Information

Related Research Units

Neurosurgery Research
Edward Smith Laboratory Research
Vascular Biology Program Research

Research Overview

Edward Smith is engaged in basic science research on primary brain tumors and cerebrovascular disease. His research is primarily centered on understanding the molecular mechanisms of tumorigenesis and angiogenesis in the central nervous system. This work has included basic science and translational studies investigating adult and pediatric brain tumors, moyamoya disease and cerebral vascular malformations. This research has implicated a class of extracellular matrix proteins, called matrix metalloproteinases (MMPs), in the advent and progression of several disease states of the central nervous system, including brain tumors, moyamoya and vascular malformations.

A major research interest, in conjunction with the lab of Marsha Moses, PhD, director of the Vascular Biology Program, is the study of pediatric cerebral arteriovenous malformations (AVMs). A number of projects have been generously supported by the Lucas Warner AVM research fund, resulting in exciting discoveries relevant to the diagnosis, understanding and treatment of these challenging lesions. Smith's research has been supported by funding from several other sources, including the National Institutes of Health, and is carried out in collaboration with a number of investigators in associated fields, particularly Moses.

Goals of Smith's work include:

  • development of a panel of non-invasive biomarkers for the detection and follow-up of neurosurgical disease;
  • development of novel therapeutic interventions for neurosurgical disease based on regulation of vascular stability;
  • investigation of the molecular mechanisms regulating tumorigenesis and angiogenesis in the central nervous system.

Research Background

Edward R. Smith received an MD from Columbia University. He completed a neurosurgery residency at Massachusetts General Hospital (MGH) and a fellowship in pediatric neurosurgery at Boston Children's Hospital. He also completed a postdoctoral research fellowship at MGH and a clinical research fellowship at Children's. Smith is currently the director of Pediatric Cerebrovascular Surgery and co-director of both the Stroke and Skull Base Centers at Children's. He has been recognized for his clinical work with regional and national awards. He was recently named Chair of the Ethics Committee of the American Society of Pediatric Neurosurgery and voted President-elect of the Young Neurosurgeons Committee of the American Association of Neurologic Surgery. Smith also teaches at Tufts and Harvard Medical Schools, having taught neuroanatomy for over 10 years. He has been an invited speaker at grand rounds at institutions locally and nationally. He gives lectures for the critical care team, the nursing staff and the first responder teams at Children's, and teaching rounds for the neurosurgical department at Children's and Brigham and Women's Hospital.

Education

Undergraduate School

Dartmouth College
1992 Hanover NH

Medical School

Columbia University
1996 New York NY

Internship

Surgery Massachusetts General Hospital
1997 Boston MA

Residency

Neurosurgery Massachusetts General Hospital
2003 Boston MA

Fellowship

Pediatric Neurosurgery Boston Children's Hospital
2004 Boston MA

Graduate School

Massachusetts Institute of Technology
2023 Cambridge MA

Media

Caregiver Profile

Meet Dr. Edward Robert Smith

Publications

  1. Cavernous Malformations of the Central Nervous System. Reply. N Engl J Med. 2024 Jun 06; 390(21):2036. View Abstract
  2. Pediatric Cerebral Vascular Malformations : Current and Future Perspectives. J Korean Neurosurg Soc. 2024 May; 67(3):326-332. View Abstract
  3. Rupture risk and outcomes of giant aneurysms in pediatric patients: a multi-institutional case series and systematic review. J Neurosurg Pediatr. 2024 Mar 01; 33(3):276-284. View Abstract
  4. Discovery and Characterization of Ephrin B2 and EphB4 Dysregulation and Novel Mutations in Cerebral Cavernous Malformations: In Vitro and Patient-Derived Evidence of Ephrin-Mediated Endothelial Cell Pathophysiology. Cell Mol Neurobiol. 2023 Dec 27; 44(1):12. View Abstract
  5. Prevention of postoperative stroke in pediatric moyamoya patients: a standardized perioperative care protocol. J Neurosurg Pediatr. 2024 Feb 01; 33(2):185-189. View Abstract
  6. Posterior cerebral territory ischemia in pediatric moyamoya: Surgical techniques and long-term clinical and radiographic outcomes. Childs Nerv Syst. 2024 Mar; 40(3):791-800. View Abstract
  7. Disease specific urinary biomarkers in the central nervous system. Sci Rep. 2023 11 07; 13(1):19244. View Abstract
  8. Benefits and limitations of a dual faculty neurosurgeon approach to resection of pediatric craniopharyngioma. Childs Nerv Syst. 2024 Mar; 40(3):647-653. View Abstract
  9. Direct Transverse Sinus Puncture for Transvenous Coil Embolization of Vein of Galen Malformations: Innovating Existing Techniques. Oper Neurosurg (Hagerstown). 2023 Dec 01; 25(6):e352-e358. View Abstract
  10. Yield of genetic evaluation in non-syndromic pediatric moyamoya patients. Childs Nerv Syst. 2024 Mar; 40(3):801-808. View Abstract
  11. Non-vascular intracranial lesions in three children with PHACE association. Pediatr Dermatol. 2024 Mar-Apr; 41(2):284-288. View Abstract
  12. Rare variants in ANO1, encoding a calcium-activated chloride channel, predispose to moyamoya disease. Brain. 2023 09 01; 146(9):3616-3623. View Abstract
  13. Pediatric Moyamoya Revascularization Perioperative Care: A Modified Delphi Study. Neurocrit Care. 2024 Apr; 40(2):587-602. View Abstract
  14. Evolution of clinical and translational advances in the management of pediatric arteriovenous malformations. Childs Nerv Syst. 2023 10; 39(10):2807-2818. View Abstract
  15. Comment on: Highlight: Is smoke the signal for surgery? Should the moyamoya syndrome "puff of smoke" trigger cerebral revascularization surgery in children with sickle cell disease? Pediatr Blood Cancer. 2023 10; 70(10):e30520. View Abstract
  16. Cerebral revascularization surgery reduces cerebrovascular events in children with sickle cell disease and moyamoya syndrome: Results of the stroke in sickle cell revascularization surgery retrospective study. Pediatr Blood Cancer. 2023 07; 70(7):e30336. View Abstract
  17. Genetic dysregulation of an endothelial Ras signaling network in vein of Galen malformations. bioRxiv. 2023 Mar 21. View Abstract
  18. Morning glory disc anomaly and its implications in moyamoya arteriopathy: a retrospective case series. J Neurosurg Pediatr. 2023 Mar 17; 1-7. View Abstract
  19. Diffusion-Weighted Magnetic Resonance Imaging Demonstrates White Matter Alterations in Watershed Regions in Children With Moyamoya Without Stroke or Silent Infarct. Pediatr Neurol. 2023 06; 143:89-94. View Abstract
  20. Letter: Wide Arterial Sparing Encephalo-Duro-Synangiosis for Moyamoya: Surgical Technique and Outcomes. Oper Neurosurg (Hagerstown). 2023 05 01; 24(5):e391. View Abstract
  21. External validation of the R2eD AVM scoring system to assess rupture risk in pediatric AVM patients. J Neurosurg Pediatr. 2023 05 01; 31(5):469-475. View Abstract
  22. Magnetic Resonance Imaging/Angiography Versus Catheter Angiography for Annual Follow-up of Pediatric Moyamoya Patients: A Cost Outcomes Analysis. Neurosurgery. 2023 06 01; 92(6):1243-1248. View Abstract
  23. Practice variability in the perioperative management of pediatric moyamoya disease in North America. J Stroke Cerebrovasc Dis. 2023 Apr; 32(4):107029. View Abstract
  24. Pediatric Moyamoya Syndrome Secondary to Tuberculous Meningitis: A Case Report. Neurol Clin Pract. 2023 Feb; 13(1):e200121. View Abstract
  25. Increasing precision in the management of pediatric neurosurgical cerebrovascular diseases with molecular genetics. J Neurosurg Pediatr. 2023 03 01; 31(3):228-237. View Abstract
  26. The composition of landmark vein of Galen malformation research: the emergence of endovascular treatments. Childs Nerv Syst. 2023 03; 39(3):733-741. View Abstract
  27. Pediatric Moyamoya Biomarkers: Narrowing the Knowledge Gap. Semin Pediatr Neurol. 2022 10; 43:101002. View Abstract
  28. A national analysis of 9655 pediatric cerebrovascular malformations: effect of hospital volume on outcomes. J Neurosurg Pediatr. 2019 10 01; 24(4):397-406. View Abstract
  29. Noninvasive Thermal Evaluation of Ventriculoperitoneal Shunt Patency and Cerebrospinal Fluid Flow Using a Flow Enhancing Device. Neurosurgery. 2019 08 01; 85(2):240-249. View Abstract
  30. Erratum. Results of more than 20 years of follow-up in pediatric patients with moyamoya disease undergoing pial synangiosis. J Neurosurg Pediatr. 2019 08 01; 24(2):215. View Abstract
  31. ITGA2 as a potential nanotherapeutic target for glioblastoma. Sci Rep. 2019 04 17; 9(1):6195. View Abstract
  32. General Principles for Preoperative Planning and Microsurgical Treatment of Pediatric Brain Arteriovenous Malformations: 2-Dimensional Operative Video. Oper Neurosurg (Hagerstown). 2019 Apr 01; 16(4):E114. View Abstract
  33. Results of more than 20 years of follow-up in pediatric patients with moyamoya disease undergoing pial synangiosis. J Neurosurg Pediatr. 2019 05 01; 23(5):586-592. View Abstract
  34. Letter to the Editor. Bypass and revascularization in young moyamoya patients. J Neurosurg Pediatr. 2019 04 01; 23(4):534-535. View Abstract
  35. General Principles for Pial Synangiosis in Pediatric Moyamoya Patients: 2-Dimensional Operative Video. Oper Neurosurg (Hagerstown). 2019 Jan 01; 16(1):E14-E15. View Abstract
  36. Acute fatal hemorrhage from previously undiagnosed cerebral arteriovenous malformations in children: a single-center experience. J Neurosurg Pediatr. 2018 09; 22(3):244-250. View Abstract
  37. Whole Exome Sequencing Reveals a Monogenic Cause of Disease in ˜43% of 35 Families With Midaortic Syndrome. Hypertension. 2018 04; 71(4):691-699. View Abstract
  38. Human genetics and molecular mechanisms of vein of Galen malformation. J Neurosurg Pediatr. 2018 04; 21(4):367-374. View Abstract
  39. Multimodal treatment approach in a patient with multiple intracranial myxomatous aneurysms. J Neurosurg Pediatr. 2018 03; 21(3):315-321. View Abstract
  40. Sickle Cell Disease. N Engl J Med. 2017 07 20; 377(3):304. View Abstract
  41. Incidence, clinical features, and treatment of familial moyamoya in pediatric patients: a single-institution series. J Neurosurg Pediatr. 2017 May; 19(5):553-559. View Abstract
  42. Real-Time Ultrasound-Guided Catheter Navigation for Approaching Deep-Seated Brain Lesions: Role of Intraoperative Neurosonography with and without Fusion with Magnetic Resonance Imaging. Pediatr Neurosurg. 2017; 52(2):80-86. View Abstract
  43. Using urinary bFGF and TIMP3 levels to predict the presence of juvenile pilocytic astrocytoma and establish a distinct biomarker signature. J Neurosurg Pediatr. 2016 Oct; 18(4):396-407. View Abstract
  44. National Analysis of 2454 Pediatric Moyamoya Admissions and the Effect of Hospital Volume on Outcomes. Stroke. 2016 05; 47(5):1303-11. View Abstract
  45. Structural causes of ischemic and hemorrhagic stroke in children: moyamoya and arteriovenous malformations. Curr Opin Pediatr. 2015 Dec; 27(6):706-11. View Abstract
  46. The natural history of cerebral cavernous malformations in children. J Neurosurg Pediatr. 2016 Feb; 17(2):123-128. View Abstract
  47. Moyamoya Biomarkers. J Korean Neurosurg Soc. 2015 Jun; 57(6):415-21. View Abstract
  48. Safety of neuroangiography and embolization in children: complication analysis of 697 consecutive procedures in 394 patients. J Neurosurg Pediatr. 2015 Oct; 16(4):432-8. View Abstract
  49. Down syndrome and moyamoya: clinical presentation and surgical management. J Neurosurg Pediatr. 2015 Jul; 16(1):58-63. View Abstract
  50. Intracranial aneurysms in the youngest patients: characteristics and treatment challenges. Pediatr Neurosurg. 2015; 50(1):18-25. View Abstract
  51. Microsurgical treatment of arteriovenous malformations in pediatric patients: the Boston Children's Hospital experience. J Neurosurg Pediatr. 2015 Jan; 15(1):71-7. View Abstract
  52. Moyamoya syndrome associated with Alagille syndrome: outcome after surgical revascularization. J Pediatr. 2015 Feb; 166(2):470-3. View Abstract
  53. Benign triton tumor: multidisciplinary approach to diagnosis and treatment. Pediatr Dev Pathol. 2014 Sep-Oct; 17(5):400-5. View Abstract
  54. Management of brain arteriovenous malformations. Lancet. 2014 May 10; 383(9929):1635. View Abstract
  55. Spontaneous regression of an epidermoid cyst of the cavernous sinus. J Clin Neurosci. 2014 Aug; 21(8):1433-5. View Abstract
  56. Occipital pial synangiosis. Acta Neurochir (Wien). 2014 Jul; 156(7):1297-300. View Abstract
  57. Pial synangiosis in patients with moyamoya younger than 2 years of age. J Neurosurg Pediatr. 2014 Apr; 13(4):420-5. View Abstract
  58. Treatment of Moyamoya disease in the adult population with pial synangiosis. J Neurosurg. 2014 Mar; 120(3):612-7. View Abstract
  59. Resection of supratentorial lobar cavernous malformations in children: clinical article.. J Neurosurg Pediatr. 2013 Oct; 12(4):367-73. View Abstract
  60. Imaging after direct and indirect extracranial-intracranial bypass surgery. AJR Am J Roentgenol. 2013 Jul; 201(1):W124-32. View Abstract
  61. Cavernous malformations of the basal ganglia in children. J Neurosurg Pediatr. 2013 Aug; 12(2):171-4. View Abstract
  62. Predictors of neoplastic disease in children with isolated pituitary stalk thickening. Pediatr Blood Cancer. 2013 Oct; 60(10):1630-5. View Abstract
  63. Vascular collateralization along ventriculoperitoneal shunt catheters in moyamoya disease. J Neurosurg Pediatr. 2013 Jun; 11(6):710-2. View Abstract
  64. Moyamoya disease with mesial temporal sclerosis. J Neurosurg Pediatr. 2013 Jun; 11(6):713-6. View Abstract
  65. Moyamoya syndrome associated with neurofibromatosis Type 1: perioperative and long-term outcome after surgical revascularization. J Neurosurg Pediatr. 2013 Apr; 11(4):417-25. View Abstract
  66. Chorea in the clinical presentation of moyamoya disease: results of surgical revascularization and a proposed clinicopathological correlation. J Neurosurg Pediatr. 2013 Mar; 11(3):313-9. View Abstract
  67. Moyamoya arteriopathy. Curr Treat Options Neurol. 2012 Dec; 14(6):549-56. View Abstract
  68. 168 Moyamoya Syndrome Associated with Neurofibromatosis Type 1 in Children. Neurosurgery. 2012 Aug 01; 71(2):E566. View Abstract
  69. Spontaneous occlusion of the circle of Willis in children: pediatric moyamoya summary with proposed evidence-based practice guidelines. A review. J Neurosurg Pediatr. 2012 Apr; 9(4):353-60. View Abstract
  70. Angioarchitectural features associated with hemorrhagic presentation in pediatric cerebral arteriovenous malformations. J Neurointerv Surg. 2013 May; 5(3):191-5. View Abstract
  71. Intraoperative anaphylaxis induced by the gelatin component of thrombin-soaked gelfoam in a pediatric patient. Ann Allergy Asthma Immunol. 2012 Mar; 108(3):209-10. View Abstract
  72. Dural arteriovenous fistulae in pediatric patients: associated conditions and treatment outcomes. J Neurointerv Surg. 2013 Jan 01; 5(1):6-9. View Abstract
  73. Pial arteriovenous fistulae in pediatric patients: associated syndromes and treatment outcome. J Neurointerv Surg. 2013 Jan 01; 5(1):10-4. View Abstract
  74. Incidentally discovered lesions. Neurosurg Focus. 2011 Dec; 31(6):1 p preceding E1. View Abstract
  75. Discovery of asymptomatic moyamoya arteriopathy in pediatric syndromic populations: radiographic and clinical progression. Neurosurg Focus. 2011 Dec; 31(6):E6. View Abstract
  76. Features of the lumbar spine on magnetic resonance images following sectioning of filum terminale. J Neurosurg Pediatr. 2011 Oct; 8(4):384-9. View Abstract
  77. Moyamoya disease in children with congenital dwarfing conditions. Pediatr Neurosurg. 2010; 46(5):373-80. View Abstract
  78. A rare case of psammomatoid ossifying fibroma in the sphenoid bone reconstructed using autologous particulate exchange cranioplasty. J Neurosurg Pediatr. 2011 Mar; 7(3):238-43. View Abstract
  79. Dural scalp and intracranial hemangiomas causing hydrocephalus and venous sinus thrombosis in an infant. J Child Neurol. 2011 Jun; 26(6):777-81. View Abstract
  80. Idiopathic syrinx in the pediatric population: a combined center experience. J Neurosurg Pediatr. 2011 Jan; 7(1):30-6. View Abstract
  81. Cavernous malformations. Neurosurg Clin N Am. 2010 Jul; 21(3):483-90. View Abstract
  82. Moyamoya: epidemiology, presentation, and diagnosis. Neurosurg Clin N Am. 2010 Jul; 21(3):543-51. View Abstract
  83. Preface: introduction to pediatric vascular neurosurgery. Neurosurg Clin N Am. 2010 Jul; 21(3):xiii-xiv. View Abstract
  84. Introduction: moyamoya disease. Neurosurg Focus. 2009 Apr; 26(4):E1. View Abstract
  85. Pial synangiosis in patients with moyamoya syndrome and sickle cell anemia: perioperative management and surgical outcome. Neurosurg Focus. 2009 Apr; 26(4):E10. View Abstract
  86. Intracranial vasospasm with subsequent stroke after traumatic subarachnoid hemorrhage in a 22-month-old child. J Neurosurg Pediatr. 2009 Apr; 3(4):311-5. View Abstract
  87. Seckel syndrome and moyamoya. J Neurosurg Pediatr. 2009 Apr; 3(4):320-4. View Abstract
  88. Moyamoya disease and moyamoya syndrome. N Engl J Med. 2009 Mar 19; 360(12):1226-37. View Abstract
  89. Identification of an association between Robinow syndrome and moyamoya. Pediatr Neurosurg. 2009; 45(1):69-72. View Abstract
  90. A novel TP53 germline mutation in a family with a history of multiple malignancies: case report and review of the literature. Pediatr Neurosurg. 2008; 44(6):501-8. View Abstract
  91. Pediatric brain tumors. Adv Pediatr. 2009; 56:249-69. View Abstract
  92. Foramen magnum stenosis from overgrowth of the opisthion in a child with achondroplasia. J Neurosurg Pediatr. 2008 Aug; 2(2):136-8. View Abstract
  93. 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. Stroke. 2008 Sep; 39(9):2644-91. View Abstract
  94. Urinary biomarkers predict brain tumor presence and response to therapy. Clin Cancer Res. 2008 Apr 15; 14(8):2378-86. View Abstract
  95. Progression of disease in unilateral moyamoya syndrome. Neurosurg Focus. 2008; 24(2):E17. View Abstract
  96. Acute hydrocephalus secondary to obstruction of the foramen of monro and cerebral aqueduct caused by a choroid plexus cyst in the lateral ventricle. Case report. J Neurosurg. 2007 Sep; 107(3 Suppl):236-9. View Abstract
  97. Tethered cord syndrome. Neurosurg Clin N Am. 2007 Jul; 18(3):531-47. View Abstract
  98. A recurrent craniopharyngioma illustrates the potential usefulness of urinary matrix metalloproteinases as noninvasive biomarkers: case report. Neurosurgery. 2007 Jun; 60(6):E1148-9; discussion E1149. View Abstract
  99. Multiple pilocytic astrocytomas of the cerebellum in a 17-year-old patient with neurofibromatosis type I. Childs Nerv Syst. 2007 Oct; 23(10):1191-4. View Abstract
  100. Wound complications associated with the use of bovine serum albumin-glutaraldehyde surgical adhesive in pediatric patients. Neurosurgery. 2007 Apr; 60(4 Suppl 2):305-9; discussion 309. View Abstract
  101. Is there a "July phenomenon" in pediatric neurosurgery at teaching hospitals? J Neurosurg. 2006 Sep; 105(3 Suppl):169-76. View Abstract
  102. Lumbar intramedullary spinal schistosomiasis presenting as progressive paraparesis: case report. Neurosurgery. 2006 May; 58(5):E996; discussion E996. View Abstract
  103. Moyamoya syndrome associated with Down syndrome: outcome after surgical revascularization. Pediatrics. 2005 Nov; 116(5):e694-701. View Abstract
  104. 913 Moyamoya Associated with Down Syndrome: Outcome after Surgical Revascularization. Neurosurgery. 2005 Aug 01; 57(2):434-435. View Abstract
  105. Surgical management of moyamoya syndrome. Skull Base. 2005 Feb; 15(1):15-26. View Abstract
  106. Cerebral pathophysiology and critical care neurology: basic hemodynamic principles, cerebral perfusion, and intracranial pressure. Semin Pediatr Neurol. 2004 Jun; 11(2):89-104. View Abstract
  107. Neurosurgical aspects of critical care neurology. Semin Pediatr Neurol. 2004 Jun; 11(2):169-78. View Abstract
  108. Craniotomy for resection of pediatric brain tumors in the United States, 1988 to 2000: effects of provider caseloads and progressive centralization and specialization of care. Neurosurgery. 2004 Mar; 54(3):553-63; discussion 563-5. View Abstract
  109. In-hospital mortality rates after ventriculoperitoneal shunt procedures in the United States, 1998 to 2000: relation to hospital and surgeon volume of care. J Neurosurg. 2004 Feb; 100(2 Suppl Pediatrics):90-7. View Abstract
  110. Far posterior subtemporal approach to the dorsolateral brainstem and tentorial ring: technique and clinical experience. Neurosurgery. 2003 Feb; 52(2):364-8; discussion 368-9. View Abstract
  111. Proposed use of prophylactic decompressive craniectomy in poor-grade aneurysmal subarachnoid hemorrhage patients presenting with associated large sylvian hematomas. Neurosurgery. 2002 Jul; 51(1):117-24; discussion 124. View Abstract
  112. Surgical approaches to vascular anomalies of the child's brain. Curr Opin Neurol. 2002 Apr; 15(2):165-71. View Abstract
  113. Massive growth of a meningioma into the brachial plexus and thoracic cavity after intraspinal and supraclavicular resection. Case report and review of the literature. J Neurosurg. 2002 Jan; 96(1 Suppl):107-11. View Abstract

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