A Rare Case of Internal Jugular Venous Malformation Treated by Surgical Excision.

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.

Aneurisma de la vena yugular interna, presentación de caso en adulto / Internal jugular venous aneurysm in an adult: case report

Se presenta el caso de una paciente de 77 años, en quien, durante una cirugía de resección de tumor cutáneo, se observa incidentalmente aumento de volumen cervical derecho de características dinámicas. El hallazgo corresponde a un aneurisma de la vena yugular interna derecha, diagnóstico de mayor frecuencia en población pediátrica, habitualmente de curso benigno.

A 77-year-old woman in whom, during a skin tumor resection surgery, a right cervical swelling with dynamic characteristics was observed. The finding is compatible with an aneurysm of the right internal jugular vein, more commonly obsered in children, usually with a benign course.

Anomalous Posterior Branching of the Internal Jugular Vein: A Report of Two Patients.

The internal jugular vein (IJV) is an important vein encountered during most routine major head and neck surgeries. The IJV is known to infrequently present with anatomical variations, commonly duplication and fenestration. This report presents two cases that highlight an unusual anatomical variation of the IJV, namely the posterior tributary, which was encountered during neck dissection for papillary carcinoma of the thyroid and metastatic cervical lymph nodes. The first case was a 50-year-old woman with papillary carcinoma of the thyroid and regional metastasis, who underwent extensive neck dissection. During dissection, an anomalous posterior tributary of the IJV was discovered, originating around 3 cm above the omohyoid tendon-IJV junction. In case 2, a 40-year-old woman with a history of thyroidectomy exhibited a similar anomaly during neck dissection. In both cases, the posterior tributary was observed branching into two divisions. These cases emphasize the significance of recognizing anatomical variations to avoid inadvertent damage during surgical procedures. Anomalies like the posterior IJV tributary could have implications for surgical planning, emphasizing the importance of thorough exploration and understanding of individual variations. Awareness of such variations will help facilitate surgeons in safely performing neck dissections.

Jugular foramen and venous collaterals may help to discriminate congenital from post-thrombotic jugular stenosis.

PURPOSE: Unilateral jugular stenosis is easily mistaken as jugular hypoplasia for their similar jugular appearances. This study aimed to propose a scheme to differentiate acquired internal jugular vein stenosis (IJVS) from congenital jugular variation through two case examples. METHODS: We presented a dynamic evolution process of the IJVS formation, through a case of a 17-year-old female with paroxysmal nocturnal hemoglobinuria (PNH)-associated right internal jugular venous thrombosis (IJVT), which resulted in post-thrombotic IJVS in the rare context of rapid recanalization. Meanwhile, we compared her images with images of a 39-year-old healthy male with hypoplastic IJV to determine the differences between the acquired IJVS and congenital dysplasia. RESULTS: Based on the first case, we noticed the whole formative process of acquired IJVS from nothing to something. Meantime, we found that acquired IJVS was surrounded by abnormal corkscrew collaterals as imaged on contrast-enhanced magnetic resonance venography (CE-MRV), and the ipsilateral jugular foramen (JF) was normal-sized as displayed on computer tomography (CT). Conversely, jugular hypoplasia was with ipsilateral stenotic JF and without serpentine collaterals. CONCLUSION: JF morphology and venous collaterals may be deemed as surrogate identifiers to distinguish acquired unilateral IJVS from jugular hypoplasia.

State of the Art: Venous Causes of Pulsatile Tinnitus and Diagnostic Considerations Guiding Endovascular Therapy.

Venous variants and pathologic abnormalities are the most common causes of pulsatile tinnitus. These conditions include causes of turbulence within normally located veins and sinuses, and abnormally enlarged or abnormally located veins in close transmissive proximity to the conductive auditory pathway. Such disorders include pathologic abnormalities of the lateral sinus (transverse sinus stenosis and sigmoid sinus wall anomalies), abnormalities and variants of the emissary veins, and anomalies of the jugular bulb and jugular vein. Despite being the most common causes for pulsatile tinnitus, venous variants and pathologic abnormalities are often overlooked in the workup of pulsatile tinnitus. Such oversights can result in delayed patient care and prolonged patient discomfort. Advances in both cerebrovascular imaging and endovascular techniques allow for improved diagnostic accuracy and an increasing range of endovascular therapeutic options to address pulsatile tinnitus. This review illustrates the venous causes of pulsatile tinnitus and demonstrates the associated endovascular treatment. © RSNA, 2021.

Duplication of the external jugular vein: a language barrier of database search in classic anatomical studies.

OBJECTIVE: Many anatomical variations of the superficial veins of the head and neck have been reported throughout the literature. Accordingly, anatomists and surgeons must have a comprehensive understanding of these variations to avoid confusion. Duplication of the external jugular vein (EJV) is occasionally observed during routine cadaveric dissections; however, this variation seems to be reported less often than actual experience suggests. Therefore, to gain a better understanding of its anatomical and clinical implications, an analysis of the available data should be available. Thus, in this article, we reviewed the current available literature for studies reporting duplication of the EJV. METHODS: We conducted a search using PubMed and Google Scholar with the following keywords: "duplication of the external jugular vein," "division of the external jugular vein," and "fenestration of the external jugular vein," "double external jugular vein," and "doubled external jugular vein." As a case illustration, we also describe a case of a duplicated EJV found during a right neck dissection of a female cadaver. RESULTS: Twenty sides across sixteen different studies were analyzed including the present case. All studies were published between 2009 and 2020. EJV division patterns were classified as either duplication, fenestration, fenestration followed by duplication, or double fenestrations. CONCLUSIONS: We have reviewed the literature regarding cases documenting duplication/fenestration of the EJV. As it is often difficult to find recent studies that report on classic anatomical variations, therefore, revisiting older articles and textbooks is necessary for achieving a "comprehensive" review, especially across different languages.

External jugular vein pierced by supraclavicular branches in a neonatal cadaver: a case report.

Anatomical variations in the venous structure and drainage patterns in the neck are not uncommon. However, this is the first known report on the external jugular vein being pierced by supraclavicular branches. In the lateral cervical region of a neonatal cadaver, the supraclavicular branches penetrated the external jugular vein superior to the clavicle, resulting in a circular venous channel formed around the nerve trunk. Variations such as these are important to note in order to minimize possible intra-operative complications sustained during surgical interventions such as venous catherization or nerve grafts.

Persistent Conductive Hearing Loss After Tympanostomy Tube Placement Due to High-Riding Jugular Bulb.

High-riding jugular bulb (HRJB) is a rare condition not often observed in the clinical setting that occurs in 1% to 3% of cases. The jugular bulb is not present at birth, and the precise size and location likely depends on a myriad of postnatal events. This report describes the case of a male adolescent who experienced persistent conductive hearing loss (CHL) unilaterally following bilateral tympanostomy tube placement. Subsequent workup included computed tomography, which identified a very high jugular bulb eroding the posterior semicircular canal and occluding the round window niche. The patient had no hearing or vestibular symptoms aside from CHL and continues to be observed on a regular basis. HRJB is a rare disorder that has been known to erode the posterior semicircular canal, resulting in possible tinnitus, vertigo, dizziness, and/or sensorineural hearing loss. CHL has been reported in HRJB cases, although it is uncommon. HRJB may result in CHL through a third-window defect shunting hydromechanical energy away from the round window or due to middle ear blockage. Imaging is useful in ascertaining rare causes of CHL, such as HRJB. Because HRJB is not easily fixable, it is important to recognize it as a rare cause of CHL for appropriate patient counseling. Possible interventions should be tailored to the patient after careful consideration of contralateral anatomy and likely benefits. Laryngoscope, 131:E1272-E1274, 2021.

Stereotactic Gamma Knife Radiosurgery for Extracranial Arteriovenous Malformations.

INTRODUCTION: Head and neck extracranial arteriovenous malformations (AVMs) are rare pathological conditions which pose diagnostic and reconstruction challenges. Stereotactic radiosurgery (SRS) is nowadays an established treatment method for brain AVMs, with high obliteration and low complication rates. Here we describe the first report of head extracranial AVMs successfully treated by Gamma Knife (GK) as a retrospective historical cohort. METHODS: Over a 9-year period, 2 cases of extracranial AVMs were treated by GK Perfexion (Elekta Instruments AB, Stockholm, Sweden) at a single institution. A stereotactic frame and multimodal imaging, including digital subtraction angiography (DSA), were used. The prescribed dose was 24 Gy at the 50% isodose line. RESULTS: The first case was of a patient with pulsating tinnitus and left superficial parotido-condylian AVM. Embolization achieved partial obliteration. Tinnitus disappeared during the following 6 months after GK. The second case was a patient with repetitive gingival hemorrhages and right superior maxillary AVM, fed by the right internal maxillary and facial arteries. Embolization achieved partial obliteration with recurrence of symptoms. GK was further performed. DSA confirmed complete obliteration in both patients. CONCLUSIONS: Single-fraction GK radiosurgery appears to be safe and effective for extracranial AVMs. We recommend prescribing doses that are comparable to the ones used for brain AVMs (i.e., 24 Gy). A stereotactic frame is an important tool to ensure higher accuracy in the context of these particular locations. However, in selected cases, a mask could be applied either for single fraction purposes (if in a non-mobile location) or for hypofractionation, in case of larger volumes. These findings should be validated in larger cohorts, inclusively in terms of dose prescription.

Jugular venous catheter-associated thrombosis and fatal pulmonary embolism: A case report.

INTRODUCTION: Central venous catheters related thrombosis (CRT) insertion has been shown to increase the risk of venous thromboembolism, particularly pulmonary embolism (PE). Nevertheless, deaths cased due to PE have been rarely reported. PATIENT CONCERNS: A central venous catheter was introduced through the right jugular vein during the operation due to severe septic shock from a 57-year-old male patient. Two days after surgery, the hemodynamics was stable. On the 7th day, and low molecular weight heparin calcium (4100 units, once a day) was added for anticoagulation to prevent venous thromboembolism. On the 15th day, during the process of central venous catheter removal, the patient suddenly lost consciousness, suffered cardiac arrest, and received emergency cardiopulmonary resuscitation. DIAGNOSIS: Jugular venous catheter-associated thrombosis and fatal PE. An acute bedside ultrasound showed a thrombus drifting with the blood stream in the right jugular vein. The lower section of the xiphoid process by echocardiography showed decreased systolic amplitude of the right atrium and right ventricle, widened and fixed inferior vena cava, and no variation with respiration. Para-sternal left ventricular long axis section showed that the right ventricular outflow tract was significantly extended, and the contraction amplitude of the anterior and posterior walls of the left ventricle decreased. Left ventricular short axis section indicated a right ventricle enlargement and ventricular septum deviation of left ventricle, showing "D" sign. Apical 4-chamber view showed that the right ventricular ratio increased and the contractile capacity decreased. INTERVENTIONS: One and a half million units of urokinase were immediately given trough intravenous drip. OUTCOMES: Twenty minutes after thrombolysis, the patient's autonomic heart rhythm was recovered, but continued to suffer from hypotension and coma, followed by multiple organ failure, and died 50 hours later. CONCLUSION: Recent clinical practice guidelines recommend against the routine use of any anticoagulant thromboprophylaxis in patients with central venous catheters, but for patients at particularly high risk for CRT, consideration can be given to using higher doses of anticoagulant as prophylaxis, although there are virtually no data to support this approach.

Two different variants of the jugulocephalic vein with supraclavicular course.

The jugulocephalic anastomosis is a rare anatomical variant which normally undergoes atrophy during embryonic development. We found 2 cases of the jugulocephalic vein variant with supraclavicular course in Korean male cadavers. In a 50-year-old cadaver, the right cephalic vein ascended anterior to the clavicle, and terminated into the external jugular vein as well as to the axillary vein through a classic branch. In a 76-year-old cadaver, the left cephalic vein ascended supraclavicular course without any branch to the axillary vein, and terminated to the external jugular vein. We discussed the embryological explanation as well as its frequency since this jugulocephalic vein variant could cause unpredicted danger during clinical procedures.

Unexpected complication of arteriovenous fistula of the left common carotid to internal jugular vein following central venous catheterization.

Incidence of inadvertent arterial puncture secondary to central venous catheter insertion is not common with an arterial puncture rate of <1%. This is due to the advancements and wide availability of ultrasound to guide its insertion. Formation of arteriovenous fistula after arterial puncture is an unexpected complication. Till date, only five cases (including this case) of acquired arteriovenous fistula formation has been described due to inadvertent common carotid puncture. The present case is a 26-year-old man sustained traumatic brain injuries, chest injuries and multiple bony fractures. During resuscitative phase, attempts at left central venous catheter via left internal jugular vein under ultrasound guidance resulted in inadvertent puncture into the left common carotid artery. Surgical neck exploration revealed that the catheter had punctured through the left internal jugular vein into the common carotid artery with formation of arteriovenous fistula. The catheter was removed successfully and common carotid artery was repaired. Postoperatively, the patient recovered and clinic visits revealed no neurological deficits. From our literature review, the safest method for removal is via endovascular and open surgical removal. The pull/push technique (direct removal with compression) is not recommended due to the high risk for stroke, bleeding and hematoma formation.

Anatomic Alert: Spinal accessory nerve traversing a fenestrated internal jugular vein.

We present a case of the spinal accessory nerve traversing a fenestrated internal jugular vein. Awareness of this variant may be important in neurosurgical procedures that involve upper cervical exposures.

Transvenous Stent-assisted Coil Embolization for Management of Dehiscent High Jugular Bulb With Tinnitus and Contralateral Hypoplastic Venous Sinuses.

OBJECTIVES: This study aimed to evaluate the safety and efficacy of transvenous stent-assisted coil embolization for dehiscent high jugular bulb (HJB) with tinnitus and contralateral hypoplastic venous sinus. STUDY DESIGN: Case series with chart review. SETTING: Tertiary academic medical center. METHODS: From September 2008 to October 2018, a series of patients with dehiscent HJB presenting with intractable pulsatile tinnitus abated only by ipsilateral jugular vein compression were included. Patients underwent transvenous stent-assisted coil embolization for selective obstruction to the dome of the HJB due to hypoplastic contralateral transverse or sigmoid venous sinus. Technical safety and clinical efficacy were retrospectively analyzed. Clinical outcome measurements included pure-tone audiogram, tinnitus character, and tinnitus handicap inventory and evaluated based on the change during the first 6 months after the procedure. RESULTS: Subjects included five patients with dehiscent HJB and troublesome pulsatile tinnitus who refused surgery (n = 4), or who experienced recurrence after surgical covering and reinforcement using autologous cartilage (n = 1). The mean age of the five patients (only female) was 45 years. Transvenous stent-assisted coil embolization was technically successful in all patients with symptomatic dehiscent HJB, with no procedure-related complications. Temporary postprocedural headache was observed in two patients, but resolved within 3 days. Symptoms were completely resolved in all cases. There was no recurrence or aggravation of tinnitus during follow-up period. CONCLUSIONS: Transvenous stent-assisted coil embolization for dehiscent HJB with tinnitus and contralateral hypoplastic transverse or sigmoid venous sinus could be a technically safe and clinically effective treatment strategy while preserving cranial venous drainage.

Congenital jugular vein phlebectasia or aneurysma: A rare entity in prenatal diagnosis.

Vascular malformations arising from the wall of the external jugular vein are rare and appeared most commonly in pediatric population. Here, we present a case of vascular malformation in the left external jugular vein diagnosed in a fetus during third trimester ultrasound. This is the first described case in prenatal diagnosis.

Fetal carotid-jugular fistula: A case report.

RATIONALE: Fetal carotid-jugular fistula is an extremely rare clinical entity that presents as an abnormal passage between the carotid artery and the jugular vein. It is difficult to treat and the chance for a cure is very low. The fetal carotid-jugular fistula causes congestive heart failure and death of the fetus. PATIENT CONCERNS: We report a case of fetal carotid-jugular fistula diagnosed at 27 weeks of pregnancy. She had no history of viral infection, no history of toxic and radiation exposure, no trauma during pregnancy, and no known family history of malformations or genetic disease. DIAGNOSES: Ultrasound revealed fetal left carotid-jugular fistula formation, massive reflux in the fetal tricuspid, a large amount of fetal pericardial effusion, fetal left ear microtia and full heart enlargement. INTERVENTIONS: The pregnant patient experienced termination of the pregnancy at 27 weeks. OUTCOMES: There were no complications in the patient. Post-termination, diagnosis of carotid-jugular fistula and left ear microtia was confirmed in the fetus. LESSONS: Our case indicated that the congenital neck artery and venous fistula of the fetus are extremely rare, and its most serious clinical symptom is congestive heart failure leading to intrauterine cessation of pregnancy. In addition, it is difficult to treat and the chance for a cure is very low. At present, there is no treatment record related to the fetal carotid artery and venous fistula, so it is very important to make a correct diagnosis as early as possible for the health of pregnant women.