Spinal cord lipomas: lessons learned in the era of total resection.

PURPOSE: Recent years have seen a paradigm shift towards total/near-total resection in spinal cord lipoma surgery. As this procedure is technically challenging, surgical candidates need to be selected appropriately through accurate image assessment and classification. The purpose of this paper is to describe a surgical series of paediatric spinal cord lipomas, their diagnosis, results and complications. METHODS: We undertook a retrospective review of paediatric patients with spinal cord lipomas who underwent surgery between 2008 and 2022. The variables studied were age, gender, preoperative symptoms according to the Necker Functional Score (NFS), type of lipoma according to Morota's classification, functional and radiological surgical outcomes using the cord-sac ratio (CSR), need for re-operation, complications and follow-up. RESULTS: A total of 25 patients (average age 36 months) underwent surgery. According to Morota's classification, MRI showed 13 type 1 lipomas, two type 2, two type 3 and eight type 4. The preoperative NFS was 16.06, with urological abnormalities being the most frequent manifestation. Total/near-total resection was attempted since 2015. Five patients with type 1 lipoma required re-operation due to clinical deterioration with suspected retethering, all of them with a CSR > 0.3. The series average CSR was 0.417. CONCLUSIONS: This paper highlights the importance of proper classification for a correct surgical approach to obtain favourable results and minimise possible complications. Based on our experience, given our results regarding the percentage of fistulae and retethering rates, we limited radical resection to symptomatic type 1 lipomas. Our future aim is to obtain better CSR rates and to decrease the retethering percentage.

Neuroendoscopic septostomy in unilateral and bilateral ventricular hydrocephalus.

PURPOSE: This study describes the results of septostomy (SPT) in terms of success and analysis of follow-up in a series of pediatric patients diagnosed with unilateral ventricular hydrocephalus (UHV) and biventricular hydrocephalus (BVH). METHODS: A total of 29 pediatric patients diagnosed with UVH or BVH were included in this study. In UVH, a neuroendoscopic SPT was performed (sometimes accompanied by NEFPFMO). In those diagnosed with BVH, an SPT combined with VPS was carried out. Demographic, etiological, clinical, and diagnostic variables and percentage of treatment success were collected. RESULTS: SPT was successful during follow-up when no VPS was required in UVH and only unilateral VPS was implanted in BVH. At the time of surgery, 16 patients needed a ventriculoperitoneal shunt. The first SPT was successful in 22 patients, requiring a second surgery in 7 patients, from the oncology group diagnosed with BHV. CONCLUSIONS: The surgical management of UVH and BVH still has some disclosure points to be reviewed. However, SPT seems to be a secure, non-traumatic, and efficient procedure.

Major Internal Carotid Artery Injury During Endoscopic Skull Base Surgery: Case Report.

Major vascular structures are always at risk during complex skull base surgery, particularly with use of the endoscopic endonasal approach, and intraoperative damage of the internal carotid artery (ICA) can be a devastating complication. Herein, we report a case of a young patient who had a major injury of the left ICA during endoscopic resection of a recurrent petrous bone chordoma. Massive bleeding was controlled by a Foley balloon inserted and kept in the resection area. Urgent angiography revealed a persistent leak from the petrous segment of the left ICA, and the vessel was sacrificed with coiling, since a balloon occlusion test showed good collateral blood flow. The patient woke up from anesthesia without a neurological deficit. Salvage resection of recurrent skull base neoplasms deserves specific attention because of the possibility of major vascular damage. In cases of intraoperative ICA injury, its management requires immediate decisions, and the available possibilities for endovascular therapy should always be considered.

Conventional posterior cranial vault expansion: indications and results-review of the literature.

In the late 1980s, craniofacial surgery units reported suboptimal cosmetic results, cranial volume restriction, and intracranial hypertension after anterior cranial vault remodeling in bilateral coronal synostosis associated with severe brachyturricephaly. A possible explanation was a severe associated growth restriction of the posterior calvaria with radiological synostosis at the lambda sutures. "Conventional" or "fixed" posterior cranial vault expansion techniques were developed to address these limitations, sometimes as the first surgical step in a two-staged protocol of total calvarial reconstruction, combined with suboccipital decompression in cases of symptomatic cerebellar tonsillar herniation or, more easily, to resolve the characteristic occipital flattening of lambdoid synostosis. Various surgical approaches have been described; however, the indications for and timing of surgical treatment and postoperative evaluation of results still remain controversial. Although more invasive, conventional posterior cranial vault expansion has proven to be safe and offers a remodeled and protective bony vault immediately after surgery, but the underlying cranial base malformation remained untreated, with implications in the postoperative growth of the facial skeleton. Overcorrection, rigid stabilization, and grafting are also concerns to be addressed.

Quality of life in school-age children with shunt implantation due to neonatal posthemorrhagic hydrocephalus.

PURPOSE: To assess the functional outcome in school-age children shunted in the neonatal period due to post-hemorrhagic hydrocephalus (PHH), using the HOQ-Spanish version (HOQ-sv), and to analyze predictors of quality of life in this group. METHODS: A cross-sectional study was performed between 2015 and 2018. Parents of pediatric patients with PHH attending our neurosurgery outpatient clinic were invited to complete the HOQ-sv and to enroll in the study. Clinical variables regarding the patients' neonatal course and surgical outcome were recorded. A descriptive analysis was done, and independent variables related to the HOQ scores were studied in univariate and multivariate analyses with regression trees. RESULTS: The study comprised a total of 52 patients. The mean overall HOQ score was 0.67 (on a scale from 0 [worse] to 1 [best]). The quality of life for the PHH children at school age was related to perinatal factors (gestational age at birth, time until shunt surgery, length of hospitalization at the time of shunt implantation, and comorbidity), shunt complications (symptomatic overdrainage, number of shunt revisions, and shunt revisions related to infection during the first year after treatment), and clinical background (seizures, spasticity, Gross Motor Function Classification System level or visual impairment). CONCLUSION: HOQ dimension scores in school-age children shunted due to PHH in our center were similar to those of referral centers for other etiologies of pediatric hydrocephalus. Future goals should be the prevention of complications related to worse outcomes at the time of diagnosis and to try to improve shunt performance later.

Cervical myelomeningocele with CSF leakage: a case-based review.

BACKGROUND: The cystic spinal dysraphism of the cervical and upper thoracic region (CDCT) accounts for only 3.9 to 8% of spina bifida cystica lesions. The presence of external cerebrospinal fluid (CSF) leakage is infrequent and very few authors have reported about surgical complications. CASE REPORT: We present the case of a new born diagnosed of CDCT C1-C3 with a stalk of fibrovascular tissue, CSF leakage, hydrocephalus, and type Chiari II malformation, discuss about the chosen surgical technique and the associated complications, and make a review of the literature focusing on the main aspects of CDCT. CONCLUSIONS: The CDCT with a stalk of neuroglial and/or fibrovascular tissue originates from the dorsal surface of the spinal cord and penetrates into a cervical cystic sac. The presence of CSF leakage, hydrocephalus, and/or type Chiari II malformation can influence to decide the most appropriate surgical technique.

Shunt independence in paediatric hydrocephalus: our 16-year experience and review.

PURPOSE: Shunt independence remains a matter of debate for neurosurgeons, and little information on this subject is available in the literature. The aims of the study were to analyse the incidence of shunt removal in a series of paediatric patients and to describe our experience with attempts at shunt removal. METHODS: Thirty of 212 paediatric patients shunted between 2000 and 2016 at our institution were studied for shunt independence. Variables related to hydrocephalus aetiology, shunt complications, independence trial peculiarities and follow-up were recorded and a descriptive analysis of the data was performed. RESULTS: Two patients (0.94%) refused to be included in a shunt independence trial and were analysed separately. In the other 28 patients, 29 shunt independence trials were performed, of which 19 (65.52%) were successful, giving a global rate of shunt independence of 8.96% (19/212) in our population. Secondary endoscopic third ventriculostomy was the most frequent type of independence trial and achieved the highest success rate (75%). Spontaneous independence was achieved in just 4/7 cases (57.14%). Planned removal of the shunt in overdrainage cases had a 50% success rate, with transient measures to control intracranial pressure frequently required. CONCLUSIONS: Shunt independence trials should be considered for selected patients in a closely monitored setting. Secondary endoscopic third ventriculostomy at the time of shunt malfunction has the highest success rate whereas planned removal of the shunt in overdrainage is an invasive procedure with more likelihood of failure. Shunt independence should not be presumed.

Suprasellar ganglioglioma presenting as a middle cerebral artery infarction.

BACKGROUND: Gangliogliomas are a relatively rare neoplasm with a major incidence in the pediatric population. As the temporal lobes are the most common site, patients usually present with seizures. CASE REPORT: We report the case of a 9-year-old child with an extensive suprasellar ganglioglioma presenting with a cerebral infarction due to direct compression of the medium cerebral artery. CONCLUSIONS: Suprasellar lesions can compress adjacent vascular structures, so an accurate diagnosis is necessary for an early treatment.

Purely neuroendoscopic resection of intraventricular tumors with an endoscopic ultrasonic aspirator.

Surgery for intraventricular tumors remains a controversial and evolving field, with endoscopic resection becoming more popular. We present a series of nine consecutive cases of purely endoscopic resection of intraventricular tumors with the aid of an ultrasonic aspirator specific for neuroendoscopy. Nine patients (five men, four women) aged 18-74 years (mean 43.7) underwent surgery. The most common symptom was headache. In all cases, magnetic resonance imaging showed single supratentorial intraventricular lesions (five lateral ventricle, four third ventricle). The average maximum diameter was 20.5 mm (range 11-42). Associated hydrocephalus was found in eight cases at diagnosis. Five patients underwent complete macroscopic resection. Three underwent subtotal resection and one underwent partial resection (two thirds of the tumor). The mean endoscopic procedure time was 70 min (37-209). The eight patients with associated hydrocephalus also underwent endoscopic septostomy to improve cerebral spinal fluid circulation, with one patient additionally requiring endoscopic third ventriculostomy and another requiring Monro foraminoplasty. One patient required ventriculoperitoneal shunting. The mean post-operative follow-up was 15.1 months (range 2-33). At the time of analysis, no patient showed recurrence or regrowth of the operated lesion. The histological diagnoses and degree of resection were three subependymomas with complete resection, three colloid cysts with two complete and one subtotal resection, one pilocytic astrocytoma with partial resection (approximately two thirds of the lesion), one epidermoid tumor with subtotal resection, and one central neurocytoma with subtotal resection. The endoscopic ultrasonic surgical aspirator can be a safe and effective tool for the removal of intraventricular tumors, even in firmer solid lesions.

Shunt overdrainage syndrome: review of the literature.

Shunt overdrainage in patients with hydrocephalus still represents a challenge for neurosurgeons, in part due to the lack of agreement or uniformity concerning the entity. Important problems exist relating to the real incidence of the entity, its definition, classification, and the pathophysiological theories behind the various treatment strategies proposed. Recent reports have suggested that the evidence about overdrainage and its consequences is not so robust as presumed. Consequently, the topic requires more detailed examination. In this review, we comment on all the main facets related with shunt overdrainage.

Factors related to shunt survival in paediatric hydrocephalus. Could failure be avoided?

BACKGROUND: Improved shunt survival and a better understanding of factors related to failure in paediatric hydrocephalus still pose a challenge for neurosurgeons, in order to avoid morbidity and mortality, as well as the economic impact of repeated revision surgeries. For these reasons, an analysis is performed on the factors related to the first shunt failure in the long-term follow-up of a series in a single centre. METHODS: A retrospective review was conducted on 166 hydrocephalic paediatric patients shunted for the first time between 2000 and 2014. An analysis was made of the statistical relationships between first shunt failure and the demographic, aetiological, surgical and clinical variables. RESULTS: Of the 166 shunts implanted in our centre during the study period, 111 required revision over a mean follow-up period of 93 months. Factors significantly related to failure were post-haemorrhagic and post-infectious hydrocephalus, meningitis episode, posterior burr hole, differential pressure valve, standard catheter in first surgery, and symptomatic over-drainage. Multivariable analysis showed that previous meningitis and symptomatic over-drainage were risk factors for shunt failure, whereas frontal burr hole location and antibiotic-impregnated catheters were protective factors. Cox regression determined that independent factors significantly related to a worse shunt survival, were shunt infection and symptomatic over-drainage. CONCLUSIONS: Meningitis, symptomatic over-drainage, frontal burr hole, and antibiotic impregnated catheters are significant prognostic factors for shunt survival. Shunt infection and symptomatic over-drainage are independent factors significantly related to a shorter shunt survival. Prospective, randomised, controlled trials are required to validate these results.

Surgical outcome of the shunt: 15-year experience in a single institution.

PURPOSE: Surgical outcome after shunt insertion in pediatric hydrocephalus can vary greatly. Although first shunt survival rates and complications have been studied by several teams, much less is known about survival and complications of subsequent systems. The goals of this study were to evaluate the surgical outcome in a series of pediatric patients followed for a long time and establish the differences between first and subsequent extracranial shunt survival and complications. METHODS: We undertook a retrospective study in pediatric patients treated with ventriculoperitoneal shunts between 2000 and 2015 at our institution. Surgical outcome was assessed, and different shunt survival curves were studied with Kaplan-Meier. Complications related to each shunt failure were examined and compared. RESULTS: A total of 166 patients underwent 425 procedures, with a mean follow-up period of 93 months. The median number of shunt revision surgeries was 2. Shunt survival rates were better with the first shunt compared to those with the subsequent shunts. The main complication necessitating system revision surgery was overdrainage, the frequency of proximal and distal dysfunctions was similar in all the shunt failures, and isolated ventricle and infection were more frequent in younger patients. Shunt-related infections accounted for 7 % of the procedures, and the shunt independence rate was 10 %. CONCLUSIONS: The frequency of complications related to shunt failure in pediatric patients changes during follow-up. A strict protocol of overdrainage detection and active treatment could explain the need for repeat surgeries and the progressively shorter shunt survival time in our series.

Increased levels of tumour necrosis factor alpha (TNFα) but not transforming growth factor-beta 1 (TGFß1) are associated with the severity of congenital hydrocephalus in the hyh mouse.

AIMS: Here, we tested the hypothesis that glial responses via the production of cytokines such as transforming growth factor-beta 1 (TGFß1) and tumour necrosis factor alpha (TNFα), which play important roles in neurodegenerative diseases, are correlated with the severity of congenital hydrocephalus in the hyh mouse model. We also searched for evidence of this association in human cases of primary hydrocephalus. METHODS: Hyh mice, which exhibit either severe or compensated long-lasting forms of hydrocephalus, were examined and compared with wild-type mice. TGFß1, TNFα and TNFαR1 mRNA levels were quantified using real-time PCR. TNFα and TNFαR1 were immunolocalized in the brain tissues of hyh mice and four hydrocephalic human foetuses relative to astroglial and microglial reactions. RESULTS: The TGFß1 mRNA levels were not significantly different between hyh mice exhibiting severe or compensated hydrocephalus and normal mice. In contrast, severely hydrocephalic mice exhibited four- and two-fold increases in the mean levels of TNFα and TNFαR1, respectively, compared with normal mice. In the hyh mouse, TNFα and TNFαR1 immunoreactivity was preferentially detected in astrocytes that form a particular periventricular reaction characteristic of hydrocephalus. However, these proteins were rarely detected in microglia, which did not appear to be activated. TNFα immunoreactivity was also detected in the glial reaction in the small group of human foetuses exhibiting hydrocephalus that were examined. CONCLUSIONS: In the hyh mouse model of congenital hydrocephalus, TNFα and TNFαR1 appear to be associated with the severity of the disease, probably mediating the astrocyte reaction, neurodegenerative processes and ischaemia.

[Factors related to endoscopic third ventriculostomy prognosis in the paediatric population]. / Factores relacionados con el pronóstico de la ventriculostomía premamilar endoscópica en pacientes pediátricos.

Endoscopic third ventriculostomy (ETV) for paediatric patients has different success rates between the published series, making its recommendation controversial. Different definitions of ETV success, hydrocephalus aetiology or patient age at diagnosis may influence the outcome of the ETV procedure. The aim of this work was to analyse our clinical series and to examine the influence of different factors on ETV outcome. This was a retrospective study of 45 patients who had undergone ETV at our Paediatric Hospital between 2003 and 2009. Successful outcome was defined as a combination of features including clinical improvement or stability, with at least 1 positive radiological parameter. The influence of age, hydrocephalus aetiology, existence or not of previous shunt and the type of endoscopic procedure were analysed in relation to ETV outcome. Up to 29% of patients were younger than 1 year. The most frequent causes of hydrocephalus were: brain tumour, aqueductal stenosis and myelomeningocele. The overall success rate was 69%, with a mean follow-up period of 26 months and mean ETV survival of 14 months. We obtained statistically significant differences in ETV success between patients aged over and under 6 moths. Our ETV success rate can be considered safe and effective for the treatment of paediatric hydrocephalus. To our knowledge, ETV is most effective in patients aged 6 months and over.

Success criteria in pediatric neuroendoscopic procedures. Proposal for classification of results after 67 operations.

PURPOSE: Controversial issues exist concerning criteria for patient selection and long-term success in pediatric neuroendoscopic procedures. We designed a classification of success grades applicable to high-pressure and chronic hydrocephalus and also to those cases in which different endoscopic maneuvers are performed during the same procedure. We then evaluated the success rate and complications in our series. METHODS: A total of 59 patients underwent 67 neuroendoscopic procedures between January 2003 and January 2011. A retrospective study was made of the preoperative history, operative reports, and postoperative imaging findings and medical records. A 5-grade scale was developed to assess the type of success depending on clinical and radiological data. Complications related to the surgical procedure were also recorded. RESULTS: Two patients were excluded from the success analysis due to insufficient follow-up time. The final results for the first procedures in 57 patients were complete and permanent success (grade I) in 49.1%, complete but transitory success (grade II) in 10.5%, partial success (grade III) in 12.3%, doubtful success (grade IV) in 5.3%, and failure (grade V) in 22.8%. In eight cases a second procedure followed the failure of the first: grade I success was achieved in seven cases (87.5%) and grade V in one case (12.5%). The highest success rates were achieved in cases of hydrocephalus caused by tumors or arachnoid cysts and the lowest in slit ventricle syndrome. CONCLUSIONS: A common classification of degrees of success, such as that proposed here, would aid the development of comparative and cooperative studies.

Shunt Overdrainage: Reappraisal of the Syndrome and Proposal for an Integrative Model.

Although shunt overdrainage is a well-known complication in hydrocephalus management, the problem has been underestimated. Current literature suggests that the topic requires more examination. An insight into this condition is limited by a lack of universally agreed-upon diagnostic criteria, heterogeneity of published series, the multitude of different management options and misunderstanding of relationships among pathophysiological mechanisms involved. We carried out a review of the literature on clinical, radiological, intracranial pressure (ICP), pathophysiological and treatment concepts to finally propose an integrative model. Active prophylaxis and management are proposed according to this model based on determination of pathophysiological mechanisms and predisposing factors behind each individual case. As pathophysiology is progressively multifactorial, prevention of siphoning with gravitational valves or antisiphon devices is mandatory to avoid or minimize further complications. Shunt optimization or transferal and neuroendoscopy may be recommended when ventricular collapse and cerebrospinal fluid isolation appear. Cranial expansion may be useful in congenital or acquired craniocerebral disproportion and shunting the subarachnoid space in communicating venous hydrocephalus and idiopathic intracranial hypertension.

Functional outcome in pediatric hydrocephalus: results of applying the Spanish version of the Hydrocephalus Outcome Questionnaire.

OBJECTIVE The aims of the study were to assess whether the Spanish version of the Hydrocephalus Outcome Questionnaire (HOQ) could be useful for the evaluation and comparison of outcomes in the patients in the authors' region with those in patients in other institutions and to analyze predictors of functional outcome in patients with a shunt. METHODS The authors performed this cross-sectional study between March 2015 and April 2016. All the parents of a pediatric patient with hydrocephalus who attended the Hospital Regional Universitario de Málaga neurosurgery outpatient clinic were invited to enroll in the study and complete the HOQ-Spanish version. Age at diagnosis and at the time of the study, clinical data, shunt complications, and socioeconomic factors were also recorded. A descriptive analysis was performed, and independent variables related to the HOQ scores were studied. RESULTS A total of 132 patients participated in the study (100% participation rate). The mean ages were 16.74 months (range 0-142 months) at diagnosis and 10.45 years (range 5-16 years) at the time of the study. The mean overall HOQ score was 0.68 (on a scale from 0 [worse] to 1.0 [best]). Factors related to a worse quality of life were seizures, motor or visual impairment, shunt infection, need for shunt revision, need for more than 2 shunt revisions (range 0-8 revisions), symptomatic overdrainage, and older age at the time of the study. CONCLUSIONS The HOQ-Spanish version is useful in the authors' region; the dimension scores found in this study were comparable to those previously reported by referral centers. Future goals should be to prevent shunt complications, routinely get children to reach functional status, and refer patients for adjuvant therapy promptly.