Intraoperative Neurophysiological Monitoring in Tethered Cord Syndrome Surgery: Predictive Values and Clinical Outcome.

INTRODUCTION: "Tethered cord syndrome" (TCS) refers to a congenital abnormality associated with neurological signs and symptoms. The aim of surgery is to prevent or arrest their progression. This study reports a retrospective case series of tethered cord syndrome surgeries, supported by intraoperative neurophysiological monitoring. METHODS: The case series comprises 50 surgeries for tethered cord syndrome in which multimodal intraoperative neurophysiological monitoring was performed using motor evoked potentials (transcranial motor evoked potentials [TcMEPs]), tibial nerve somatosensory evoked potentials (TNSEPs), and pudendal-anal reflex (PAR). The intraoperative neurophysiological monitoring results are reported and correlated with clinical outcomes. RESULTS: Sensitivity, specificity, and negative predictive value were high for TcMEPs and TNSEPs, while PAR exhibited low sensitivity and positive predictive value but high specificity and negative predictive value. Fisher's exact test revealed a significant correlation between changes in TcMEPs, TNSEPs, and clinical outcome ( P < 0.000 and P = 0.049 respectively), but no correlation was detected between PAR and urinary/anal function ( P = 0.497). CONCLUSIONS: While TcMEPs and TNSEPs were found to be reliable intraoperative neurophysiological monitoring parameters during tethered cord syndrome surgery, PAR had low sensitivity and positive predictive value probably because the reflex is not directly related to bladder function and because its multisynaptic pathway may be sensitive to anesthetics. New onset muscle weakness and sensory deficits were related to postoperative changes in TcMEPs and TNSEPs, whereas changes in PAR did not predict bladder/urinary impairment. Urinary deficits may be predicted and prevented with other neurophysiological techniques, such as the bladder-anal reflex.

Giant intradural extramedullary spinal ependymoma, a rare arachnoiditis-mimicking condition: case report and literature review.

BACKGROUND AND IMPORTANCE: Ependymomas are tumours arising from the ependymal cells lining the ventricles and the central canal of the spinal cord. They represent the most common intramedullary spinal cord tumour in adults and are very rarely encountered in an extramedullary location. Only 40 cases of intradural extramedullary (IDEM) ependymomas have been reported, all of which were diagnosed pre-operatively as IDEM ependymomas on contrast-enhanced MRI. CLINICAL PRESENTATION: We report a 23-year old male presenting with rapidly worsening signs and symptoms of spinal cord disease. A spinal MRI demonstrated a posterior multi-cystic dilatation extended between T1 and T12. Post-contrast sequences showed peri-medullar leptomeningeal enhancement and the diagnosis of spinal arachnoiditis was made. The patient underwent surgery and the spinal cord appeared circumferentially wrapped by an irregular soft tissue. The tissue was sub-totally removed and the pathological diagnosis was ependymoma WHO grade II. The patient experienced an excellent neurological recovery and no further treatments were administered. A small residue is now stable at 2.5 years follow-up. CONCLUSIONS: Giant IDEM ependymomas are rare entities and pre-operative diagnosis can be challenging in some cases. Surgery represents the main treatment option being resolutive in most cases.

Acute bilateral foot drop due to lumbar disc herniation treated by bilateral interlaminar approach: case report and literature review.

Foot drop due to lumbar disc herniation (LDH) is a relatively common finding in spinal practice. Bilateral foot drop (BFD) due to LDH is an extremely rare condition with only a few reported cases. We describe the case of a middle-aged man presenting with a rapid onset BFD with back and leg pain. Urgent MRI revealed an L4-L5 centrally located LDH with bilateral compression of the L5 nerve roots and the cauda equina centrally. About 4 h after presentation surgery was performed adopting a bilateral L4-L5 interlaminar approach and the prolapsed disc was removed. Nine months after surgery, the patient showed a complete recovery of his deficit. We discuss the advantages of this approach in this urgent situation and we compare it with other techniques.

Sporadic cauda equina hemangioblastoma: surgical management in a pregnant woman.

Hemangioblastoma is a rare benign vascular tumour of the central nervous system that occurs either sporadically or in association with Von Hippel-Lindau syndrome. Hemangioblastoma can be found throughout the central nervous system and usually present with late manifestations due to their slow growth rate. Cauda equina hemangioblastomas are extremely rare, and in the literature, no cases are reported on the surgical management of pregnant patients with this condition. We report the case of a young woman with back pain and sphincter dysfunctions in whom an L1-L2 hemangioblastoma was diagnosed. We describe the perioperative and surgical strategies we adopted to successfully treat the patient and preserve the fetus well-being.

Trans-thoracic versus retropleural approach for symptomatic thoracic disc herniations: comparative analysis of 94 consecutive cases.

PURPOSE: The authors illustrate their results in the surgical treatment of symptomatic thoracic disc herniations (TDHs) by comparing the traditional open to the less invasive retropleural lateral approaches. METHODS: Retrospective review of 94 consecutive cases treated at a single Institution between 1988 and 2014. Fifty-two patients were males, 42 females, mean age was 53.9 years. Mean follow-up was 46.9 months (12-79 months). 33 patients were diagnosed with a giant thoracic disc herniation (GTDH). Upon admission, the most common symptoms were: motor impairment (91.4%, n = 86), neuropathic radicular pain with VAS > 4 (50%), bladder and bowel dysfunction (57.4% and 41.4% respectively) and sensory disturbances (29.7%). The surgical approach was based upon level, laterality and presence or absence of calcified lesions. RESULTS: Decompression was performed in 7 cases via a thoraco-laparo-phrenotomy and in 87 cases via an antero-lateral thoracotomy. Out of the latter cases, 49 (56%) were trans-thoracic trans-pleural approaches (TTA) and 38 (44%) were less invasive retropleural approaches (MIRA). At follow-up, there were 59.5% neurologically intact patients according to the McCormick Scale, while 64.8% and 67% had no bladder or bowel dysfunction respectively. Complications occurred in 24 patients (25.5%). Pulmonary complications were the commonest (12.7%) with pleural effusion being significantly more common in patients treated with TTA compared to MIRA (20% vs 5.2%: X2 4.13 P:0.042). Severe post-operative neuralgia (VAS 7-10) was also significantly more frequent in the TTA group (22.4% vs 2.6% X2 7.07 p 0.0078). CONCLUSIONS: MIRA is a safe and effective technique to obtain adequate TDH decompression and is associated with lower morbidity compared to TTA.

12 Years delayed postoperative spinal recurrence of craniopharyngioma. Case report and literature review.

A case of delayed spinal adamantinomatous craniopharyngioma recurrence is presented. A 54-year-old male patient was admitted in our Emergency Department complaining of urinary disorders and leg pains. He underwent surgical removal of intraventricular craniopharyngioma 12 years previously. On MR imaging a well-circumscribed intradural cistyc mass at the T12 level was reavealed. A T11 and T12 laminotomy was performed and total removal of the tumour was achieved. Histology examination showed adamantinous craniopharyngioma. The authors believe that this represents the third case described of spinal craniofaryngioma recurrence and the first involving the dorsal spine.

Basal ganglia haemorrhages: efficacy and limits of different surgical strategies.

To assess the role of surgery in patients with spontaneous basal ganglia haemorrhages, we evaluated poor outcome (mortality and prolonged unawareness) one month after 'open' surgery in patients with haematomas larger than 30 cm(3). One hundred and twenty-seven patients were traced over a 5-year period. Excluding deeply comatose patients (Glasgow Coma Scale [GCS] 3-4, n = 39), we analysed the remaining 88 patients, dividing them into two homogeneous groups according to the modality of treatment: aggressive or palliative. Multivariate analysis was applied both to the overall population and to the two groups in order to determine factors prognostic for poor outcome. Aggressive treatment was defined as surgery as the first-choice treatment modality aimed at 'complete' evacuation. Palliative treatment was defined as delayed surgery and/or surgery aimed at clot removal only to obtain internal decompression. Efficacy was assessed in patients having the same initial GCS score in both groups. Factors significantly associated with outcome were preoperative complications, volume, timing of operation, residual clots and postoperative complications. Outcomes were significantly better for aggressive surgery (17% vs. 68%, p < 0.001). On analysing the two treatment groups, volume and GCS were found to be significantly correlated with outcome in the palliative treatment group, while pre- and postoperative complications were significantly correlated with outcome in the aggressive treatment group. As judged by preoperative GCS score, aggressive treatment is always effective while palliative treatment is valid for GCS 9 or more. It clearly emerged that early surgery, aimed at removing all the clots, improves the outcome in patients with spontaneous ganglionic haemorrhages (excluding deeply comatose patients) and has wider indications than palliative surgery. This aggressive strategy is negatively affected by pre- and postoperative general complications.

Primary high-grade B-cell lymphoma of the choroid plexus.

Primary central nervous system lymphoma (PCNSL) is an extranodal non-Hodgkin lymphoma arising from and confined to the neuraxis. It represents about 3-4% of all primitive brain neoplasms and is mainly located in deep supratentorial regions. A ventricular involvement is quite frequent, but in the vast majority of cases it is secondary to an extension from a subependymal location. Amongst the primary ventricular forms occasionally reported, a choroid plexus origin is extremely rare; to date, only three cases have been described in the English literature. We report the case of a 71-year-old left-handed woman admitted to our department after a progressive 2-week history of confusion, motor speech disturbances and left hemiparesis. A brain MRI scan displayed an intraventricular lesion located in the right atrium, about 2.5cm in its major axis, homogeneously enhancing after gadolinium administration, surrounded by edema in the homolateral deep hemispheric region; the main diagnostic hypothesis was meningioma. She underwent a parieto-occipital craniotomy and a navigation-assisted posterior parietal transcortical approach, achieving complete removal of the intraventricular lesion originating from the choroid plexus. The histological diagnosis was of high-grade diffuse large B-cell lymphoma. Both an MRI scan of the entire craniospinal axis and a thoracic and abdominal CT scan showed no other neoplastic lesions; a bone marrow biopsy was normal; a final diagnosis of primary central nervous system lymphoma was made. CSF cytological analysis did not show any signs of lymphoma dissemination. Adjuvant chemotherapy with methotrexate was administered, and at the last follow-up (26 months) our patient is clinically and radiologically disease-free. Primary choroid plexus lymphoma is a very rare tumor that should be included in the differential diagnosis of intraventricular neoplasms. It usually occurs in the lateral ventricles (with a predilection for the atrium) of adult people (>50 years of age), apparently with a male prevalence. Generally, the radiological picture mimics that of a meningioma, despite the fact that meningiomas usually reach a greater volume before clinical onset. Surgery is almost always necessary to obtain an adequate tissue sample and frequently leads to a radical removal of the lesion. Low-grade marginal zone B-cell, T-cell and high-grade diffuse large B-cell (present case) forms have been described. With a proper adjuvant therapy a relatively good outcome is observed.

Leksell gamma knife radiosurgery for cerebral arteriovenous malformations in pediatric patients.

OBJECTS: The authors report their experience of gamma knife radiosurgery (GKR) in a large series of pediatric cerebral arteriovenous malformations (cAVMs). The advantages, risks and failures of this approach are presented and discussed. METHODS: Gamma knife radiosurgery was performed on 63 children aged < or =16 years. Haemorrhage was the clinical onset in 50 out of 63 cases. The mean pre-GK cAVM volume was 3.8 cm(3). Fifty-eight out of 63 cAVMs were Spetzler-Martin grades I-III. Most lesions (47 out of 63) were in eloquent or deep-seated brain regions. CONCLUSION: Gamma knife radiosurgery-related complications occurred in 2 out of 47 cases with an available follow-up (1 had transient and 1 permanent morbidity). No bleeding occurred during the latency period. In 39 children with >36-month follow-up, complete cAVM occlusion was angiographically documented in 31, with a 3- and 4-year actuarial obliteration rate of 72 and 77% respectively. High rates of complete obliteration and very low frequency of permanent morbidity with no bleeding during the latency period encourage widespread application of GKR in the treatment of pediatric cAVMs.