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Paragangliomas are the most common tumors at jugular foramen and pose a great surgical challenge. Careful clinical history and physical examination must be performed to adequately evaluate neurological deficits and its chronologic evolution, also to delineate an overview of the patient performance status. Complete imaging evaluation including MRI and CT scans should be performed, and angiography is a must to depict tumor blood supply and sigmoid sinus/internal jugular vein patency. Screening for multifocal paragangliomas is advisable, with a whole-body imaging. Laboratory investigation of endocrine function of the tumor is necessary, and adrenergic tumors may be associated with synchronous lesions. Preoperative prepare with alpha-blockage is advisable in norepinephrine/epinephrine-secreting tumors; however, it is not advisable in exclusively dopamine-secreting neoplasms. Best surgical candidates are young otherwise healthy patients with smaller lesions; however, treatment should be individualized each case. Variations of infratemporal fossa approach are employed depending on extensions of the mass. Regarding facial nerve management, we avoid to expose or reroute it if there is preoperative function preservation and prefer to work around facial canal in way of a fallopian bridge technique. If there is preoperative facial nerve compromise, the mastoid segment of the nerve is exposed, and it may be grafted if invaded or just decompressed. A key point is to preserve the anteromedial wall of internal jugular vein if there is preoperative preservation of lower cranial nerves. Careful multilayer closure is essential to avoid at most cerebrospinal fluid leakage. Residual tumors may be reoperated if growing and presenting mass effect or be candidate for adjuvant stereotactic radiosurgery.

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INDICATIONS CORRIDOR AND LIMITS OF EXPOSURE: This approach is intended for tumors centered in the jugular foramen with extensions between intracranial and extracranial spaces, possible spread to the middle ear, and variable bony destruction. 1,2. ANATOMIC ESSENTIALS NEED FOR PREOPERATIVE PLANNING AND ASSESSMENT: Jugular foramen paragangliomas are complex lesions that usually invade and fill related venous structures. They present complex relationships with skull base neurovascular structures as internal carotid artery, lower cranial nerves (CNs), middle ear, and mastoid segment of facial nerve. In this way, it is essential to perform an adequate preoperative vascular study to evaluate sinus patency and the tumor blood supply, besides a computed tomography scan to depict bone erosion. ESSENTIAL STEPS OF THE PROCEDURE: Mastoidectomy through an infralabyrinthine route up to open the lateral border of jugular foramen, allowing exposure from the sigmoid sinus to internal jugular vein. Skeletonization of facial canal without exposure of facial nerve is performed and opening of facial recess to give access to the middle ear in way of a fallopian bridge technique. 2-10. PITFALLS/AVOIDANCE OF COMPLICATIONS: If there is preoperative preservation of lower CN function, it is important to not remove the anteromedial wall of the internal jugular vein and jugular bulb. In addition, facial nerve should be exposed just in case of preoperative facial palsy to decompress or reconstruct the nerve. VARIANTS AND INDICATIONS FOR THEIR USE: Variations are related mainly with temporal bone drilling depending on the extensions of the lesion, its source of blood supply, and preoperative preservation of CN function.Informed consent was obtained from the patient for the procedure and publication of his image.Anatomy images were used with permission from:• Ceccato GHW, Candido DNC, and Borba LAB. Infratemporal fossa approach to the jugular foramen. In: Borba LAB and de Oliveira JG. Microsurgical and Endoscopic Approaches to the Skull Base. Thieme Medical Publishers. 2021.• Ceccato GHW, Candido DNC, de Oliveira JG, and Borba LAB. Microsurgical Anatomy of the Jugular Foramen. In: Borba LAB and de Oliveira JG. Microsurgical and Endoscopic Approaches to the Skull Base. Thieme Medical Publishers. 2021.

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BACKGROUND: Among the several approaches described to the jugular foramen (JF), the retrosigmoid infralabyrinthine (suprajugular) approach was one of the most recently described. OBJECTIVE: To describe the indications, limitations, and operative nuances of the suprajugular approach. METHODS: We provided a pertinent review of the anatomy, indications, preoperative evaluation, surgical steps and nuances, and postoperative management. RESULTS: The suprajugular approach is suitable for tumors occupying the intracranial compartment with limited extension into the JF. Volume, width, and configuration of the foramen dictate the feasibility of the approach. Tumors invading the venous system are not suitable for this approach. Preoperative 3-dimensional MRI and computed tomography are used to evaluate intrajugular extension, relationship between the tumor and the jugular bulb (JB), venous system invasion, and shape of the JF. During surgery, exposition of the entire posterior border of the sigmoid sinus is needed and removing the bone over the JB. After identification of the JF, the jugular notch and intrajugular process of the roof of the foramen are removed and intrajugular resection is completed. In cases of high-riding JB, it may be gently pushed down to allow visualization of the anterior foramen. In cases of JB laceration, it may be repaired using a muscle patch and usually does preclude further resection. CONCLUSION: The suprajugular approach is variation of the retrosigmoid approach that, when properly indicated, provides excellent exposure of the medial JF, with most anatomical variations and intraoperative complications predicted by a comprehensive preoperative evaluation.

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Introdução. Retorno venoso extracraniano anormal é um importante fator relacionado às craniossinostoses complexas, sendo responsável por alta morbimortalidade. Associa-se a essa circulação, a estenose ou atresia do forame jugular, existindo dúvida na literatura se a presença de circulação colateral venosa é causa da estenose de forame, gerando hipertensão venosa; se o desenvolvimento da circulação venosa acontece como consequência da hipertensão intracraniana ou se existem componentes intrínsecos levando à formação de veias anômalas. Objetivo. Analisar o retorno venoso completo (intra e extracraniano) pré-operatório de pacientes com craniossinostoses complexas e sindrômicas e avaliar a sua relação com a: morfologia da base do crânio, funcionalidade dos forames e seios durais, hipertensão intracraniana e diagnostico sindrômico e molecular. Método. Estudo Retrospectivo e prospectivo de pacientes com craniossinostose complexa ou sindrômica submetidos à angiotomografia com fase venosa no Instituto Fernandes Figueira a partir de 2014. Para avaliação da gravidade do retorno venoso extracraniano foi elaborada uma classificação vascular, categorizando os pacientes em leves, moderados e graves. Essa classificação consistiu em uma pontuação em cada região de circulação colateral estudada, variando de 0 a 3. Desta forma, sendo 9 regiões de interesse (frontais, parietais, mastoides, condilares esquerda e direita e circulação transoccipital), a classificação variou entre 0 quando ausência completa de circulação colateral até 27, considerado a possibilidade mais grave apresentada. De acordo com o Cluster Hierárquico, os pacientes foram classificados em 3 categorias de circulação venosa extracraniana: Leve ­ pontuação vascular entre 0 e 9; Moderada ­ pontuação vascular entre 10 e 18; Grave ­ pontuação vascular entre 19 e 27. Os 3 grupos Moderada ­ pontuação vascular entre 10 e 18; Grave ­ pontuação vascular entre 19 e 27. Os 3 grupos foram comparados quanto à morfologia da base do crânio (áreas, medidas anteroposteriores e volumes do forame jugular, forame magno e fossa posterior), quanto ao retorno venoso intracraniano (seios venosos durais, funcionalidade do forame jugular), quanto à hipertensão intracraniana (número de suturas acometidas, presença de malformação de Chiari I, hidrocefalia, ventriculomegalia) e quanto às síndromes apresentadas (manifestações fenotípicas e estudo molecular). Resultados. Dentre o total de 45 pacientes, 44,4% (n= 20) pertenciam ao grupo leve, 37,8% (n= 17) ao grupo moderado e 17,8% (n= 8) ao grupo grave. A circulação venosa extracraniana não se correlacionou com as medidas anteroposteriores, transversas, de área e de volume dos forames jugulares, forame magno ou da fossa posterior, com o número de suturas acometidas, com a classificação dos seios venosos intracranianos e da fossa posterior, com a hidrocefalia ou ventriculomegalia. A malformação de Chiari I é mais frequente em pacientes mais graves (p valor <0,001). As mutações identificadas se correlacionaram com a gravidade da circulação venosa extracraniana (pvalor <0,001). Conclusões. A estratificação de pacientes em uma classificação vascular permite a avaliação mais adequada dos fatores que historicamente são relacionados à circulação venosa colateral em Craniossinostoses complexas e sindrômicas. As alterações venosas extracranianas não se correlacionaram diretamente com as alterações morfológicas da base do crânio, com a funcionalidade dos seios durais ou com sinais de hipertensão intracraniana. Essas alterações parecem ser resultado de manifestações intrínsecas relacionadas ao genótipo, sendo as mutações no gene FGFR2 mais gravemente relacionadas à circulação venosa extracraniana. Pacientes com síndrome de Pfeiffer apresentaram a classificação venosa extracraniana mais grave, seguido pelas síndromes de Crouzon, Apert, Jackson-Weiss, Saerthre-Chotzen e craniossinostoses complexas negativas. As veias emissárias mais frequentemente encontradas foram as condilares e mastóides, seguidas da circulação transóssea occipital. Atresia funcional do forame jugular se correlaciona com a gravidade da circulação venosa extracraniana e pode ser consequência da atividade molecular intrínseca no endotélio durante a formação do sistema venoso.

Introduction. Abnormal extracranial venous outflow is an important factor related to complex craniosynostosis, that can lead to high morbidity and mortality. It has been associated with stenosis or atresia of the jugular foramen. In fact, there is doubt in the literature if the collateral venous circulation is caused by Jugular foramen stenosis, generating venous hypertension or if the development of the venous circulation happens as a consequence of intracranial hypertension or whether if there are intrinsic components leading to the formation of anomalous veins. Objective. To analyze the preoperative complete venous outflow (intra and extracranial) of patients with complex and syndromic craniosynostosis and to evaluate its relationship with: morphology of the skull base, functionality of the jugular foramina and dural sinuses, intracranial hypertension and syndromic and molecular diagnosis. Method. A retrospective and prospective study of patients with complex or syndromic craniosynostosis on CT angiography with venous phase at Instituto Fernandes Figueira from 2014 to 2022. To assess the severity of extracranial venous drainage, a vascular classification was developed, categorizing patients into mild, moderate and severe. The classification consisted of a score in each region of collateral circulation studied, ranging from 0 to 3. Thus, with 9 regions of interest (frontal, parietal, mastoid, left and right condylar and transoccipital circulation), the classification ranged from 0 when absence collateral circulation up to 27, considered the most serious possibility presented. According to the Hierarchical Cluster, patients were in 3 extracranial circulation categories: Mild ­ vascular assessment between 0 and 9; Moderate ­ vascular assessment between 10 and 18; Severe ­ vascular assessment between 19 and 27. The 3 groups were compared regarding skull base morphology (areas, measurements of jugular foramen and foramen magnum, and posterior fossa volumes), intracranial venous outflow (dural venous sinuses, jugular foramen functionality), and intracranial hypertension (number of affected sutures, presence of Chiari I malformation, hydrocephalus, ventriculomegaly) and the syndromes presented (phenotypic manifestations and molecular study). Results. Among the 45 patients, 44.4% (n=20) belonged to the mild group, 37.8% (n=17) to the moderate group and 17.8% (n=8) to the severe group. The extracranial venous circulation does not correlate with the anteroposterior, transverse, area and volume measurements of the jugular foramen, foramen magnum or posterior fossa, with the number of affected sutures, with the intracranial venous sinuses and the posterior fossa, with the hydrocephalus or ventriculomegaly. Chiari I malformation correlates with the most severe patients (p value <0.001). The mutations identified correlate with the severity of the extracranial venous circulation (p-value <0.001). Conclusions. The stratification of patients in a vascular classification allows a more adequate evaluation of the factors that are historically related to the collateral venous circulation in complex and syndromic craniosynostosis. Extracranial venous changes did not directly correlate with morphological changes in the skull base, functionality of the dural sinuses, or with signs of intracranial hypertension. These alterations seem to be the result of intrinsic manifestations related to the genotype, with mutations in the FGFR2 gene most severely related to extracranial venous circulation. Patients with Pfeiffer syndrome had the most severe extracranial venous classification, followed by Crouzon, Apert, Jackson-Weiss, Saerthre-Chotzen and complex negative craniosynostosis. The emissary veins most frequently found were the condylar and mastoid veins, followed by the occipital transosseous circulation. Functional atresia of the jugular foramen correlates with the severity of extracranial venous circulation and may be a consequence of intrinsic molecular activity in the endothelium during formation of the venous system.

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Glomus jugular tumors, also known as paragangliomas (PGLs), are rare and related to several clinical syndromes described. These are located in the carotid body, the jugular glomus, the tympanic glomus and the vagal glomus. The symptoms are directly related to the site of involvement and infiltration. These lesions have slow growth, are generally benign and hypervascularized, have a peak incidence between the age of 30 to 50 years old; however, when associated with hereditary syndromes, they tend to occur a decade earlier. Several familial hereditary syndromes are associated with PGLs, including Von Hippel- Lindau disease (VHL) in< 10% of the cases. The diagnosis and staging of PGLs are based on imaging and functional exams (bone window computed tomography [CT] with a "ground moth" pattern and magnetic resonance imaging (MRI) with a "salt and pepper" pattern). The cerebral angiography is a prerequisite in patients with extremely vascularized lesions, whose preoperative embolization is necessary. The histopathological finding of cell clusters called "Zellballen" is a characteristic of PGLs. Regarding the jugular foramen, the combination of two or three surgical approaches may be necessary: (1) lateral group, approaches through themastoid; (2) posterior group, through the retrosigmoid access and its variants; and (3) anterior group, centered on the tympanic and petrous bone. In the present paper, we report a case of PGL of the jugular foramen operated on a young female patientwho underwent a surgery with a diagnosis ofVonHippel-Lindau Disease (VHL) at the Neurosurgery Service of the Hospital Heliópolis, São Paulo, state of São Paulo, Brazil in 2018, by the lateral and posterior combined route.

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SUMMARY: The objective of this study was to determine the occurrence of anatomical variants in the exocranial surface of the jugular foramen, specifically, the presence of single or double and complete or incomplete septation. A cross-sectional anatomical study was performed using 96 Brazilian dry human skulls (53 male and 43 female). One examiner determined the number (single or double) and type (i.e. complete or incomplete) of osseous septation at the outer surface of jugular foramens. Data went through statistical analysis on GraphPad Prism 6.01. Our results shown that Male individuals where more likely to present normal jugular foramens (male = 71.69%, female = 34.88%; p = 0.003). However, one incomplete septation occurred more often on the right side of female individuals (1 incomplete septation, male = 16.98%; 1 incomplete septation, female = 34.88%; p = 0.044). Similarly, one complete septation (i.e. the presence of two fully divided jugular compartments) also occurred more often on the right side of female individuals (1 complete septation, male = 9.43%; 1 complete septation, female = 25.58%; p = 0.038). Anatomical variants of the jugular foramen regarding single or double complete or incomplete septations were more likely to be found on the right side of female individuals, whose also presented a higher rate of jugular foramens with any type of septation than regular non-altered jugular foramens.

RESUMEN: El objetivo de la presente investigación fue determinar la presencia de variaciones anatómicas en la superficie exocraneal del foramen yugular, especificamente, la presencia de septos únicos o dobles, completos o incompletos. El estudio fue realizado en 96 cráneos secos (53 masculinos y 43 femeninos) de indivíduos Brasileños. Se determinaron septos óseos completos o incompletos y número de ellos. Los resultados obtenidos fueron tratados estadísticamente con el programa GraphPad Prism 6.01. Los sujetos de sexo masculino fueron más propensos a presentar forámenes yugulares normales (sexo masculino: 71,69%; sexo femenino: 34,88%, p= 0,003). Sin embargo, se observaron septos incompletos con mayor frecuencia en el lado derecho y en el sexo femenino (sexo masculino: 16,98%; sexo femenino: 34,88%, p=0,044). Adicionalmente, una septación completa (presencia de dos compartimientos yugulares, divididos completamente), se presentaron más frecuentemente en el lado derecho de indivíduos femeninos (sexo masculino: 9,43%; sexo femenino: 25,58%, p= 0,038). Las variantes anatómicas del foramen yugular, en relación a septos simples o dobles, completos o incompletos, se encontraron con mayor frecuencia en el lado derecho de las mujeres, las que presentaron un alto rango de forámenes yugulares con algún tipo de septos respecto a los forámenes yugulares regulares no alterados.

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Abstract Introduction The anatomical complexity of the jugular foramen makes surgical procedures in this region delicate and difficult. Due to the advances in surgical techniques, approaches to the jugular foramen became more frequent, requiring improvement of the knowledge of this region anatomy. Objective To study the anatomy of the jugular foramen, internal jugular vein and glossopharyngeal, vagus and accessory nerves, and to identify the anatomical relationships among these structures in the jugular foramen region and lateral-pharyngeal space. Methods A total of 60 sides of 30 non-embalmed cadavers were examined few hours after death. The diameters of the jugular foramen and its anatomical relationships were analyzed. Results The diameters of the jugular foramen and internal jugular vein were greater on the right side in most studied specimens. The inferior petrosal sinus ended in the internal jugular vein up to 40 mm below the jugular foramen; in 5% of cases. The glossopharyngeal nerve exhibited an intimate anatomical relationship with the styloglossus muscle after exiting the skull, and the vagal nerve had a similar relationship with the hypoglossal nerve. The accessory nerve passed around the internal jugular vein via its anterior wall in 71.7% of cadavers. Conclusion Anatomical variations were found in the dimensions of the jugular foramen and the internal jugular vein, which were larger in size on the right side of most studied bodies; variations also occurred in the trajectory and anatomical relationships of the nerves. The petrosal sinus can join the internal jugular vein below the foramen.

Resumo Introdução A complexidade anatômica do forame jugular torna a realização de procedimentos cirúrgicos nessa região delicada e difícil. Devido aos avanços obtidos nas técnicas cirúrgicas, as abordagens do forame jugular têm sido feitas com maior frequência, o que requer uma melhoria correspondente no conhecimento de sua anatomia. Objetivo Estudar a anatomia do forame jugular, da veia jugular interna e dos nervos glossofaríngeo, vago e acessório, assim como as relações anatômicas entre estas estruturas na região do forame jugular e no espaço parafaríngeo. Método Foram examinados 60 lados de 30 cadáveres frescos algumas horas após a morte. Os diâmetros e suas relações anatômicas foram analisados. Resultados Os diâmetros do forame jugular e da veia jugular interna foram maiores no lado direito na maioria dos espécimes estudados. O seio petroso inferior terminava na veia jugular interna até 40 mm abaixo do forame jugular, em 5% dos casos. O nervo glossofaríngeo exibiu uma relação íntima anatômica com o músculo estiloglosso após a sua saída do crânio e o nervo vago exibiu uma relação semelhante com o nervo hipoglosso. O nervo acessório passou em torno da veia jugular interna via sua parede anterior em 71,7% dos cadáveres. Conclusão Foram encontradas variações anatômicas nas dimensões do forame jugular e da veia jugular interna, que apresentaram tamanhos maiores à direita na maioria dos espécimes estudados; variações também ocorreram na trajetória e nas relações anatômicas dos nervos. O seio petroso pode se unir à veia jugular interna abaixo do forame.

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INTRODUCTION: The anatomical complexity of the jugular foramen makes surgical procedures in this region delicate and difficult. Due to the advances in surgical techniques, approaches to the jugular foramen became more frequent, requiring improvement of the knowledge of this region anatomy. OBJECTIVE: To study the anatomy of the jugular foramen, internal jugular vein and glossopharyngeal, vagus and accessory nerves, and to identify the anatomical relationships among these structures in the jugular foramen region and lateral-pharyngeal space. METHODS: A total of 60 sides of 30 non-embalmed cadavers were examined few hours after death. The diameters of the jugular foramen and its anatomical relationships were analyzed. RESULTS: The diameters of the jugular foramen and internal jugular vein were greater on the right side in most studied specimens. The inferior petrosal sinus ended in the internal jugular vein up to 40mm below the jugular foramen; in 5% of cases. The glossopharyngeal nerve exhibited an intimate anatomical relationship with the styloglossus muscle after exiting the skull, and the vagal nerve had a similar relationship with the hypoglossal nerve. The accessory nerve passed around the internal jugular vein via its anterior wall in 71.7% of cadavers. CONCLUSION: Anatomical variations were found in the dimensions of the jugular foramen and the internal jugular vein, which were larger in size on the right side of most studied bodies; variations also occurred in the trajectory and anatomical relationships of the nerves. The petrosal sinus can join the internal jugular vein below the foramen.

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