Laminoplasty with tented duraplasty for Hirayama disease.

BACKGROUND: Hirayama disease (HD) is a characterized by progressive amyotrophy of the upper limbs due to a forward displacement of the cervical dura during neck flexion. METHODS: Unlike other treatment options aiming at preventing cervical flexion (e.g., collar or arthrodesis), laminoplasty with tented duraplasty addresses dural dysplasia. Technically, the procedure consists in enlarging the dural sac by performing an expansile duraplasty that is secured to the yellow ligaments, in association with an open-book laminoplasty. CONCLUSION: Laminoplasty with tented duraplasty is a surgical option addressing the cause of HD to prevent further neurological deterioration while preserving cervical motion.

Deep retroinsular and parieto-opercular origin of vestibular symptoms: A stereoelectrocenphalography (SEEG) study.

Several studies have shown that the retroinsular and posterior parietal operculum regions play a central role in vestibular processing. Electrical stimulations performed during stereoelectroencephalography (SEEG) in patients with focal drug-resistant epilepsy could contribute to the analysis of this area. Among the 264 SEEGs performed in both an adult and a paediatric epilepsy surgery centre, we retrospectively identified 24 patients (9%) reporting vertigo during electrical stimulations (ES). In seven of them (29% of patients experiencing vertigo during ES), it was evoked by stimulating the retroinsular region. The reported responses were mostly not rotatory sensations but actually illusions of body, limb or limb segment movement. The involved area is limited. Moreover, two patients reported having the same symptoms at the beginning of their seizures starting in the same region. Our case study confirms the pivotal role of the retroinsular and posterior parietal operculum areas in vestibular responses, and we therefore advise the exploration of this region when patients report an illusion of body movement at the beginning of their seizures.

Relationship between mild traumatic brain injury and the gut microbiome: A scoping review.

There is increasing evidence for the important role of gut microbiota (GMB) in the development and progression of neurologic pathologies. Some studies have shown that modifying the microbiome profile can confer benefits to patients. Mild traumatic brain injury (mTBI) is a common occurrence in the general population. Although most patients recover, in a minority, disabling symptoms can persist for several months. We carried out a review of the literature to assess the effect of mTBI on GMB and to determine whether alleviating dysbiosis can improve clinical outcomes in mTBI patients. We performed searches in Medline/PubMed and Embase using the keywords "MTBI" AND "microbiome" OR "microbiota". Additional articles were identified by manual searches and using the Google search engine. In animal models, a clear perturbation of GMB was reported following TBI and probiotic supplementation (Lactobacillus acidophilus or Clostridium butyricum) improved neurologic function. There were no studies on changes in GMB after mTBI in humans; however, pre- or probiotic supplementation reduced the infection rate in patients with severe TBI and shortened the time spent in the intensive care unit without conferring any neurologic benefits. Thus, although the findings from animal models are promising, clinical studies are needed to determine whether therapeutic strategies that restore gut microbiome profile can improve long-term outcomes of patients with mTBI.

Microsurgical resection of a radicular hemangioblastoma with subarachnoid hemorrhage: how I do it.

BACKGROUND: Hemangioblastomas (HBL) are benign tumors occurring sporadically or associated with Von Hippel-Lindau syndrome (VHL). METHOD: We present the pre-, per-, and postoperative course of a rare case with radicular HBL presenting with subarachnoid hemorrhage (SAH) in the frame of VHL. We describe the microsurgical approach. CONCLUSION: Complete microsurgical in bloc resection has been performed. Postoperative course was uneventful.

Paragangliomas of the spine: a retrospective case series in a national reference French center.

INTRODUCTION: Primary paragangliomas (PG) of the spine are extremely rare entities. The present study reviews our experience over a period of 30 years. METHODS: This is a retrospective, single center, observational study. Patients surgically treated for a spinal PG with confirmed anatomopathological diagnosis were included. The McCormick classification was used as a reference for clinical evaluation. Follow-up MRI and clinical assessment took place at 6 weeks, 3 months, 6 months, and 1 year after surgery and on yearly basis after. RESULTS: Six cases have been operated in our institution. The mean age was 37.8 (median 35.5, 30-53). The mean follow-up period was 9.6 years (median 9.5, 1-23). Preoperative duration of symptoms varied between a few hours to 4 years. Low back pain was most common sign. One presented with hemorrhage and acute onset of paraplegia. All patients underwent single surgery, with the exception of one case, which had two surgeries on the same anatomical site and a third surgery on another location of the same tumor type. Preoperatively, McCormick scale was I in four cases, and II and IV in one case, respectively. Postoperatively, all patients in McCormick I retained the same class; one patient in McCormick II passed to McCormick III; the case in McCormick IV recovered to McCormick II. Five of eight surgeries achieved total resection, while two surgeries accomplished a partial microsurgical excision and one a gross total resection. Three patients had spinal leptomeningeal dissemination. Two of them benefited from extended spine radiotherapy, while the other of a "wait-and-scan" policy. Spinal leptomeningeal dissemination was stable in all patients at last follow-up. CONCLUSION: We consider surgery as primary treatment in all PG. In our experience, preoperative diagnosis is difficult and caution must be taken to perioperative course in these cases. We do not routinely perform postoperative radiation if there is a residual tumor. We regularly perform clinical and radiological follow-up, so as to be able to document recurrent cases, which have been reported even up to 30 years after primary surgical excision.

Transsphenoidal resection for pituitary adenoma in elderly versus younger patients: a systematic review and meta-analysis.

INTRODUCTION: Pituitary adenomas (PA) are common intracranial tumors. In the context of the aging of the population, the question is whether postsurgical outcomes are comparable to the younger ones. The primary objective of the present study was to review published resection and recurrence rates after transsphenoidal resection. The secondary aim was to evaluate visual improvement and complication rates. METHODS: The authors searched PubMed and Medline databases, of published English series, with no time frame limit, evaluating outcomes of transsphenoidal resection in populations aged more or less than 65, 70, and 80 years. We performed a systematic review and meta-analysis. RESULTS: Median overall resection rates for younger population was 70.7% (range 54-76.8) and for elderly one was 65.7% (range 16.6-78.2) (two-sample t test, p = 0.35). The only statistically significant difference for gross total resection rates (GTR) favored patients aged less than 80 (p = 0.01). There was no statistically significant difference among recurrence rates. There was a statistically significant difference for visual improvement favoring patients aged more than 80 (p = 0.03). For the age groups of less versus more than 70, there was a statistically significant difference for overall complication rate favoring younger groups (p < 0.05). CONCLUSION: Present data shows GTR rates favoring younger patients. Recurrence rates remain similar over the mean follow-up period. Moreover, visual improvement favors patients aged more than 80. Overall complication rates favor patients younger than 70, which might be also related to additional comorbidities, frequently present in seniors. Transsphenoidal surgery is safe and effective even for older patients.

Surgical resection of medulla oblongata hemangioblastomas: outcome and complications.

OBJECT: The purpose of this study was to analyze the surgical outcome and complications of a single-center series of medulla oblongata (MO) hemangioblastomas. METHODS: We retrospectively reviewed the medical charts of all medulla oblongata hemangioblastomas operated on at our institution between 1996 and 2015. All patients had a pre- and postoperative MRI and a minimum follow-up of 6 months. Patients were scored according to the Karnofsky Performance Scale (KPS) and McCormick Scale at the moment of admission, discharge and the last follow-up. RESULTS: Thirty-one surgical procedures were performed on 27 patients (16 females and 11 males). The mean age was 33 years, and 93 % of patients had von Hippel Lindau (VHL) disease. Three patients experienced very complicated postoperative courses, with one case ending in the death of the patient. Two patients required tracheostomy. According to McCormick's classification, 7 (23 %) of the 31 operations resulted in aggravation and 23 (74 %) in no change. Considering the seven patients with aggravation at discharge, four patients (60 %) returned to their preoperative status, one (14 %) improved but remained below his preoperative McCormick grade and two (29 %) did not improve. At last follow-up, KPS was ameliorated in 53 %, stable in 40 % and worsened in 7 % of cases. CONCLUSION: Surgery of medulla oblongata hemangioblastomas is a challenging procedure characterized by an acceptable morbidity. Transient morbidity is not negligible even if the long-term outcome is in most cases favorable. A compromised neurological condition seems to be the best predictor of unfavorable outcome.

A Staged Approach for Surgical Management of Basilar Invagination.

BACKGROUND AND OBJECTIVES: Patients with basilar invagination (BI) can be treated with several surgical options, ranging from simple posterior decompression to circumferential decompression and fusion. Here, we aimed at examining the indications and outcomes associated with these surgical strategies to devise a staged algorithm for managing BI. METHODS: We conducted a retrospective cohort study in 2 neurosurgical centers and included patients with a BI, as defined by a position of the dens tip at least 5 mm above the Chamberlain line. Other craniovertebral junction anomalies, such as atlas assimilation, platybasia, and Chiari malformations, were documented. C1-C2 stability was assessed with a dynamic computed tomography scan. RESULTS: We included 30 patients with BI with a mean follow-up of 56 months (min = 12, max = 166). Posterior decompression and fusion (n = 8) was only performed in cases of obvious atlanto-axial instability (eg, increased atlanto-dental interval or hypermobility on flexion/extension), while anterior decompression (transoral or transnasal) was reserved to patients with lower cranial nerves deficits (eg, swallowing dysfunction) and irreducible anterior compression (n = 9). Patients with posterior signs (eg, Valsalva headaches) or myelopathy but without C1-C2 instability nor anterior signs were managed with an isolated foramen magnum decompression, with or without duraplasty (n = 13). Complications were more frequent for combined procedures, including neurological deterioriation (n = 4) and tracheostomy (n = 2), but reinterventions were more likely in patients undergoing posterior decompression alone (n = 3). CONCLUSION: Patient selection is key to determine the appropriate surgical strategy for BI: In our experience, combined approaches are only needed for patients with irreducible and symptomatic anterior compression, while fusion should be restricted to patient with obvious signs of atlanto-axial instability. Other BI patients can be managed by foramen magnum decompression alone to minimize surgical morbidity.

Spinal Anterior Dural Dissection: Moving From Differential to Unifying Diagnosis.

BACKGROUND AND OBJECTIVES: Cerebrospinal fluid (CSF) collections extending longitudinally at the anterior aspect of the spinal dura have been reported in association with various conditions and under multiple names. The aim of this study was to report cases associated with brachial amyotrophy (BA) and examine its relationship with other clinical variants. METHODS: We conducted a retrospective cohort study including patients who presented with a motor deficit of the upper limbs and an anterior interdural CSF collection on spinal MRI. We performed a systematic review of the literature to include cases revealed by BA. RESULTS: Seven patients presenting with BA and a confirmed dural dissection on spinal MRI were included. All patients were male with a slowly progressing history of asymmetrical and proximal motor deficit of the upper limbs. Chronic denervation affecting mostly C5 and C6 roots was found on electroneuromyography. Spinal MRI demonstrated an anterior CSF collection dissecting the interdural space and exerting a traction on cervical motor roots. Dynamic computed tomography myelogram localized the dural defect every time it was performed (4/7 cases), and surgical closure was possible for 3 patients, leading to resolution of the collection. Literature review yielded 18 other published cases of spinal dural dissections revealed by BA, including 4 in association with spontaneous intracranial hypotension and 4 others in association with superficial siderosis. CONCLUSION: We propose a unifying diagnosis termed "spinal anterior dural dissection" (SADD) to encompass spinal dural CSF collections revealed by BA (SADD-BA), spontaneous intracranial hypotension (SADD-SIH), or superficial siderosis (SADD-SS).

Long-Term Outcomes after Incomplete Resection of Intramedullary Grade II Ependymomas: Is Adjuvant Radiotherapy Justified?

Ependymomas are the most common intramedullary tumors in adults. While gross total resection is the aim of surgery, tumor infiltration might limit resection. In cases of subtotal removal, the necessary adjuvant management remains unclear. The aim of our study was to assess the need for adjuvant radiotherapy after an incomplete resection of grade II intramedullary ependymomas (IME-II). We retrospectively reviewed all cases of IME-II operated upon at a single tertiary neurosurgical center from 2009 to 2018. Patients with anaplastic or myxopapillary ependymomas, and patients with a follow-up of less than three years, were excluded. We included 46 patients: 19 (41.3%) had a gross total resection; 21 (45.7%) had a subtotal resection; and 6 (13%) had a partial resection. None of the patients underwent adjuvant radiotherapy. Over a median follow-up of 79 months (range = 36-186), seven patients presented a radiological tumor progression with a mean delay of 50.9 months (range = 18-85), of which two were symptomatic (4.3%). Progression-free survival (PFS) was 90.1% at 5 years and 76.8% at 10 years. The extent of the resection was the only significant risk factor for secondary tumor progression (p = 0.012). Four of the seven patients with recurring IME-II were treated: three patients had a second surgery, leading to two GTR and one STR, followed by radiotherapy in one case, and one patient underwent radiotherapy alone. In this study, the rate of symptomatic progression and retreatment after incomplete resection of IME-II without adjuvant radiotherapy was low, suggesting a conservative approach in such cases.

Long-term outcome of surgical treatment for idiopathic spinal arachnoid web: A case series.

OBJECTIVE: Spinal arachnoid web (SAW) is a rare condition of the spine with limited long-term follow-up data in the literature. The longest reported follow-up period was an average 3.2 years. The objective of this study is to report our long-term results of patients who underwent surgical treatment for symptomatic idiopathic SAW. METHODS: We conducted a retrospective review of cases of idiopathic SAW that were operated between 2005-2020. We collected preoperative and last follow-up (LFU) data on motor force, sensory loss, pain, upper motor neuron (UMN) sign, gait disorder, sphincter dysfunction, syringomyelia, hyperintensity on T2-MRI, appearance of newer symptoms and number of reoperations. RESULTS: Our study included 9 patients with a mean follow-up period of 3.6years (range 2-9.1years). The surgical intervention involved a standard centered laminectomy, durotomy and arachnoid lysis. At presentation, motor weakness was present in 77.8% of patients, sensory loss in 66.7%, pain in 88.9%, sphincter dysfunction in 33.3%, UMN sign in 22%, gait disorder 55.6%, syringomyelia in 55.6% and MRI T2 hyperintensity in 55.6% of patients. At LFU, there was an improvement in all symptoms and signs to varying degrees. No new neurological symptoms appeared postoperatively, and there was no recurrence during the follow-up period. CONCLUSION: Our results demonstrate that the reported immediate and short-term favorable outcomes following arachnoid lysis for symptomatic SAW persist over a long-term period and the risk of readhesion-correlated neurological deterioration following conventional surgical intervention is low.

Surgical management of pituicytomas: a single-center case series.

OBJECTIVE: Pituicytomas (PT) are rare benign sellar and/or supra sellar tumors which surgical treatment might be challenging, owing to the hypervascularity of the tumor. Here, we report our experience with PTs, describe their clinical and radiological features, and propose an optimal therapeutic strategy. METHODS: This is a retrospective single-center study, reporting the clinical manifestations, radiological characteristics, histopathological features, treatment strategies and long-term outcomes of four patients who have been treated for a PT at Bicêtre University Hospital in Paris, France, over the past 7 years. RESULTS: Four patients were operated mean age at presentation was 60.25 years. Main symptoms, which tended to be progressive, included visual field defects and pituitary-hypothalamic dysfunction. Radiologically, all our cases resented with sellar and suprasellar localization with cavernous invasion in 75% of cases. Endoscopic transphenoidal approach was performed as first surgery for all cases. Transcranial route was done in one case. During surgery, important bleeding was observed in all cases. Gross total resection was obtained with a single surgery in two cases and a second surgery was necessary in the two remaining cases. Pathological diagnosis was confirmed in all cases. No recurrences were noted after an average follow-up of 3.6 years. CONCLUSIONS: PT is a rare differential diagnosis of sellar and/or parasellar tumors, whose hemorrhagic nature can become a challenge during surgery. We added four more cases to the literature to make physicians establish Piticytomas at suspicion diagnosis for sellar and/or suprasellar masses. Knowing the diagnosis, a preparation should be made preoperatively to avoid possible complications peroperatively.

Interval between onset of symptoms and diagnosis of medulloblastoma in children: distribution and determinants in a population-based study.

Hospital-based studies have reported long delays in the diagnosis of paediatric brain tumours. Our objective was to describe the duration between onset of symptoms and diagnosis of medulloblastoma in children and study their clinical determinants in a population-based study. This retrospective cohort study included all paediatric medulloblastoma from a region of France from 1990 to 2005. The median interval from symptom onset until diagnosis for these 166 patients was 65 days and did not decrease during the study period. The most frequent manifestations were: vomiting (88%), headaches (79%), psychomotor regression (60% of children under 3 years), psychological symptoms (27%), strabismus (26%), and asthenia (25%). For one third of the children under 3 years, the diagnosis was made only after life-threatening signs of intracranial hypertension appeared. The prediagnosis interval was significantly longer (median 91 vs. 60 days, p = 0.001) in children with psychological symptoms (27%). Causes for intervals that exceeded the median (65 days) included inconsistent (25%) or late (36%) combination of headaches and vomiting, a period of spontaneous symptom remission (14%-20%), no (24%) or late (57%) neurological signs, psychological symptoms (35%), and a normal neurological examination (27%). Time to medulloblastoma diagnosis in children remains fairly long, despite advances in imaging. Primary-care physicians must be suspicious not only of suggestive neurological signs, but also of non-specific symptoms that persist or are multiple. A meticulous neurological examination and cerebral imaging for such patients might facilitate earlier diagnosis.

Surgical Management after Chiari Decompression Failure: Craniovertebral Junction Revision versus Shunting Strategies.

Revision surgery after posterior fossa decompression for Chiari malformation is not uncommon and poses both strategic and technical challenges. We conducted a single-center retrospective cohort study including all adult patients who underwent revision surgery after posterior fossa decompression for Chiari type I malformation between 2010 and 2019. Among 311 consecutive patients operated on for Chiari malformation at our institution, 35 patients had a least one revision surgery with a mean follow-up of 70.2 months. Mean delay for revision was 28.8 months. First revision surgery was performed at the level of the foramen magnum in 25/35 cases and consisted in duraplasty revision in all cases, arachnolysis (51.4%), additional bone decompression (37.1%), tonsillar coagulation or resection (25.7%), 4th ventricle to cervical subarachnoid spaces shunt (5.7%). Most repeat revisions consisted in CSF diversion procedures, with either ventriculo-peritoneal or syringo-peritoneal shunts. Mean number of interventions per patient was 3.2, with 22.9% of patients undergoing 4 or more surgeries. Based on our experience, we propose that revision at the level of the foramen magnum should be considered as a first-line strategy for Chiari decompression failure. Shunting procedures can be performed in case of extensive arachnoiditis or repeated failures.

Validity of algorithms for identifying mild traumatic brain injury in the French national emergency department database OSCOUR: a retrospective multicentre validation study protocol.

INTRODUCTION: The French emergency department (ED) surveillance network OSCOUR transmits data on ED visits to Santé publique France (the national public health agency). As these data are collected daily and are almost exhaustive at a national level, it would seem relevant to use them for national epidemiological surveillance of mild traumatic brain injury (mTBI). This article presents the protocol of a planned study to validate algorithms for identifying mTBI in the OSCOUR database. Algorithms to be tested will be based on International Classification of Diseases (ICD)-10 codes. METHODS AND ANALYSIS: We will perform a multicentre validation study of algorithms for identifying mTBI in OSCOUR. Different combinations of ICD-10 codes will be used to identify cases of mTBI in the OSCOUR database. A random sample of mTBI cases and non-cases will be selected from four EDs. Medical charts will serve as the reference standard to validate the algorithms. The sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) of the different algorithms, as well as their 95% CIs, will be calculated and compared. ETHICS AND DISSEMINATION: The ethics committee of the French National Data Protection Authority (CNIL) approved this study (n° 921152, 1 August 2021). Results will be submitted to national and international peer-reviewed journals and presented at conferences dedicated to trauma and to methodologies for the construction and validation of algorithms.

Somatomotor or somatosensory facial manifestations in patients with temporobasal epilepsies.

OBJECTIVE: The semiology of temporo-basal epilepsy has rarely been analysed in the literature. In this paper, we report three patients with proven basal temporal epilepsy with somatomotor or somatosensory facial ictal semiology, highly suggestive of insulo-opercular onset. METHODS: The three patients had a temporobasal lesion and their drugresistant epilepsy was cured with resection of the lesion (follow-up duration: 7-17 years). We reviewed the medical charts, non-invasive EEG data as well as the stereoelectroencephalography (SEEG) performed in two patients. Quantitative analysis of ictal fast gamma activity was performed for one patient. RESULTS: Early ictal features were orofacial, either somatomotor in two patients or ipsilateral somatosensory in one. The three patients had prior sensations compatible with a temporal lobe onset. Interictal and ictal EEG pointed to the temporal lobe. The propagation of the discharge to the insula and operculum before the occurrence of facial features was seen on SEEG. Facial features occurred 7-20 seconds after electrical onset. Quantitative analysis of six seizures in one patient confirmed the visual analysis, showing statistically significant fast gamma activity originating from basal areas and then propagating to insuloopercular regions after a few seconds. SIGNIFICANCE: We report three cases of lesional temporo-basal epilepsy responsible for orofacial semiology related to propagation of insulo-opercular ictal discharge. In MRI-negative patients with facial manifestations, this origin should be suspected when EEG is suggestive. These observations may contribute to our understanding of brain networks.

Clinical Image of a Spinal Ependymoma Discovered 8 Years after Initial Misdiagnosis as an Idiopathic Syringomyelia.

With the increasing frequency of neuroimaging, incidental intramedullary cavities are diagnosed more frequently. We present a case of asymptomatic incidental intramedullary cervical cavity diagnosed as an idiopathic syringomyelia as initial magnetic resonance imaging (MRI) showed an isolated cystic image without contrasted component. The patient had no subsequent MRI follow-up, but eventually showed symptoms 8 years later. By this stage of the disease, the MRI appearance had changed, showing a solid and enhanced component. The patient underwent surgical resection and histopathology concluded a papillary ependymoma (grade 2). This case illustrates how asymptomatic intramedullary cavities may hide an underlying tumoral process and why these cavities should not be considered as idiopathic syringomyelia by default, except after prolonged MRI follow-up.

Reintervention rate of arachnolysis versus shunting for nonforaminal syringomyelia.

OBJECTIVE: Surgical treatment for nonforaminal syringomyelia related to spinal arachnoiditis is still controversial. The authors sought to assess respective outcomes and rates of reintervention for shunting and spinal cord untethering (arachnolysis) in spinal arachnoiditis with syringomyelia. METHODS: This retrospective cohort study was conducted at a single reference center for syringomyelia. Patients undergoing arachnolysis and/or shunting interventions for nonforaminal syringomyelia were screened. RESULTS: The study included 75 patients undergoing 130 interventions. Arachnolysis without shunting was performed in 48 patients, while 27 patients underwent shunting. The mean follow-up between the first surgery and the last outpatient visit was 65.0 months (range 12-379 months, median 53 months). At the last follow-up, the modified McCormick score was improved or stabilized in 83.4% of patients after arachnolysis versus 66.7% after shunting. Thirty-one (41.3%) patients underwent reintervention during follow-up, with a mean delay of 33.2 months. The rate of reintervention was 29.2% in the arachnolysis group versus 63.0% in the shunting group (chi-square = 8.1, p = 0.007). However, this difference was largely driven by the extension of the arachnoiditis: in patients with focal arachnoiditis (≤ 2 spinal segments), the reintervention rate was 21.6% for arachnolysis versus 57.1% for shunting; in patients with extensive arachnoiditis, it was 54.5% versus 65.0%, respectively. Survival analysis assessing the time to the first reintervention demonstrated a better outcome in both the arachnolysis (p = 0.03) and the focal arachnoiditis (p = 0.04) groups. CONCLUSIONS: Arachnolysis led to fewer reinterventions than shunting in patients with nonforaminal syringomyelia. There was a high risk of reintervention for patients with extensive arachnopathies, irrespective of the surgical technique.

Management of Chiari malformation type I and syringomyelia during pregnancy and delivery.

OBJECTIVE: Although a recurrent question in clinical practice, the management of Chiari malformation type I (CMI) and/or syringomyelia during pregnancy and delivery is still debated. The aim of this study was to investigate the modalities of delivery and anesthesia in women presenting with CMI and/or syringomyelia at a national reference center, and to question their potential role in the natural history of these conditions. STUDY DESIGN: We conducted a retrospective cohort study using a standardized questionnaire, a customized clinical severity score and data from medical records. RESULTS: 83 patients were included in the final analysis: 32 had CMI without syringomyelia, 27 had CMI with syringomyelia and 24 had non-foraminal syringomyelia. Most patients (55/83) were not diagnosed at the time of their pregnancy, 12 had surgery before being pregnant and 16 were diagnosed but not operated. Most women underwent vaginal delivery (62 %) and neuraxial (i.e. epidural or spinal) anesthesia (69 %). However, the proportion of cesarean procedures increased to 53.6 % and even 83.3 % when considering only patients already diagnosed or operated on, respectively. Nonetheless, neither vaginal compared to cesarean delivery (change in clinically severity score: -1.5 ± 0.4 versus -0.9 ± 0.4, p = 0.4) nor neuraxial compared to general anesthesia (-1.2 ± 0.3 versus -1.5 ± 0.6, p = 0.7) were associated with increased clinical deterioration. CONCLUSION: Although individual evaluation is mandatory, this study supports that neither delivery nor anesthesia modalities affect the natural history for the vast majority of patients with CMI and/or syringomyelia.