Neurofibromatosis Type 1 (NF1) is a rare autosomal dominant disorder that has a wide array of clinical manifestations. NF1 Vasculopathies constitute 0.4% to 6.4% of the findings and they often develop in the arterial circulation while venous involvement is rare. We present a case of a 73-year-old male with NF1 with an incidental finding of right neck swelling for 2 months. Different radiological modalities were performed, identifying the lesion as an internal jugular vein aneurysm. The patient was managed conservatively as he was asymptomatic in relation to the swelling. NF1 venous vasculopathies are rare but they have detrimental consequences such as rupture and severe hemorrhage in view of the fragility of the aneurysmal wall and the infiltration of the neurofibroma into the vessel. Hence, high clinical suspicion and selective imaging and follow-up is advisable for physicians.

This paper reports a case of an internal jugular venous malformation (IJVM) and route of treatment in a patient with limited symptoms. After history and imaging studies, a determination of surgical excision was made to rule out possible malignancy and future problems such as thrombosis. The mass was resected, and part of the IJVM was ligated. The mass had no identifiable malignancy, and the patient recovered fully with no complications. The paper highlights the importance of identifying venous malformations and highlights the reasoning behind the course of action.

The preservation of the spinal accessory nerve represents a key goal in head and neck oncologic surgery during selective neck dissection. This study aims to illustrate the anatomical variants of the XI cranial nerve, delving into the relationship between the spinal nerve and the internal jugular vein, as well as the surgical implications. Two cases of patients who underwent oncologic surgery with neck dissection are described. Both cases found the spinal accessory nerve passing through the fenestration of the internal jugular vein. Alongside this case series, an independent literature review was conducted using the Medline and PubMed databases. In the majority of cases (67% - 96%), the spinal accessory nerve traces a lateral course to the internal jugular vein. Less frequently, the XI cranial nerve courses medial to the internal jugular vein. More rarely, as described in this case series, the nerve crosses through the fenestration of the vein (0.48% - 3.3%).

Usually, the external jugular vein (EJV) is located superficially over the sternocleidomastoid muscle and joins the subclavian vein or the venous angle. The internal jugular vein (IJV) lies deeply in close relation with the common carotid artery and vagus nerve, enveloped by the carotid sheath. Normally, there is no direct connection between those vessels. During a routine neck dissection, we found a rare anastomosis between IJV and EJV. The anastomosis was localized on the level of the cricoid cartilage. It was approximately 1 cm long, with the diameter of the lumen being 0.3 cm. There was no obstruction along the length of the vessel. The direction was oblique and followed the blood flow from IJV to EJV. The observed variation has high clinical importance related to numerous procedures executed in the neck region, such as placement of hemodialysis catheter in patients with renal failure, insertion of central venous line in the care of critically ill patients, and radical neck dissections.

Head and neck trauma-induced pulsatile tinnitus (PT) should be approached with caution, as it can rarely be attributed to an arteriovenous fistula (AVF). We present a 26-year-old male with a history of blunt trauma who presented delayed PT with direct AVF between the ascending pharyngeal artery (APA) and the internal jugular vein (IJV). The patient underwent occlusion of the fistula with transarterial embolization using coils and PT was completely resolved, confirming successful treatment. The delayed manifestation of PT in the APA-IJV fistula is probably due to the gradual formation of a pseudoaneurysm and subsequent AVF. This case highlights the importance of investigating PT in head trauma patients, as it can be a sign of AVF and possible complications. Overall, this case contributes to understanding delayed PT with AVF and emphasizes the importance of prompt diagnosis and treatment of AVF in patients with head and neck trauma.

BACKGROUND: Eagle jugular syndrome (EJS), recently identified as a cause of cerebrovascular disease (CVD) due to venous obstruction by an elongated styloid process (SP), is reported here alongside a case of concurrent de novo cerebral cavernous malformation (CCM). This study aims to explore the potential causal relationship between EJS and de novo CCM through a comprehensive literature review.
METHODS: Systematic literature reviews, spanning from 1995 to 2023, focused on EJS cases with definitive signs and symptoms and de novo CCM cases with detailed clinical characteristics. Data on the pathophysiology and clinical manifestations of EJS, as well as potential risk factors preceding de novo CCM, were collected to assess the relationship between the two conditions.
RESULTS: Among 14 patients from 11 articles on EJS, the most common presentation was increased intracranial hypertension (IIH), observed in 10 patients (71.4%), followed by dural sinus thrombosis in four patients (28.6%). In contrast, 30 patients from 28 articles were identified with de novo CCM, involving 37 lesions. In these cases, 13 patients developed CCM subsequent to developmental venous anomalies (43%), seven following dural arteriovenous fistula (dAVF) (23%), and two after sinus thrombosis (6%). In a specific case of de novo brainstem CCM, the development of an enlarged condylar emissary vein, indicative of venous congestion due to IJV compression by the elongated SP, was noted before the emergence of CCM.
CONCLUSIONS: This study underscores that venous congestion, a primary result of symptomatic EJS, might lead to the development of de novo CCM. Thus, EJS could potentially be an indicator of CCM development. Further epidemiological and pathophysiological investigations focusing on venous circulation are necessary to clarify the causal relationship between EJS and CCM.

A rare foreign body accompanied by thrombosis in the right internal jugular vein was accidentally observed. We collected the medical history of this special patient, analyzed the causes and characteristics of the foreign body and thrombosis formation, and subsequently observed the changes in thrombosis. Finally, we discussed the diagnostic value of ultrasound for such rare intravascular lesions.

Internal jugular vein (IJV) stenosis is associated with several neurological disorders including idiopathic intracranial hypertension (IIH) and pulsatile tinnitus. In cases of extreme bony compression causing stenosis in the infracondylar region, surgical decompression might be necessary. We aim to examine the safety and efficacy of surgical IJV decompression.
We retrospectively reviewed patients who received surgical IJV decompression via the extreme lateral infracondylar (ELI) approach between July 2020 and February 2022.
Fourteen patients with IJV stenosis were identified, all with persistent headache and/or tinnitus. Six patients were diagnosed with IIH, three of whom failed previous treatment. Of the eight remaining patients, two failed previous treatment. All underwent surgical IJV decompression via styloidectomy, release of soft tissue, and removal of the C1 transverse process (TP). Follow-up imaging showed significant improvement of IJV stenosis in eleven patients and mild improvement in three. Eight patients had significant improvement in their presenting symptoms, and three had partial improvement. Two patients received IJV stenting after a lack of initial improvement. Two patients experienced cranial nerve paresis, and one developed a superficial wound infection.
The ELI approach for IJV decompression appears to be safe for patients who are not ideal endovascular candidates due to bony anatomy. Confirming long-term efficacy in relieving debilitating clinical symptoms requires longer follow-up and a larger patient cohort. Carefully selected patients with symptomatic bony IJV compression for whom there are no effective medical or endovascular options may benefit from surgical IJV decompression.

The intricacies of human anatomy continue to astound, as underscored by this unusual case of a 45-year-old female patient who presented to our esteemed otolaryngology department with T3N1MO squamous cell carcinoma of the lip. The preoperative diagnostic imaging of this patient divulged an enigmatic venous anomaly involving the internal jugular vein (IJV). Our team meticulously orchestrated a wide local excision of the primary tumor and modified radical neck dissection with Abbe Estlander flap reconstruction. Identification of the anomaly during the preoperative phase helped in meticulous planning and preparation. Thus, the surgical team was well-prepared for neck dissection and successfully navigated the rare IJV fenestration without incurring nerve or vascular injuries. This remarkable case accentuates the importance of maintaining a profound understanding of potential anatomical aberrations while performing intricate surgical procedures such as neck dissections. Heightened awareness can circumvent inadvertent damage to critical structures, ultimately safeguarding patient well-being. In this captivating report, we explain the preoperative suspicion, intraoperative identification, and subsequent outcome of a rare fenestration of the IJV encountered during a challenging neck dissection.

During microvascular decompression (MVD) for trigeminal neuralgia (TGN), the mastoid emissary veins (MEV) are routinely sacrificed using the suboccipital retrosigmoid approach. The technical nuances when the MEV is an important collateral venous pathway for the obstructive internal jugular vein (IJV) have not been described thus far. Herein, we demonstrate for the first time a modified surgical technique for MVD to preserve the MEV. A 62-year-old man with a 10-year history of TGN refractory to carbamazepine was referred to our hospital for MVD. Preoperative imaging revealed that the superior cerebellar artery was the offending vessel. Computed tomography angiography also revealed that his contralateral IJV pathway was hypoplastic, and the ipsilateral pathway was severely stenosed by the external compression of the elongated styloid process and the transverse process of the first cervical vertebra. The ipsilateral MEV and the connecting occipital veins were enlarged as the sole collateral pathways of intracranial venous drainage. A modified MVD technique, including an upside-down L-shaped skin incision, layer-by-layer dissection of the occipital muscles, and denuding of the intraosseous part of the MEV, was used to cure the TGN with the preservation of the venous pathway. After surgery, the pain completely diminished without any complications. In conclusion, such technical modifications would be applicable in cases where the MEV needs to be preserved during posterior fossa surgery. Preoperative screening of the venous system is also recommended.