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Pediatric low-grade gliomas (pLGGs) in the cerebellar vermis present unique challenges due to their intricate anatomical location and potential impact on critical neurological functions. Surgical intervention remains a cornerstone in the management of these tumors, aiming to achieve maximal tumor resection while preserving neurological function. In this review, the authors will discuss anatomical consideration and will explore current surgical techniques and strategies employed in the treatment of cerebellar vermis pLGGs such as the midline and lateral suboccipital approaches, as well as endoscopic-assisted technique. Additionally, we will emphasize the importance of intraoperative neurophysiological monitoring (IONM) in ensuring safe and effective tumor resection. Overall, this review provides insights into the neurosurgical approach of pLGGs in the cerebellar vermis.
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BACKGROUND: Velopharyngeal insufficiency (VPI) is a known complication of transoral surgery, with a reported incidence of 8.1%. The main factor related to VPI is the split of the soft palate. However, dead space resulting from transoral decompression may play a pivotal role in the pathogenesis of the dysfunction. In our experience, functionally significant dead space is almost constantly present after transoral decompression. This is probably due to malformation in children and postoperative scarring, thus configuring a nosological entity that we could define as "syndrome of the nasopharyngeal dead space." Palatal prosthesis and pharyngoplasty have been proposed, though these surgical procedures are technically tricky and with possible complications, such as OSA symptoms, snoring, and nasopharyngeal stenosis. METHODS: We proposed an effortless and minimally invasive procedure to treat this condition based on lipofilling the nasopharynx posterior wall endoscopically. To test the procedure's functional result, the submucosa of the nasopharynx posterior wall was initially filled with resorbable material, namely fibrin glue and autologous blood. The result was optimal but regressed after one month. Then, we repeated the procedure by lipofilling with autologous abdominal fat, resulting in a more stable anatomical and functional outcome at six months follow-up. RESULTS: The patient had a prompt significant improvement of his complaints (rhinolalia and oronasal regurgitation) and a correct projection of the nasopharynx posterior wall, with correct closure during phonation and absence of oronasal reflux. CONCLUSIONS: The "syndrome of the nasopharyngeal dead space" should be correctly identified after transoral surgery. It can be effectively treated with lipofilling of the posterior nasopharyngeal wall, a simple and minimally invasive procedure.
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Insuficiencia Velofaríngea , Humanos , Masculino , Tejido Adiposo/trasplante , Tejido Adiposo/cirugía , Endoscopía/métodos , Nasofaringe/cirugía , Paladar Blando/cirugía , Complicaciones Posoperatorias/etiología , Complicaciones Posoperatorias/cirugía , Resultado del Tratamiento , Insuficiencia Velofaríngea/cirugía , Insuficiencia Velofaríngea/etiología , NiñoRESUMEN
OBJECTIVE: To investigate the Italian experience on the surgical and radiosurgical treatment of drug-resistant epilepsy due to hypothalamic hamartoma (HH) in the period 2011-2021 in six Italian epilepsy surgery centers, and to compare safety and efficacy profiles of the different techniques. METHODS: We collected pseudo-anonymized patient's data with at least 12 months of follow-up. Surgical outcome was defined according to Engel classification of seizure outcome. Univariate analysis was performed to assess the risk of post-operative seizures, categorized in dichotomous variable as favorable and unfavorable; explanatory variables were considered. Mann-Whitney or Chi-squared test were used to assess the presence of an association between variables (p < 0.05). RESULTS: Full presurgical and postoperative data about 42 patients from 6 epilepsy surgery centers were gathered. Engel class I was reached in the 65.8% and 66.6% of patients with gelastic and non-gelastic seizures, respectively. Other than daily non-gelastic seizures were associated with seizure freedom (p = 0.01), and the radiological type presented a trend toward significance (p = 0.12). SIGNIFICANCE: Endoscopic disconnection and laser interstitial thermal therapy are effective in the treatment of HH-related epilepsy, with a tolerable safety profile. Both gelastic and non-gelastic seizures can be treated, also in patients with a long history of seizures. PLAIN LANGUAGE SUMMARY: This study collected data about 42 patients with HH-related epilepsies. Endoscopic disconnection and laser therapy are both effective and safe in the treatment of hypothalamic hamartoma-related epilepsies.
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Hamartoma , Enfermedades Hipotalámicas , Radiocirugia , Humanos , Hamartoma/cirugía , Enfermedades Hipotalámicas/cirugía , Radiocirugia/métodos , Italia , Femenino , Masculino , Niño , Preescolar , Adolescente , Epilepsia Refractaria/cirugía , Resultado del Tratamiento , Adulto , Lactante , Adulto Joven , Estudios Retrospectivos , Procedimientos Neuroquirúrgicos/métodosRESUMEN
OBJECTIVE: The potential advantages of exoscopy have been discussed theoretically for a long time. Such a concept holds significance, especially in the specific setting of the posterior cranial fossa (PCF), characterized by complex anatomy and long and narrow surgical corridors with relatively extreme working angles. We aimed to: 1) analyze the institutional preliminary case-based data on the use of the Robotic 3D Exoscope AEOS Aesculap in three different PCF approaches: retrosigmoid (RSA), midline suboccipital (MSA), and supracerebellar infratentorial via feedbacks was collected using a 20-point questionnaire, and 2) perform a comprehensive review of the literature concerning the use of EX in PCF surgery. RESULTS: A total of 38 patients with neurosurgical pathologies underwent a neurosurgical procedure using the EX (Robotic 3D exoscope AEOS Aesculap) at our institution between January and March 2022. 21 surgeons were involved in the abovementioned PCF surgeries and answered the questionnaire. The main perceived advantages were in terms of ergonomics (67%), magnification (52%), and visualization of extreme angles. The main reported disadvantage was color vision (16, 76%), followed by manual mobility (24%). Concerning the review, the search of the literature yielded a total of 177 results. Upon full-text review, 17 articles were included, including 153 patients. CONCLUSIONS: In conclusion, our study provides a comprehensive evaluation of the advantages and challenges associated with using the exoscope in posterior fossa surgery, setting a precedent as the first to report on a questionnaire-based analysis of exoscope utilization in this specific domain.
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INTRODUCTION: The association between trigonocephaly and Sylvian fissure arachnoid cysts (ACs) has been occasionally reported in the literature. However, the real incidence of this association and its clinical relevance remain unknown. METHODS: The authors collected and retrospectively reviewed all clinical charts and CT scans of patients surgically treated for trigonocephaly at the Pediatric Neurosurgical Department of Fondazione Policlinico Universitario "Agostino Gemelli" IRCCS from January 2014 to June 2023. RESULTS: During the study period, 136 patients with trigonocephaly underwent surgery. Analysis of the clinical charts revealed that in 39.7% of the cases (54/136), preoperative CT scan depicted the presence of a Sylvian fissure AC. Of these, AC was bilateral in 23 cases and unilateral in the remaining 31. All unilateral ACs were on the left side. The ACs were classified as Galassi grade I in 52 cases (96.3%) and Galassi grade II in 2 cases (3.7%). Interestingly, in 1 case we reported a Galassi grade I AC enlargement during follow-up, thereby necessitating surgical fenestration. CONCLUSION: ACs and trigonocephaly are well-known conditions for pediatric neurosurgeons; however, their association is poorly defined. Despite the lack of reports on the incidence and clinical significance of this association, it is worth knowing that radiological follow-up is essential in monitoring AC evolution.
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Quistes Aracnoideos , Humanos , Quistes Aracnoideos/cirugía , Quistes Aracnoideos/diagnóstico por imagen , Quistes Aracnoideos/complicaciones , Estudios Retrospectivos , Masculino , Femenino , Lactante , Craneosinostosis/cirugía , Craneosinostosis/diagnóstico por imagen , Craneosinostosis/complicaciones , Preescolar , Tomografía Computarizada por Rayos X , NiñoRESUMEN
OBJECTIVE: The present study evaluated whether minimally invasive approaches to orbital lesions could improve surgical, clinical, and aesthetic outcomes compared with more invasive ones. This is the first study specifically addressing this topic in children. METHODS: Children consecutively operated on from January 2010 to January 2020 were analyzed. Thirty patients matched the inclusion criteria and were divided into group A: 14 cases treated with traditional surgical approaches; and group B: 16 cases managed by minimally invasive approaches. RESULTS: There were no significant differences between the 2 groups in terms of demographic data and extent of tumor resection. Mean surgical time for the approach (40 minutes vs. 70 minutes, P < 0.0001), surgical complication such as periorbital edema (37% vs. 78%, P = 0.02) and dural tear (0 vs. 21%, P = 0.05), and procedures cost (P < 0.0001) were significantly reduced in group B. Regarding clinical outcomes, group B showed a significant reduction both in terms of postoperative pain (mean score based on visual pain scale was 2.9 vs. 4.1 P = 0.003) and mean hospitalization time (4.5 days vs. 5.5 days, P = 0.0004). The cosmetic outcome according to the Sloan classification was significantly better in group B as well (81% vs. 36% class I patients, P = 0.005). CONCLUSIONS: The use of mini-invasive approaches to orbital tumor has clear advantages in terms of surgical, clinical, and cosmetic outcomes in comparable patients; therefore, they should be preferred whenever feasible. Craniotomic approaches remain necessary for very large tumors.
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Procedimientos Quirúrgicos Mínimamente Invasivos , Neoplasias Orbitales , Humanos , Niño , Procedimientos Quirúrgicos Mínimamente Invasivos/métodos , Masculino , Femenino , Neoplasias Orbitales/cirugía , Resultado del Tratamiento , Preescolar , Adolescente , Procedimientos Neuroquirúrgicos/métodos , Estudios Retrospectivos , Tempo Operativo , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/etiologíaRESUMEN
BACKGROUND: Family members dealing with the devastating impact of a cancer diagnosis are now facing even greater vulnerability due to the COVID-19 pandemic. Alongside the already overwhelming trauma, they must also bear the distressing burden of the infection risks. The purpose of this study was to examine and explore the effects in parents of pediatric cancer patients two years after the start of the COVID-19 pandemic to compare these data with the previous data. METHODS: We conducted a single-center prospective observational study, enrolling 75 parents of 42 pediatric oncology patients. Four questionnaires (IES-R; PSS; STAI-Y and PedsQL) were given to the parents 2 years after the first evaluation. RESULTS: The bivariate matrix of correlation found a strong significant positive correlation between IES-R and PSS scores (r = 0.526, p < 0.001) as in T1. Stress symptoms (t = 0.00, p < 0.001) and levels of anxiety (trait) (t = 0.32, p < 0.001) remained unchanged; anxiety state levels appeared to have increased (t = 0.425, p < 0.001); there was a significant decrease in the PedsQL tot (t = 5.25, p < 0.001). CONCLUSIONS: The COVID-19 pandemic has influenced the levels of stress and anxiety of parents and the quality of life of patients, also correlating with the traumatic impact of the diagnosis.
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OBJECTIVE: Dysphagia is a significant complication in fourth ventricle surgery. Corticobulbar motor evoked potentials (CB-MEPs) of the lower cranial nerves may provide real-time information possibly correlating with postoperative swallowing dysfunction, and the vagus nerves may prove ideal for this purpose. However, the literature is heterogeneous, non-systematic, and inconclusive on this topic. The object of this retrospective study was to evaluate the correlation between CB-MEPs of the vagus nerve and postoperative worsening or new-onset swallowing deficits in intraaxial fourth ventricle surgery. METHODS: In 21 consecutive patients undergoing surgery for fourth ventricle intraaxial tumors between February 2018 and October 2022, endotracheal tubes with two applied electrodes contacting the vocal cords were used to record vagus nerve MEPs including values at baseline, the end of surgery, and the minimum value during the operation. From the mean value of right and left vagus nerve MEP amplitudes, the minimum-to-baseline amplitude ratio (MBR) and final-to-baseline amplitude ratio (FBR) were calculated. These indexes were correlated with postoperative swallowing function. RESULTS: Given their clinical significance, receiver operating characteristic curves were obtained to evaluate the performance of these indexes in predicting postoperative swallowing function. The area under the curve (AUC) was 0.850 (p < 0.001) and the best cutoff for FBR was 67.55% for the worsening of swallowing in the postoperative period. The AUC was 0.750 (p = 0.026) and the best cutoff was 46.37% in MBR for the absence of a swallowing disorder at the late follow-up. CONCLUSIONS: This study confirmed that vagus nerve MEPs are reliable predictors of postoperative swallowing function in fourth ventricle surgery and can be feasibly used as an intraoperative monitoring technique.
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The present review aims to describe the state of the art of research studies investigating the citrullination post-translational modification in adult and pediatric brain tumors. After an introduction to the deimination reaction and its occurrence in proteins and polypeptide chains, the role of the citrullination post-translational modification in physiological as well as pathological states, including cancer, is summarized, and the recent literature and review papers on the topic are examined. A separate section deals with the specific focus of investigation of the citrullination post-translational modification in relation to brain tumors, examining the state of the art of the literature that mainly concerns adult and pediatric glioblastoma and posterior fossa pediatric tumors. We examined the literature on this emerging field of research, and we apologize in advance for any possible omission. Although only a few studies inspecting citrullination in brain tumors are currently available, the results interestingly highlighted different profiles of the citrullinome associated with different histotypes. The data outlined the importance of this post-translational modification in modulating cancer invasion and chemoresistance, influencing key factors involved in apoptosis, cancer cell communication through extracellular vesicle release, autophagy, and gene expression processes, which suggests the prospect of taking citrullination as a target of cancer treatment or as a source of potential diagnostic and prognostic biomarkers for potential clinical applications in the future.
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Pediatric optic pathway gliomas (OPG) are low-grade brain tumors characterized by slow progression and invalidating visual loss. Common therapeutic strategies include surgery, radiotherapy, chemotherapy, and combinations of these modalities, but despite the different treatment strategies, no actual treatment exists to prevent or revert visual impairment. Nowadays, several reports of the literature show promising results regarding NGF eye drop instillation and improvement of visual outcome. Such results seem to be related with the NGF-linked prevention in caspase activation, which reduces retinal ganglion cell loss.Reducing retinal ganglion cell loss results clinically in visual field improvement as well as visual electric potential and optical coherence tomography gain. Nonetheless, visual acuity fails to show significant changes.Visual impairment represents nowadays one of the major issues in dealing with OPGs. Secondary to the interesting results offered by NGF eye drop administration, further studies are warranted to better comprehend potential treatment strategies.
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Neurofibromatosis 1 , Glioma del Nervio Óptico , Baja Visión , Niño , Humanos , Glioma del Nervio Óptico/terapia , Visión Ocular , Agudeza Visual , Campos Visuales , Trastornos de la Visión/etiología , Baja Visión/complicaciones , Neurofibromatosis 1/complicacionesRESUMEN
BACKGROUND: In spite of the continuous progresses in pediatric neurosurgery, adamantinomatous craniopharyngioma (AC) remains a challenging tumor due to its proximity to optic pathways, pituitary gland, hypothalamus, and Willis' circle, which can result in significant endocrine, cognitive, and neurological morbidity after treatment with subsequent impact on the patient's quality of life (QoL). The relevance that QoL has today explains the changes in the management of AC observed over the time. The goal of the present article is to provide a historical background, to show the milestones in the changes of the AC treatment, and to analyze the current main options to manage such a challenging tumor. MATERIAL AND METHODS: The pertinent literature has been reviewed. Moreover, a comparison between the past and recent personal series is reported. RESULTS: Three main eras have been identified. The first (named Cushing era) was characterized by the need to realize a harmless surgery and to define the best way to approach AC; the second (microscope era) was characterized by a tremendous technical and technological development, with remarkable results in term of safe tumor resection and control but relatively poor QoL outcomes; and the third one (current period) is characterized by an increasing integration between surgery and adjuvant treatments, with relatively minor tumor control but significant improvement of QoL (comparable overall survival). The authors' experience reflects these changes. Two groups of children were compared: 52 cases (mean follow-up: 17.5 years) belong to the historical series (group 1, 1985-2003, aggressive surgical management) and 41 (mean follow-up: 8.5 years) to the current one (Group 2, 2004-2021, integrated management). No significant differences between the two groups were detected about recurrence rate, surgical mortality, and overall survival. However, Group 2 showed significant lower rates of postoperative panhypopituitarism, obesity, and visual deterioration. CONCLUSIONS: Radical surgery allows for a good AC control with a low rate of recurrence but high risk of permanent morbidity. Despite the greater number of recurrences and surgeries, the more conservative policy, based on a combination of treatments, seems to provide the same tumor control with a better QoL. The advances in trans-nasal and trans-ventricular endoscopy, in proton therapy and in the management of the AC cyst are the main factors that allowed such an improvement.
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Craneofaringioma , Neoplasias Hipofisarias , Niño , Humanos , Craneofaringioma/diagnóstico por imagen , Craneofaringioma/cirugía , Craneofaringioma/patología , Calidad de Vida , Resultado del Tratamiento , Neoplasias Hipofisarias/diagnóstico por imagen , Neoplasias Hipofisarias/cirugía , Estudios RetrospectivosRESUMEN
BACKGROUND: Medulloblastoma (MB) is the most common cerebellar malignancy during childhood. Among MB, MYC-amplified Group 3 tumors display the worst prognosis. MYC is an oncogenic transcription factor currently thought to be undruggable. Nevertheless, targeting MYC-dependent processes (i.e. transcription and RNA processing regulation) represents a promising approach. METHODS: We have tested the sensitivity of MYC-driven Group 3 MB cells to a pool of transcription and splicing inhibitors that display a wide spectrum of targets. Among them, we focus on THZ531, an inhibitor of the transcriptional cyclin-dependent kinases (CDK) 12 and 13. High-throughput RNA-sequencing analyses followed by bioinformatics and functional analyses were carried out to elucidate the molecular mechanism(s) underlying the susceptibility of Group 3 MB to CDK12/13 chemical inhibition. Data from International Cancer Genome Consortium (ICGC) and other public databases were mined to evaluate the functional relevance of the cellular pathway/s affected by the treatment with THZ531 in Group 3 MB patients. RESULTS: We found that pharmacological inhibition of CDK12/13 is highly selective for MYC-high Group 3 MB cells with respect to MYC-low MB cells. We identified a subset of genes enriched in functional terms related to the DNA damage response (DDR) that are up-regulated in Group 3 MB and repressed by CDK12/13 inhibition. Accordingly, MYC- and CDK12/13-dependent higher expression of DDR genes in Group 3 MB cells limits the toxic effects of endogenous DNA lesions in these cells. More importantly, chemical inhibition of CDK12/13 impaired the DDR and induced irreparable DNA damage exclusively in MYC-high Group 3 MB cells. The augmented sensitivity of MYC-high MB cells to CDK12/13 inhibition relies on the higher elongation rate of the RNA polymerase II in DDR genes. Lastly, combined treatments with THZ531 and DNA damage-inducing agents synergically suppressed viability of MYC-high Group 3 MB cells. CONCLUSIONS: Our study demonstrates that CDK12/13 activity represents an exploitable vulnerability in MYC-high Group 3 MB and may pave the ground for new therapeutic approaches for this high-risk brain tumor.
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Neoplasias Cerebelosas , Meduloblastoma , Humanos , Meduloblastoma/tratamiento farmacológico , Meduloblastoma/genética , Regulación hacia Arriba , Anilidas , Neoplasias Cerebelosas/tratamiento farmacológico , Neoplasias Cerebelosas/genética , Proteína Quinasa CDC2 , Quinasas Ciclina-Dependientes/genéticaRESUMEN
BACKGROUND AND DEFINITION: In recent years thanks to the growing use of radiological assessment, Chiari I malformation became one of the major diseases for a neurosurgeon to deal with. CIM can be classified according to the extent of cerebellar tonsil tip into the foramen magnum being a protrusion over five mm considered pathological. Such a disease is a heterogeneous condition with a multifactorial pathogenetic mechanism that can subdivided into a primary and secondary form. Regardless of the form, it seems that CIM is the result of an imbalance between the volume of the braincase and its content. Acquired CIMs are secondary to conditions causing intracranial hypertension or hypotension while the pathogenesis of primary forms is still controversial. PATHOGENESIS AND TREATMENT: There are several theories in the literature but the most accepted one implies an overcrowding due to a small posterior cranial fossa. While asymptomatic CIM do not need treatment, symptomatic ones prompt for surgical management. Several techniques are proposed being the dilemma centered in the need for dural opening procedures and bony decompression ones. CONCLUSION: Alongside the paper, the authors will address the novelty presented in the literature on management, diagnosis and pathogenesis in order to offer a better understanding of such a heterogeneous pathology.
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Malformación de Arnold-Chiari , Descompresión Quirúrgica , Humanos , Descompresión Quirúrgica/métodos , Malformación de Arnold-Chiari/diagnóstico por imagen , Malformación de Arnold-Chiari/cirugía , Malformación de Arnold-Chiari/complicaciones , Foramen Magno/cirugía , Cráneo/cirugía , Fosa Craneal Posterior/cirugía , Imagen por Resonancia MagnéticaRESUMEN
Background: Neurofibromatosis type 1 (NF1)-related gliomas appear to have a clinical behavior different from that of sporadic cases. The purpose of the study was to investigate the role of different factors in influencing the tumor response rate of children receiving chemotherapy for their symptomatic glioma. Methods: Between 1995 and 2015, 60 patients with low-grade glioma (42 sporadic cases and 18 cases with NF1) were treated. Patients with brainstem gliomas were excluded. Thirty-nine patients underwent exclusive or postsurgical chemotherapy (vincristine/carboplatin-based regimen). Results: Disease reduction was achieved in 12 of the 28 patients (42.8%) with sporadic low-grade glioma and in 9 of the 11 patients (81.8%) with NF1, with a significant difference between the 2 groups (P < 0.05). The response to chemotherapy in both the patient groups was not significantly influenced by sex, age, tumor site, and histopathology, although disease reduction occurred more frequently in children aged under 3 years. Conclusions: Our study showed that pediatric patients with low-grade glioma and NF1 are more likely to respond to chemotherapy than those with non-NF1.
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Protocolos de Quimioterapia Combinada Antineoplásica , Glioma , Neurofibromatosis 1 , Niño , Preescolar , Humanos , Glioma/diagnóstico , Glioma/terapia , Neurofibromatosis 1/diagnóstico , Neurofibromatosis 1/terapia , Vincristina , CarboplatinoRESUMEN
BACKGROUND: Iniencephaly is a rare, complex, and severe form of neural tube defect (NTD), usually associated with wide systemic involvement and poor prognosis. The malformation involves the occiput and the inion, variably combined with rachischisis of the upper cervical and thoracic spine. Although most patients are either stillborn or perish within a few hours after birth, there are reports linking iniencephaly and long survival. For the neurosurgeon, associated encephalocele and secondary hydrocephalus represent the main challenges in this setting of patients, together with proper prenatal counseling. MATERIAL AND METHODS: Herein, the authors performed a thorough review of the pertinent literature, looking for reports of long-term survivors. RESULTS: To date, only five long-term survivors have been reported, with surgical repair attempted in four of them. Furthermore, the authors added their personal experience about two children with long-term survival who underwent surgery, thus accurately comparing them with the analogous cases previously reported in literature, with the ultimate aim of providing novel information on the pathology and the therapeutic options needed to offer proper treatment to these patients. CONCLUSIONS: Although no distinctive anatomic differences have been prior identified between long-term survivors and other patients, some variations emerged to occur, regarding age of presentation, the extension of the CNS malformation, the systemic involvement, and the surgical procedures offered. Although the authors shed some light on the topic, further studies are warranted to better define this rare and complex disease, and survival.
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Hidrocefalia , Defectos del Tubo Neural , Embarazo , Niño , Femenino , Humanos , Defectos del Tubo Neural/cirugía , Defectos del Tubo Neural/complicaciones , Encefalocele/cirugía , Columna Vertebral , Hidrocefalia/complicaciones , Huesos FacialesRESUMEN
INTRODUCTION: Intraoperative ultrasound (IOUS) may aid the resection of space-occupying brain lesions, though technical limits may hinder its reliability. METHODS: IOUS (MyLabTwice®, Esaote, Italy) with a microconvex probe was utilized in 45 consecutive cases of children with supratentorial space-occupying lesions aiming to localize the lesion (pre-IOUS) and evaluate the extent of resection (EOR, post-IOUS). Technical limits were carefully assessed, and strategies to enhance the reliability of real-time imaging were accordingly proposed. RESULTS: Pre-IOUS allowed us to localize the lesion accurately in all of the cases (16 low-grade gliomas, 12 high-grade gliomas, eight gangliogliomas, seven dysembryoplastic neuroepithelial tumors, five cavernomas, and five other lesions, namely two focal cortical dysplasias, one meningioma, one subependymal giant cell astrocytoma, and one histiocytosis). In 10 deeply located lesions, IOUS with hyperechoic marker, eventually coupled with neuronavigation, was useful to plan the surgical route. In seven cases, the administration of contrast ensured a better definition of the vascular pattern of the tumor. Post-IOUS allowed the evaluation of EOR reliably in small lesions (<2 cm). In large lesions (>2 cm) assessing EOR is hindered by the collapsed surgical cavity, especially when the ventricular system is opened, and by artifacts that may simulate or hide residual tumors. The main strategies to overcome the former limit are inflation of the surgical cavity through pressure irrigation while insonating, and closure of the ventricular opening with Gelfoam before insonating. The strategies to overcome the latter are avoiding the use of hemostatic agents before IOUS and insonating through normal adjacent brain instead of corticotomy. These technical nuances enhanced the reliability of post-IOUS, with a total concordance to postoperative MRI. Indeed, the surgical plan was changed in about 30% of cases, as IOUS showed a residual tumor that was left behind. CONCLUSION: IOUS ensures reliable real-time imaging in the surgery of space-occupying brain lesions. Limits may be overcome with technical nuances and proper training.
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BACKGROUND: Laminotomy has been introduced in surgical practice to reduce complications of laminectomy after surgery of tumors in the spinal canal. However, the posterior ligament complex, which is routinely interrupted to remove the laminoplasty segment and gain access to the spinal canal, has a tendency not to heal and can lead to progressive kyphosis and collapse. CASE PRESENTATION: A 5-month-old boy affected by a thoracolumbar extradural tumor extending along seven spinal levels was operated on. The tumor was exposed and completely resected by a one-piece laminotomy with preservation of the integrity of the posterior tension band at both extremities. After 1-year radiological examination ruled out spinal deformity. CONCLUSION: The technique herein presented, which we named in situ laminotomy, allows to fully preserve the posterior tension band without reducing the exposure of the spinal canal in multilevel tumors. Additionally, the technique makes also the reconstruction of the spine elements very easy and rapid. However, longer follow-up is necessary to prove the effectiveness of this procedure in preventing long-term deformity and instability.
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Neoplasias de la Médula Espinal , Neoplasias de la Columna Vertebral , Masculino , Humanos , Niño , Lactante , Laminectomía/métodos , Neoplasias de la Columna Vertebral/diagnóstico por imagen , Neoplasias de la Columna Vertebral/cirugía , Neoplasias de la Columna Vertebral/complicaciones , Columna Vertebral/cirugía , Neoplasias de la Médula Espinal/diagnóstico por imagen , Neoplasias de la Médula Espinal/cirugía , Neoplasias de la Médula Espinal/complicaciones , Canal Medular/cirugíaRESUMEN
PURPOSE: Malformations of the craniovertebral junction (CVJ) range from mild, asymptomatic conditions to severe forms of instability with basilar invagination. Rarely, there have been accounts of forms of so-called paramedian basilar invagination, with abnormal bone masses invading the lateral portion of the foramen magnum. All these entities have been comprehensively classified both from an anatomical and embryological standpoint. METHODS: Here, we report a case of a unique CVJ malformation which is not included in any existing classification framework and could represent a novel pathologic entity. We also provide an overview of the pertinent literature. RESULTS: The patient was a 14-year-old boy with a recent onset of spastic tetraparesis. Radiological studies documented a malformation of the atlas which invaginated through the foramen magnum, causing anterolateral medullary incarceration. Surgical treatment involved posterior decompression with resection of the abnormal bone and occipito-cervical fusion. CONCLUSION: Our report enriches the panorama of CVJ malformations, showing how anatomical knowledge and embryological insights constitute the basis for the correct assessment and treatment of these complex entities.
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Malformación de Arnold-Chiari , Platibasia , Fusión Vertebral , Masculino , Humanos , Adolescente , Foramen Magno/diagnóstico por imagen , Foramen Magno/cirugía , Foramen Magno/patología , Descompresión Quirúrgica , Malformación de Arnold-Chiari/diagnóstico por imagen , Malformación de Arnold-Chiari/cirugíaRESUMEN
The management of myelomeningocele study trial showed significant prognostic improvement in fetal repair before 26 weeks of gestation. Hence, surgery in utero represents the best treatment option for open-neural tube defects (NTDs). Fetal surgery of open-NTDs has specific inclusion and exclusion criteria, which can be adequately studied with fetal MRI. The main concern: the spine (spinal defects other than Myelomeningocele and Myeloschisis, the level of the lesion higher than T1 or lower than S1 and the degree of kyphosis ≥ 30°), the skull/brain (no cerebellum herniation and Chiari II malformation and the presence of any intracranial abnormality unrelated to open NTDs), the uterus (cervix length less than 2 cm, multiple gestations and placental and uterine abnormalities) and any other fetal abnormality not attributed to spinal defect. In this review, we describe the fundamental role of fetal MRI in supporting therapeutic decisions in pre-surgery intrauterine planning through the accurate and comprehensive description of findings, providing a proposal of a structured report. In addition, we describe how post-surgical MRI is important in investigating the effectiveness of surgery and detecting repairing complications.