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Luigi Albano, Lina Raffaella Barzaghi and Pietro Mortini

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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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Alberto Franzin, Pietro Panni, Giorgio Spatola, Antonella del Vecchio, Alberto L. Gallotti, Carmen R. Gigliotti, Andrea Cavalli, Carmine A. Donofrio and Pietro Mortini

OBJECTIVE

There are few reported series regarding volume-staged Gamma Knife radiosurgery (GKRS) for the treatment of large, complex, cerebral arteriovenous malformations (AVMs). The object of this study was to report the results of using volume-staged Gamma Knife radiosurgery for patients affected by large and complex AVMs.

METHODS

Data from 20 patients with large AVMs were prospectively included in the authors' AVM database between 2004 and 2012. A staging strategy was used when treating lesion volumes larger than 10 cm3. Hemorrhage and seizures were the presenting clinical feature for 6 (30%) and 8 (40%) patients, respectively. The median AVM volume was 15.9 cm3 (range 10.1–34.3 cm3). The mean interval between stages (± standard deviation) was 15 months (± 9 months). The median margin dose for each stage was 20 Gy (range 18–25 Gy).

RESULTS

Obliteration was confirmed in 8 (42%) patients after a mean follow-up of 45 months (range 19–87 months). A significant reduction (> 75%) of the original nidal volume was achieved in 4 (20%) patients. Engel Class I–II seizure status was reported by 75% of patients presenting with seizures (50% Engel Class I and 25% Engel Class II) after radiosurgery. After radiosurgery, 71.5% (5/7) of patients who had presented with a worsening neurological deficit reported a complete resolution or amelioration. None of the patients who presented acutely because of hemorrhage experienced a new bleeding episode during follow-up. One (5%) patient developed radionecrosis that caused sensorimotor hemisyndrome. Two (10%) patients sustained a bleeding episode after GKRS, although only 1 (5%) was symptomatic. High nidal flow rate and a time interval between stages of less than 11.7 months were factors significantly associated with AVM obliteration (p = 0.021 and p = 0.041, respectively). Patient age younger than 44 years was significantly associated with a greater than 75% reduction in AVM volume but not with AVM obliteration (p = 0.024).

CONCLUSIONS

According to the results of this study, volume-staged GKRS is an effective and safe treatment strategy for large, complex, cerebral AVMs for which microsurgery or endovascular approaches could carry substantially higher risks to the patient. Radiation doses up to 20 Gy can be safely administered. The time interval between stages should be shorter than 11.7 months to increase the chance of obliteration. High nidal flow and a patient age younger than 44 years were factors associated with nidus obliteration and significant nidus reduction, respectively.

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Marco Losa, Micol Valle, Pietro Mortini, Alberto Franzin, Camillo Ferrari da Passano, Marco Cenzato, Stefania Bianchi, Piero Picozzi and Massimo Giovanelli

Object. Radiation therapy diminishes the risk of recurrence of incompletely removed nonfunctioning pituitary adenoma (NPA). The authors evaluated the efficacy and safety of gamma knife surgery (GKS) in patients with residual NPA following surgical debulking of the tumor.

Methods. Fifty-four patients, 26 men and 28 women, ranging in age from 29 to 72 years underwent gamma knife treatment. Baseline and follow-up studies involved magnetic resonance imaging, hormone evaluation, and neuroophthalmological examination 6 and 12 months after GKS and at yearly intervals thereafter. The mean follow up after GKS was 41.1 ± 3.1 months. Two of 52 patients undergoing follow up had a recurrence 40 and 49 months after GKS. In both of these patients the treated lesion had reduced in size, but a new lesion appeared in the contralateral side of the sella turcica. The recurrence-free interval at 5 years was 88.2% (95% confidence interval 72.6–100%). Tumor volume decreased from a baseline value of 2.3 ± 0.2 to 1.7 ± 0.2 cm3 at the last follow up (p < 0.001). Twenty-two patients (42.3%) had a 20% or greater reduction in tumor volume. The administered radiation dose had been significantly higher in patients who experienced tumor reduction. Visual function and motility did not deteriorate in any patient. New cases of hypogonadism, hypothyroidism, and hypoadrenalism occurred in 12.5, 8.6, and 2.3%, respectively, of assessable patients at risk.

Conclusions. Gamma knife surgery was effective in controlling the growth of residual NPA after previously performed maximal surgical debulking. The major advantage of GKS compared with fractionated radiotherapy seems to be a lower risk of side effects, especially a lower risk of hypopituitarism.

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Marco Losa, Pietro Mortini, Laura Urbaz, Paolo Ribotto, Tristana Castrignanò and Massimo Giovanelli

Object

The question of whether preoperative therapy with somatostatin analogs can improve surgical outcome in acromegaly has not been definitively answered. In this paper, the authors report the effects of preoperative treatment with somatostatin analogs in a large sample of patients with acromegaly.

Methods

Between 1990 and 2003, 399 consecutive patients with acromegaly underwent surgery at the Istituto Scientifico San Raffaele. Thirty-three patients who had previously undergone surgery or radiation treatment, 48 patients treated with somatostatin analogs for fewer than 3 months, and patients who had stopped therapy for too long a time before surgery were excluded from the study. One hundred forty-three patients who had received somatostatin analogs prior to surgery (Group 1) were randomly matched to 143 patients who had never been treated with somatostatin analogs (Group 2). Matching criteria were tumor size and invasiveness into the cavernous sinus. Before surgery, Group 1 patients showed reduction of growth hormone levels to less than 50% of baseline in 64% of cases, but insulin-like growth factor–I was normalized in only 19.5%. Surgical remission occurred in 81 Group 1 patients (56.6%) and in 91 Group 2 patients (63.6%; p = 0.28). No significant difference in the remission rate was observed when cases were analyzed according to tumor size or invasiveness. Logistic regression analysis confirmed that pretreatment with somatostatin analogs was not associated with surgical outcome. Surgical morbidity was mild and similar in Group 1 and Group 2 patients (7 and 5.6%, respectively; p = 0.81). Surgical remission and complication rates in patients with acromegaly who received treatment with somatostatin analogs prior to surgery were not significantly different from those of matched patients who did not receive these agents.

Conclusions

At present, the routine use of presurgical therapy with somatostatin analogs for patients with acromegaly cannot be recommended.

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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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Federico Biglioli, Valeria Colombo, Dimitri Rabbiosi, Filippo Tarabbia, Federica Giovanditto, Alessandro Lozza, Silvia Cupello and Pietro Mortini

OBJECTIVE

Facial palsy is a well-known functional and esthetic problem that bothers most patients and affects their social relationships. When the time between the onset of paralysis and patient presentation is less than 18 months and the proximal stump of the injured facial nerve is not available, another nerve must be anastomosed to the facial nerve to reactivate its function. The masseteric nerve has recently gained popularity over the classic hypoglossus nerve as a new motor source because of its lower associated morbidity rate and the relative ease with which the patient can activate it. The aim of this work was to evaluate the effectiveness of masseteric–facial nerve neurorrhaphy for early facial reanimation.

METHODS

Thirty-four consecutive patients (21 females, 13 males) with early unilateral facial paralysis underwent masseteric–facial nerve neurorrhaphy in which an interpositional nerve graft of the great auricular or sural nerve was placed. The time between the onset of paralysis and surgery ranged from 2 to 18 months (mean 13.3 months). Electromyography revealed mimetic muscle fibrillations in all the patients. Before surgery, all patients had House-Brackmann Grade VI facial nerve dysfunction. Twelve months after the onset of postoperative facial nerve reactivation, each patient underwent a clinical examination using the modified House-Brackmann grading scale as a guide.

RESULTS

Overall, 91.2% of the patients experienced facial nerve function reactivation. Facial recovery began within 2–12 months (mean 6.3 months) with the restoration of facial symmetry at rest. According to the modified House-Brackmann grading scale, 5.9% of the patients had Grade I function, 61.8% Grade II, 20.6% Grade III, 2.9% Grade V, and 8.8% Grade VI. The morbidity rate was low; none of the patients could feel the loss of masseteric nerve function. There were only a few complications, including 1 case of postoperative bleeding (2.9%) and 2 local infections (5.9%), and a few patients complained about partial loss of sensitivity of the earlobe or a small area of the ankle and foot, depending on whether great auricular or sural nerves were harvested.

CONCLUSIONS

The surgical technique described here seems to be efficient for the early treatment of facial paralysis and results in very little morbidity.

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Marco Losa, Wolfgang Saeger, Pietro Mortini, Claudio Pandolfi, Maria Rosa Terreni, Gianluca Taccagni and Massimo Giovanelli

✓ Acromegaly is usually caused by a growth hormone (GH)-secreting pituitary adenoma, and hypersecretion of GH-releasing hormone (GHRH) from a hypothalamic or neuroendocrine tumor accounts for other cases. The authors report on the unusual association of acromegaly with a granular cell tumor of the neurohypophysis.

A 42-year-old woman with a 10-year history of acral enlargement, headache, and menstrual abnormalities was referred to our department for a suspected GH-secreting pituitary adenoma. The patient's basal GH levels were mildly elevated at 4.8 µg/L, were not suppressed in response to an oral glucose tolerance test, and increased paradoxically after administration of thyrotropin-releasing hormone. The patient's insulin-like growth factor—1 (IGF-1) level was elevated at 462 µg/L, whereas a magnetic resonance image of the sella turcica revealed an intra- and suprasellar lesion that was compatible with a diagnosis of pituitary adenoma. A transsphenoidal approach to remove the lesion, which was mainly suprasellar, was successful during a second operative attempt, resulting in the clinical and biochemical regression of the patient's acromegaly. Four months postoperatively, the patient's basal GH level was 0.9 µg/L and her IGF-1 level was 140 µg/L. Histological analysis of the operative specimen demonstrated a granular cell tumor of the neurohypophysis, which when stained proved negative for pituitary hormones and GHRH.

This case represents the first reported association between a granular cell tumor of the neurohypophysis and acromegaly. Granular cell tumor of the neurohypophysis could be added to the restricted list of neoplastic causes of acromegaly secondary to hypersecretion of a GH-releasing substance.

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Matteo Alicandri-Ciufelli, Giacomo Pavesi and Livio Presutti