used a stereotactic surgical approach to access the vein after a failed intervention attempt. The majority (80%) of the patients were also treated via transarterial embolization of the larger feeders. Long-term outcome was not available in four patients. Fourteen patients (66.7%) had reportedly normal development. Five patients (23.8%) were developmentally delayed. Two patients (9.5%) died. Median follow-up was 18 months (range 9–39 months).

FIG. 3.

A: Artistic sketch depicting a “choroidal” (type III) VoGM. Multiple arterial feeders join the venous aneurysm at many fistulous points. B: Right internal carotid angiogram, lateral view. Two enlarged choroidal arteries and a “nidus-like” fine vessel network feed the venous varix via multiple fistulous connections. C: Axial T2 MRI. A network of feeders to the aneurysm is present in the subarachnoid space.

Illustrative Case

Twins were born at full term. Four days prior, one of the twins, a baby girl, was noted to harbor a VoGM. She was clinically normal, so no medical treatment was required. Her Bicêtre score was 21. She was discharged home on postdelivery day 4. The girl is now 9 years old, and no intervention was needed. Her development is normal. Her VoGM is complex with multiple nidus-like feeders and many fistulous sites (Fig. 3). She does not have deep intraventricular venous drainage.

Type IV: Choroidal With Deep Venous Drainage

Generally, the venous drainage of the VoGMs is via the prosencephalic vein of Markowski—the persistent vein of Galen precursor vein. Lasjaunias observed that in his treated cases, the deep venous system of the healthy brain was separate from the VoGM: “The deep venous system has to find an alternate route to drain. Thalamostriate veins open into the posterior and inferior thalamic veins, which secondarily join a subtemporal or lateral mesencephalic vein, which then join the superior petrosal sinus, demonstrating a typical epsilon-shape ε on lateral angiogram.”³

However, so far unreported, to our knowledge, there is unfortunately also an aggressive type of choroidal VoGM in which deep venous drainage is recruited. Specifically, in two of our own patients and one patient in the literature (not described by the authors of the corresponding article),³⁴ we discovered deep venous drainage of choroidal VoGM in addition to drainage into the vein of Galen. The deep drainage follows the pathway described by Lasjaunias above. The typical “ε” appearance, or “number 3” appearance, of the engorged deep venous drainage system is clearly visible on lateral angiography in those patients (Fig. 4). On magnetic resonance angiography (MRA), the vein could be mistaken for one of the feeders because they are close by. However, the feeding arteries are in the choroidal fissures/subarachnoid space. In contrast, the deep veins can be spotted on axial T2 MRI scans within the ventricles (Fig. 4). It is those patients who develop the feared “melting brain” syndrome, even despite aggressive treatment attempts.

FIG. 4.

A: Artistic sketch depicting a “choroidal” (type IV) VoGM with deep venous drainage. Characteristic is the “number 3” configuration of the deep draining vein. The thalamostriate vein opens into the posterior and inferior thalamic veins, which drain into a subtemporal or lateral mesencephalic vein, which joins the superior petrosal sinus. The presence of deep venous drainage predicts impending melting brain syndrome. B: Right internal carotid angiogram, lateral view. Note the major deep venous drainage with extensive cortical venous reflux. C: Axial T2 MRI. The engorged bilateral thalamostriate veins together with the VoGM create the appearance of a scarab. The brain mass is already reduced secondary to venous hypertension combined with right heart failure.

Median age at treatment for the three type IV VoGM patients was 1 week (range 0.5–1.5 weeks). Treatment was always emergent. Mortality was 100%. Two babies were treated with multiple intraarterial embolizations. One baby was treated with transvenous embolization. Care was withdrawn during the first month in all patients. There was no obvious complication of the procedure, and the cardiac status actually improved with embolization. However, follow-up MRI revealed progressive loss of brain substance, also known as “melting brain syndrome.”

Illustrative Case

A 2.9-kg boy was born at term and developed respiratory distress days after birth. Secondary to CHF with right heart failure, he required intubation and mechanical ventilation. His Bicêtre score decreased to 10. The likely poor prognosis was discussed with the family. After thorough discussion, the decision was made to give the baby a chance with an attempt at transarterial embolization. Catheter angiography demonstrated prominent deep venous drainage of the VoGM in addition to outflow via the vein of Galen. Using a transumbilical approach, two sessions of 90% NBCA embolization were performed. The major feeding arteries were closed off. This resulted in improvement of the cardiac function. The child, however, did not progress neurologically. A 1-month follow-up MRI demonstrated recession of brain mass consistent with the melting brain syndrome. The patient transitioned to comfort care.

Discussion

Observations

In this article, we identify four different types of VoGMs, supplementing the two main variants already described by Lasjaunias.³ The four types are characterized by specific anatomical features, which can be identified by MRI/MRA, even prior to birth or the first day of life. All types are associated with specific clinical outcomes and treatment options.

Type I: Mural Simple

The type I (mural simple) VoGM has a more benign course and may even resolve spontaneously. The patients can be followed clinically and are typically discharged soon after birth. An intervention, usually a single-stage procedure, can be performed later, preferably >6 months after birth. Risk of the intervention is much lower than that of types II–IV. The interventionalist has numerous treatment options to choose from and can often deploy a treatment strategy he or she is most familiar with. Transfemoral, transarterial embolization with coils, Onyx, glue, WEB (Microvention), and MVP Amplatzer (Medtronic) are all reasonable options. We favor one embolization of the main feeder with high-concentration glue as described by Berenstein and Lasjaunias.^2,3

Type II: Mural Complex

The type II VoGM (mural complex) is defined by its mainly large feeders converging mostly on a single fistulous connection. Despite its often complex appearance, it still represents a mural lesion, not a choroidal network. The large feeders present relatively safe targets for embolization, in contrast to the smaller feeders of the choroidal types. The larger feeders of the type II VoGM converge on a common pouch within the wall of the malformation, a fistulous point in the sense of William Hunter.¹ The interventionalist can gradually (in multiple stages) close off this fistulous point. Nonetheless, the lesions are often high flow and already threaten the life of the newborn patient. Emergent intervention is required in approximately two-thirds of the patients. NBCA via transarterial approach, best transumbilical, is the treatment of choice. Outcome is less favorable than that of type I VoGMs.

Type III: Choroidal

The majority of VoGMs fall into this group. The main feature of the type III choroidal VoGM is the presence of multiple fistulous connections, complicating the treatment. Type III has been described previously in detail by Yaşargil as a type III and by Lasjaunias as “choroidal.”^3,6,7 Smaller vessels cannot be catheterized and embolized safely. Therefore, the transvenous approach comes into play. Lasjaunias, however, had unfavorable outcomes (death) in the only two patients (<1%) he treated via the transvenous route after multiple successful transarterial embolizations.³ Nonetheless, embolization of the venous pouch, via either transvenous or transarterial access, can be a successful treatment option.^29,30,32 It should also be recognized that some patients may not need treatment at all (see illustrative case for type III). Lasjaunias reports spontaneous occlusion of even choroidal VoGMs.³ The primary goal is still embolization of the main feeders to manage heart failure in the newborn and hydrocephalus in the infant. Overall, the choroidal AVMs are defined by a mix of larger and smaller feeders. Due to its complexity, often more treatment sessions are required, increasing the risk compared with mural VoGMs. Families need to be counseled accordingly.

Type IV: Choroidal With Deep Venous Drainage

The key element of the type IV choroidal VoGM is its engorged deep venous drainage system, which appears as a “3” or “ε” on lateral angiography. The enlarged thalamostriate vein can also be noted on axial T2 MRI inside the lateral ventricle. This enlarged thalamostriate vein indicates the impending melting brain syndrome. Currently, there is no established treatment for this type of VoGM. Palliative care and transition to comfort care without intervention are accepted. An attempt at prenatal treatment³⁵ or transvenous embolization against all odds is unlikely to succeed.

Limitations

This retrospective, qualitative study has two major limitations. First, the case numbers are small. VoGMs are exceptionally rare with an incidence of only 1 in 3 million.^36–51 Therefore, we elected to supplement our case series with additional cases from the literature to further characterize VoGMs subtypes I–IV in greater detail. This constitutes the second major limitation, namely selection bias, because authors generally tend to report their better cases only. Consequently, the outcomes reported for types I–III may be worse in the general population than reported here. Similarly, complication rates and rates of developmental delay may be higher than reported in this summary. Nonetheless, the addition of the reviewed cases from the literature supports the correlation of the different VoGM subtypes with a specific treatment and prognosis. Outcome progressively worsens from grade I to grade IV; treatment urgency and complexity escalate with increasing grades.

Lessons

There is great variability among patients with VoGMs regarding their needs and projected outcomes. The spectrum ranges from relatively straightforward elective treatment with normal development for VoGM type I (mural simple) to 100% mortality despite emergent treatment for VoGM type IV (choroidal with deep venous drainage). This report aims to provide practical guidance to providers tackling these highly complex lesions and set expectations for outcome.

Disclosures

The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

Author Contributions

Conception and design: EF Hauck, Yarden, LI Hauck. Acquisition of data: EF Hauck, Yarden, Bibawy, Mirshahi. Analysis and interpretation of data: EF Hauck, Yarden, Bibawy, Mirshahi, Grant. Drafting the article: EF Hauck, Yarden. Critically revising the article: EF Hauck, Yarden, Bibawy, Grant. Reviewed submitted version of manuscript: EF Hauck, Mirshahi, Grant. Approved the final version of the manuscript on behalf of all authors: EF Hauck. Statistical analysis: EF Hauck. Administrative/technical/material support: EF Hauck. Study supervision: EF Hauck. Collaboration with the artist and figure creation: EF Hauck.

Supplemental Information

Previous Presentations

This work was previously presented as an oral presentation at Neurosurgery in Jackson Hole, Jackson Hole, WY, on March 1, 2023.