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

2021 ◽  
Vol 56 (6) ◽  
pp. 584-590
Author(s):  
Gaurav Gupta ◽  
Michael S. Rallo ◽  
David Y. Goldrich ◽  
Vinayak Narayan ◽  
Neil Majmundar ◽  
...  
Keyword(s):  
Jugular Foramen ◽  
Jugular Bulb ◽  
Venous Hypertension ◽  
Cerebral Injury ◽  
Vein Of Galen ◽  
Microsurgical Decompression ◽  
Venous Stenosis ◽  
Initial Reduction ◽  
Treatment Paradigm ◽  
Angioplasty And Stenting

<b><i>Introduction:</i></b> 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. <b><i>Case Presentation:</i></b> 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. <b><i>Discussion/Conclusion:</i></b> 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.

scholarly journals Stenting of a Venous Stenosis in Vein of Galen Aneurysmal Malformation

2001 ◽  
Vol 7 (3) ◽  
pp. 237-240 ◽  
Author(s):  
S. Brew ◽  
W. Taylor ◽  
A. Reddington
Keyword(s):  
Cardiac Failure ◽  
Neonatal Period ◽  
Jugular Bulb ◽  
Venous Hypertension ◽  
High Flow ◽  
Arteriovenous Shunt ◽  
Vein Of Galen ◽  
Venous Outflow ◽  
Venous Stenosis ◽  
Parenchymal Damage

The vein of Galen aneurysmal malformation (VGAM) is a high flow arteriovenous shunt at the choroidal level. In the neonatal period, it typically presents with cardiac failure. Venous stenoses, occlusions and anomalies are often present. In the absence of adequate venous outflow pathways, severe, irreversible cerebral parenchymal damage may occur due to intracranial venous hypertension, altered hydrodynamics and ischaemia. We present a case of deployment of a stent across a focal superior jugular bulb stenosis in an effort to avert this outcome.

scholarly journals Is jugular bulb stenosis in vein of Galen aneurysmal malformation associated with bony remodeling of the jugular foramina?

2016 ◽  
Vol 18 (1) ◽  
pp. 92-96
Author(s):  
Guillaume Saliou ◽  
Peter Dirks ◽  
Lee-Anne Slater ◽  
Timo Krings
Keyword(s):  
Jugular Vein ◽  
Jugular Foramen ◽  
Jugular Bulb ◽  
Age Group ◽  
Vein Of Galen ◽  
Male Predominance ◽  
Surgical Bypass ◽  
History Of ◽  
Sick Children

OBJECTIVE The etiology of jugular bulb stenosis (JBS) or occlusion in the context of vein of Galen aneurysmal malformations (VGAMs) is unknown. It can lead to decompensation of a lesion that was previously clinically stable. The aim of this study was to describe the natural history of JBS or occlusion in VGAM and to determine whether there is an association with bony remodeling of the jugular foramina. METHODS The authors identified all cases of JBS greater than 70% bilaterally involving patients seen at The Hospital for Sick Children between January 2007 and June 2014. The foramen diameters were measured on sagittal CT imaging, on a slice passing at the level of the jugular vein. The jugular foramen diameters were also compared to measurements obtained in a matched population of the same age group who had no VGAM and had undergone cerebral CT for a reason other than vascular disease. RESULTS Eight patients (6 male and 2 female) with bilateral JBS were included in this series. The median duration of clinical follow-up was 2.5 years (IQR 1.7–4.2 years). JBS was associated with bony narrowing the jugular foramina in 7 of the 8 patients over time. Between 1 and 2 years of age, patients with a VGAM demonstrated jugular foramen narrowing in comparison with a matched population (p = 0.015). CONCLUSIONS Jugular bulb stenosis or occlusion in VGAM may be associated with narrowing of the jugular foramina. These conditions seem to have a male predominance. If treatment is required, bony narrowing of the jugular foramina should be taken into account when deciding whether angioplasty and stent placement or surgical bypass might be appropriate therapeutic options.

scholarly journals Extracranial invasion of a recurrent, transformed anaplastic pleomorphic xanthoastrocytoma: a case report

2018 ◽  
Vol 45 (S1) ◽  
pp. S2-S3
Author(s):  
K.D. Langdon ◽  
D. Krivosheya ◽  
M.O. Hebb ◽  
B. Wehrli ◽  
L.C. Ang
Keyword(s):  
Posterior Fossa ◽  
Jugular Foramen ◽  
Jugular Bulb ◽  
Neck Mass ◽  
Pleomorphic Xanthoastrocytoma ◽  
Braf V600e ◽  
Rare Tumour ◽  
Who Grade ◽  
Original Tumour ◽  
The Right

Pleomorphic xanthoastrocytoma (PXA) is a rare tumour comprising <1% of all primary central nervous system tumours and the majority (~98%) occur supratentorially. We report on a 40-year-old female with a past medical history of a rare posterior fossa/cerebellar PXA who presented with a right-sided neck mass, decreased shoulder power and longstanding right tongue deviation with right-sided hemi-atrophy. The patient had prior tumour debulking. Recent MRI demonstrated an enhancing posterior fossa mass extending to the skull base at the jugular foramen and another mass in the upper neck along the jugular bulb with displacement and encasement of the right common carotid artery down to C5. Resection of the neck mass reveals an anaplastic PXA. The tumour has close approximation with adjacent peripheral nerves and is positive in 2 lymph nodes. Comparison with the original tumour molecular and immunohistochemical profiles reveals a conserved BRAF V600E mutation but the transformed malignant glioma now expresses dot-like EMA positivity and ATRX is completely lost (mutated). Transformation of a PXA (WHO Grade II) into an anaplastic PXA (WHO Grade III) has been well documented, but extracranial extension is extraordinarily rare. We report herein the first documented case of a posterior fossa PXA that underwent malignant transformation and extracranial invasion to the parapharyngeal space.

Anterior Facial Nerve Rerouting in Cranial Base Surgery: A Comparison of Three Techniques

1996 ◽  
Vol 115 (1) ◽  
pp. 82-88 ◽  
Author(s):  
Peter G. Von Doersten ◽  
Robert K. Jackler
Keyword(s):  
Facial Nerve ◽  
Jugular Foramen ◽  
Jugular Bulb ◽  
Cranial Base ◽  
Clinical Impression ◽  
Canal Wall ◽  
Posterior Aspect ◽  
Pivot Point ◽  
Carotid Canal ◽  
Canal Wall Down

Anterior rerouting of the facial nerve is a maneuver designed to enhance exposure of the jugular foramen and carotid canal during resection of cranial base tumors. Our clinical impression is that the degree of additional exposure afforded by moving the facial nerve varies considerably according to both anatomic variations and the technique used. Three possible techniques exist based on the extent of facial nerve mobilization and point of rotation: canal wall up-second genu pivot point (CWU-2G); canal wall down-second genu pivot point (CWD-2G); and canal wall down-first genu pivot point (CWD-1G). We anatomically studied 20 human cadaver heads to establish clinically relevant guidelines for the selective use of these techniques. At the level of the dome of the jugular bulb, the facial nerve mobilized anteriorly a mean of 4.2 mm for CWU-2G, 10 mm for CWD-2G, and 14 mm for CWD-1G. Detailed analysis of numerous measurements and rotation angles suggests that the typical exposure afforded by the various rerouting techniques is as follows: CWU-2G, complete exposure of the jugular bulb; CWD-2G, exposure of the jugular bulb and a mean of 6 mm of the posterior aspect of the carotid artery; and CWD-1G, exposure of the jugular bulb and entire carotid genu. Minimizing the amount of facial nerve manipulation needed to achieve sufficient surgical exposure helps optimize postoperative functional status.

Neuroprotection for Carotid Endarterectomy and Carotid Artery Stenting

2017 ◽  
Author(s):  
Zirka H. Anastasian ◽  
Eric J. Heyer
Keyword(s):  
Carotid Artery ◽  
Cognitive Decline ◽  
Carotid Endarterectomy ◽  
Carotid Artery Stenting ◽  
Cerebral Injury ◽  
Embolic Stroke ◽  
Neurological Injury ◽  
Anesthetic Techniques ◽  
Neurological Outcomes ◽  
Angioplasty And Stenting

Carotid endarterectomy (CEA) and carotid artery stenting (CAS) pose risks of cerebral injury and adverse neurological outcomes consisting of perioperative stroke and cognitive decline. This chapter examines the mechanisms of and risks for neurological injury associated with these procedures and the various strategies that are employed to protect the brain and minimize the risk of stroke and cognitive decline, including surgical and anesthetic techniques, blood pressure management, and statin administration. During CEA, neuromonitoring is used to guide surgical technique in order to prevent ischemic stroke due to hypoperfusion during carotid artery cross-clamping and embolic stroke during unclamping. For CAS, cerebral protective devices are the primary neuroprotection technique, with the focus on preventing embolic stroke during manipulation of wires, angioplasty, and stenting.

Variations in the Structure of the Jugular Foramen of Human Skull

2013 ◽  
Vol 10 (2) ◽  
pp. 45-49 ◽  
Author(s):  
SM Akram Hossain ◽  
SM Moshadeq Hossain ◽  
Fakhrul Amin Mohammad Hasanul Banna
Keyword(s):  
Internal Jugular Vein ◽  
Jugular Vein ◽  
Jugular Foramen ◽  
Jugular Bulb ◽  
Cross Sectional ◽  
Medical College ◽  
Geographical Variations ◽  
Pass Through ◽  
The Right ◽  
Study Type

Context: The jugular foramen is one of the most fascinating foramen present at the base of the skull attracting the imagination of many Anatomists worldwide as many important structures pass through it, and amongst them the intriguing structure is the internal jugular vein. The shape and size of the jugular foramen is related to the size of the internal jugular vein and the presence or absence of a prominent superior bulb. As most of the textbooks of Anatomy describe that the right jugular foramen is usually larger than the left jugular foramen. Henceforth the present study was undertaken in 55 skulls from the dept. of Anatomy. Measurements were taken with the help of sliding vernier caliper. Study type: Cross-sectional descriptive type. Place and period of study: Department of Anatomy, Rajshahi Medical College, Rajshahi and Pabna Medical College, Pabna from April 2010 to June 2011. Materials and Methods: Total fifty five (55) human adult skulls were collected from the Anatomy department of Rajshahi Medical College, Rajshahi and Pabna Medical College, Pabna at different times of the study period. The study was conducted to observe variations in the structure of the jugular foramen of the human’s skull. Result: Out of 55 skulls (110 foramina) studied, the presence of dome indicating the presence of jugular bulb was found bilaterally in 100% of cases. 58.18% of cases showed that the size of right foramina were larger than the left foramina whereas 20% of cases showed that right foramina were equal to the left and in 21.82% of cases the left foramina were larger than the right side foramina. An important observation in the present study was the presence of either complete or partial septation in the jugular foramen. Conclusion: The findings of the study reveals that there are some differences among some parameters. The variations are might be due to the geographical variations of the skeletons. It needs further study with larger sample size from different geographical areas of Bangladesh. DOI: http://dx.doi.org/10.3329/bja.v10i2.17281 Bangladesh Journal of Anatomy, July 2012, Vol. 10 No. 2 pp 45-49

Microsurgical Resection of Glomus Jugulare Tumors With Facial Nerve Reconstruction: 3-Dimensional Operative Video

2018 ◽  
Vol 16 (1) ◽  
pp. E1-E1 ◽  
Author(s):  
Duarte N C Cândido ◽  
Jean Gonçalves de Oliveira ◽  
Luis A B Borba
Keyword(s):  
Facial Nerve ◽  
Infratemporal Fossa ◽  
Tumor Resection ◽  
Cranial Nerves ◽  
Jugular Foramen ◽  
Jugular Bulb ◽  
Senior Author ◽  
Nerve Reconstruction ◽  
Glomus Jugulare ◽  
Carotid Canal

Abstract Paragangliomas are tumors originating from the paraganglionic system (autonomic nervous system), mostly found at the region around the jugular bulb, for which reason they are also termed glomus jugulare tumors (GJT). Although these lesions appear to be histologically benign, clinically they present with great morbidity, especially due to invasion of nearby structures such as the lower cranial nerves. These are challenging tumors, as they need complex approaches and great knowledge of the skull base. We present the case of a 31-year-old woman, operated by the senior author, with a 1-year history of tinnitus, vertigo, and progressive hearing loss, that evolved with facial nerve palsy (House-Brackmann IV) 2 months before surgery. Magnetic resonance imaging and computed tomography scans demonstrated a typical lesion with intense flow voids at the jugular foramen region with invasion of the petrous and tympanic bone, carotid canal, and middle ear, and extending to the infratemporal fossa (type C2 of Fisch's classification for GJT). During the procedure the mastoid part of the facial nerve was identified involved by tumor and needed to be resected. We also describe the technique for nerve reconstruction, using an interposition graft from the great auricular nerve, harvested at the beginning of the surgery. We achieved total tumor resection with a remarkable postoperative course. The patient also presented with facial function after 6 months. The patient consented with publication of her images.

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