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.

Ectopic recurrence of craniopharyngioma in the posterior fossa: Case report and review of the literature / Recurrencia ectópica de craneofaringioma en fosa posterior: presentación de casos y revisión de la literatura

Craniopharyngiomas are benign epithelial tumors which may very occasionally recur in ectopic locations. We present two cases of ectopic recurrence, both in the posterior fossa, and provide a review of the literature with basic statistics. Two patients admitted to our institution with posterior fossa lesions underwent gross total resection. Pathological studies showed adamantinomatous craniopharyngiomas (ACP). Both patients had a prior history of suprasellar tumor surgery. We also reviewed the related data and undertook a basic statistical analysis. We found 67 cases of ectopic recurrent craniopharyngioma (including the present cases): 51 cases were adamantinomatous (76%), 6 papillary (9%) and 10 unknown (15%). 18 cases occurred in the posterior fossa, all of them diagnosed as the ACP subtype. The intervals until recurrence were 15.15 years for posterior fossa recurrences and 5.75 years for supratentorial cases. Student t test showed significant differences in time to recurrence (p 0.002). Gross total resection was performed in 53 cases (79%), subtotal resection + radiotherapy in 3 (5%) and 11 (16%) cases were treated with other options. Ectopic recurrence is a rare but possible event. Those in the posterior fossa may appear later than in the supratentorial space. ACP is likely to be the most common subtype in these cases, possibly due to its more aggressive behavior compared to the papillary subtype. Long term follow-up should be performed to detect ectopic recurrences. Ectopic recurrences are often surgically accessible and gross total resection should be achieved. (AU)

Los craneofaringiomas son tumores epiteliales benignos que pueden recurrir ocasionalmente en localizaciones ectópicas. Presentamos 2 casos de recurrencias ectópicas, ambos en fosa posterior, y realizamos una revisión de la literatura con análisis estadístico básico. Dos pacientes ingresaron en nuestro servicio con lesiones en fosa posterior en quienes se indicó resección quirúrgica. El diagnóstico histopatológico fue de craneofaringioma adamantinomatoso en ambos casos. Ambos pacientes fueron intervenidos años atrás por un tumor supraselar. Se hallaron 67 casos descritos de recurrencia ectópica de craneofaringioma (incluyendo el presente estudio): 51 fueron adamantinomatoso (76%), 6 papilar (9%) y 10 desconocidos (15%). Dieciocho casos ocurrieron en la fosa posterior, todos con diagnóstico de craneofaringioma adamantinomatoso. El intervalo hasta la recurrencia fue de 15,15 años para aquellas en la fosa posterior y de 5,75 años para recurrencias supratentoriales. El test de Student mostró diferencias estadísticamente significativas en el tiempo de recurrencia (p 0,002). En 53 casos se obtuvo una resección completa (79%), resección subtotal + radioterapia en 3 (5%) y 11 casos se usaron otras modalidades de tratamiento (16%). La recurrencia ectópica es un evento raro, pero posible. Aquellas en la fosa posterior pueden aparecer más tarde que a nivel supratentorial. Los craneofaringiomas adamantinomatosos son los subtipos más probables en esos casos, posiblemente debido a su comportamiento más agresivo comparado con los papilares. Un seguimiento a largo plazo debe llevarse a cabo para detectar dichas recurrencias ectópicas, ya que estas suelen ser quirúrgicamente accesibles, y debe obtenerse una resección completa. (AU)

Ectopic recurrence of craniopharyngioma in the posterior fossa: Case report and review of the literature.

Craniopharyngiomas are benign epithelial tumors which may very occasionally recur in ectopic locations. We present two cases of ectopic recurrence, both in the posterior fossa, and provide a review of the literature with basic statistics. Two patients admitted to our institution with posterior fossa lesions underwent gross total resection. Pathological studies showed adamantinomatous craniopharyngiomas (ACP). Both patients had a prior history of suprasellar tumor surgery. We also reviewed the related data and undertook a basic statistical analysis. We found 67 cases of ectopic recurrent craniopharyngioma (including the present cases): 51 cases were adamantinomatous (76%), 6 papillary (9%) and 10 unknown (15%). 18 cases occurred in the posterior fossa, all of them diagnosed as the ACP subtype. The intervals until recurrence were 15.15 years for posterior fossa recurrences and 5.75 years for supratentorial cases. Student t test showed significant differences in time to recurrence (p 0.002). Gross total resection was performed in 53 cases (79%), subtotal resection+radiotherapy in 3 (5%) and 11 (16%) cases were treated with other options. Ectopic recurrence is a rare but possible event. Those in the posterior fossa may appear later than in the supratentorial space. ACP is likely to be the most common subtype in these cases, possibly due to its more aggressive behavior compared to the papillary subtype. Long term follow-up should be performed to detect ectopic recurrences. Ectopic recurrences are often surgically accessible and gross total resection should be achieved.

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.

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.

Factors related to shunt survival in paediatric hydrocephalus. Could failure be avoided? / Factores relacionados con la supervivencia del shunt en hidrocefalia pediátrica. ¿Es posible prevenir el fallo valvular?

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

Antecedentes: Mejorar la supervivencia del shunt y conocer los factores relacionados con el fallo valvular continúa siendo un reto en neurocirugía pediátrica, con el propósito de evitar la morbimortalidad y el impacto económico de las revisiones valvulares repetidas. Por este motivo, se estudiaron los factores relacionados con el primer fallo valvular en una serie propia con un seguimiento prolongado. Métodos: Estudio retrospectivo de 166 pacientes pediátricos con hidrocefalia derivada por primera vez entre los años 2000-2014. Se analizó la asociación estadística entre el primer fallo valvular y las variables demográficas, etiológicas, quirúrgicas y clínicas. Resultados: De 166 válvulas implantadas durante el periodo de estudio, 111 requirieron revisión en un seguimiento medio de 93 meses. Los factores relacionados de forma significativa con el fallo valvular fueron las etiologías posthemorrágica y postinfecciosa; una meningitis previa; trépano posterior, válvula de presión diferencial o catéter estándar en la primera intervención, y sobredrenaje sintomático. El análisis multivariante mostró que la meningitis previa y el sobredrenaje sintomático fueron factores de riesgo para la disfunción valvular, mientras que el trépano frontal y los catéteres con impregnación antibiótica fueron factores protectores. La regresión de Cox determinó que la infección del shunt y el sobredrenaje sintomático fueron los factores relacionados de forma independiente con una menor supervivencia valvular. Conclusiones: Meningitis, sobredrenaje sintomático, trépano frontal y catéteres de impregnación antibiótica son factores pronósticos significativos en la supervivencia valvular. La infección del shunt y el sobredrenaje sintomático son factores independientes relacionados de forma significativa con una menor supervivencia valvular. Estudios controlados, prospectivos y aleatorizados son necesarios para validar estos resultados

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.