Cirurgia híbrida na exérese de tumor glômico Shamblin II / Hybrid surgery in excision of a Shamblin II glomus tumor

O tumor glômico é uma neoplasia benigna rara originada de células paraganglionares da crista neural que se desenvolve na camada adventícia do vaso. São tumores não encapsulados e altamente vascularizados. Paciente feminina, 64 anos, foi diagnosticada com tumor glômico hipervascularizado com 5 cm posteriormente à bifurcação carotídea esquerda e oclusão de carótida contralateral. Optou-se por realizar embolização através de acesso endovascular seguida de punção percutânea direta, guiada por angiografia, para preenchimento da área remanescente. Após embolização, realizou-se a exérese cirúrgica do tumor com menor sangramento e maior facilidade para encontrar o plano de clivagem das estruturas adjacentes. Em acompanhamento tardio, a paciente apresenta-se sem recidiva tumoral. O tumor foi classificado como pertencente ao grupo Shamblin II, o qual inclui tumores apresentando de 4 a 6 cm com inserção arterial moderada. Através dessa dupla abordagem, foi possível notar uma redução relativa do sangramento intraoperatório e facilitação de identificação do plano de clivagem, colaborando para sua exérese e evitando o pinçamento cirúrgico

Glomus tumors are rare benign neoplasms originating from paraganglionic cells of the neural crest developing in the adventitious layer of the vessel. They are nonencapsulated and highly vascularized. A 64-year-old female patient was identified with a hypervascularized glomus tumor measuring 5 cm, posterior to the left carotid bifurcation and contralateral carotid occlusion. We performed preoperative embolization via endovascular access followed by direct percutaneous puncture, guided by angiography, to fill the remaining area. After embolization, surgical excision of the tumor was performed with reduced bleeding and it was easier to find the cleavage planes to adjacent structures. At late follow-up, the patient is free from tumor recurrence. The tumor was classified as Shamblin II, measuring 4 to 6 cm with moderate arterial insertion. Through this double approach we observed a relative reduction in intraoperative bleeding and improved identification of the cleavage plane, facilitating excision and avoiding surgical clamping

[Complications and pitfalls in surgery of the ear/lateral skull base]. / Fehler und Gefahren: Ohrchirurgie und Chirurgie der lateralen Schädelbasis.

Surgery of the ear and the lateral skull base is a fascinating, yet challenging field in otorhinolaryngology. A thorough knowledge of the associated complications and pitfalls is indispensable for the surgeon, not only to provide the best possible care to his patients, but also to further improve his surgical skills.Following a summary about general aspects in pre-, intra- and postoperative care of patients with disorders of the ear/lateral skull base, this article covers the most common pitfalls and complications in stapes surgery, cochlear implantation, surgery of vestibular schwannomas, and jugulotympanal paragangliomas. Based on these exemplary procedures, basic "do's and don'ts" of skull base surgery are explained, which the reader can easily transfer to other disorders. Special emphasis is laid on functional aspects, such as hearing, balance and facial nerve function. Furthermore, the topics of infection, bleeding, skull base defects, quality of life and indication for revision surgery are discussed.An open communication about complications and pitfalls in ear/lateral skull base surgery among surgeons is a prerequisite for the further advancement of this fascinating field in ENT surgery. This article is meant to be a contribution to this process.

[The arrest of bleeding from the sigmoid sinus and the upper segments of internal jugular vein].

The objective of the present work was to develop an efficacious non-injurious technique for the arrest of hemorrhage from the sigmoid sinus and the upper segments of the internal jugular vein. The authors proposed a relatively simple and highly effective method to be used to the blockade of the sigmoid sinus. It was employed to treat 8 patients none of whom suffered complications in the late postoperative period (the follow-up study lasting from 6 months to 2 years after the intervention).

Cranial nerve preservation and outcomes after stereotactic radiosurgery for jugular foramen schwannomas.

OBJECTIVE: Jugular foramen region schwannomas are rare intracranial tumors that usually present with multiple lower cranial nerve deficits. For some patients, complete surgical resection is possible but may be associated with significant morbidity. Stereotactic radiosurgery is a minimally invasive alternative or adjunct to microsurgery for such tumors. We reviewed our clinical and imaging outcomes after patients underwent gamma knife radiosurgery for management of jugular foramen schwannomas. METHODS: Thirty-four patients with 35 tumors (one patient had bilateral tumors) underwent radiosurgery between May 1990 and December 2005. Twenty-two patients had previous microsurgical resection and all patients experienced various cranial neuropathies. A median of six isocenters were used. Median marginal and maximum doses were 14 and 28 Gy, respectively. RESULTS: None of the patients were lost to evaluation and the mean duration of follow-up was 83 months. Tumors regressed in 17 patients, remained stable in 16, and progressed in two. Five- and 10-year actuarial control rates were 97 and 94%, respectively. Preexisting cranial neuropathies improved in 20% and remained stable in 77% after radiosurgery. One patient worsened. The function of all previous intact nerves was preserved after radiosurgery. CONCLUSION: Stereotactic radiosurgery proved to be a safe and effective management for newly diagnosed or residual jugular foramen schwannomas. Long-term tumor control rates and stability or improvement in cranial nerve function was confirmed.

[The management of high jugular bulb and its active bleeding during middle ear surgery].

OBJECTIVE: To study the incidence of high jugular bulb (HJB) in the patients with chronic otitis media and to explore the management of its active bleeding during middle ear surgery. METHOD: From January 2005 to August 2006, consecutive 287 patients undergoing surgical intervention for chronic otitis media were retrospectively studied. The high resolution CT (HRCT) scan and the surgical technique for diagnosis and management of bleeding of jugular bulb were also discussed. RESULT: Sixty-five patients (22.6%) were diagnosed as HJB by HRCT scan, including 21 men and 44 women (P<0.01), with their ages ranged from 7 to 68 years (mean 41 years). Fourteen cases of them were on the left side, and 31 were on the right side (P<0.05). Twenty cases showed bilateral HJB. Five cases were found dehiscent high jugular bulb by CT scan and were confirmed in operation. Active bleeding was encountered in one case while elevating the pathological eardrum in hypotympanum. It was treated by pressure compression using collagen gelfoam cushion covered by muscle to protect the dehiscent bulb. The patients proceed through the planned surgery without complications. CONCLUSION: HJB might cause hemorrhage during middle ear surgery, but preliminary attention, correct treatment and awareness of the pitfalls may lessen the operation risk.

Tumors of the jugular foramen: diagnosis and management.

OBJECTIVE: Jugular foramen tumors are rare cranial base lesions that present diagnostic and management difficulties. With the availability of new diagnostic procedures these tumors have been more precisely studied and questions of whether, when, and how these lesions should be treated often arise. Data from 106 consecutive patients surgically treated in the past 17 years were retrospectively analyzed to identify surgical outcomes. METHODS: The tumors were approached by a multidisciplinary team composed of neurosurgeons, ear, nose, and throat surgeons, and neuroradiologists. Hypervascular lesions were embolized 3 to 5 days before surgery. The same surgical technique was used to resect all tumors. The surgical defect was covered with vascularized myofascial flaps. The internal carotid artery was infiltrated in two patients, and a saphenous graft bypass was carried out before removal of the lesions. The facial nerve was reconstructed with nerve grafts (great auricular nerve) or XII/VII anastomosis in five cases. Postoperative radiotherapy was carried out for malignant and invasive tumors. RESULTS: Paragangliomas were the most frequent lesions, followed by schwannomas and meningiomas. Complete excision was possible in 89% of benign tumors and 80% of paragangliomas. Lower cranial nerve deficit was the most frequent complication (10 patients, 9.4%), transient in 4 patients. Facial and cochlear nerve paralysis occurred in 8 patients (7.5%). The function of the facial nerve recovered spontaneously in 3 patients. Four patients (3.7%) developed postoperative cerebrospinal fluid leakage. Four patients (3.7%) died after surgery. CONCLUSION: Radical removal of benign jugular foramen tumors is the treatment of choice and may be curative. Large lesions can be radically excised in one surgical procedure with preservation of lower cranial nerves. Cranial base reconstruction with vascularized myofascial flaps reduces the incidence of postoperative cerebrospinal fluid leakage. Damage of the lower cranial nerves is the most serious surgical complication.

Intradural jugular foramen tumors.

Eleven patients with jugular foramen lesions with or without extradural extension were operated at University Hospital of Campinas (UNICAMP), in Campinas, Brazil, between 1998 and 2001. Neck dissection, mastoidectomy without transposition of the facial nerve and myofascial flap reconstruction of the cranial base with an especially developed technique were carried out in 7 patients. Four patients were operated using retrosigmoid craniectomy. Total excision was accomplished in 9 cases. All patients did not show evidence of disease progression at least after 2 years follow-up. There was no mortality. New lower cranial nerve deficits occurred in 5 patients. Nine maintain or improved their preoperative status based on Karnofsky and Glasgow Outcome Scale. A complex anatomy of this region demand wide exposures for treat those tumors. For this reason, an adequate approach for curative resection of most lesions and an efficient skull base reconstruction decreasing postoperative morbidity are essential.

Intradural jugular foramen tumors

Onze pacientes com lesões expansivas do forame jugular associadas ou não a componente extradural foram submetidos a ressecção cirúrgica no Hospital das Clínicas da Universidade Estadual de Campinas (UNICAMP) entre 1998 e 2001. Foi utilizada cirurgia combinada com dissecção do pescoço, mastoidectomia sem transposição do nervo facial e técnica de reconstrução miofascial da base do crânio desenvolvida por um dos autores. Quatro pacientes foram operados via craniectomia retrosigmoidea. Ressecção total foi feita em 9 pacientes, subtotal em um e parcial em outro. Nenhum dos 11 pacientes teve progressão da doença em 2 anos de acompanhamento. Não houve mortalidade. Cinco pacientes tiveram déficits adicionais de nervos cranianos baixos. Nove pacientes mantiveram ou melhoraram suas pontuações de acordo com a escala de Karnofsky. Para adequada abordagem do paciente com tumor de forame jugular, são necessários bom conhecimento anatômico da região, exposição cirúrgica ampla, e técnica de reconstrução eficiente para obter cura com baixas taxas de complicações pós-operatórias.

Intradural jugular tubercle reduction to enhance exposure via the transcondylar approach: technical note.

OBJECTIVE: The jugular tubercle can present an obstruction to adequate visualization of posteroinferior cerebellar artery (PICA) or vertebrobasilar junction aneurysms approached via a far lateral transcondylar approach. Reduction of the tubercle via an intradural approach was performed to enhance exposure. The technical details of this maneuver are discussed and demonstrated. METHODS: Two patients with PICA aneurysms were treated via the far lateral transcondylar approach. Intradural reduction of the jugular tubercle was performed to enhance exposure in each case. RESULTS: Exposure of the parent artery and aneurysm was enhanced in each case by intradural reduction of the jugular tubercle, providing several millimeters of added space. Both PICA aneurysms were successfully clipped with no intraoperative complications. CONCLUSION: Several millimeters of additional exposure can be obtained when treating a PICA or vertebrobasilar junction aneurysm via the far lateral transcondylar approach by removing the jugular tubercle via an intradural approach.

Anatomic landmarks of the glossopharyngeal nerve: a microsurgical anatomic study.

OBJECTIVE: Compared with other lower cranial nerves, the glossopharyngeal nerve (GPhN) is well hidden within the jugular foramen, at the infratemporal fossa, and in the deep layers of the neck. This study aims to disclose the course of the GPhN and point out landmarks to aid in its exposure. METHODS: The GPhN was studied in 10 cadaveric heads (20 sides) injected with colored latex for microsurgical dissection. The specimens were dissected under the surgical microscope. RESULTS: The GPhN can be divided into three portions: cisternal, jugular foramen, and extracranial. The rootlets of the GPhN emerge from the postolivary sulcus and course ventral to the flocculus and choroid plexus of the lateral recess of the fourth ventricle. The nerve then enters the jugular foramen through the uppermost porus (pars nervosa) and is separated from the vagus and accessory nerves by a fibrous crest. The cochlear aqueduct opens to the roof of this porus. On four sides in the cadaver specimens (20%), the GPhN traversed a separate bony canal within the jugular foramen; no separate canal was found in the other cadavers. In all specimens, the Jacobson's (tympanic) nerve emerged from the inferior ganglion of the GPhN, and the Arnold's (auricular branch of the vagus) nerve also consisted of branches from the GPhN. The GPhN exits from the jugular foramen posteromedial to the styloid process and the styloid muscles. The last four cranial nerves and the internal jugular vein pass through a narrow space between the transverse process of the atlas (C1) and the styloid process. The styloid muscles are a pyramid shape, the tip of which is formed by the attachment of the styloid muscles to the styloid process. The GPhN crosses to the anterior side of the stylopharyngeus muscle at the junction of the stylopharyngeus, middle constrictor, and hyoglossal muscles, which are at the base of the pyramid. The middle constrictor muscle forms a wall between the GPhN and the hypoglossal nerve in this region. Then, the GPhN gives off a lingual branch and deepens to innervate the pharyngeal mucosa. CONCLUSION: Two landmarks help to identify the GPhN in the subarachnoid space: the choroid plexus of the lateral recess of the fourth ventricle and the dural entrance porus of the jugular foramen. The opening of the cochlear aqueduct, the mastoid canaliculus, and the inferior tympanic canaliculus are three landmarks of the GPhN within the jugular foramen. Finally, the base of the styloid process, the base of the styloid pyramid, and the transverse process of the atlas serve as three landmarks of the GPhN at the extracranial region in the infratemporal fossa.

[Attical fixation of the malleus head associated with obstruction of the round window niche by a high jugular bulb]. / Blocage attical de la tête du marteau associé à une obstruction de la fenêtre ronde par le golfe de la jugulaire.

OBJECTIVE: To report the case of a 52 year-old-woman presenting with a left mixed hearing loss. Surgical exploration revealed an attical fixation of the malleus head associated with obliteration of the round window by a high jugular bulb. METHOD: Surgical exploration revealed this rare association. Attical fixation of the malleus head was treated by a partial prosthesis. The mobility of the chain was normal at the end of the surgical exploration but it was not possible to search for the round window sign because the niche of the round window was obliterated by an high Jugular bulb. RESULTS: Audiogram was unchanged even if CT-scan confirm the good position of the partial prosthesis. CONCLUSION: Surgical treatment of an attical fixation of the malleus head do not seem to be necessary if it is associated with a simultaneous obliteration of the round window. Indeed, obstruction of the round window by a high Jugular bulb can caused a conductive hearing loss.

Efficiency of gamma knife radiosurgery in the treatment of glomus jugulare tumors.

This study was performed to assess the impact of gamma knife radiosurgery (RS) in the treatment of glomus jugulare tumors. Between February 1993 and February 1999, thirteen patients (9 women, 4 men; mean age 63.5 years, range 29 to 79 years) underwent stereotactic radiosurgery for glomus jugulare tumors with the Leksell Gamma Knife at the Neurosurgical Department of the University of Vienna. Four patients, mean age 74.5 years, range 67 to 79 years, underwent radiosurgery as the only treatment. Nine patients received radiosurgery as adjuvant therapy after previous treatment had failed: surgical resection in 9 patients and additional fractionated external beam radiation in two of these patients. Pretreatment evaluation included the staging of all tumors according to the Fisch Classification: De1 (7), De2 (1), Di1 (4) and Di2 (1). The mean follow-up period was 4.2 years, range 0.7 to 6.7 years. Ten patients, 77 %, were treated prior to 1997, the mean follow-up period being 5 years. Six patients showed no clinical changes, while six experienced an improvement of their clinical symptoms and neurological deficits. One patient was lost to follow-up. Radiation-induced transient cranial nerve neuropathies were observed in two patients. Serial MRI scans revealed tumor control in all patients, with unaltered tumor size in 10 and shrinkage in three patients. The results indicate that RS is an attractive treatment option for glomus jugulare tumors and will occupy an increasingly important role in the management of these tumors in selected patients.

Radiología y timpanometría en el glomus yugular / Radiology and tympanogram in the yugular glomus

Los paragangliomas yugulotimpánicos son tumores relativamente frecuentes en el oído. Han sido fuente de múltiples publicaciones pero en ninguna de las revisadas se ha demostrado, a pesar de ser casi constantemente mencionada, la curva timpanométrica característica en dientes de sierra junto con un relato exhaustivo de los hallazgos mediante técnicas de imagen. Se presenta un nuevo caso en el que se ha podido completar con abundante iconografía, entre la cual se incluye una curva timpanometría característica. Se revisan los datos más importantes de estos tumores y se incluye estudios de imagen con un amplio comentario (AU)

[Jugular bulb anomaly].

OBJECTIVE: To differentiate and treat correctly the jugular bulb anomalies, as well as to avoid the unnecessarily enlarged operations. METHODS: Four cases of jugular bulb anunalies were reported and analysed. RESULTS: Two of 4 cases were misdiognosed as jugular glomus tumors; One was considered to be jugular bulb anomaly, but was done with an enlarged operation; another one was still accompanied with benign intracranial hypertension syndrome, was inappropriately treated. CONCLUSION: The jugular bulb anomaly was often misdiagnosed, therefore the anomaly should be differentiated from the jugular glomus tumor carefully.

The variable relationship between the lower cranial nerves and jugular foramen tumors: implications for neural preservation.

Tumors involving the jugular foramen (JF) have a variable relationship to the neurovascular structures (jugular vein, cranial nerves IX-XI) that traverse this conduit through the skull base. The surgeon familiar with the site of origin, growth pattern, and geometry of each of the common lesions affecting this region with respect to surrounding nerves and vessels is at a considerable advantage when undertaking a function-sparing procedure. Anatomically, the JF has two vascular compartments that may be affected by tumor: the jugular bulb laterally and a passage for the inferior petrosal sinus medially. Tumors may also penetrate the JF along the fibro-osseous diaphragm, which divides these two vascular channels. The lower cranial nerves lie on either side of this partition, which is connected to the posterior cranial fossa via a curved, funnel-shaped cone of dura. Tumors that arise within or penetrate the JF lateral to this neural plane displace the nerves medially, a position favorable for their preservation during tumor extirpation. By contrast, medially positioned tumors displace the cranial nerves onto the lateral tumor surface, where they interpose between surgeon and tumor-an unfavorable location. Glomus tumors consistently arise in the lateral aspect of the JF, displacing the lower cranial nerves medially. This positioning accounts for the high rate of neural preservation in small and medium-size glomus tumors that have not invaded the foramen's central partition. Meningiomas that arise lateral to the JF (e.g., the posterior petrous surface, sigmoid sinus) favorably displace the lower cranial nerves medially. By contrast, tumors that originate medial to the JF (e.g., clivus, foramen magnum) are unfavorable, laterally displacing the multiple small rootlets that coalesce into cranial nerves IX-XI into a vulnerable location. Schwannomas arise within the neural plane and have a variable geometry that depends, in part, upon the nerve of origin. Theoretically, tumors that arise from the ninth nerve, which is located on the lateral surface of the neural plane, should be more favorable than those originating from the tenth or eleventh nerves, which lie on its deep surface. The propensity of these three tumor types toward thrombosis of the jugulosigmoid complex also carries important surgical implications. Because glomus tumors arise from the jugular bulb, the jugulosigmoid complex is nearly always occluded. In both meningiomas and schwannomas, however, the jugular system may occasionally remain patent. This is important to recognize through angiography and/or magnetic resonance venography, since sacrifice of a patent, dominant system risks intracerebral venous infarction.

[Differential diagnosis of neurinomas of the jugular foramen and glomus jugulare: a clinical case]. / Diagnóstico diferencial entre neurinomas de agujero rasgado posterior y glomus yugular: Caso clínco.

Neurinomas or schwannomas of the jugular foramen are rare tumors whose differentiation from tumors of the jugular body may be difficult because they share common symptoms. Since schwannomas at this site are less aggressive than tumors of the jugular body and certain elements of their treatment differ, it is important to make a correct diagnosis promptly. A well-documented case of schwannoma of the jugular foramen is reported and its differential diagnosis is discussed in the light of the literature.

Management of the high jugular bulb in the translabyrinthine approach.

A high jugular bulb is a frequent problem in the translabyrinthine approach. This article described a safe technique for effective inferior displacement of the high bulb.