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Luis A. B. Borba, Samir Ale-Bark and Charles London

Object

Glomus jugulare tumors are benign lesions located in the jugular foramen that may or may not extend into the middle ear, petrous apex, and upper neck; these growths sometimes invade intradurally. The surgical management of these tumors is a challenge to neurosurgeons and skull base surgeons. Because of their abundant vascularity, deep location, complex anatomy, and difficult surgical approach, their treatment, has been a controversial issue for many years. Despite advancements in nonsurgical techniques, the only treatment with proven efficacy is radical surgical removal. The authors present a series of patients treated with radical removal, in which the feasibility of removing glomus jugulare tumors with low morbidity and a surgical approach limited to tumor removal are discussed. The extent of surgical exposure is tailored with emphasis placed on the routine anterior transposition of the facial nerve.

Methods

Between May 1997 and March 2004, 24 patients with glomus jugulare tumors were treated; 17 patients were women and seven were men. Their mean age at the time of diagnosis was 50 years (range 18–71 years). The most common symptom was hearing loss in 77%, followed by dysphagia and dysphonia in 55% of patients. In seven patients the clinical presentation was a facial palsy. Radical tumor removal was achieved in 23 patients. An anterior facial nerve transposition was not needed in any case. No surgery-related death was recorded in this series, although one patient died of a pulmonary embolism 70 days after the procedure. A one-stage procedure was performed in 23 patients and a two-stage procedure was used in the other patient. Cerebrospinal fluid leakage occurred in two patients. The lower cranial nerve function was worse in eight patients; however, only one had a new deficit. The facial nerve was preserved in all patients except one, in whom a large intradural tumor caused a temporary facial palsy. In the patients with pre-operative facial palsy, the tumor only compressed the nerve in three and it invaded the nerve in four. The nerve was decompressed in the cases with no invasion and a graft was placed in the others. The greater auricular nerve was used as a graft in three and the sural nerve was used in one. On follow-up review, the facial nerve function was House–Brackmann Grade 3 in three patients and Grade 2 in three. After 6 months of follow up with no improvement, one patient was referred for a facial muscle transfer.

Conclusions

The surgical technique must be tailored to each case. The authors believe that the standard surgical approach to jugular foramen tumors with anterior transposition of the facial nerve should be avoided, and that the extent of surgical exposure must be tailored to each case based on the extent of the tumor and the clinical symptoms. Lower morbidity rates and radical removal can be achieved with a good surgical plan.

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Ossama Al-Mefty and Luis A. B. Borba

✓ Because of their critical location, invasive nature, and aggressive recurrence, skull base chordomas are challenging and, at times, frustrating tumors to treat. Both radical surgical removal and high-dose radiation therapy, particularly proton beam therapy, reportedly are effective in tumor control and improve survival rates. The authors posit that these tumors are best treated with radical surgery and proton—photon beam therapy. During the last 5 years, they treated 25 patients (15 females and 10 males) who harbored pathologically diagnosed skull base chordomas. The mean age of the patients was 38.4 years (range 8–61 years). Previous surgery or radiation therapy was performed at other institutions in seven and two patients, respectively. The authors performed 33 surgical procedures on 23 patients. Radical removal (defined as absence of residual tumor on operative inspection and postoperative imaging) was achieved in 10 patients; subtotal resection (defined as resection of > 90% of the tumor) was achieved in 11 patients; and partial resection (defined as resection of < 90% of the tumor) was achieved in two patients. Radical surgical removal included not only the excision of soft-tumor tissue, but also extensive drilling of the adjacent bone. Adjuvant therapy consisted of postoperative combined proton—photon beam therapy (given to 17 patients and planned for one patient) and conventional radiation therapy (two patients); three patients received no adjunct therapy. To date, four patients have died. One patient who had undergone previous surgery and sacrifice of the internal carotid artery died postoperatively from a massive stroke; one patient died from adenocarcinoma of the pancreas without evidence of recurrence; and two patients died at 25 and 39 months of recurrent tumor. Permanent neurological complications included third cranial nerve palsy (one patient) and hemianopsia (one patient); radiation necrosis occurred in three patients. Of the 21 patients followed for more than 3 months after surgery, 16 have had no evidence of recurrence and five (including the two mortalities noted above) have had recurrent tumors (four diagnosed clinically and one radiologically). The mean disease-free interval was 14.4 months. A longer follow-up period will, hopefully, support the early indication that radical surgical removal and postoperative proton—photon beam therapy is an efficacious treatment. The use of skull base approaches based on the tumor classification introduced in this paper is associated with low mortality and morbidity rates.

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Marcio S. Rassi, Jean G. de Oliveira and Luis A. B. Borba

Surgical removal of foramen magnum meningiomas poses great challenges due their deep location within the central skull base and their proximity to vital neurovascular structures. This video depicts the operative nuances of surgical management for a 59-year-old female who presented with a right-sided spinocranial meningioma. Simpson Grade I resection was achieved through a right transcondylar approach. The patient’s postoperative period was unremarkable, and she was discharged home on postoperative Day 5 for periodic follow-up. The transcondylar approach safely exposes the craniocervical junction at the anterior aspect of the neuraxis and still allows the surgeon to access the tumor through a parallel plane, with minimum morbidity.

The video can be found here: https://youtu.be/P0-kXjAkw9U.

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Luis A. B. Borba, Ossama Al-Mefty, Robert E. Mrak and James Suen

✓ Chordomas are rare tumors that usually occur in adults. This report describes four cases of intracranial chordomas treated in patients 20 years of age or younger by the senior author (O.A.M.) during a 4-year period. The authors also reviewed the literature on pediatric patients, which revealed that the clinical presentations, histological patterns, and behaviors of these tumors differ considerably depending on whether the patient is younger or older than 5 years of age. The younger population had a wider range of presenting symptoms, a greater prevalence of atypical histological findings with aggressive behavior, a greater range of cellularity than the classic chordomas, and a higher instance of metastasis; it showed no chondroid component compared to a 17.1% instance in the older patients. The prognosis for patients with a chordoma is related directly to the histological pattern of the tumor; the atypical chordoma carries a poor prognosis. The prognoses for children older than 5 years of age with a classic or chondroid tumor were not significantly different (p = 0.788). At follow up, the difference in survival rates between patients undergoing surgery plus radiation therapy and those who had surgery alone was statistically significant (p = 0.00446). No correlation was found between radical resection or radiation therapy and an improved prognosis for patients younger than 5 years of age who had a tumor with an atypical histological pattern. This study identifies and delineates the distinction between these age groups and provides a review of the potential prognostic factors.

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Ossama Al-Mefty, Luis A. B. Borba, Nobuo Aoki, Edgardo Angtuaco and T. Glenn Pait

✓ Ventral extradural lesions at the craniovertebral junction are commonly exposed through the transoral or transmaxillary approach. The disadvantages of these approaches include: 1) difficulty in reaching laterally located lesions; 2) ineligibility of patients with an intradental distance of less than 25 mm or severe macroglossia; 3) the need for a separate procedure for stabilization and fusion; and 4) the risk of infection from transgressing a contaminated field. In this report, the authors describe the use of the transcondylar approach to extradural nonneoplastic lesions of the anterior craniovertebral junction for decompression and stabilization. Advantages of this approach include: 1) a short distance to the lesion; 2) a wide surgical envelope; 3) direct visualization of the dural sac, eliminating manipulation of the brainstem or upper spinal cord; 4) easy identification and control of the ipsilateral vertebral artery; 5) direct visualization and preservation of the lower cranial nerves; and 6) a sterile field. In addition, occipitocervical fusion and instrumentation can be performed during the same procedure. The transcondylar approach, based on anatomical studies in cadavers, was used to treat eight patients with ventral nonneoplastic lesions at the craniocervical junction. The technique and results are described.

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Jean G. de Oliveira, Carlos R. Massella Jr., Carlos Vanderlei M. de Holanda, Miguel Giudicissi-Filho and Luis A. B. Borba

The recommendation for the great majority of high-grade brain arteriovenous malformations (bAVMs) is observation, except for those patients with recurrent hemorrhages, progressive neurological deficits, steal-related symptoms, or AVM-related aneurysms, for whom intervention should be considered. These are general recommendations, and the decision should be made on an individual basis. Surgical resection of an AVM in the central lobe may cause postoperative sensorimotor deficits since this anatomical region includes the pre- and postcentral gyri on the lateral surface and the paracentral lobule on the medial surface.

The authors present a patient with a ruptured high-grade bAVM in the central lobe who underwent previous surgery for hematoma evacuation and previous radiosurgery, and whose indication for reoperation was proposed based on progressive hemiparesis. Microsurgical resection was possible after a wide frontoparietal craniotomy, which made all the nidus borders accessible. This case illustrates the anatomy and surgical technique for large nidus AVMs in eloquent areas, showing that complete microsurgical resection is possible with good clinical outcome.

The video can be found here: https://youtu.be/Cpd1PK6BLIM.

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Guilherme H. W. Ceccato, Rodolfo F. M. da Rocha, Julia Goginski, Pedro H. A. da Silva, Gabriel S. de Fraga, Marcio S. Rassi and Luis A. B. Borba

Brainstem cavernous malformations are especially difficult to treat because of their deep location and intimate relation with eloquent structures. This is the case of a 26-year-old female presenting with dizziness, dysmetria, nystagmus and unbalance. Imaging depicted a lesion highly suggestive of a cavernous malformation in the left inferior cerebellar peduncle. Following a suboccipital midline craniotomy, the cerebellomedullary fissure was dissected and the lesion was identified bulging the surface. The malformation was completely removed with constant intraoperative neurophysiological monitoring. The patient presented improvement of initial symptoms with no new deficits. Surgical resection of brainstem cavernous malformations can be successfully performed, especially when superficial, using the inferior cerebellar peduncle as an entry zone.

The video can be found here: https://youtu.be/-GGZe_CaZnQ.

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Pedro Helo dos Santos Neto, Johnni Oswaldo Zamponi Jr., Rogério Hamerschmidt, Gislaine Richter Minhoto Wiemes, Marcio S. Rassi and Luis A. B. Borba

Hearing loss is the most common symptom of vestibular schwannomas (VSs). The management of these lesions includes observation, radiosurgery, and microsurgical resection. Hearing preservation and rehabilitation are the major challenges after the tumor treatment. A 43-year-old man with previous left-sided profound hearing loss and tinnitus presented with a 2-mm left-sided intracanalicular VS. The decision was made to perform a simultaneous cochlear implantation (CI) and microsurgical resection of the tumor. The patient did well postoperatively, with significant improvement of tinnitus, sound localization, and speech recognition in noise. Previous reports of simultaneous CI and VS resection in patients with neurofibromatosis type 2 and sporadic VS in the only hearing ear have been described. The role of CI in patients with VS and normal contralateral hearing has been recently described, showing positive outcomes due to the binaural benefits. Tinnitus also can be treated by the implantation of the cochlear device. The simultaneous microsurgical removal of VS and implantation of a cochlear device is a feasible approach in patients with unilateral hearing loss and severe tinnitus.

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Luis A. B. Borba, João Cândido Araújo, Jean G. de Oliveira, Miguel Giudicissi Filho, Marlus S. Moro, Luis Fernando Tirapelli and Benedicto O. Colli

Object

The goal of this paper is to analyze the extension and relationships of glomus jugulare tumor with the temporal bone and the results of its surgical treatment aiming at preservation of the facial nerve. Based on the tumor extension and its relationships with the facial nerve, new criteria to be used in the selection of different surgical approaches are proposed.

Methods

Between December 1997 and December 2007, 34 patients (22 female and 12 male) with glomus jugulare tumors were treated. Their mean age was 48 years. The mean follow-up was 52.5 months. Clinical findings included hearing loss in 88%, swallowing disturbance in 50%, and facial nerve palsy in 41%. Magnetic resonance imaging demonstrated a mass in the jugular foramen in all cases, a mass in the middle ear in 97%, a cervical mass in 85%, and an intradural mass in 41%. The tumor was supplied by the external carotid artery in all cases, the internal carotid artery in 44%, and the vertebral artery in 32%. Preoperative embolization was performed in 15 cases. The approach was tailored to each patient, and 4 types of approaches were designed. The infralabyrinthine retrofacial approach (Type A) was used in 32.5%; infralabyrinthine pre- and retrofacial approach without occlusion of the external acoustic meatus (Type B) in 20.5%; infralabyrinthine pre- and retrofacial approach with occlusion of the external acoustic meatus (Type C) in 41%; and the infralabyrinthine approach with transposition of the facial nerve and removal of the middle ear structures (Type D) in 6% of the patients.

Results

Radical removal was achieved in 91% of the cases and partial removal in 9%. Among 20 patients without preoperative facial nerve dysfunction, the nerve was kept in anatomical position in 19 (95%), and facial nerve function was normal during the immediate postoperative period in 17 (85%). Six patients (17.6%) had a new lower cranial nerve deficit, but recovery of swallowing function was adequate in all cases. Voice disturbance remained in all 6 cases. Cerebrospinal fluid leakage occurred in 6 patients (17.6%), with no need for reoperation in any of them. One patient died in the postoperative period due to pulmonary complications. The global recovery, based on the Karnofsky Performance Scale (KPS), was 100% in 15% of the patients, 90% in 45%, 80% in 33%, and 70% in 6%.

Conclusions

Radical removal of glomus jugulare tumor can be achieved without anterior transposition of the facial nerve. The extension of dissection, however, should be tailored to each case based on tumor blood supply, preoperative symptoms, and tumor extension. The operative field provided by the retrofacial infralabyrinthine approach, or the pre- and retrofacial approaches, with or without closure of the external acoustic meatus, allows a wide exposure of the jugular foramen area. Global functional recovery based on the KPS is acceptable in 94% of the patients.

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Jean G. de Oliveira, Luis A. B. Borba, Aziz Rassi-Neto, Samuel M. de Moura, Santiago L. Sanchez-Júnior, Márcio S. Rassi, Carlos Vanderlei M. de Holanda and Miguel Giudicissi-Filho

Object

Intracranial aneurysms may grow closer to anterior optic pathways, causing mass effect over these anatomical structures, including visual deficit. The authors retrospectively reviewed a series of aneurysms in patients presenting with visual field deficit caused by mass effect, to analyze the aneurysm's characteristics, the neurosurgical management of these aneurysms, as well as their clinical, visual, and radiological outcomes.

Methods

The authors reviewed the medical charts, neuroimaging examination results, and surgical videos of 15 patients presenting with visual symptoms caused by an aneurysm's mass effect over the anterior optic pathways. These patients were treated at the Department of Neurosurgery, Center of Neurology and Neurosurgery Associates, Hospital Beneficência Portuguesa de São Paulo, Brazil. Statistical analysis was performed to identify the variables related to partial or total recovery of the visual symptoms.

Results

All patients underwent microsurgical clip placement and emptying of their aneurysms. After a mean follow-up of 38.5 months, the mean postoperative Glasgow Outcome Scale score was 4.33, and the visual outcomes were as follows: 1 patient (6.6%) unchanged, 7 (46.6%) improved, and 7 (46.6%) experienced complete recovery from visual deficits. The variables that influenced the visual outcomes were the size of the aneurysm (p = 0.039), duration of the visual symptoms (p = 0.002), aneurysm wall calcification (p = 0.010), and intraluminal thrombosis (p = 0.007). Postoperative examination using digital subtraction angiography showed complete aneurysm occlusion in 14 (93.3%) of the 15 patients.

Conclusions

Intracranial aneurysms causing mass effect over the anterior optic pathways usually present with complex features. The best treatment option must include not only the aneurysm occlusion but also relief of the mass effect. Microsurgical clip placement with reduction of aneurysmal mass effect achieved improvement in visual ability or recovery from visual impairment, as well as total aneurysm occlusion, in 93.3% of the study group. Therefore, this option is well supported as the first choice of treatment for intracranial aneurysms presenting with mass effect over the anterior visual pathways.