Il carcinoma del sacco endolinfatico è un tumore molto raro come evidente dai dati presenti in letteratura ove anche centri di esperienza riferiscono tuttavia casistiche limitate. E un tumore ...maligno a lenta crescita, con tendenza allinvasione locale e scarsa tendenza alla disseminazione metastatica. Linsorgenza spesso tardiva dei sintomi e la difficoltà ad eseguire indagini bioptiche a livello della sede di origine, parete posteriore della rocca, ha reso la diagnosi di questo tumore spesso difficile, nonostante si riconoscano attualmente dati radiologici patognomonici della neoplasia. Patterns tipici di presentazione sono evidenziabili alla RM con mezzo di contrasto e alla TC per osso a strato sottile, rendendo nella maggior parte dei casi possibile la diagnosi radiologica. Lincidenza del tumore del sacco endolinfatico è maggiore nei pazienti affetti da sindrome di von Hippel Lindau (VHL), con una frequenza del 25% che fa parte del quadro sindromico. Negli anni dal 2012 al 2015 abbiamo osservato 7 casi, uno di essi con manifestazione della patologia bilaterale, tutti affetti da VHL. Quattro tra questi sono stati sottoposti a chirurgia presso il nostro centro per un totale di 5 procedure chirurgiche. Ogni caso è stato descritto dettagliatamente analizzando sintomi, intervallo tra comparsa dei sintomi, diagnosi e terapia. Non vi è stata morbidità post-operatoria aggiuntiva nei pazienti in cui la diagnosi e la terapia sono state precoci, mentre la gestione di tumori localmente avanzati è stata associata a deficit neurologici postoperatori, in particolare del VII, IX e X nervo cranico. I siti anatomici critici di coinvolgimento della malattia che hanno coinciso con un pianificato rischio di danno neuronale sono risultati essere il canale di Falloppio, il forame giugulare, lapice della rocca petrosa. Lestensione intradurale nella fossa cranica posteriore è stato un altro elemento caratterizzante i tumori in stadio avanzato. I tumori del sacco endolinfatico che lo screening permette di evidenziare precocemente nei pazienti VHL, hanno buona prognosi quando affrontati precocemente, compatibilmente con le esigenze terapeutiche della malattia di base.
Negli ultimi 20 anni il miglioramento dellassistenza neonatale ha determinato un progressivo aumento dei bambini che sopravvivono in presenza di gravi malformazioni o patologie congenite. Questi ...bambini richiedono una riabilitazione prolungata, talora multidisciplinare e complessa. Purtroppo, unorganizzazione adeguata alla riabilitazione della disfagia, dei disturbi della comunicazione e della respirazione non è sempre disponibile, non è sempre coordinata in equipe multidisciplinari che operino sia negli ospedali che sul territorio e non è facile mantenere tutte le figure professionali coinvolte al passo con le sempre più rapide innovazioni. Scopo del presente lavoro è presentare un aggiornamento su alcuni aspetti tuttora controversi della riabilitazione in età pediatrica.
This study was conducted on patients with squamous cell carcinoma of the external auditory canal and temporal bone treated with surgery alone or surgery plus postoperative radiotherapy. It was ...designed as a retrospective investigation with complete long-term follow-up covering the years from 1983 to 2008. The setting was a tertiary referral centre. Forty-one consecutive cases underwent surgery involving en bloc lateral or subtotal temporal bone resection, parotidectomy and neck dissection plus radiotherapy in advanced cases. The Pittsburgh staging system was adopted. No cases were lost to follow-up, which ranged from 2 to 220 months, while for survivors ranged from 60 to 220 months and included clinical examinations and imaging. Outcome was expressed as NED (no evidence of disease), DOC (dead of other causes), DOD (dead of disease), AWD (alive with disease), disease-free survival (DFS) and disease-specific survival (DSS). Results were expressed with raw data and Kaplan Meyer curves. Patients with T1 and T2 disease had a DFS of 67% and a DSS of 92%. For T3 and T4 cases, the DFS was 41% and DSS was 48%. All treatment failures were due to local recurrences. The cases classified as T4 because the lesion extended from the cartilage canal to the periauricular soft tissues, or from the anterior wall to the parotid space, had a better outcome than the other T4 cases: this different prognosis suggests the need to stage tumours differently. Nodal disease coincided with a worse outcome due to local recurrence.
Tractography of the facial nerve based on single-shell diffusion MR imaging is thought to be helpful before surgery for resection of vestibular schwannoma. However, this paradigm can be vitiated by ...the isotropic diffusion of the CSF, the convoluted path of the facial nerve, and its crossing with other bundles. Here we propose a multishell diffusion MR imaging acquisition scheme combined with probabilistic tractography that has the potential to provide a presurgical facial nerve reconstruction uncontaminated by such effects.
Five patients scheduled for vestibular schwannoma resection underwent multishell diffusion MR imaging (b-values = 0, 300, 1000, 2000 s/mm
). Facial nerve tractography was performed with a probabilistic algorithm and anatomic seeds located in the brain stem, cerebellopontine cistern, and internal auditory canal. A single-shell diffusion MR imaging (b-value = 0, 1000 s/mm
) subset was extrapolated from the multishell diffusion MR imaging data. The quality of the facial nerve reconstruction based on both multishell diffusion MR imaging and single-shell diffusion MR imaging sequences was assessed against intraoperative videos recorded during the operation.
Single-shell diffusion MR imaging-based tractography was characterized by failures in facial nerve tracking (2/5 cases) and inaccurate facial nerve reconstructions displaying false-positives and partial volume effects. In contrast, multishell diffusion MR imaging-based tractography provided accurate facial nerve reconstructions (4/5 cases), even in the presence of ostensibly complex patterns.
In comparison with single-shell diffusion MR imaging, the combination of multishell diffusion MR imaging-based tractography and probabilistic algorithms is a more valuable aid for surgeons before vestibular schwannoma resection, providing more accurate facial nerve reconstructions, which may ultimately improve the postsurgical patient's outcome.
The current treatment options for acoustic neuromas (AN) - observation, microsurgery and radiotherapy - should assure no additional morbidity on cranial nerves VII and VIII. Outcomes in terms of ...disease control and facial function are similar, while the main difference lies in hearing. From 2012 to 2016, 91 of 169 patients (54%) met inclusion criteria for the present study, being diagnosed with unilateral, sporadic, intrameatal or extrameatal AN up to 1 cm in the cerebello-pontine angle; the remaining 78 patients (46%) had larger AN and were all addressed to surgery. The treatment protocol for small AN included observation, translabyrinthine surgery, hearing preservation surgery (HPS) and radiotherapy. Hearing function was assessed according to the Tokyo classification and the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) classification. Sixty-one patients (71%) underwent observation, 19 (22%) HPS and 6 (7%) translabyrinthine surgery; 5 patients were lost to follow-up. Median follow-up was 25 months. In the observation group, 24.6% of patients abandoned the wait-and-see policy for an active treatment; the risk of switching from observation to active treatment was significant for tumour growth (p = 0.0035) at multivariate analysis. Hearing deteriorated in 28% of cases without correlation with tumour growth; the rate of hearing preservation for classes C-D was higher than for classes A-B (p = 0.032). Patients submitted to HPS maintained an overall preoperative hearing class of Tokyo and AAO-HNS in 63% and 68% of cases, respectively. Hearing preservation rate was significantly higher for patients presenting with preoperative favourable conditions (in-protocol) (p = 0.046). A multi-option management for small AN appeared to be an effective strategy in terms of hearing outcomes.
Intracochlear schwannomas can occur either as an extension of a larger tumor from the internal auditory canal, or as a solitary labyrinthine tumor. They are currently removed via a translabyrinthine ...approach extended to the basal turn, adding a transotic approach for tumors lying beyond the basal turn. Facial bridge cochleostomy may be associated with the translabyrinthine approach to enable the whole cochlea to be approached without sacrificing the external auditory canal and tympanum. We describe seven cases, five of which underwent cochlear schwannoma resection with facial bridge cochleostomy, one case with the same procedure for a suspect tumor and one, previously subjected to radical tympanomastoidectomy, who underwent schwannoma resection via a transotic approach. Facial bridge cochleostomy involved removing the bone between the labyrinthine and tympanic portions of the fallopian canal, and exposing the cochlea from the basal to the apical turn. Patients’ recovery was uneventful, and long-term magnetic resonance imaging showed no residual tumor. Facial bridge cochleostomy can be a flexible extension of the translabyrinthine approach for tumors extending from the internal auditory canal to the cochlea. The transcanal approach is suitable for the primary exclusive intralabyrinthine tumor. The indications for the different approaches are discussed.
Endolymphatic sac tumour (ELST) is infrequent, as emerges from small series reported in the literature. It is a slow-growing malignancy with local aggressiveness and a low risk of distant metastases. ...It is often misdiagnosed because of the late onset of symptoms and difficulty in obtaining a biopsy. Its frequency is higher in von Hippel-Lindau (VHL) disease (a genetic systemic syndrome involving multiple tumours), with a prevalence of around 25%. The diagnosis is based on radiology, with specific patterns on contrast-enhanced MRI and typical petrous bone erosion on bone CT scan. Our experience of ELST in the years between 2012-2015 concerns 7 cases, one of which was bilateral, in patients with VHL disease. Four of the 7 patients underwent 5 surgical procedures at our institution. Each case is described in detail, including clinical symptoms, and the intervals between symptom onset, diagnosis and therapy. Postoperative morbidity was low after early surgery on small tumours, whereas extensive surgery for large tumours was associated with loss of cranial nerve function (especially VII, IX, X). The critical sites coinciding with loss of neurological function were the fallopian canal, jugular foramen, petrous apex and intradural extension into the posterior cranial fossa. Early surgery on small ELST is advocated for patients with VHL disease, in whom screening enables a prompt diagnosis and consequently good prognosis.
Lateral approaches to the skull base ZANOLETTI, E; MARTINI, A; EMANUELLI, E ...
Acta otorhino-laryngologica italica,
10/2012, Letnik:
32, Številka:
5
Journal Article
Recenzirano
Odprti dostop
Progress in the study of skull base anatomy and the different lesions involving it has led to more and more precise knowledge of the site. New diagnostic tools have allowed better definition of ...various diseases, and the use of an operative microscope and modern surgical instruments have all contributed to the development of microsurgery of the skull base. Refinements in microsurgical techniques have led to the description of multiple approaches to the skull base. Lateral approaches to the skull base have been described by pioneering surgeons who created the basis of this surgery and further contributed with their own experience. Refinements and modifications to the original technique have taken place over years, to the point that nowadays the large number of approaches described by oto- and neurosurgeons have led to some confusion. We have attempted to simplify this by retracing it to the original, basic steps based on the most representative publications and personal experience.
Long-term hearing was examined in 200 patients operated on during 1976-2000 with removal of acoustic neuroma with a retrosigmoid approach and hearing preservation technique. Ninety-four cases ...preserved hearing, and 91 cases were followed-up for at least 6 years (range 6 to 21 years). Hearing was measured with the PTA 0.5 KHz to 4 KHz and the SDS, and followed the guidelines of the American Academy of Otolaryngology and Head Neck Surgery (AAOHNS classification). Change (or stability) of the short to long-term class was the main outcome measure. Postoperative hearing of class A and B was preserved at long-term and within class B in 87% of cases. A total of 13% presented a decrease of class to C or D. Small size and good preoperative hearing correlated with good short- and long-term hearing corrected.
PDF
