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Nicola Boari, Alfio Spina, Lodoviga Giudice, Francesca Gorgoni, Michele Bailo and Pietro Mortini

OBJECTIVE

Advantages of the fronto-orbitozygomatic (FOZ) approach have been reported extensively in the literature; nevertheless, restoration of normal anatomy and the esthetic impact of surgery are increasingly important issues for patients and neurosurgeons. The aim of this study was to analyze functional and cosmetic outcomes in a series of 169 patients with different pathologies who underwent surgery in which the FOZ approach was used.

METHODS

Between January 2000 and December 2014, 250 consecutive patients underwent surgery with an FOZ approach as the primary surgical treatment. Follow-up data were available for only 169 patients; 103 (60.9%) of these patients were female and 66 (39.1%) were male, and their ages ranged from 6 to 77 years (mean 46.9 years; SD 15.6 years). Mean follow-up time was 66 months (range 6–179 months; SD 49.5 months). Evaluation of clinical outcomes was performed with a focus on 4 main issues: surgical complications, functional outcome, cosmetic outcome, and patient satisfaction. The additional time needed to perform orbitotomy and orbital reconstruction was also evaluated.

RESULTS

The permanent postoperative complications included forehead hypesthesia (41.4%) and dysesthesia (15.3%), frontal muscle weakness (10.3%), exophthalmos (1.4%), enophthalmos (4.1%), diplopia (6.6%; 2% were related to surgical approach), and persistent periorbital and eyelid swelling (3%). Approximately 90% of the patients reported subjectively that surgery did not affect their quality of life or complained of only minor problems that did not influence their quality of life significantly. The mean time needed for orbitotomy and orbital reconstruction was approximately half an hour.

CONCLUSIONS

Comprehensive knowledge of the potential complications and overall clinical outcomes of the FOZ approach can be of great utility to neurosurgeons in balancing the well-known benefits of the approach with potential additional morbidities.

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Nicola Boari, Fabio Roberti, Federico Biglioli, Anthony J. Caputy and Pietro Mortini

Object

The authors describe a modified Le Fort I maxillotomy with medial and posterior antrectomy and removal of the pterygoid plates, aimed at improving the lateral surgical exposure during open transmaxillary surgery for pathological conditions involving the clivus. A cadaveric microanatomical study was conducted to compare the planimetric exposures allowed by the transmaxillary transpterygoid (TMTP) approach and the standard Le Fort I maxillotomy (STM).

Methods

Six cadaveric specimens that had been fixed with glutaraldehyde and injected with latex were dissected to obtain morphometric measurements after both TMTP and STM approaches. The anatomical areas exposed by the surgical approaches were calculated using ImageJ 1.37a software.

Results

As expected, the TMTP approach allowed for a greater surgical exposure, with an incremental area exposed ranging from 4.9 to 7.6 cm2 (mean ± standard deviation 6.4 ± 1.2 cm2, 95% CI 5.4–7.4 cm2). The amount of additional anatomical area visualized, as recorded as a percentage increase after the TMTP approach when compared with the STM approach, ranged from 83 to 109% (mean 99%).

Conclusions

The lateral surgical exposure allowed by the STM approach is limited by the pterygoid plates. The TMTP approach significantly improves the exposure of the anatomical regions lateral to the clivus, allowing access to the pterygopalatine and medial infratemporal fossae. In comparison with the STM, the TMTP approach allows for a surgical exposure that is nearly double. The authors conclude that the TMTP approach provides a significant improvement in the surgical exposure of the lateral paraclival areas, when compared with the STM approach.

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Nicola Boari, Filippo Gagliardi, Andrea Cavalli, Marco Gemma, Luca Ferrari, Paola Riva and Pietro Mortini

OBJECTIVE

Skull base chordomas (SBCs) are rare dysembryogenetic invasive tumors with a variable tendency for recurrence. According to previous studies, the recurrence rate seems to be affected by both clinical variables and tumor biological features. The authors present the results of treatment of SBCs in a large series of patients and investigate the role of 1p36 chromosomal region loss of heterozygosity (LOH) as a prognostic factor.

METHODS

Between 1990 and 2011, 45 patients were treated for SBCs. The mean follow-up was 76 months (range 1–240 months). An LOH analysis was performed in 27 cases. Survival analysis was performed to determine clinical and biological parameters correlating with clinical outcome.

RESULTS

The 5- and 10-year overall survival rates were 67% and 57%, respectively. Five- and 10-year progression-free survival rates were 58% and 44%, respectively. Multivariate analysis showed that extent of resection, adjuvant radiation therapy, and absence of rhinopharynx invasion were positive independent predictors of overall survival. The latter 2 variables and a younger patient age were positive independent predictors of progression-free survival. Twenty-one patients showed 1p36 LOH. All events of recurrence and death clustered in the group of patients with 1p36 LOH; however, this biological marker was not statistically significant on multivariate analysis.

CONCLUSIONS

Resection is the treatment of choice in primary and recurrent SBC. Patient age, rhinopharynx invasion at diagnosis, extent of tumor removal, and postoperative radiation therapy influence SBC prognosis. Genetic analysis, even while showing interesting results, did not reveal 1p36 LOH as an independent predictor of clinical outcome.

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Matteo Alicandri-Ciufelli, Giacomo Pavesi and Livio Presutti

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Nicola Boari, Michele Bailo, Filippo Gagliardi, Alberto Franzin, Marco Gemma, Antonella del Vecchio, Angelo Bolognesi, Piero Picozzi and Pietro Mortini

Object

Since the 1990s, Gamma Knife radiosurgery (GKRS) has become the first-line treatment option for small- to medium-size vestibular schwannomas (VSs), especially in patients without mass effect–related symptoms and with functional hearing. The aim of this study was to assess the safety and efficacy of GKRS, in terms of tumor control, hearing preservation, and complications, in a series of 379 consecutive patients treated for VS.

Methods

Of 523 patients treated at the authors' institution for VS between 2001 and 2010, the authors included 379 who underwent GKRS as the primary treatment. These patients were not affected by Type 2 neurofibromatosis and had clinical follow-up of at least 36 months. Clinical follow-up (mean and median 75.7 and 69.5 months, respectively) was performed for all patients, whereas audiometric and quantitative radiological follow-up examinations were obtained for only 153 and 219 patients, respectively. The patients' ages ranged from 23 to 85 years (mean 59 years). The mean tumor volume was 1.94 ± 2.2 cm3 (median 1.2 cm3, range 0.013–14.3 cm3), and the median margin dose was 13 Gy (range 11–15 Gy). Parameters considered as determinants of the clinical outcome were long-term tumor control, hearing preservation, and complications. A statistical analysis was performed to correlate clinical outcomes with the radiological features of the tumor, dose-planning parameters, and patient characteristics.

Results

Control of the tumor with GKRS was achieved in 97.1% of the patients. In 82.7% of the patients, the tumor volume had decreased at the last follow-up, with a mean relative reduction of 34.1%. The rate of complications was very low, with most consisting of a transient worsening of preexisting symptoms. Patients who had vertigo, balance disorders, or facial or trigeminal impairment usually experienced a complete or at least significant symptom relief after treatment. However, no significant improvement was observed in patients previously reporting tinnitus. The overall rate of preservation of functional hearing at the long-term follow-up was 49%; in patients with hearing classified as Gardner-Robertson (GR) Class I, this value was 71% and reached 93% among cases of GR Class I hearing in patients younger than 55 years.

Conclusions

Gamma Knife radiosurgery is a safe and effective treatment for VS, achieving tumor control in 97.1% of cases and resulting in a very low morbidity rate. Younger GR Class I patients had a significantly higher probability of retaining functional hearing even at the 10-year follow-up; for this reason, the time between symptom onset, diagnosis, and treatment should be shortened to achieve better outcomes in functional hearing preservation.