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Alex Alfieri and Giampietro Pinna


There is little information about the long-term effectiveness and complications following decompressive surgery for syringomyelia related to Chiari malformation Type I (CM-I).


Examining long-term clinical and radiological follow-up, the authors studied a mixed retrospective and prospective single-institution cohort of 109 consecutive surgically treated adult patients with syringomyelia and CM-I. All patients underwent a standardized surgical protocol: decompression of the craniocervical junction, arachnoid exploration, and shrinkage of the cerebellar tonsils. Factors predicting outcome were investigated.


The retrospective arm consisted of 41 cases treated between 1990 and 1994, and the prospective arm comprised 68 patients treated between 1994 and 2001. The mean overall age was 45.9 years, and 58.8% of the population was female. The median follow-up period was 12.7 years. The most frequent initial symptoms were pain and sensory and gait disturbances. There was no perioperative death or neurological deterioration. The comprehensive perioperative complication rate was approximately 11%, with 3 cases (2.7%) of CSF leakage. Regression analysis showed that the best combination of clinical and radiological outcome predictors was age and duration of symptoms. Clinical follow-up confirmed surgical result stability with clinical improvement of greater than 90% of the spinal and cranial manifestations over a long-term period. Two patients had radiological recurrences of syringomyelia without clinical signs 85 and 124 months after surgery.


Certain clinical predictors of poor clinical and radiological prognosis were identified—namely, age at time of surgery and symptom duration. The results of the study provide additional long-term data that support the effectiveness and safety of relieving CSF block at the craniocervical junction in CM-I–related syringomyelia.

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Giampietro Pinna, Franco Alessandrini, Alex Alfieri, Marcella Rossi, and Albino Bricolo

Cerebrospinal fluid (CSF) flow abnormalities are known to be present in Chiari I malformation and to underlie the origin and progression of associated syringomyelia. The incidence of syrinx formation, however, is variable for unknown reasons. The aim of this study was to investigate whether differences in CSF flow dynamics in patients with Chiari I malformation may account for the different clinical and radiological presentation.

Presurgical and postsurgical phase-contrast magnetic resonance imaging investigations were prospectively conducted in 47 adult patients with symptomatic Chiari I malformation. Patients were divided into two groups according to the presence (32 cases) or absence (15 cases) of syrinx. Cerebrospinal fluid flow patterns were evaluated at four regions of interest: prebulbar cistern, foramen magnum, and the ventral and dorsal spinal subarachnoid spaces at the C-5 level. A temporal analysis of CSF flow waveforms was performed with measurement of cranial- and caudal-directed flow durations. All patients underwent a craniocervical decompressive procedure. Preoperatively, a prolonged caudal-directed (systolic) flow pattern was observed in patients with syringomyelia, as compared with normal control values obtained in 15 healthy volunteers. Conversely, a decreased systolic duration was observed in Chiari I patients who had malformation without syrinx. These trends were not statistically significant because of the considerable degree of overlap with the control values recorded in both groups. Additional comparison of the observed preoperative values obtained in patients with and those without syringomyelia indicated that the difference in systolic flow duration was significant at the ventral spinal subarachnoid space level (p = 0.003) and remarkable at the other levels, although not reaching statistical significance. Cerebrospinal fluid flow was minimal or absent at the foramen magnum (dorsal aspect) due to tonsillar herniation, precluding reliable quantitative measurement at this level. There was no evidence of communication between the fourth ventricle and syrinx in any case. Postoperatively, unobstructed CSF flow was recorded across the enlarged foramen magnum and into the artificial cisterna magna in all patients. A gradual restoration of near-normal flow patterns was observed in both groups. Inside the syrinx, fluid motion gradually tapered, no longer being detectable in 12 patients (37.5%) 1 year postsurgery.

In patients with Chiari I malformation and associated syringomyelia different CSF flow patterns were demonstrated as compared with patients in whom syrinx was absent. Analysis of this study's findings supports the hypothesis that in Chiari I malformation an elongated systolic flow may prolong the condition of increased spinal subarachnoid pressure caused by the junctional obstruction, thus favoring CSF penetration into the spinal cord. It may be also proposed that a shortened systolic flow may be insufficient to maintain a hypertensive condition for enough time to induce syrinx formation.

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Nicolò Marchesini, Nicola Tommasi, Franco Faccioli, Giampietro Pinna, and Francesco Sala


Cauda equina ependymoma (CEE) is a rare tumor for which little information is available on the oncological and clinical outcomes of patients. In this study the authors aimed to address functional, oncological, and quality-of-life (QOL) outcomes in a large series of consecutive patients operated on at their institution during the past 20 years.


The records of 125 patients who underwent surgery between January 1998 and September 2018 were reviewed. Analyzed variables included demographic, clinical, radiological, surgical, and histopathological features. Neurological outcomes were graded according to the McCormick and Kesselring scales. The QOL at follow-up was evaluated by administering the EQ-5DL questionnaire.


On admission, 84% of patients had a McCormick grade of I and 76.8% had a Kesselring score of 0. At follow-up (clinical 8.13 years; radiological 5.87 years) most scores were unchanged. Sacral level involvement (p = 0.029) and tumor size (p = 0.002) were predictors of poor functional outcome at discharge. Tumor size (p = 0.019) and repeated surgery (p < 0.001) were predictors of poor outcome. A preoperative McCormick grade ≥ III and Kesselring grade ≥ 2 were associated with worse outcomes (p = 0.035 and p = 0.002, respectively). Myxopapillary ependymoma (MPE) was more frequent than grade II ependymoma (EII). The overall rate of gross-total resection (GTR) was 91.2% and rates were significantly higher for patients with EII (98%) than for those with MPE (84%) (p = 0.0074). On multivariate analysis, the only factor associated with GTR was the presence of a capsule (p = 0.011). Seventeen patients (13.7%) had recurrences (13 MPE, 4 EII; 76.4% vs 23.6%; p = 0.032). The extent of resection was the only factor associated with recurrence (p = 0.0023) and number of surgeries (p = 0.006). Differences in progression-free survival (PFS) were seen depending on the extent of resection at first operation (p < 0.001), subarachnoid seeding (p = 0.041), piecemeal resection (p = 0.004), and number of spine levels involved (3 [p = 0.016], 4 [p = 0.011], or ≥ 5 [p = 0.013]). At follow-up a higher proportion of EII than MPE patients were disease free (94.7% vs 77.7%; p = 0.007). The QOL results were inferior in almost all areas compared to a control group of subjects from the Italian general population. A McCormick grade ≥ 3 and repeated surgeries were associated with a worse QOL (p = 0.006 and p = 0.017).


An early diagnosis of CEE is important because larger tumors are associated with recurrences and worse functional neurological outcomes. Surgery should be performed with the aim of achieving an en bloc GTR. The histological subtype was not directly associated with recurrences, but some of the features more commonly encountered in MPEs were. The outcomes are in most cases favorable, but the mean QOL perception is inferior to that of the general population.

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Richard Ferch, Alberto Pasqualin, Giampietro Pinna, Franco Chioffi, and Albino Bricolo

Object. This study was performed to further elucidate technical and patient-specific risk factors for perioperative stroke in patients undergoing temporary arterial occlusion during the surgical repair of their aneurysms.

Methods. One hundred twelve consecutive patients in whom temporary arterial occlusion was performed during surgical repair of an aneurysm were retrospectively analyzed. Confounding factors (inadvertent permanent vessel occlusion and retraction injury) were identified in six cases (5%) and these were excluded from further analysis. The demographics for the remaining 106 patients were analyzed with respect to age, neurological status, aneurysm characteristics, intraoperative rupture, duration of temporary occlusion, and number of occlusive episodes; end points considered were outcome at 3-month follow up and symptomatic and radiological stroke.

Conclusions. Overall 17% of patients experienced symptomatic stroke and 26% had radiological evidence of stroke attributable to temporary arterial occlusion. A longer duration of clip placement, older patient age, a poor clinical grade (Hunt and Hess Grades IV–V), early surgery, and the use of single prolonged clip placement rather than repeated shorter episodes were associated with a higher risk of stroke based on univariate analysis. Intraoperative aneurysm rupture did not affect stroke risk. On multivariate analysis, only poorer clinical grade (p = 0.001) and increasing age (p = 0.04) were significantly associated with symptomatic stroke risk.

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Fabio Raneri, Oriela Rustemi, Giampaolo Zambon, Giulia Del Moro, Salima Magrini, Yuri Ceccaroni, Elisabetta Basso, Francesco Volpin, Martina Cappelletti, Jacopo Lardani, Stefano Ferraresi, Franco Guida, Franco Chioffi, Giampietro Pinna, Giuseppe Canova, Domenico d’Avella, Francesco Sala, and Lorenzo Volpin


The purpose of this study was to analyze the effect of the coronavirus disease 2019 (COVID-19) outbreak and of the subsequent lockdown on the neurosurgical services of the Veneto region in Italy compared to the previous 4 years.


A survey was conducted in all 6 neurosurgical departments in the Veneto region to collect data about surgical, inpatient care and endovascular procedures during the month of March for each year from 2016 to 2020. Safety measures to avoid infection from SARS-CoV-2 and any COVID-19 cases reported among neurosurgical patients or staff members were considered.


The mean number of neurosurgical admissions for the month of March over the 2016–2019 period was 663, whereas in March 2020 admissions decreased by 42%. Emergency admissions decreased by 23%. The average number of neurosurgical procedures was 697, and declined by 30% (range −10% to −51% in individual centers). Emergency procedures decreased in the same period by 23%. Subarachnoid hemorrhage and spontaneous intracerebral hemorrhage both decreased in Veneto—by 25% and 22%, respectively. Coiling for unruptured aneurysm, coiling for ruptured aneurysm, and surgery for ruptured aneurysm or arteriovenous malformation diminished by 49%, 27%, and 78%, respectively. Endovascular procedures for acute ischemic stroke (AIS) increased by 33% in 2020 (28 procedures in total). There was a slight decrease (8%) in brain tumor surgeries. Neurosurgical admissions decreased by 25% and 35% for head trauma and spinal trauma, respectively, while surgical procedures for head trauma diminished by 19% and procedures for spinal trauma declined by 26%. Admissions and surgical treatments for degenerative spine were halved. Eleven healthcare workers and 8 patients were infected in the acute phase of the pandemic.


This multicenter study describes the effects of a COVID-19 outbreak on neurosurgical activities in a vast region in Italy. Remodulation of neurosurgical activities has resulted in a significant reduction of elective and emergency surgeries compared to previous years. Most likely this is a combined result of cancellation of elective and postponable surgeries, increase of conservative management, increase in social restrictions, and in patients’ fear of accessing hospitals. Curiously, only endovascular procedures for AIS have increased, possibly due to reduced physical activity or increased thrombosis in SARS-CoV-2. The confounding effect of thrombectomy increase over time cannot be excluded. No conclusion can be drawn on AIS incidence. Active monitoring with nasopharyngeal swabs, wearing face masks, and using separate pathways for infected patients reduce the risk of infection.

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Marco Cenzato, Davide Colistra, Giorgia Iacopino, Christian Raftopoulos, Ulrich Sure, Marcos Tatagiba, Robert F. Spetzler, Alexander N. Konovalov, Andriy Smolanka, Volodymir Smolanka, Roberto Stefini, Carlo Bortolotti, Paolo Ferroli, Giampietro Pinna, Angelo Franzini, Philipp Dammann, Georgios Naros, Davide Boeris, Paolo Mantovani, Domenico Lizio, Mariangela Piano, and Enrica Fava


In this paper, the authors aimed to illustrate how Holmes tremor (HT) can occur as a delayed complication after brainstem cavernoma resection despite strict adherence to the safe entry zones (SEZs).


After operating on 2 patients with brainstem cavernoma at the Great Metropolitan Hospital Niguarda in Milan and noticing a similar pathological pattern postoperatively, the authors asked 10 different neurosurgery centers around the world to identify similar cases, and a total of 20 were gathered from among 1274 cases of brainstem cavernomas. They evaluated the tremor, cavernoma location, surgical approach, and SEZ for every case. For the 2 cases at their center, they also performed electromyographic and accelerometric recordings of the tremor and evaluated the post-operative tractographic representation of the neuronal pathways involved in the tremorigenesis. After gathering data on all 1274 brainstem cavernomas, they performed a statistical analysis to determine if the location of the cavernoma is a potential predicting factor for the onset of HT.


From the analysis of all 20 cases with HT, it emerged that this highly debilitating tremor can occur as a delayed complication in patients whose postoperative clinical course has been excellent and in whom surgical access has strictly adhered to the SEZs. Three of the patients were subsequently effectively treated with deep brain stimulation (DBS), which resulted in complete or almost complete tremor regression. From the statistical analysis of all 1274 brainstem cavernomas, it was determined that a cavernoma location in the midbrain was significantly associated with the onset of HT (p < 0.0005).


Despite strict adherence to SEZs, the use of intraoperative neurophysiological monitoring, and the immediate success of a resective surgery, HT, a severe neurological disorder, can occur as a delayed complication after resection of brainstem cavernomas. A cavernoma location in the midbrain is a significant predictive factor for the onset of HT. Further anatomical and neurophysiological studies will be necessary to find clues to prevent this complication.