Pediatric non-galenic pial arteriovenous fistula's characteristics and outcomes: a systematic review.

INTRODUCTION: Pediatric non-galenic pial arteriovenous fistulas (pAVFs) are rare vascular malformations that are characterized by a pial arterial-venous connection without an intervening capillary bed. Outcomes and treatment strategies for pAVFs are highly individualized, owing to the rarity of the disease and lack of large-scale data guiding optimal treatment approaches. METHODS: We performed a systematic review of pediatric patients (< 18 years at diagnosis) diagnosed with a pAVF by digital subtraction angiogram (DSA). The demographics, treatment modalities, and outcomes were documented for each patient and clinical outcome data was collected. Descriptive information stratified by outcome scores were classified as follows: 1 = excellent (no deficit and full premorbid activity), 2 = good (mild deficit and full premorbid activity), 3 = fair (moderate deficit and impaired activity), 4 = poor (severe deficit and dependent on others), 5 = death. RESULTS: A total of 87 studies involving 231 patients were identified. Median age at diagnosis was 3 years (neonates to 18 years). There was slight male preponderance (55.4%), and 150 subjects (81.1%*) experienced excellent outcomes after treatment. Of the 189 patients treated using endovascular approaches, 80.3% experienced excellent outcomes and of the 15 patients surgically treated subjects 75% had an excellent outcome. The highest rate of excellent outcomes was achieved in patients treated with Onyx (95.2%) and other forms of EvOH (100%). High output heart failure and comorbid vascular lesions tended to result in worse outcomes, with only 54.2% and 68% of subjects experiencing an excellent outcome, respectively. *Outcomes were reported in only 185 patients. CONCLUSION: pAVFs are rare lesions, necessitating aggregation of patient data to inform natural history and optimal treatment strategies. This review summarizes the current literature on pAVF in children, where children presenting with heart failure as a result of high flow through the lesion were less likely to experience an excellent outcome. Prospective, large-scale studies would further characterize pediatric pAVFs and enable quantitative analysis of outcomes to inform best treatment practices.

The role of surgical disconnection for posterior fossa pial arteriovenous fistulas and dural fistulas with pial supply: an illustrative case series.

BACKGROUND: Pial arteriovenous fistulas (pAVFs) are rare vascular malformations characterized by high-flow arteriovenous shunting involving a cortical arterial supply directly connecting to venous drainage without an intermediate nidus. Dural arteriovenous fistulas (dAVFs) can infrequently involve additional pial feeders which can introduce higher flow shunting and increase the associated treatment risk. In the posterior fossa, arteriovenous fistula (AVF) angioarchitecture tends to be particularly complex, involving either multiple arterial feeders-sometimes from both dural and pial origins-or small caliber vessels that are difficult to catheterize and tend to be intimately involved with functionally critical brainstem or upper cervical cord structures. Given their rarity, published experience on microsurgical or endovascular treatment strategies for posterior fossa pAVFs and dAVFs with pial supply remains limited. METHODS: Retrospective chart review from 2019-2023 at a high-volume center identified six adult patients with posterior fossa pAVFs that were unable to be fully treated endovascularly and required microsurgical disconnection. These cases are individually presented with a technical emphasis and supported by comprehensive angiographic and intraoperative images. RESULTS: One vermian (Case 1), three cerebellopontine angle (Cases 2-4) and two craniovertebral junction (Cases 5-6) posterior fossa pAVFs or dAVFs with pial supply are presented. Three cases involved mixed dural and pial arterial supply (Cases 1, 4, and 6), and one case involved a concomitant microAVM (Case 2). Endovascular embolization was attempted in four cases (Cases 1-4): The small caliber and tortuosity of the main arterial feeder prevented catheterization in two cases (Cases 1 and 3). Partial embolization was achieved in Cases 2 and 4. In Cases 5 and 6, involvement of the lateral spinal artery or anterior spinal artery created a prohibitive risk for endovascular embolization, and surgical clip ligation was pursued as primary management. In all cases, microsurgical disconnection resulted in complete fistula obliteration without evidence of recurrence on follow-up imaging (mean follow-up 27.1 months). Two patients experienced persistent post-treatment sensory deficits without significant functional limitation. CONCLUSIONS: This illustrative case series highlights the technical difficulties and anatomical limitations of endovascular management for posterior fossa pAVFs and dAVFs with pial supply and emphasizes the relative safety and utility of microsurgical disconnection in this context. A combined approach involving partial preoperative embolization-when the angioarchitecture is permissive-can potentially decrease surgical morbidity. Larger studies are warranted to better define the role for multimodal intervention and to assess associated long-term AVF obliteration rates in the setting of pial arterial involvement.

Expect the unexpected: a case of spontaneous thrombosis of a pial arteriovenous fistula in a preterm newborn with review of the literature.

INTRODUCTION: Pial arteriovenous fistulas (pAVF) are rare vascular malformations, especially in children and newborns. In neonates, the most common symptom is congestive heart failure. CASE PRESENTATION: We report a case of an asymptomatic preterm newborn incidentally diagnosed with pAVF during a routine cranial ultrasound (cUS) on the third day of life. Cerebral magnetic resonance (MRI) confirmed the diagnosis. A wait-and-see approach was chosen by the multidisciplinary team. The cUS and the MRI on day 14 of life showed the spontaneous resolution of the lesion. CONCLUSIONS: This case underlines the challenges in identifying pAVF in the first weeks of life and demonstrates a possible positive outcome for affected neonates.

Mixed pial and dural arteriovenous fistula after craniotomy: case report and literature review.

Dural arteriovenous fistula (AVF) is known to occur after craniotomy, but mixed pial and dural AVF after craniotomy has not been reported. A 45-year-old man who had undergone surgical clipping of an unruptured aneurysm 2 years previously presented with small subcortical hemorrhage from mixed pial and dural AVF. Surgical disconnection could not be cured completely due to the granulomatous tissue around the aneurysm, and the presence of an undetected shunt. Postoperative digital subtraction angiography showed a new pial AVF supplied by the middle cerebral artery pial branches. Many branches were associated with the remnant aneurysm and pial AVF, so we did not try to embolize the fistula. Gamma knife surgery was performed as adjuvant radiotherapy, which achieved angiographically complete occlusion of the shunt points. Multimodal approaches including surgery, endovascular intervention, and radiotherapy are needed for radiological and clinical cure of mixed pial and dural AVF. Long-term follow up is essential.

A case of craniocervical junction pial arteriovenous fistula causing postoperative medullary and spinal cord edema.

OBJECTIVES: Pial arteriovenous fistulas (pAVFs) are direct connections between the pial artery and vein without an intervening nidus. We report a rare case of craniocervical junction (CCJ) pAVF causing medullary and spinal cord edema resulting from surgical removal of the varix with remnant shunt after coil embolization. CASE DESCRIPTION: A 16-year-old man presented with subarachnoid hemorrhage. Digital subtraction angiography revealed a CCJ pAVF with multiple fistulas at the 2 varices (varix A and varix B), which was fed by the bilateral lateral spinal arteries and anterior spinal artery (ASA), and drained into the median posterior vermian vein with varix (varix C) and anterior spinal vein (ASV). Varices A and B were embolized using coils, but the shunts remained in varix C. Then, varix C was surgically removed. After this operation, medullary and spinal cord edema occurred. Digital subtraction angiography showed the ASV drainage responsible for edema. Finally, surgical removal of varices A and B was performed. However, arteriovenous shunts, supplied by the ASA and drained into the ASV via the intrinsic vein, were found in the medulla oblongata and coagulated, resulting in disappearance of edema. CONCLUSIONS: Edema was probably caused by concentration of drainage from the arteriovenous shunt in the medulla oblongata into the ASV by surgical removal of varix C acting as another draining route. High flow AVF can induce angiogenesis and secondary arteriovenous shunt. Precise analysis of the angioarchitecture is important to treat such cases without complications.

Occult Brain Arteriovenous Malformation Superimposed on a Pial Arteriovenous Fistula: Case Report.

INTRODUCTION: Pial arteriovenous fistulas are characterized by an abnormal connection between an intracranial artery and vein without an intervening nidus. Their predominant symptoms largely arise from mass effect, shunting, or hemorrhage. Most conservatively managed cases progress to death, but endovascular and/or surgical intervention is often successful. CASE PRESENTATION: We present the unique case of a 15-year-old girl with spontaneous intracranial hemorrhage from a single-vessel arteriovenous fistula. Although preoperative imaging failed to show a distinct nidus, intraoperative indocyanine green angiography performed after successful clipping of the primary fistulous site revealed residual shunting from a peri-lesional arteriovenous malformation. DISCUSSION/CONCLUSION: This case demonstrates the importance of intraoperative imaging and meticulous circumferential inspection of these lesions to detect residual vascular shunting.

Pediatric pial arteriovenous fistula located at the bottom of the callosal sulcus presenting with intraventricular hemorrhage: a case report and literature review.

BACKGROUND: A pial arteriovenous fistula (pAVF) is a rare condition characterized by a direct connection between a cerebral artery and cerebral vein without an intervening nidus. The prognosis is poor in untreated cases with hemorrhagic manifestations, indicating that surgical treatment is desirable. We describe a successful endovascular treatment for a pediatric case of ruptured pAVF located at the bottom of the sulcus. CLINICAL DESCRIPTION: An 11-year-old girl presented with severe headache and mild disturbance of consciousness. Head computed tomography showed hemorrhage in the callosal sulcus and ventricle. Cerebral angiography showed an arteriovenous shunt without a nidus. The branching artery from the pericallosal artery was connected directly to the thalamostriate vein without varix, and the shunt point was located at the bottom of the callosal sulcus. The patient was diagnosed with pAVF involving a single feeder and single drainer. Emergency endovascular transarterial embolization was performed using 20% N-butyl cyanoacrylate, and the shunt disappeared completely without complications. The patient was discharged with no neurological deficits. CONCLUSION: Endovascular treatment is feasible, safe, and effective for pediatric cases of deeply located pAVF.

Congenital pial AVF along the falx cerebri with complete agenesis of the corpus callosum and bilateral parasagittal pachygyria-polymicrogyria secondary to chronic ischemia.

OBJECTIVE: Pial arteriovenous fistula (AVF) is an extremely rare entity due to direct arterial connection with the venous plexus without an intervening capillary network. The objective of this article is to describe a unique case of congenital pial AVF along the interhemispheric falx with complete callosal agenesis and malformation of cortical development within the bilateral anterior cerebral artery territories. We also demonstrated the distinctive feature of temporal stability of the extensive intracranial abnormalities without active intervention. Less than 100 cases have been reported thus far, most of which involve the adult rather than pediatric age group. A comprehensive literature review of congenital pial AVF will also be included. CASE DESCRIPTION: A 5-year-old child presented with headache and complex partial seizures. Imaging of the brain revealed the presence of polymicrogyria-pachygyria in the parasagittal frontoparietal lobes with associated underlying white matter hypodensities. Complete agenesis of the corpus callosum was also seen. In addition, enlarged and tortuous vessels were noted along the interhemispheric falx with no appreciable nidus. Bilateral dilated and tortuous ACAs were seen supplying the network of abnormal vessels along the falx. The radiological findings were stable on a follow-up MRI 12 years later. CONCLUSION: Our reported case adds to current limited knowledge of this rare entity in the pediatric age group, which is traditionally treated aggressively and urgently. Our case demonstrated temporal stability of this lesion with no detrimental complications observed. This suggests that the outcome of pial AVFs with conservative treatment may not be as grim as previously thought.

Pial Arteriovenous Fistula and Capillary Malformation-Arteriovenous Malformation Associated with RASA1 Mutation: 2 Pediatric Cases with Successful Surgical Management.

We present case reports of 2 pediatric patients who were both found to have pial arteriovenous fistulas (AVFs) with subsequent genetic analysis revealing mutations in the RASA1 gene. Considering their family history of distinct cutaneous lesions, these mutations were likely inherited as opposed to de novo mutations. Patient 1 had large capillary malformations on the left side of the face and neck, associated with macrocephaly, and presented at the age of 32 months with speech delay, right-sided weakness, and focal seizures involving the right side of the body. Patient 2 presented with proptosis at the age of 9 months, but was otherwise neurologically intact. Given the chance for definitive single-stage control of vascular shunt (obviating chances for radiation exposure with endovascular treatment) and surgically accessible location of these intracranial lesions, both patients were treated with surgery with excellent clinical and radiological outcome. In general, given the high mortality secondary to severe congestive heart failure when treated conservatively, the goal of treatment in cortical AVF in young children, even when asymptomatic, is rapid control of the shunt. This was achieved successfully in our cases - both patients experienced significant symptomatic improvement following surgery and remained neurologically stable in the subsequent follow-up visits.

Multiple Dural and Pial Arteriovenous Fistulae in a Twenty-Four-Year-Old Woman in the Setting of Superior Sagittal Sinus Thrombosis: Case Report and Review of Literature.

CASE: A 24-year-old woman presented with headache, nausea, and vomiting, and was found to have chronic superior sagittal sinus (SSS) thrombosis and multiple dural arteriovenous fistulae (dAVFs). Despite anticoagulant therapy and successful recanalization of her sinus, her fistulae persisted, and she developed additional separate pial arteriovenous fistulae (pAVFs). Her fistulae were treated with staged endovascular embolization, open clipping, and gamma knife radiosurgery over the course of 10 months. Complete resolution of SSS thrombosis and all arteriovenous fistulae (AVFs) was noted on cerebral angiogram performed 18 months from initial presentation. DISCUSSION: dAVFs have frequently been associated with venous sinus thrombosis. Sinus thrombosis resulting after endovascular or surgical treatment of dural arteriovenous fistulous connections has been reported in literature and is considered a possible complication of treatment. Multiple dAVFs and pAVFs are rare and often require multimodal staged approaches for definitive treatment. CONCLUSION: We report a case of chronic sagittal sinus thrombosis resulting in multiple AVFs requiring staged multimodal treatment with successful resolution of the fistulous connections. Furthermore, upon reviewing the literature addressing multiple dAVFs and the treatment of such lesions using endovascular, microsurgical, and stereotactic radiosurgery techniques, we elucidate the success a multimodal approach to therapy can afford for the unique challenges associated with multiple lesions.

Infratentorial Pial Arteriovenous Fistula in the Elderly.

BACKGROUND: Intracranial pial arteriovenous fistulas (pAVFs) are rare vascular lesions, which may present with, but are distinct from arteriovenous malformation and dural arteriovenous fistula. They most often manifest during infancy or early childhood, but rarely in adulthood. METHODS: We report an exceptionally rare case of infratentorial pAVF in a 73-year-old man, who presented with progressive gait disturbance due to cerebellar edema resulting from arteriovenous shunts. RESULTS: The patient was successfully treated by endovascular flow reduction followed by surgical extirpation of the fistula. The diagnosis was confirmed by pathological findings. CONCLUSIONS: Pial arteriovenous fistula is rare, but can occur in the elderly. Combination of endovascular flow reduction and surgical disconnection yielded an excellent clinical outcome.

Spontaneous occlusion of a pial arteriovenous fistula after angiography: The role of iodinated contrast media.

Intracranial non-galenic pial arteriovenous fistula (PAVF) is an extremely rare vascular malformation, where one or more pial arteries feeds directly into a cortical vein without any intervening nidus. Though occasionally they can be asymptomatic, neurological symptoms such as headache, seizure, or focal neurological deficit are more common presenting features. Life threatening or fatal hemorrhage is not uncommon, hence needed to be treated more often than not. Spontaneous occlusion of PAVF is reported only four times before. We report a 49-year-old gentleman, who was diagnosed to have a PAVF, possibly secondary to trauma. He presented 5 months and 22 days from initial digital subtraction angiography (DSA) for treatment, and follow-up angiogram showed complete obliteration. He denied any significant event, medication or alternate treatment during this period. His clinical symptoms were stable as well. We postulate iodinated contrast medium induced vasculopathy as a possible cause, which has been described for other vascular pathologies, but never for PAVF.

Treatment of filum terminale pial arteriovenous fistulas in the sacral region: A case report and systematic review.

Spinal filum terminale pial arteriovenous fistulas (FT PAVFs) are uncommon. Most FT PAVFs are located in the lumbar region; far fewer are located in the sacral region. Due to the rarity of sacral FT PAVFs, the precise surgical dissection and removal of these lesions are challenging. Here, we report an FT PAVF in the sacral region. The patient was a 45-year-old male who suffered from progressive weakness and numbness of the bilateral lower limbs; his symptoms gradually worsened. Digital subtraction angiography (DSA) showed an AVF at the sacral canal at the S3-4 level. Microsurgical treatment with intraoperative DSA was performed, and the FT PAVF was resected. After the operation, the patient gradually recovered. Follow-up magnetic resonance imaging revealed a recession in the dilation of the spinal cord venous plexuses. A literature review was also performed, and a total of 14 FT PAVFs of the sacral region were identified. The patients identified in the literature review had an average age of 58.9 ± 12.9 years, and 92.9% of the patients were male. Spinal cord edema was present in 85.7% of the FT PAVF patients. Regarding treatment, 64.3% of the FT PAVF patients underwent microsurgical resection, 28.6% patients underwent endovascular treatment, and 7.1% patients underwent a hybrid operation; good outcomes were achieved with all three methods. Therefore, FT PAVF of the sacral region is a unique lesion whose angioarchitecture needs to be identified carefully; prompt treatment is necessary, and microsurgery can yield good outcomes.

Adult Infratentorial Pial Arteriovenous Fistula Treated with Detachable Coils: A Case Report and a Review of Literature.

An appropriate therapeutic option for pial arteriovenous fistula (PAVF) can vary according to the angioarchtecture of the lesion. We present a case of adult infratentorial PAVF treated by transarterial coil embolization. A 26-year-old man was referred to our institution for an asymptomatic intracranial vascular lesion. Cerebral angiograms revealed PAVF fed by three arteries in the right cerebellomedullary cistern. The feeding arteries were accurately identified by three-dimensional rotational angiography and were successfully embolized using coils while normal arterial flow was preserved. This case report suggests that stepwise transarterial coil embolization can cure PAVF under detailed evaluation of its angioarchitecture.

Embolization of cerebellar pial arteriovenous fistula presented with congestive edema of the brainstem and cervical cord.

Cerebellar pial arteriovenous fistula (PAVF) in adults presenting with congestive edema of the brainstem and cervical cord has rarely been reported. Here, we report such a case. A 59-year-old man presented with progressive weakness and numbness of the limbs and hiccups for 6 months. On physical examination, his limbs had grade III muscle strength, and he was unable to stand or walk. He also had occasional incontinence and retention. There was a positive Babinski sign in both lower limbs. Below the neck, he had hypoesthesia of the skin. Magnetic resonance imaging (MRI) showed edema of the brainstem and upper cervical cord. Digital subtraction angiography (DSA) confirmed there was a cerebellar nongalenic-type PAVF lesion; the feeding arteries were the branches of the posterior inferior cerebellar artery (PICA); and the draining vein was a single cortical vein that ultimately entered the venous systems of the brainstem and upper cervical cord. Casting Onyx-18 via the PICA obliterated the PAVF. Postoperatively, the patient recovered gradually. Two and a half months later, he could walk using a crutch and had no incontinence. Six-month follow-up MRI and DSA revealed recession of the edema of the brainstem and cervical cord and no recurrence of this PAVF. One year later, he was nearly normal. In conclusion and extremely rarely, cerebellar nongalenic-type PAVF can present with congestive edema of the brainstem and high cervical cord. This treatment by Onyx-18 embolization can resolve PAVF.

Craniocervical Junction Dural Arteriovenous Fistula and Pial Arteriovenous Fistula Presenting Concomitantly in Separate Locations with Subarachnoid Hemorrhage.

Craniocervical junction dural arteriovenous fistula and pial arteriovenous fistula are rare cerebrovascular lesions. While their pathophysiology is different, both conditions can cause intracranial hemorrhage attributable to venous congestion. We present, to our knowledge, the first case report of craniocervical junction dural arteriovenous fistula and pial arteriovenous fistula presenting concomitantly in separate locations with subarachnoid hemorrhage. This case appears to have been due to increased venous hypertension caused by a merging of the venous drainage of the 2 lesions, resulting in hemorrhage.

Pial arteriovenous fistula associated with vein of Galen dilatation in adult: A case report and MRI findings.

Pial arteriovenous fistula (PAVF) is a rare intracranial vascular lesion where direct communication exists between one or more pial arteries and a cerebral vein, without an intervening nidus and located in the subpial meningeal space. When the drainage of PAVF involves a dilated, but already formed vein of Galen (VOG), it should be distinguished from other vascular lesions located in this area, because their angio-architecture, natural history and treatment options are different. A 33-year-old female was admitted to our hospital with a history of new-onset generalized tonic-clonic seizures. Clinical examination showed no neurological deficit. Magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) depicted an arteriovenous fistula that was fed by the pial branches from left posterior cerebral artery and drained into the medial atrial vein before joining the VOG confluence and causing VOG dilatation. No nidus between the feeding arteries and draining vein, dural feeding arteries, or anatomical variations commonly seen with true vein of Galen aneurysmal malformations (VOGM) were found. These finding suggested a diagnosis of a PAVF associated with vein of Galen dilatation, which was confirmed by digital subtraction angiography. The patient was treated with transarterial glue embolization in 1 section, resulting in nearly complete occlusion of the fistula. Conventional MRI and MRA are noninvasive modalities that can provide valuable information regarding the anatomic localization of the fistula point, the feeding arteries, the venous sac, and their relationship with surrounding structures. These techniques are helpful for accurate diagnosis and treatment planning.

Mini-Pterional Craniotomy for Microsurgical Ligation of a Sylvian Fissure Arteriovenous Fistula.

Pial arteriovenous fistulae (pAVF) are rare lesions, arising from direct fistulation between an artery and vein, with absence of a nidus. We present the surgical treatment of a 22-year-old female found to have a right middle cerebral artery (MCA) pAVF in the Sylvian fissure. The patient underwent a right mini-pterional approach, and careful dissection of the arterial feeder, venous varix and fistulation point. The fistulation point was tested with a temporary clip and intra-operative indocyanine green (ICG) videoangiography. All arterial feeders were identified and ligated. Complete obliteration was confirmed using ICG and post-operative digital subtraction angiography (DSA). The patient made a good recovery with no neurological deficits, and her pulsatile tinnitus stopped. Pial arteriovenous fistulae are rare lesions amenable for a surgical cure in the appropriate context.

Intracranial arteriovenous shunts in infants: A decade of experience from a quaternary pediatric center.

BACKGROUND AND PURPOSE: Intracranial arteriovenous shunts (IAVS) are rare vascular diseases in infants. They can be categorized into vein of Galen aneurysmal malformation (VGAM), pial arteriovenous fistula (PAVF), and dural arteriovenous fistula associated with dural sinus malformation (DAVF/DSM). We sought to review the clinical presentation, imaging characteristics, endovascular treatment (EVT), and outcomes of IAVS in infants presenting to a quaternary pediatric referral center over one decade. METHODS: A retrospective review of a prospectively maintained database was performed of all infants diagnosed with IAVS between January 2011 and January 2021 in a quaternary pediatric referral center. For each patient, demographic data, clinical presentation, imaging findings, management strategies, and outcomes were reviewed and discussed. RESULTS: Over the study period, 38 consecutive infants were diagnosed with IAVS. Patients with VGAM (23/38, 60.5%) presented with congenital heart failure (CHF) (14/23), hydrocephalus (4/23), and seizures (2/23), and three patients were asymptomatic. Eighteen patients with VGAM underwent EVT. Among those, 13 patients (72.2%) were successfully treated with an angiographic cure and three patients (3/18, 17%) died. Patients with PAVF (9/38, 23.7%) presented with CHF (5/9), intracranial hemorrhage (2/9), and seizures (2/9), and all of them were successfully treated endovascularly. Patients with Type I DAVF/DSM (4/6, 66.6%) presented with mass effect (2/4), cerebral venous hypertension (1/4), CHF (1/4), and cerebrofacial venous metameric syndrome (1/4). Patients with type II DAVF/DSM (2/6, 33.3%) presented with a thrill behind the ear. Patients with DAVF/DSM were treated endovascularly, five patients were cured, and one with type I DAVF/DSM died. CONCLUSION: Intracranial arteriovenous shunts are rare but potentially life-threatening neurovascular pathologies in infants. Endovascular treatment is challenging but feasible in carefully selected patients.

Transarterial and transvenous pial arteriovenous Fistula embolization: A video case report.

Cerebral pial arteriovenous fistulas (pAVFs) are rare and complex high-flow vascular malformations found in pediatric and adolescent populations.1 They are often divided into two groups based on the pattern of venous drainage, galenic or nongalenic. Nongalenic pAVFs are typically supratentorial and carry a high risk of rupture. Their angioarchitecture is very complex with various patterns of feeding arteries and draining veins not originating from dural vessels or the vein of Galen.2 The natural history has not been well established; however, mortality estimates range as high 63%.1 Presentations include hemorrhage, seizure, congestive heart failure, and elevated intracranial pressure.3 We describe the case of an adolescent girl with acute onset of headaches that led to the discovery of an occipital, nongalenic pAVF. Transarterial and transvenous embolizations performed during a single procedure resulted in complete obliteration of the fistula. No complications arose, and the patient remained at her neurological baseline.