Management of Jugular Bulb Stenosis in Pediatric Vein of Galen Malformation: A Novel Management Paradigm

Authors

Gaurav Gupta, Department of Neurosurgery, Rutgers Robert Wood Johnson Medical School, New Brunswick, New Jersey, USA.
Michael S. Rallo, Department of Neurosurgery, Rutgers Robert Wood Johnson Medical School, New Brunswick, New Jersey, USA.
David Y. Goldrich, Division of Otology and Neurotologic Skull Base Surgery, Department of Otolaryngology, Head and Neck Surgery, Rutgers Robert Wood Johnson Medical School, New Brunswick, New Jersey, USA.
Vinayak Narayan, Department of Neurosurgery, Rutgers Robert Wood Johnson Medical School, New Brunswick, New Jersey, USA.
Neil Majmundar, Department of Neurosurgery, Rutgers Robert Wood Johnson Medical School, New Brunswick, New Jersey, USA.
Sudipta Roychowdhury, Department of Neurosurgery, Rutgers Robert Wood Johnson Medical School, New Brunswick, New Jersey, USA.
Anil Nanda, Department of Neurosurgery, Rutgers Robert Wood Johnson Medical School, New Brunswick, New Jersey, USA.
P Ashley Wackym, Division of Otology and Neurotologic Skull Base Surgery, Department of Otolaryngology, Head and Neck Surgery, Rutgers Robert Wood Johnson Medical School, New Brunswick, New Jersey, USA.

Document Type

Article

Abstract

INTRODUCTION: Pediatric vein of Galen malformations (VOGMs) are fistulous intracranial malformations arising congenitally within the choroidal fissure that can present with an array of neurological and cardiac sequelae. Associated venous stenosis may result in intracranial venous hypertension and ischemia leading to severe, irreversible cerebral injury. Management of neonatal VOGMs typically involves staged embolization and angioplasty/stenting for relief of venous stenosis. Rarely, jugular foraminal narrowing has been identified as causing jugular bulb stenosis. CASE PRESENTATION: We present the case of a 22-month-old female diagnosed with VOGM prenatally who displayed persistent intracranial venous hypertension despite multiple neuroembolization procedures during the neonatal period. Following initial reduction in arteriovenous shunting, she once again developed venous hypertension secondary to jugular bulb stenosis for which angioplasty was attempted. Failure of angioplasty to relieve the venous hypertension prompted skull base imaging, which revealed jugular foraminal ossification and stenosis. Microsurgical jugular foraminotomy followed by balloon angioplasty and stenting significantly reduced jugular pressure gradients. Restenosis requiring re-stenting developed postoperatively at 9 months, but the patient has remained stable with significant improvement in cortical venous congestion. DISCUSSION/CONCLUSION: This case demonstrates the efficacy of microsurgical decompression of the jugular foramen and endovascular angioplasty/stenting as a novel treatment paradigm for the management of intracranial venous hypertension in the setting of VOGM.

Medical Subject Headings

Cerebral Veins; Child; Constriction, Pathologic (surgery); Embolization, Therapeutic; Female; Humans; Infant; Infant, Newborn; Intracranial Hypertension; Vein of Galen Malformations (diagnostic imaging, surgery)

Publication Date

1-1-2021

Publication Title

Pediatric neurosurgery

E-ISSN

1423-0305

Volume

56

Issue

6

First Page

584

Last Page

590

PubMed ID

34614493

Digital Object Identifier (DOI)

10.1159/000517653

Recommended Citation

Gupta, Gaurav; Rallo, Michael S.; Goldrich, David Y.; Narayan, Vinayak; Majmundar, Neil; Roychowdhury, Sudipta; Nanda, Anil; and Wackym, P Ashley, "Management of Jugular Bulb Stenosis in Pediatric Vein of Galen Malformation: A Novel Management Paradigm" (2021). Neurosurgery. 2212.
https://scholar.barrowneuro.org/neurosurgery/2212