Reflections upon the intrauterine repair of myelomeningocele.

The intrauterine repair of myelomeningocele presents certain advantages and has gained widespread acceptance. It significantly reduces the incidence of Chiari-2 anomalies and hydrocephalus, and it is thought to enhance the neurologic outcome. Nevertheless, several issues remain unsettled and there are no negligible disadvantages. After working with patients with myelomeningocele for 30 years, I thought about how we currently treat them. There are ethical, organizational, neurological, obstetrical, and postnatal aspects worth discussing.

PEG hydrogel sealant versus fibrin glue in posterior fossa surgery: an economic comparison across five European countries.

Aim: Posterior cranial fossa (PCF) surgery is associated with complications, including cerebrospinal fluid (CSF) leakage. Dural sealants such as polyethylene glycol (PEG)-based hydrogels and fibrin glue can prevent CSF leaks, with evidence suggesting PEG hydrogels may outperform fibrin glue. However, the budget impact of using PEG hydrogels in PCF surgeries in Europe is unclear. Materials & methods: A decision tree was developed based on a previous US model, to assess the budget impact of switching from fibrin glue to PEG hydrogel in PCF surgery across five European countries. Input costs were derived from published sources for the financial year 2022/2023. Health outcomes, including CSF leaks, were considered. Results: The model predicted that using PEG hydrogel instead of fibrin glue in PCF surgery can lead to cost savings in five European countries. Cost savings per patient ranged from EUR 419 to EUR 1279, depending on the country. Sensitivity analysis showed that the incidence of CSF leaks and pseudomeningoceles had a substantial impact on the model's results. Conclusion: PEG hydrogels may be a cost-effective alternative to fibrin glue in PCF surgery. The model predicted that cost savings would be mainly driven by a reduction in the incidence of postoperative CSF leaks, resulting in reduced reliance on lumbar drains, reparative surgery and shortened hospital stays.

Surgical Treatment of Complex Craniofacial Fractures.

OBJECTIVE: Severe craniofacial fractures may present different needs in treating intracranial lesions over facial injuries. This paper examines the results of our strategy, consisting of a single-stage combined neurosurgical-maxillofacial treatment. METHODS: A retrospective review was conducted of 33 consecutive patients with complex fractures of the anterior cranial fossa and facial skeleton, who required elective surgery for craniofacial reconstruction. Patients who required emergency surgery for intracranial clots or penetrating wounds were excluded. In all cases, all or almost all the anterior skull-base was injured with compound fractures of the frontal sinus, the orbital roofs, the lamina cribrosa, and the planum sphenoidale. In all cases, the prioritization of treatment was carefully discussed, and surgical timing and strategy were agreed. RESULTS: There was 1 dead. Olfactory injuries were always found intraoperatively. There were no mucoceles, CSF-leak recurrences, cranial infections, or neurologic worsening. The functional and neurologic results were highly satisfactory. CONCLUSIONS: The one-stage surgical treatment of complex craniofacial fractures has numerous advantages, including the possibility of reducing facial fractures without the risk of CSF leaks. It also eliminates the need for repeated procedures in fragile patients, and the need to dismantle the facial reconstruction if the skull base repair is performed later. The main issue is the surgical timing, considering that the maxillofacial surgeon usually favors early facial repair, whereas the neurosurgeon generally prefers delayed manipulation of the contused frontal lobes. A timeframe of 10-14 days after trauma may be a good compromise for safe procedures with excellent neurologic and functional outcomes.

Reoperation in Chiari-1 Malformations.

(1) Background: The issue of unsuccessful surgery for Chiari-1 malformation (CM-1), as well as its potential causes and possible solutions, remains poorly documented and studied. (2) Methods: From a retrospective review of a personal series of 98 patients undergoing treatment for CM-1 during the past 10 years, we created two study groups. Group 1: 8 patients (8.1%) requiring additional surgeries owing to postoperative complications (7 cerebrospinal fluid leakage, 1 extradural hematoma); 7 patients (7.1%) undergoing reoperations for failed decompression during the follow-up. Group 2: During the same period, we also managed 19 patients who had previously been operated on elsewhere: 8 patients who required adequate CM-1 treatment following extradural section of the filum terminale; 11 patients requiring reoperations for failed decompression. Failed decompression was managed by adequate osteodural decompression, which was associated with tonsillectomy (6 cases), subarachnoid exploration (8 cases), graft substitution (6 cases), and occipito-cervical fixation/revision (1 case). (3) Results: There was no mortality or surgical morbidity in Group 1. However, one patient's condition worsened due to untreatable syrinx. In Group 2, there were two cases of mortality, and surgical morbidity was represented by functional limitation and pain in the patient who needed revision of the occipitocervical fixation. Twenty patients improved (58.8%), 6 remained unchanged (32.3%), 1 worsened (2.9%) and 2 died (5.9%). (4) Conclusions: The rate of complications remains high in CM-1 treatment. Unfortunately, a certain rate of treatment failure is unavoidable, but it appears that a significant number of re-operations could have been avoided using proper indications and careful technique.

Towards a common language in neurosurgical outcome evaluation: the NEON (NEurosurgical Outcome Network) proposal.

BACKGROUND: The aim of this study was to achieve a consensus on the minimum set of outcome measures and predictors to be used in the neurosurgical practice and on the timing of outcome assessment. METHODS: A consensus building approach was employed. All neurosurgical departments in Lombardy (Italy) were invited to participate by the Carlo Besta Neurologic Institute IRCCS Foundation. Three workshops were organized during which a multidisciplinary group called Neurosurgical Outcome Network (NEON) was created and the methodology to select outcome measures, predictors, and timing of outcome assessment was established. Eight working groups were created for the different neurosurgical diseases (neuro-oncological, skull base, vascular, traumatic, spinal, peripheral nervous system, malformation, functional) and 8 workshops were organized to identify the outcome measures and predictors specific for each of the neurosurgical diseases based on the experts' clinical practice and the existing literature. RESULTS: A total of 20 neurosurgical departments participated in this study. Specific outcome measures, predictors and the timing of outcome assessment were identified for each of the 8 neurosurgical diseases. Moreover, a list of variables common to all pathologies were identified by the NEON group as further data to be collected. CONCLUSIONS: A consensus on the minimum set of outcome measures and predictors and the timing of outcome assessments for 8 neurosurgical diseases was achieved by a group of neurosurgeons of the Lombardy region, called NEON. These sets could be used in future studies for a more homogeneous data collection and as a starting point to reach further agreement also at national and international level.

Posterior fossa syndrome in a population of children and young adults with medulloblastoma: a retrospective, multicenter Italian study on incidence and pathophysiology in a histologically homogeneous and consecutive series of 136 patients.

INTRODUCTION: Posterior fossa syndrome (PFS) is a set of debilitating complications that can occur after surgery for posterior fossa tumors. This study aimed to assess the preoperative radiological and surgical risk factors for the onset of PFS in a histologically homogeneous population of children with medulloblastoma and compare it to a similar population of young adults. METHODS: Included patients underwent posterior fossa surgery for medulloblastoma at 11 Italian neurosurgical wards (2003-2019) and were referred to Fondazione IRCCS Istituto Nazionale dei Tumori in Milan (INT) for postoperative treatments. We collected patients' pre- and post-operative clinical, surgical and radiological data from the INT charts. To compare the distribution of variables, we used the Mann-Whitney and Fisher tests for continuous and categorical variables, respectively. RESULTS: 136 patients (109 children and 27 young adults) were included in the study. Among children, 29 (27%) developed PFS, and all of them had tumors at midline site with invasion of the fourth ventricle. Radiological evidence of involvement of the right superior (39% versus 12%; p = 0.011) or middle cerebellar peduncles (52% versus 18%; p = 0.002) seemed more common in children who developed PFS. Young adults showed an expected lower incidence of PFS (4 out of 27; 15%), that may be due to anatomical, physiological and oncological elements. CONCLUSIONS: This study confirmed some factors known to be associated with PFS onset and shed light on other debated issues. Our findings enhance an already hypothesized role of cerebellar language lateralization. The analysis of a population of young adults may shed more light on the often-neglected existence of PFS in non-pediatric patients.

Diagnosis and treatment of Chiari malformation type 1 in children: the International Consensus Document.

BACKGROUND: Chiari malformation type 1 (CM1) is a rare condition where agreed classification and treatment are still missing. The goal of this study is to achieve a consensus on the diagnosis and treatment of CM1 in children. METHODS: A multidisciplinary panel formulated 57 provisional statements based on a review of the literature. Thirty-four international experts (IE) participated in a Delphi study by independently rating each statement on a 4-point Likert scale ("strongly disagree," "disagree," "agree," "strongly agree"). Statements that were endorsed ("agree" or "strongly agree") by < 75% of raters were re-formulated, or new statements were added, and another Delphi round followed (up to a maximum of three). RESULTS: Thirty-five IE were contacted and 34 agreed to participate. A consensus was reached on 30/57 statements (52.6%) after round 1. Three statements were added, and one removed. After round 2, agreement was reached on 56/59 statements (94.9%). Finally, after round 3, which took place during the 2019 Chiari Consensus Conference (Milan, Italy), agreement was reached on 58/59 statements (98.3%) about four main sections (Definition and Classification, Planning, Surgery, Isolated Syringomyelia). Only one statement did not gain a consensus, which is the "definition of radiological failure 24 month post-surgery." CONCLUSIONS: The consensus document consists of 58 statements (24 on diagnosis, 34 on treatment), serving clinicians and researchers following children with CM1. There is a clear need for establishing an international network and registry and to promote collaborative studies to increase the evidence base and optimize the long-term care of this patient population.

Diagnosis and treatment of Chiari malformation and syringomyelia in adults: international consensus document.

BACKGROUND: Syringomyelia and Chiari malformation are classified as rare diseases on Orphanet, but international guidelines on diagnostic criteria and case definition are missing. AIM OF THE STUDY: to reach a consensus among international experts on controversial issues in diagnosis and treatment of Chiari 1 malformation and syringomyelia in adults. METHODS: A multidisciplinary panel of the Chiari and Syringomyelia Consortium (4 neurosurgeons, 2 neurologists, 1 neuroradiologist, 1 pediatric neurologist) appointed an international Jury of experts to elaborate a consensus document. After an evidence-based review and further discussions, 63 draft statements grouped in 4 domains (definition and classification/planning/surgery/isolated syringomyelia) were formulated. A Jury of 32 experts in the field of diagnosis and treatment of Chiari and syringomyelia and patient representatives were invited to take part in a three-round Delphi process. The Jury received a structured questionnaire containing the 63 statements, each to be voted on a 4-point Likert-type scale and commented. Statements with agreement <75% were revised and entered round 2. Round 3 was face-to-face, during the Chiari Consensus Conference (Milan, November 2019). RESULTS: Thirty-one out of 32 Jury members (6 neurologists, 4 neuroradiologists, 19 neurosurgeons, and 2 patient association representatives) participated in the consensus. After round 2, a consensus was reached on 57/63 statements (90.5%). The six difficult statements were revised and voted in round 3, and the whole set of statements was further discussed and approved. CONCLUSIONS: The consensus document consists of 63 statements which benefited from expert discussion and fine-tuning, serving clinicians and researchers following adults with Chiari and syringomyelia.

Management of Slit Ventricle Syndrome: A Single-Center Case Series of 32 Surgically Treated Patients.

OBJECTIVE: Slit ventricle syndrome (SVS) is an iatrogenic disease occurring in patients with ventriculoperitoneal shunt. This article reports the management modalities and results in a case series from a single center. METHODS: We reviewed a series 48 hospitalized patients with severe SVS whom we managed in a 10-year period. Thirty-seven patients harboring programmable valves (P-valves) first underwent attempts at valve reprogramming. This treatment produced no effect in 21 patients, who therefore required surgical treatment. Surgery was also required by 11 patients without P-valve. Accordingly, 32 patients had to be operatively treated by shunt externalization followed by valve replacement or endoscopic third ventriculostomy basing on intracranial pressure and ventricular size. The new valve was either ProGav Mietke (Aesculap) or Medos Codman (Integra), each equipped with its own antisiphon system. In selected cases, a programmable antisiphon system (ProSa Mietke) was used. RESULTS: Surgical mortality was 3% and major morbidity accounted for 6%. Complete resolution was obtained in 55% of cases, improvement in 32%, and no effect or worsening in 13%. Only 1 patient became shunt free after endoscopic third ventriculostomy. Medos and ProGrav provided comparable outcomes, whereas ProSa was determinant in selected cases. Pediatric age, uncomplicated shunt courses, and short SVS histories were significantly favorable indicators. CONCLUSIONS: SVS management remains problematic. However, this study individuated factors that may improve the outcome, such as wider use of P-valves to treat hydrocephalus, timely diagnosis of overdrainage, and earlier and more aggressive indications to manage SVS.

Clinical considerations on a right operculo-insular cavernous angioma: an illustrative case.

The insular cortex is considered one of the most complex regions of the brain, defined as the "hub" of somatosensory areas. Here, we examine the case of a surgically treated haemorrhagic cavernoma involving the middle and posterior insular cortex, presenting both sensory, gustative and speech symptoms. By reviewing the recent findings in humans' and primates' basic research, we illustrated clinical and radiological correlations of the reported case, confirming insular role in sensitive and gustatory functions.

Intracranial Dural Arteriovenous Fistulas: The Sinus and Non-Sinus Concept.

INTRODUCTION: Dural arteriovenous fistulas (dAVFs) account for 10-15% of all intracranial arteriovenous lesions. Different classification strategies have been proposed in the course of the years. None of them seems to guide the treatment strategy. OBJECTIVE: We expose the experience of the vascular group at Niguarda Hospital and we propose a very practical classification method based on the location of the shunt. We divide dAVF in sinus and non-sinus in order to simplify our daily practice, as this classification method is simply based on the involvement of the sinuses. MATERIAL AND METHODS: 477 intracranial dural arteriovenous fistulas have been treated. 376 underwent endovascular treatment and 101 underwent surgical treatment. Cavernous sinus DAVFs and Galen ampulla malformations have been excluded from this series as they represent a different pathology per se. 376 dAVFs treated by endovascular approach: 180 were sinus and 179 were non-sinus. 101 dAVFs treated with surgical approach: 15 were sinus and 86 were non-sinus. DISCUSSION: Of the 477 intracranial dAVF the recorded mortality and severe disability was 3% and morbidity less than 4%. All patients underwent a postoperative DSA with nearly 100% of complete occlusion of the fistula. At a mean follow-up of 5 years in one case there was a non-sinus fistula recurrence, due to the presence of a partial clipping of "piè" of the vein. CONCLUSIONS: The sinus and non-sinus concept has guided our institution for years and has led to good clinical results. This paper intends to share this practical classification with the neurosurgical community.

Surgical pathway proposal for severe paralytic scoliosis in adolescents with myelomeningocele.

PURPOSE: The increased life expectancy of patients with myelomeningocele led to the necessity of new management for addressing complex spine deformities with severe pelvic obliquity. Tethered cord release (TCR) and spinal fusion were described as treatment options. However, nowadays, the surgical strategy is not well defined and high rates of mechanical failures and complications are reported. Our aim was to propose a new two-stage surgical pathway to treat a selected group of severe myelomeningocele scoliosis. METHODS: This is a retrospective series of myelomeningocele scoliosis in paralytic adolescent patients treated with concurrent TCR and posterior spinal fusion (PSF), followed by delayed anterior fusion (ASF) through minimally invasive lateral approach. Inclusion criteria were as follows: evolutive scoliosis in tethered cord syndrome and paraplegia, main curve >70°, pelvic obliquity >20°, and age between 10 and 18 years. Clinical, surgical, and radiographic parameters were evaluated preoperatively and at a mean follow-up (FU) of 2.8 years. RESULTS: Six patients out of 58 met the inclusion criteria and were included in the study (mean age 12.3 years old). The mean curve and pelvic obliquity correction were 68.8% and 82%, respectively. No loss of correction was recorded at FU. One case of cerebrospinal fluid leakage requiring revision surgery was recorded. CONCLUSION: To the best of our knowledge, this is the first study proposing a two-stage surgical pathway for severe MMC spinopelvic deformity treatment. Concurrent TCR and PSF with delayed minimally invasive ASF permitted to minimize complications providing good fusion rates without loss of correction and implant failure.

The management of pediatric severe traumatic brain injury: Italian Guidelines.

INTRODUCTION: The aim of the work was to update the "Guidelines for the Management of Severe Traumatic Brain Injury" published in 2012, to reflect the new available evidence, and develop the Italian national guideline for the management of severe pediatric head injuries to reduce variation in practice and ensure optimal care to patients. EVIDENCE ACQUISITION: MEDLINE and EMBASE were searched from January 2009 to October 2017. Inclusion criteria were English language, pediatric populations (0-18 years) or mixed populations (pediatric/adult) with available age subgroup analyses. The guideline development process was started by the Promoting Group that composed a multidisciplinary panel of experts, with the representatives of the Scientific Societies, the independent expert specialists and a representative of the Patient Associations. The panel selected the clinical questions, discussed the evidence and formulated the text of the recommendations. The documentarists of the University of Florence oversaw the bibliographic research strategy. A group of literature reviewers evaluated the selected literature and compiled the table of evidence for each clinical question. EVIDENCE SYNTHESIS: The search strategies identified 4254 articles. We selected 3227 abstract (first screening) and, finally included 67 articles (second screening) to update the guideline. This Italian update includes 25 evidence-based recommendations and 5 research recommendations. CONCLUSIONS: In recent years, progress has been made on the understanding of severe pediatric brain injury, as well as on that concerning all major traumatic pathology. This has led to a progressive improvement in the clinical outcome, although the quantity and quality of evidence remains particularly low.

Chiari malformation type 1: are we doing less with more? Illustrative case.

BACKGROUND: Classic treatment of Chiari malformation type 1 consists of foramen magnum decompression. Selected patients may require occipitocervical fixation, transoral odontoidectomy, tonsillectomy, and so forth. Treatment standardization does not yet exist, and some patients risk being overtreated. OBSERVATIONS: A 20-year-old man with headache and Chiari malformation type 1 underwent extradural bone decompression. One year later, he was managed with the extradural section of his filum terminale. Eighteen months later, the patient underwent monitoring of intracranial pressure, occipitocervical stabilization, transoral odontoidectomy, minimally invasive subpial tonsillectomy, and occipital cranioplasty. His headache never changed, and he progressively developed hemiparesis and swallowing and respiratory disturbances. Two years later, a new magnetic resonance imaging scan showed extended syringomyelia with scarce peritonsillar subarachnoid space. The umpteenth operation consisted of the removal of a constricting epidural scar, arachnoid dissection, total tonsillectomy, creation of a wide subarachnoid space, and dural sac augmentation. The patient's initial postoperative course was smooth, and his headache improved. However, 8 days after surgery, the patient acutely presented with vegetative disturbances and died because of malignant brainstem edema of unknown origin. LESSONS: The story of this patient is not so uncommon. He underwent all the possible surgical treatments rather than a timely adequate osteodural decompression. Probably, he received less with more.

Giant calcified thoracic disk herniations: ossification of PLL or autonomous entity?

BACKGROUND: Giant calcified thoracic disk herniation (GCTD) is an uncommon event, which requires surgical treatment in less than 1% of patients. GCDTs are a specific subgroup of herniated thoracic disks occupying more than 40% of the spinal canal showing calcifications associated with a certain degree of ossification. In this paper, we are reporting our whole experience in the surgical management of GCTDs through anterior approaches. We believe that they present characteristics that associate them to the circumscribed type of Ossified Posterior Longitudinal Ligament (OPLL) with a possible common pathophysiology consisting in the dural violation. METHODS: Twenty-three consecutive patients with GCDTs were managed through anterior approaches during the period 1996-2019 at the Niguarda Hospital - Milan, Italy. Clinical data, radiological features, surgical reports, histological findings, and outcomes were reviewed. RESULTS: There was no mortality, whereas permanent morbidity consisted of 1 cases of worsened paraparesis due to accidental spinal cord contusion. One patient required reoperation to repair a postoperative cerebrospinal fluid (CSF) leakage. All patients underwent postoperative MRI which showed excellent decompression of cord and dural sac in all cases. Histological study of en-bloc removed GCTD showed typical calcification patterns of the PLL. CONCLUSIONS: GCDTs may be assimilated to the so-called "circumscribed type" of OPLL. The GCDTs may show the same radiological CT and MRI pattern of OPLL. The anterior accesses now represent the standard of care for GCTDs. The use of operative microscope and intraoperative monitoring is mandatory. The risk of CSF leakage can be markedly reduced by meticulous reconstruction of the dura and the placement of spinal drainage. Adequate exposition may sometimes require one or two levels of corpectomy with consequent vertebral body reconstruction and fixation of anterior column of the spine.

Surgical management of Chiari malformation type II.

BACKGROUND: Chiari malformation type II is present in almost all patients with myelomeningocele but usually remains asymptomatic. Symptoms are generally more severe in neonates, who have the worst prognosis. The association symptoms/hydrocephalus is well known, and first treatment usually consists of ensuring adequate ventricular drainage. Craniovertebral decompression may be required in patients who do not improve after drainage. However, mechanisms of symptom development are not yet completely understood, timing and techniques of surgery are not codified, long-term evolution is poorly reported, and there are few paper reporting clinical onset and treatment in older patients. METHODS: We reviewed our personal series of 42 consecutive symptomatic patients that required surgical treatment. Age at surgery ranged from 1 week to 44 years (mean 6.6 years). Surgical timing strictly depended on clinical conditions: urgent management in the more compromised patients (usually infants) and elective treatment before severe deterioration in patients with less severe conditions. All patients first underwent external ventricular drainage, which resolved the symptomatology in 17 cases (40%). Craniocervical decompression was required by 25 patients (60%) who received no benefit from the ventricular drainage. RESULTS: Early mortality (2 cases = 4.7%) occurred only in neonates. Clinical improvement was achieved in 37 of 40 survivors (92%). During a follow-up ranging from 2 to 20 years (mean 10.3 years), late mortality consisted of 4 cases (10%), mainly due to cardio-respiratory arrest. Twenty-two patients (55%) required surgery for shunt malfunction and 4 for cord detethering. Six patients (15%) required reoperation owing to symptom recurrence. CONCLUSION: Early treatment of symptomatic Chiari II malformations may warrant satisfactory results in a significant number of patients, even in neonates. Nevertheless, overall mortality remains relatively high, throughout the patient life. Formal transition programs and adult spina bifida care processes have become crucial.

Acute presentation of Chiari 1 malformation in children.

PURPOSE: To review the literature about the acute presentation of Chiari 1 malformation in children, with a focus on acute cervical cord syndromes with impairment of the cortico-spinal tract. To analyze the possible precipitating factors and the pathophysiology of the acute onset. METHOD: Illustrative case presentation with literature review. RESULTS: The literature includes just a few dozens of children with acute presentation of Chiari 1 malformation. The more frequent presentations consist of oropharyngeal dysfunction, cranial nerve impairment, high intracranial pressure, peripheral motor deficit. Acute impairment of cervical long tracts is very rare and we could find only 16 previously reported cases of cervical cord impairment with quadriparesis or hemiparesis. CONCLUSIONS: Nowadays, a lot of asymptomatic Chiari 1 malformations are frequently observed owing to the wide diffusion of magnetic resonance imaging. This raised the question about the management of these patients. Despite severe and even lethal manifestations being reported in previously asymptomatic patients, the absolute rarity of acute deterioration does not justify prophylactic surgery. The diagnosis of Chiari malformation may be initially difficult in patients with isolated, acute, and unusual presentations but physicians should bear in mind its possibility, because prompt cranio-cervical decompression may be decisive.