Giant hypervascular lesion of the sinonasal tract invading the anterior skull base and orbit: a puzzling case.

The sinonasal tract may be involved in a wide variety of hypervascular lesions, either benign or malignant, and differential diagnosis may be challenging. We present the case of a 26-year-old man with an aggressive ethmoid hypervascular mass invading the anterior skull base and orbit. Because of massive intraoperative bleeding and difficult dissection of the lesion from the periorbita, the planned cranioendoscopic approach had to be converted into a standard craniofacial resection by a combined Lynch and coronal incision. The definitive diagnosis was consistent with lobular capillary hemangioma associated with intravascular papillary endothelial hyperplasia. Two years after surgery, the patient is free of disease. Lobular capillary hemangioma is a hypervascular lesion that may involve the sinonasal tract. The case presented herein is exceptional, both in the presentation and in the difficulties encountered in diagnosis and treatment, because of the concurrence of lobular capillary hemangioma and intravascular papillary endothelial hyperplasia.

Endoscopic surgery for malignant tumors of the sinonasal tract and adjacent skull base: a 10-year experience.

BACKGROUND: The increasing expertise in the field of transnasal endoscopic surgery recently has expanded its indications to include the management of sinonasal malignancies. We report our experience with the endoscopic management of nasoethmoidal malignancies possibly involving the adjacent skull base. METHODS: A retrospective analysis was performed of patients treated by an exclusive endoscopic approach (EEA) or a cranioendoscopic approach (CEA) from 1996 to 2006 managed by two surgical teams at the Departments of Otorhinolaryngology of the University of Brescia, and the University of Pavia/Insubria-Varese, Italy. RESULTS: One-hundred eighty-four patients were considered eligible for the present analysis. An EEA was performed in 134 patients and the remaining 50 patients underwent the CEA. The most frequent histotypes encountered were adenocarcinoma (37%), squamous cell carcinoma (13.6%), olfactory neuroblastoma (12%), mucosal melanoma (9.2%), and adenoid cystic carcinoma (7.1%). Overall, 86 (46.7%) patients received some form of adjuvant treatment. The patients were followed up for a mean of 34.1 months (range, 2-123 months). The 5-year disease-specific survival was 91.4 +/- 3.9% and 58.8 +/- 8.6% (p = 0.0004) for the EEA and CEA group, respectively. CONCLUSION: To the best of our knowledge, this is the largest series reported to date of malignant tumors of the sinonasal tract and adjacent skull base treated with pure endoscopic or cranioendoscopic techniques. A 5-year disease-specific survival of 91.4% and 58.8% for the EEA and the CEA groups, respectively, seem to indicate that endoscopic surgery, when properly planned and in expert hands, may be a valid alternative to standard surgical approaches for the management of malignancies of the sinonasal tract.

Auditory brainstem implant in a child with severely ossified cochlea.

OBJECTIVE: The hearing outcome after implanting a severely ossified cochlea has always been less satisfactory than implanting a patent one. The aim of our study is to present a case where brainstem implantation was successfully performed as an alternative to cochlear implantation in a child with bilateral severe ossification of the cochlea. STUDY DESIGN: Case presentation. This study was conducted at Gruppo Otologico, Rome, Italy, a private referral center for neurotology and skull base surgery. METHODS: The subject of our study was a 12-year-old female child with postmeningitic deafness and bilaterally ossified cochleae. This case is the first brainstem implantation performed at our center with the indication of severe ossification of the cochlea. RESULTS: Successful brainstem implantation of a device was carried out, and the hearing of the patient was restored to the degree that she can freely use the telephone after 8 months of implantation. CONCLUSION: Although more cases are needed before establishing the exact outcome of brainstem implantation in cases of deafness in the presence of severe bilateral cochlear ossification, preliminary results show the superiority of brainstem implants to conventional or even customized cochlear implants.

Surgical removal of jugular paragangliomas after stenting of the intratemporal internal carotid artery: a preliminary report.

OBJECTIVE: Jugular paragangliomas with extensive involvement of the internal carotid artery (ICA) represent a true challenge for surgeons, especially in the presence of inadequate collateral circulation through the circle of Willis. The aim of our study is to present a preliminary report of our experience with the surgical removal of three such cases using the stenting of the ICA as the method of choice for protecting and preserving the integrity of the artery. METHODS: This retrospective study was conducted at Gruppo Otologico, a private referral center for neurotology and skull base surgery. The subjects of our study are three cases of jugular paragangliomas with extensive involvement of the ICA and inadequate collateral circulation. These cases are the first three cases operated at our center after stenting of the intratemporal portion of the ICA. RESULTS: Complete surgical removal of the tumor, including the part involving the ICA, was achieved in all cases. Over a follow-up period of 22 to 30 months, no complications occurred and the patency of the stented arteries was preserved. CONCLUSION: Although more follow-up is still needed before establishing the exact long-term outcome of stenting the intratemporal ICA, our preliminary report shows that the stent has facilitated the complete surgical removal of the tumor, preserving the integrity of the ICA.

Hearing preservation surgery in vestibular schwannoma.

OBJECTIVES: To study the effect of pre-operative hearing level and tumour size on the hearing outcome of hearing preservation surgery for vestibular schwannoma. STUDY DESIGN AND SETTING: A review of literature conducted at Gruppo Otologico, a tertiary referral centre for neurotology and skull base surgery. RESULTS: A total of 1993 patients in 16 publications addressing the topic of hearing preservation surgery in vestibular schwannoma were analysed. The American Academy of Otolaryngology-Head and Neck Surgery hearing classification system was the classification upon which we based our analysis. CONCLUSION: Defining hearing preservation as class-A hearing, there was a strong inverse relationship between pre-operative hearing and post-operative hearing levels and between tumour size and post-operative hearing levels.

Management of temporal bone hemangiomas.

Temporal bone hemangiomas are rare tumors that require a high degree of clinical suspicion on the part of the otolaryngologist to be diagnosed early. The purpose of this report is to present 10 histologically confirmed cases of hemangiomas located within the temporal bone treated at the Gruppo Otologico of Piacenza-Rome, Italy. A short review of the literature is also presented. The symptomatology of these extraneural vascular tumors depends on their location. Tumors located within the internal auditory canal present mainly with hearing loss, while a facial nerve deficit is the predominant symptom in geniculate ganglion tumors. The treatment modality is also dependent on the location of the tumor, as well as the clinical features and tumor size. The final facial nerve outcome is directly affected by the duration of the facial nerve deficit, and so the treatment should not be delayed once the diagnosis is made.

Cerebrospinal fluid leak prevention after translabyrinthine removal of vestibular schwannoma.

OBJECTIVES/HYPOTHESIS: The purpose of the report was to present an update on the authors' results for prevention and management of cerebrospinal fluid (CSF) leak after translabyrinthine approach for vestibular schwannoma. STUDY DESIGN: : Retrospective case review. METHODS: The study was conducted at Gruppo Otologico (Piacenza, Italy), a tertiary referral center for neurotology and skull base surgery. In all, 710 patients underwent translabyrinthine approach for the removal of vestibular schwannoma at that institution between April 1987 and December 2002. The medical records were retrospectively reviewed to identify tumor size, the incidence of postoperative CSF leak, and its treatment. RESULTS: The overall rate of CSF leak was 1.4%. CONCLUSION: The use of proper surgical technique minimizes the risk of CSF leak. Study results show that the continued application of the authors' proposed preventive measures resulted in the maintenance of a low rate of CSF leak. Immediate management of CSF fistulae helps prevent meningitis.

Hearing preservation surgery in vestibular schwannoma: the hidden truth.

To compare the results of hearing preservation surgeries using different approaches--the enlarged middle cranial fossa approach and the retrosigmoid approach--and different classification systems, stressing the importance of preserving "normal hearing," we performed a retrospective case review in a tertiary care medical center. The charts of 107 patients with vestibular schwannoma who underwent tumor resection were reviewed. Hearing preservation was reported according to 2 different classification systems: the modified Sanna classification and the classification of the American Academy of Otolaryngology-Head and Neck Surgery. The facial nerve results were graded according to the House-Brackmann scale. The hearing preservation rates differed markedly depending on the classification used. We conclude that hearing preservation in acoustic neuroma is a more difficult proposition than most surgeons appreciate, especially in terms of serviceable hearing.

Inner ear extension of vestibular schwannomas.

OBJECTIVE: Inner ear extension of vestibular schwannomas (VSs) is a rare finding but has important clinical implications. This report reviews the treatment options and presents the experience of the Gruppo Otologico, Piacenza, Italy, in this field. STUDY DESIGN: Case report and literature review. METHODS: Five cases of VSs with inner ear extension were surgically removed. In all of them, the cochlea was partially or completely invaded by the lesion. RESULTS: In 4 cases, the inner ear extension was preoperatively identified on magnetic resonance imaging, and the surgical removal was planned through a transotic approach. In the last case, the cochlear invasion was not detected preoperatively, and the lesion was removed during a second surgery performed to seal a cerebrospinal fluid fistula. CONCLUSIONS: VSs with inner ear extension should be distinguished from pure intralabyrinthine schwannomas because of differences in clinical significance. Cochlear involvement is more frequent than vestibular involvement and is often accompanied by a dead ear. Dead ear caused by small VSs should alert the surgeon to the possibility of a cochlear extension. The presence of an intracochlear involvement requires the adoption of an approach that allows control of the cochlear turns, and we found the transotic approach to be the most suitable. Undetected cochlear extensions that are left in place may grow with time.