Chiari malformation-related headache: outcome after surgical treatment.

The outcome of headache in a series of 135 operated CM1 is presented. Favorable results were obtained in 85% of atypical and 93% typical headache with the support of a multidisciplinary approach that restricted the indications for surgery.

Discrete or diffuse intramedullary tumor? Contrast-enhanced intraoperative ultrasound in a case of intramedullary cervicothoracic hemangioblastomas mimicking a diffuse infiltrative glioma: technical note and case report.

Hemangioblastomas are benign, highly vascularized intramedullary lesions that may also extend into the intradural space. Surgery represents the standard therapy, with the goal of obtaining complete resection even at the risk of neurological morbidity. MRI is the gold standard for diagnosis and assessment of intramedullary tumors. Nevertheless, sometimes MRI may not accurately differentiate between different types of intramedullary tumors, in particular if they are associated with syringes or intra- and peritumoral cysts. This could subsequently affect surgical strategies. Intraoperative ultrasound (ioUS) has become in the last few years a very useful tool for use during neurosurgical procedures. Various ioUS modalities such as B-mode and Doppler have been applied during neurosurgical procedures. On the other hand, the use of contrast-enhanced ultrasound (CEUS) is not yet well defined and standardized in this field. We report a case of a young patient harboring a cervicothoracic intramedullary tumor, for which the preoperative neuroradiologi-cal diagnosis was in favor of a diffuse astrocytoma with nodular components whereas ioUS demonstrated 3 distinct intramedullary nodules. CEUS showed highly vascularized lesions, compatible with hemangioblastomas. These findings, particularly those obtained with CEUS, allowed better definition of the lesions for diagnosis, enhanced understanding of the physiopathological aspects, and permitted the localization of all 3 nodules, thus limiting spinal cord manipulation and allowing complete resection of the lesions, with an uneventful postoperative neurological course. To the best of our knowledge, this is the first report of the use of intraoperative CEUS in a case of intramedullary hemangioblastoma.

Surgical Management of Chiari 1.5 in Children: A Truly Different Disease?

Background: In patients with Chiari 1.5 malformation (CM1.5), a more aggressive disease course and an increased association with craniovertebral junction (CVJ) anomalies has been suggested. The best management of this subgroup of patients is not clearly defined, also due to the lack of specific series elucidating this anomaly's peculiar characteristics. Methods: We evaluated a series of 33 patients (25 females, 8 males; mean age at surgery: 13 years) fulfilling the criteria for Chiari 1.5 diagnosis who underwent posterior fossa decompression and duraplasty (PFDD) between 2006 and 2021. Results: Headache was present in all children, five presented central apnea, five had dysphagia, and three had rhinolalia. Syringomyelia was present in 19 (58%) children. Twenty patients (61%) showed various CVJ anomalies, but only one child presented instability requiring arthrodesis. The mean tonsil displacement below the foramen magnum was 19.9 mm (range: 12-30), without significant correlation with the severity of symptoms. Syringomyelia recurred or was unchanged in three patients, and one needed C1-C2 fixation. The headache disappeared in 28 children (84%). Arachnoid opening and tonsil coagulation or resection was necessary for 19 children (58%). Conclusions: In our pediatric CM series, the need for tonsil resection or coagulation was higher in CM1.5 children due to a more severe crowding.

Multidisciplinary Management of Children with Occult Spinal Dysraphism: A Comprehensive Journey from Birth to Adulthood.

Occult spinal dysraphism (OSD) comprises different forms of failure in embryogenic development that can lead to genitourinary, spinal, or lower limb alterations, thus determining progressive neurological deterioration. The correct management of children harboring OSD represents a significant issue during their life up to adulthood. However, patients often have to entertain individual consultations with each specialist. We settled on a multidisciplinary team comprising pediatric neurosurgeons, urologists, neurologists, orthopedists, and other supporting physicians. We present the results of such actions by analyzing a series of 141 children with OSD subjected to neurosurgical procedures, evaluating the impact of multidisciplinary management on outcomes. We also evaluated the specific actions according to the different ages of OSD patients from birth to adulthood to provide a schematic plan that could represent a basis for establishing and disseminating the need for a multidisciplinary approach in OSD management. The multidisciplinary team allows all consultants to see the patient together, covering specific aspects of history and examination pertinent to their management. Offering a one-stop service prevents coordination issues between the different medical teams, avoids delays or cancellations of the various appointments, optimizes cost-effectiveness, and improves efficiency and parents' satisfaction.

Surgical treatment and clinical outcome of large pediatric interhemispheric cysts with callosal agenesis: A systematic literature review with four additional patients.

OBJECTIVE: Large interhemispheric cysts (IHC) with partial or complete agenesis of corpus callosum (ACC) constitute a heterogeneous group of rare disorders. Neurosurgical treatment, in the terms of if, when and how to operate, remains unclear METHODS: We performed a surgical literature review of series or reports of IHCs with callosal anomalies; we evaluated whether revision surgeries were necessary and considered the dimensional change in the cyst postoperatively and the developmental outcome. We also reported our experience with sfour patients treated by programmable cysto-peritoneal (CP) shunting. Patients' clinical history, neuroradiological and neuropsychological performances were evaluated pre and post operatively. RESULTS: The review included 133 patients with surgically-treated IHCs. Although most authors are in agreement to perform surgery if the patients present signs of raised ICP and to not intervene in those completely asymptomatic, for other signs and symptoms the debate is still open; only few authors performed cognitive tests pre and post-operatively. Shunting procedures were successful in 60% of our reviewed cases and often lead to a major cyst collapse. Craniotomy achieves good results but is extremely invasive. Endoscopy is minimally invasive and our review demonstrated a success rate of 66%. However, endoscopy does not ensue a complete cyst collapse. Our series and review seem to suggest a possible link between parenchymal re-expansion and cognitive outcome. CONCLUSIONS: Early and effective surgery seems to obtain a greater cerebral parenchyma re-expansion and long-term cognitive evolution. Endoscopy is safe and reliable, but more data is needed on the impact of uncomplete cyst collapse on neurocognitive outcome.

Diagnosis of Ventriculoperitoneal Shunt Malfunction: A Practical Algorithm.

OBJECTIVE: This study aims to present a practical method to accurately diagnose ventriculoperitoneal shunt (VPS) malfunction and to detect the exact level at which the system has failed to tailor VPS revision at that level only. METHODS: A tertiary referral single-center algorithm for diagnosis of VPS malfunction is proposed. Based on clinical symptoms and confirmed ventricular dilatation on computed tomography, the VPS reservoir is punctured; if no cerebrospinal fluid is obtained, ventricular catheter replacement is recommended. Conversely, if cerebrospinal fluid is obtained, a sample is sent for cultural examination and the macroscopic integrity of the whole system is checked via plain radiography in the angiographic suite. Then, through the injection of iodate contrast medium into the reservoir and selective exclusion of the proximal and distal catheters, the patency and correct VPS functioning are investigated. RESULTS: A total of 102 (56 males) patients (mean age, 41.5 years; range, 1-86 years) underwent a VPS function test from 2012 to 2018: 59 cases of VPS malfunction (57.8%) were diagnosed. Ventricular catheter obstruction/damage/displacement occurred in 12/59 patients (20.3%), valve damage in 11/59 patients (18.6%), distal catheter obstruction/damage/displacement in 17/59 patients (28.8%) and 2-level (valve/proximal catheter or valve/distal catheter) obstruction/damage/displacement in 16/59 patients (27.1%). Subclinical infection was diagnosed in 3 patients (5.1%). VPS revision was performed selectively at the level of failure. CONCLUSIONS: The proposed algorithm is a practical, simple and minimally invasive technique to accurately diagnose VPS malfunction, identifying the exact level of system failure and allowing surgical VPS revision to be tailored, avoiding unnecessary complete system replacement.

Pediatric primitive intraneural synovial sarcoma of L-5 nerve root.

Primitive intraneural synovial sarcomas are rare in children. The authors report the case of a 7-year-old girl affected by intraneural synovial sarcoma of a lumbar nerve root, the first such lesion in this location described in a child. The lesion mimicked a schwannoma clinically and radiologically. There was long-lasting leg pain in a radicular distribution, and a well-demarcated intraneural tumor was seen on MRI. On this basis, the first resection was conservative. However, histological examination documented a classic biphasic synovial sarcoma, which was confirmed by immunohistochemistry. After radical resection and adjuvant treatment, complete disease control was achieved and verified at 5-year follow-up. This case strongly suggests that early diagnosis and a multidisciplinary approach to this unusual spinal lesion are essential to achieving a better prognosis.

Surgical site infections after elective neurosurgery: a survey of 1747 patients.

OBJECTIVE: To evaluate the incidence and risk factors of postsurgical site infections (SSIs) in elective neurosurgical procedures in patients treated with an ultrashort antibiotic protocol. METHODS: In this consecutive series of 1747 patients treated with elective neurosurgery and ultrashort prophylactic antibiotic therapy at the Fondazione Istituto Nazionale Neurologico "Carlo Besta" in Milan, the rate of SSIs was 0.7% (13 patients). When only clean neurosurgery was considered, there were 11 such SSIs (1.52%) in 726 craniotomies and one SSI (0.15) in 663 spinal operations. The antibiotic protocol was prolonged in every case of external communication as cerebrospinal fluid leaks or external drainages. RESULTS: The infection rate of the whole series was low (0.72%), and a risk factor identified for SSIs in clean neurosurgery was longer surgery duration. The relative risk estimate was 12.6 for surgeries lasting 2 hours and 24.3 for surgeries lasting 3 or more hours. Patients aged older than 50 years had a lower risk of developing SSI with a relative risk of 0.23 when compared with patients aged younger than 50 years. CONCLUSION: The present series reports a low incidence of SSIs for elective neurosurgery, even for high-risk complex craniotomies performed for tumor removal. Given that an antibiotic protocol prolongation was used to pretreat any early signs of infection and external communication, the protocol was appropriate for the case mix. The two identified risk factors (surgical duration > 2 hours and middle-aged patients [16-50 yr]) may be indicators of other factors, such as the level of surgical complexity and poor neurological outcome.