Cerebrovascular Surgery and Interventions Center | Selected papers

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Weinstock P; et al. Optimizing cerebrovascular surgical and endovascular procedures in children via personalized 3-dimensional printing. J Neurosurg Pediatr. 2015 Nov 1;16(5):584-9.
A small case series describes the use of 3D printing models based on patients’ brain scans to prepare for cerebrovascular procedures. A collaboration between the Cerebrovascular Surgery and Interventions Center and Boston Children’s SimPeds program.

Burch EA; Orbach DB. Pediatric CNS vascular anomalies. Pediatric Radiology. 2015Sep 1;45(3):463-72.

Lin N; et al. Safety of neuroangiography and embolization in children: Complication analysis in 697 consecutive procedures in 394 patients. J Neurosurg Pediatr. 2015 Oct 1; 16 (4):432-8.

 Gross BA; Orbach DB. Addressing challenges in 4 F and 5 F arterial access for neurointerventional procedures in infants and young children. J Neurointerv Surg 2014 May; 6:308-313.

With creative strategies for accessing tiny vessels, complex endovascular neurointerventions are possible even in the youngest infants.

Orbach DB; et al. Neurointerventions in children: Radiation exposure and its import. AJNR 2013 [epub before print]; doi: 10.3174/ajnr.A3758
Although neurointerventional procedures have dramatically improved the prognosis of serious cerebrovascular conditions, physicians need to be mindful of the risk for secondary tumors, particularly in very young patients and those undergoing multiple procedures.


Penn DL; et al. Multimodal treatment approach in a patient with multiple intracranial myxomatous aneurysms. Journal of Neurosurgery: Pediatrics. 2018 Mar 1;21(3):315-21.

Gross BA, Smith ER, Scott RM, Orbach DB. Intracranial aneurysms in the youngest patients: Characteristics and treatment challenges. Pediatr Neurosurg. 2015;50(1):18-25. 

Arteriovenous malformations (AVMs)

Koch MJ; et al. Dynamic Changes in Arteriovenous Malformations (AVMs): Spontaneous Growth and Resolution of AVM-Associated Aneurysms in Two Pediatric Patients. Pediatric neurosurgery. 2019 Oct;54(6):394-8.

Review of two pediatric cases with de nova development of AVM-associated aneurysms, highlighting risks of AVMs and treatment measures.

Penn DL; et al. General Principles for Preoperative Planning and Microsurgical Treatment of Pediatric Brain Arteriovenous Malformations: 2-Dimensional Operative Video. Operative Neurosurgery. 2019 Apr 1;16(4):E114-.

Smith ER. Structural causes of ischemic and hemorrhagic stroke in children: moyamoya and arteriovenous malformations. Current opinion in pediatrics. 2015 Dec 1;27(6):706-11.

Gross BA; et al. Microsurgical treatment of arteriovenous malformations in pediatric patients: the Boston Children's Hospital experience. J Neurosurg Pediatr 2015 Jan; 15:71-7.
A review of 94 children with cerebral AVMs who underwent microsurgical resection finds high efficacy and low complications.

Chandra RV; et al. Transarterial embolization of mandibular arteriovenous malformations using ONYX.Oral Maxillofac Surg. 2014 Aug 1;72(8):1504-10.
A chart review of three patients with facial AVMs who had endovascular embolization procedures with ONYX showed good results, and all patients were able to avoid surgery.

 Ellis MJ; et al. Angioarchitectural features associated with hemorrhagic presentation in pediatric cerebral arteriovenous malformations. J NeuroIntervent Surg 2013; 5:191-5.

Distinguishing the particular features of AVMs that cause hemorrhage in children greatly aids in managing patients with these lesions.

Thiex R; et al. The use of Onyx for embolization of central nervous system arteriovenous lesions in pediatric patients. AJNR 2010; 31:112-20.
This patient series, the largest to date, finds that Onyx embolization can be effective and safe in children. The paper points out technical aspects that render Onyx injection safe, as well as predictors of potential hazards.

Thiex R; et al. A novel association between RASA1 mutations and spinal arteriovenous anomalies. Am J Neuroradiol 2010; 31:775-9.
This collaborative study finds a novel association between RASA1 mutations and spinal arteriovenous anomalies (AVMs and AVFs) in a series of patients who were also born with capillary malformations.

Arteriovenous fistulae (AVFs)

See AP, Kochis MA, Orbach DB. Republished: Segmental upper mid-basilar artery sacrifice in a child using a Micro Vascular Plug device for treatment of a basilar arteriovenous fistula compressing the brainstem. Journal of neurointerventional surgery. 2017 Oct 1;9(10):e37.

Ashour R, Orbach DB. Lower vertebral-epidural spinal arteriovenous fistulas: a unique subtype of vertebrovertebral arteriovenous fistula, treatable with coil and Penumbra Occlusion Device embolization. Journal of neurointerventional surgery. 2016 Jun 1;8(6):643-7.

Gross BA; et al. Dural arteriovenous shunts in children. J Pediatr Neuroradiol 2013 Oct 2:263-8.
A review of dural AVFs in children, including subtypes (dural sinus malformations, infantile dural arteriovenous shunts, and adult-type dural arteriovenous shunts) and prognosis.

Walcott BP; et al. Dural arteriovenous fistulae in pediatric patients: associated conditions and treatment outcomes. J NeuroIntervent Surg 2013; 5:6-9.

Walcott BP; et al. Pial arteriovenous fistulae in pediatric patients: associated syndromes and treatment outcome. J NeuroIntervent Surg 2013; 5:10-4.

Thiex R; et al. A novel association between RASA1 mutations and spinal arteriovenous anomalies. Am J Neuroradiol 2010; 31:775-9.
This collaborative study finds a novel association between RASA1 mutations and spinal arteriovenous anomalies (AVMs and AVFs) in a series of patients who were also born with capillary malformations.

Cavernous malformations (CMs)

Gross BA, Du R, Orbach DB, Scott RM, Smith ER. The natural history of cerebral cavernous malformations in children. J Neurosurg Pediatr. 2015:1-6. PMID: 26474098.

Gross BA; et al. Cavernous malformations of the basal ganglia in children. J Neurosurg Pediatr 2013; 12:171-4.
This series of 180 patients treated at Boston Children’s confirms a benign natural history for small, asymptomatic CMs of the basal ganglia. Children with larger, symptomatic CMs had surgical removal with good results and no re-bleeding or late neurological deterioration.

Gross BA; et al. Resection of supratentorial lobar cavernous malformations in children. J Neurosurg Pediatr 2013; 12:367-73.
This review of 181 children with lobar CMs supports the use of surgery for those with symptomatic supratentorial lobar CMs, and observation for those with smaller asymptomatic lesions located within “eloquent” parts of the brain responsible for essential functions.

Singla A; et al. Cavernous malformations of the brain after treatment for acute lymphocytic leukemia: presentation and long-term follow-up. J Neurosurg Pediatr 2013; 11:127-3.
Symptomatic, particularly aggressive CMs may develop in the brain several years after chemotherapy and cranial radiation treatment for acute lymphocytic leukemia (ALL). Children treated for ALL should receive long-term monitoring for CMs, as removing them surgically appears to protect against repeated bleeding and neurological deficits.

Dural sinus malformations

Yang E; et al. Imaging features and prognostic factors in fetal and postnatal torcular dural sinus malformations, part I: review of experience at Boston Children’s Hospital. Journal of neurointerventional surgery. 2018 May 1;10(5):467-70.

Yang E; et al. Imaging features and prognostic factors in fetal and postnatal torcular dural sinus malformations, part II: synthesis of the literature and patient management. Journal of neurointerventional surgery. 2018 May 1;10(5):471-5.

Orbach DB; et al. Pathogenesis of dural sinus malformations as demonstrated by fetal imaging: a decision-making crucible for parents and clinicians. J Neurointerv Surg 2014 Jul; 6 Suppl 1:A69-70.
A study of all 9 cases of dural sinus malformation seen prenatally over the past five years by Boston Children’s Hospital’s Advanced Fetal Care Center.

Moyamoya disease

Fehnel K.P.; et al. Indirect bypass for maternal symptomatic moyamoya in the first trimester of pregnancy: case report. Journal of Neurosurgery: Pediatrics, 2019 Nov; 1(aop), pp.1-6.

Pinard A; et al. The pleiotropy associated with de novo variants in CHD4, CNOT3, and SETD5 extends to moyamoya angiopathy. Genetics in Medicine. 2019 Sep 2:1-5.

Riordan CP; et al. Results of more than 20 years of follow-up in pediatric patients with moyamoya disease undergoing pial synangiosis. Journal of Neurosurgery: Pediatrics. 2019 Mar 1;23(5):586-92.

Penn DL; et al. General principles for pial synangiosis in pediatric moyamoya patients: 2-dimensional operative video. Operative Neurosurgery. 2019 Jan 1;16(1):E14-5.

Ganesan V; et al. Moyamoya: defining current knowledge gaps. Dev Med Child Neurol 2015 Feb 13 (DOI: 10.1111/dmcn.12708).

See P; et al. Down syndrome and moyamoya: Clinical presentation and surgical management. J Neurosurg Pediatr. 2015 Jul 1;16(1):58-63.

Baird LC; et al. Moyamoya syndrome associated with Alagille syndrome: Outcome after surgical revascularization. J Pediatr 2015 Feb; 166:470-3.
A case report of five children with ALGS with moyamoya who underwent revascularization surgery.

 Jackson EM; et al. Pial synangiosis in patients with moyamoya less than 2 years of age. J Neurosurg Pediatr 2014 Apr; 13:420-5.

A review of 34 procedures over a period of 12 years finds that the majority of children who have pial synangiosis for moyamoya disease before age 2 have a good long-term prognosis.

Gross BA; et al. Occipital pial synangiosis. Acta Neurochir (Wien) 2014 Jul; 156:1297-300.
An illustrated, instructional video demonstration in a 5-year-old child shows that pial synangiosis can be successfully applied to moyamoya affecting the posterior circulation.

Lin N; et al. Treatment of moyamoya disease in the adult population with pial synangiosis. J Neurosurg 2014 Mar; 120:612-7.
A review of 66 procedures from 1985 to 2010 finds pial synangiosis to be a safe and effective way to provide more blood to the brain in adults with moyamoya disease.

Smith ER; Scott RM. Spontaneous occlusion of the circle of Willis in children: Pediatric moyamoya summary with proposed evidence-based practice guidelines. J Neurosurg Pediatrics 2012; 9:353-60.
This review summarizes current studies of pediatric moyamoya disease, proposing a framework for evidence-based treatment guidelines.

Lin N; et al. Discovery of asymptomatic moyamoya arteriopathy in pediatric syndromic populations: radiographic and clinical progression. Neurosurg Focus 2011; 31(6):E6.
Data on 418 patients who underwent surgical revascularization demonstrate that moyamoya is a progressive disorder, even when it does not cause symptoms, and supports the use of early surgical intervention to minimize morbidity from stroke. 

Scott RM; Smith ER. Moyamoya disease and moyamoya syndrome [review]. N Engl J Med 2009; 360:1226-37.
This comprehensive review of moyamoya disease covers who is at risk, presenting features, diagnosis, treatment and outcomes.

Smith ER; et al. Pial synangiosis in patients with moyamoya syndrome and sickle cell anemia: perioperative management and surgical outcome. Neurosurg Focus 2009; 26:E10.
In this series of 12 patients with sickle-cell disease and moyamoya syndrome, 11 of whom had a prior stroke, pial synangiosis appears to be safe and confers long-lasting protection against further stroke.


Ferriero DM; et al. Management of stroke in neonates and children: a scientific statement from the American Heart Association/American Stroke Association. Stroke. 2019 Mar;50(3):e51-96.

See AP; et al. Considerations in applying a new stent retriever in pediatric endovascular cerebral thrombectomy for acute ischemic stroke. Pediatric neurosurgery. 2016 May ;51(5):263-8.

Akgoz A; et al. Interventional management of acute ischemic stroke in children. J Pediatr Neuroradiol 2013 Oct 2:269-75.
A review of endovascular treatments (intra-arterial thrombolysis and mechanical thrombectomy) for pediatric acute ischemic stroke, including cautions in extrapolating from results in adults. 

Ellis MJ; et al. Endovascular therapy in children with acute ischemic stroke. Neurology 2012; 79(13 Suppl 1):S158-64.
This review identified 34 cases of pediatric acute ischemic stroke described in the medical literature to date that were treated with endovascular thrombolysis. Until further safety and efficacy are available, the authors call for a considered and cautious approach by a neurointerventional team in close collaboration with pediatric stroke neurologists or, when not available, with a team including a pediatric neurologist and adult stroke neurologist. 

Roach ES; et al. Management of stroke in infants and children: A scientific statement from a special writing group of the American Heart Association Stroke council and the Council on Cardiovascular Disease in the Young [AHA Scientific Statement]. Stroke 2008; 39:2644-91.
This report from an AHA writing group, led by Michael Scott, MD, and Edward Smith, MD, of Boston Children’s, provides evidence-based recommendations for the prevention of ischemic stroke caused by moyamoya disease, arterial dissection in the head or neck, cardiogenic embolism and other causes, and provides recommendations on evaluating and managing hemorrhagic stroke.

Vein of Galen Malformations

Arko L, Lambrych M, Montaser A, Zurakowski D, Orbach, DB. Fetal and neonatal MRI predictors of aggressive early clinical course in vein of Galen malformation. Am Journal Neurorad Jun 2020, 41 (6) 1105-1111. This study found that early neonatal and fetal MRI can accurately predict which fetuses with vein of Galen malformations will go on to have heart failure after birth.

Duran D; et. al. Mutations in Chromatin Modifier and Ephrin Signaling Genes in Vein of Galen Malformation. Neuron. 2019 02 06; 101(3):429-443.e4

Duran D; et. al. Human genetics and molecular mechanisms of vein of Galen malformation. J Neurosurg Pediatr. 2018 04; 21(4):367-374.

Click here for a complete list of publications by Edward Smith, MD.
Click here for a complete list of publications by Darren Orbach, MD, PhD.