Giant Brain Arteriovenous Malformation Managed by Staged Embolizations and Surgical Resection with Satisfactory Outcome.

Spetzler-Martin grade V (>6 cm) arteriovenous malformations (AVMs) are traditionally considered inoperable. A 35-year-old man presented with repeated seizures for 7 years, and computed tomography arteriography and magnetic resonance imaging revealed left deep hemispheric AVM. A combination of embolization and surgical resection successfully achieved a cure of the patient. Well-equipped neurosurgery facilities can best manage selective Spetzler-Martin grade V AVMs with no neurologic deficits contrary to their traditionally inoperable concept. Successful surgery offers the patient a better quality of life.

Gamma Knife Radiosurgery for Spetzler-Martin Grade III Brain Arteriovenous Malformations.

BACKGROUND: Spetzler-Martin (SM) grade III arteriovenous malformations (AVMs) show angioarchitecture heterogeneity and lack a clearly defined treatment strategy. This study aims to evaluate outcomes after treatment of SM grade III AVMs with Gamma Knife radiosurgery (GKRS). METHODS: A single-institution retrospective analysis was conducted of 307 patients with SM grade III AVMs undergoing GKRS between October 2006 and December 2020 with follow-up times of at least 24 months. SM grade III AVMs were classified into 4 subtypes: IIIA (S1E1V1), IIIB (S2E0V1), subtype IIIC (S2E1V0), and IIID (S3E0V0). RESULTS: Over a median follow-up time of 50.3 months, complete AVM obliteration was achieved in 211 patients (68.7%). Complete obliteration rates in subtypes IIIA, IIIB, IIIC, and IIID were 80.8%, 55.4%, 53.4%, and 25.0%, respectively. Annual post-GKRS hemorrhage risk was 0.8%. Significant radiosurgery-induced imaging changes occurred in 7 patients (2.3%). Three variables were identified as predictors of obliteration in final forward stepwise regression models, including volume of AVM (B = -0.011; P < 0.001), age (B = -0.004; P = 0.024), and previous AVM hemorrhage (B = 0.187; P = 0.077). CONCLUSIONS: GKRS is a safe and effective treatment for SM grade III AVMs, particularly subtype IIIA (S1E1V1). AVM volume is the key predictor of post-GKRS obliteration.

A comparison of incidental and symptomatic unruptured brain arteriovenous malformations in children.

OBJECTIVE: Patients with unruptured brain arteriovenous malformations (AVMs) may present with headaches, seizures, and/or neurological deficits. A smaller number of cases may be discovered incidentally. These lesions remain incompletely understood due to their sparse reporting. Herein, the authors describe the largest series to date comparing the presentation, angioarchitecture, and management of incidental versus symptomatic unruptured AVMs in children. METHODS: The authors performed a retrospective analysis of patients who presented with brain AVMs from 1998 to 2022 at the University of California, San Francisco. Inclusion criteria were age ≤ 18 years at the time of presentation and an angiographically proven unruptured AVM that had been diagnosed postnatally. RESULTS: Of 76 children with unruptured AVMs, 66 (86.8%) presented with headaches, seizures, and/or neurological deficit. Ten AVMs (13.1%) were incidentally discovered through unrelated disease workup (50%), cranial trauma (40%), or research study participation (10%). Compared with patients with symptomatic unruptured AVMs, patients with incidental unruptured AVMs had a smaller mean ± SD maximum nidus diameter (2.82 ± 1.1 vs 3.98 ± 1.52 cm, p = 0.025) and fewer had deep venous drainage (20% of patients vs 61%, p = 0.036). They also presented at an earlier age (10 ± 5.2 vs 13.5 ± 4 years, p = 0.043) and with longer duration to first treatment (541 ± 922 vs 196 ± 448 days, p = 0.005). During the observation period, 1 patient developed recurring headaches and demonstrated AVM nidus growth. Four AVMs greater than 3 cm in size or in a deep location were treated with radiosurgery. Six other AVMs were treated with resection, with 2 receiving preoperative embolization. Eight AVMs (80%) were obliterated on last follow-up. Postprocedural complications included 2 transient neurological deficits after resection and 1 case of delayed seizure development after radiosurgery. The mean follow-up period was 5.7 ± 5.7 years without any hemorrhage episodes. CONCLUSIONS: A substantial proportion of pediatric patients with unruptured AVMs are discovered incidentally. With earlier presentation and more elementary angioarchitecture than symptomatic unruptured AVMs, these incidental lesions provide a snapshot into the natural history of AVM before symptom development or rupture.

Longstanding overt ventriculomegaly diagnosed in adolescents, not adults: a pediatric case report.

BACKGROUND: Longstanding overt ventriculomegaly in adults (LOVA) is a new form of progressive hydrocephalus characterized by onset in early childhood and gradual progression into adulthood. Patients with LOVA are usually asymptomatic in childhood. The diagnosis of LOVA in adolescence has not been reported. CASE REPORT: A patient with macrocephaly and mild ventriculomegaly from infancy developed headache exacerbation and cognitive dysfunction at the age of 11 years. Brain magnetic resonance imaging showed mild tri-ventriculomegaly with no radiological aggravation compared to imaging at the age of 8 years. No papilledema was observed. Drainage of 15 ml of spinal fluid via a lumbar puncture relieved the headache and cognitive dysfunction. Based on repeated improvements in cognitive function and headaches after spinal fluid drainage, we diagnosed the patient with LOVA with symptom onset in early adolescence. A ventriculoperitoneal shunt was placed, and the headaches disappeared completely. The full-scale intellectual quotient, verbal comprehension, and working memory improved significantly. CONCLUSIONS: LOVA may manifest as early as adolescence. The clinical presentation, age, clinical, radiological features, and management vary, and a spinal tap exam is useful for diagnosing LOVA, even in children. The spinal tap exam may be indicated in children with longstanding ventriculomegaly and deteriorating neurological symptoms to diagnose this "treatable intellectual disability."

Outcomes of Unruptured Low-Grade Brain Arteriovenous Malformations Using TOBAS (Treatment of Brain Arteriovenous Malformations Study) Criteria.

BACKGROUND: Treatment of an unruptured brain arteriovenous malformation (bAVM) is a matter of debate, especially for low-grade bAVM (Spetzler-Martin grade I and II). The aim is to compare the outcomes of patients with low-grade unruptured bAVM after interventional or medical management in a pragmatic manner. METHODS: Adults with unruptured low-grade bAVM diagnosed between 2006 and 2016 were included. The primary end points were death from all causes and disabling stroke that resulted in a modified Rankin Scale (mRS) score >2 at last follow-up. RESULTS: Eighty-four patients presented with an unruptured Spetzler-Martin low-grade bAVM. Among these patients, 55 (65.5%) were treated and 29 (34.5%) were untreated, with no differences regarding clinical and radiologic characteristics. The modality of treatment was embolization in 25.5%, radiosurgery (alone, 30.9%; with embolization, 18.2%), and surgery (alone, 5.5%; with embolization, 20%). The rupture rate was 6.7% person-year in the untreated group; 12.7% (n = 7) of treated and 16.7% (n = 5) of untreated patients achieved the primary evaluation criteria (P = 0.744). Using a Kaplan-Meier curve, the probability of reaching this criterion at 5 years was not different between groups (P = 0.07). Complications resulting in an mRS score >2 at last follow-up occurred in 9.1%, in 80% of cases after embolization. CONCLUSIONS: This study shows no differences between treated and untreated low-grade bAVM. Embolization seems to carry a high risk of complication and should be used with caution. The small number of cases must encourage cautious interpretations especially because of the spontaneous high-rupture rate. One major interest is to investigate center habits in pathology when treatment standards are limited.

Gamma Knife Radiosurgery for Brain Arteriovenous Malformations: A 15-Year Single-Center Experience in Southern Vietnam.

BACKGROUND: In the present study, we aimed to identify the obliteration outcomes, complications, and predictors in gamma knife radiosurgery (GKRS) treatment of brain arteriovenous malformations (AVMs) at a tertiary center in a developing country in a 15-year experience. METHODS: We retrospectively reviewed the clinical data and GKRS procedures of patients who had undergone GKRS from 2006 to 2011 (cohort 1) and from 2011 to 2020 (cohort 2) at Cho Ray Hospital, Vietnam. The exclusion criteria included patients with <24 months of follow-up without obliteration or AVM-related hemorrhage during the study period. RESULTS: A total of 870 patients were included in the final analysis. The patients in cohort 1 had had significantly smaller AVMs (8.4 ± 11.6 cm3 vs. 11.2 ± 12.8 cm3; P < 0.001), and the AVMs were less frequently located in eloquent locations (46.6% vs. 65.5%; P < 0.001) than in cohort 2. The mean follow-up time was 49.6 ± 22.6 months (range, 5.9-102.6). The overall AVM obliteration rate was 66.6%. Cohort 1 had a significantly higher rate of complete obliteration compared with cohort 2 (81.0% vs. 55.1%; P < 0.001). The post-GKRS annual hemorrhage risk was 1.0%. Significant radiosurgery-induced brain edema and radiosurgery-induced cyst formation was reported in 24 (2.6%) and 4 (0.5%) patients in cohorts 1 and 2, respectively. Using multivariate analysis, we identified prior AVM hemorrhage (hazard ratio [HR], 1.430; 95% confidence interval [CI], 1.182-1.729), a higher margin dose (HR, 1.136; 95% CI, 1.086-1.188), a noneloquent location (HR, 0.765; 95% CI, 0.647-0.905), and smaller AVM volume (HR, 0.982; 95% CI, 0.968-0.997) as predictive factors for obliteration. CONCLUSIONS: GKRS is a safe and effective treatment of brain AVMs. The lack of prior AVM hemorrhage, an eloquent location, and higher AVM were unfavorable predictors for post-GKRS obliteration.

Upfront and Repeated Gamma-Knife Radiosurgery for Small (≤5 mL) Unruptured Brain Arteriovenous Malformation: A Cohort of 249 Consecutive Patients.

OBJECTIVE: According to A Randomized Trial of Unruptured Brain Arteriovenous Malformations (ARUBA), conservative treatment seems to be superior to any intervention for unruptured brain arteriovenous malformations (AVMs). This study aims to evaluate safety and efficacy of upfront and repeated Gamma-Knife Radiosurgery (GKRS) in patients harboring small AVMs fulfilling the inclusion criteria of ARUBA. METHODS: A retrospective study was conducted to evaluate outcomes of unruptured naive brain AVM with a volume ⩽ 5 mL eligible to ARUBA treated by GKRS with at least 3 years of follow-up. RESULTS: From 1992 to 2014, 249 patients fulfilled the inclusion criteria of this study. The median age was 36 years (range: 18-78 years). The median treated volume of the nidus was 1.3 mL (range: 0.4-5 mL) and 63% of the AVM were in eloquent areas. Radiosurgery-based AVM score was 1-1.8 (76%), the Spetzler-Martin grade was II-III (73%), and the Virginia Radiosurgery AVM scale was ≤1 point (75%). The overall AVM obliteration rate was 77.1% after up to 3 GKRS sessions. The median dose at the margin was 24 Gy (15-25 Gy) and the median follow-up was 45 months (range: 36-205 months). Eight patients (3.2%) experienced hemorrhage after GKRS, corresponding to a post-GKRS hemorrhage annual rate of 1.03%. Permanent symptomatic radio-induced changes rate was 2% (4 increased seizures, 1 neurologic deficit). CONCLUSIONS: The very low toxicity rate and the high occlusion rate suggest in favor of upfront and repeated GKRS for unruptured small AVMs (⩽5 mL).

Current Concepts and Perspectives on Brain Arteriovenous Malformations: A Review of Pathogenesis and Multidisciplinary Treatment.

Brain arteriovenous malformations (bAVMs) are unusual vascular pathologies characterized by the abnormal aggregation of dilated arteries and veins in the brain parenchyma and for which the absence of a normal vascular structure and capillary bed leads to direct connections between the arteries and veins. Although bAVMs have long been believed to be congenital anomalies that develop during the prenatal period, more recent studies have shown that inflammation is associated with AVM genesis, growth, and rupture. The interventional treatment options include microsurgery, stereotactic radiosurgery, and endovascular embolization, and management often comprises a multidisciplinary combination of these modalities. The appropriate selection of patients with bAVMs for interventional treatment requires balancing the risk of treatment complications against the risk of hemorrhage during the natural course of the pathology. However, no definitive guidelines have been established for the management of bAVMs. We have comprehensively reviewed the current basic and clinical studies of bAVMs and discussed the contemporary status of multidisciplinary management of bAVMs.

Improving long-term outcomes in pediatric torcular dural sinus malformations with embolization and anticoagulation: a retrospective review of The Hospital for Sick Children experience.

OBJECTIVE: Torcular dural sinus malformations (tDSMs) are rare pediatric cerebrovascular malformations characterized by giant venous lakes localized to the midline confluence of sinuses. Historical clinical outcomes of patients with these lesions were poor, though better prognoses have been reported in the more recent literature. Long-term outcomes in children with tDSMs are uncertain and require further characterization. The goal of this study was to review a cohort of tDSM patients with an emphasis on long-term outcomes and to describe the treatment strategy. METHODS: This study is a single-center retrospective review of a prospectively maintained data bank including patients referred to and cared for at The Hospital for Sick Children for tDSM from January 1996 to March 2019. Each patient's clinical, radiological, and demographic information, as well as their mother's demographic information, was collected for review. RESULTS: Ten patients with tDSM, with a mean follow-up of 58 months, were included in the study. Diagnoses were made antenatally in 8 patients, and among those cases, 4 families opted for either elective termination (n = 1) or no further care following delivery (n = 3). Of the 6 patients treated, 5 had a favorable long-term neurological outcome, and follow-up imaging demonstrated a decrease or stability in the size of the tDSM over time. Staged embolization was performed in 3 patients, and anticoagulation was utilized in 5 treated patients. CONCLUSIONS: The authors add to a growing body of literature indicating that clinical outcomes in tDSM may not be as poor as initially perceived. Greater awareness of the lesion's natural history and pathophysiology, advancing endovascular techniques, and individualized anticoagulation regimens may lead to continued improvement in outcomes.

Improvement in ventriculomegaly following cervicomedullary decompressive surgery in children with achondroplasia and foramen magnum stenosis.

The role of cervicomedullary decompression (CMD) in the care of hydrocephalic achondroplastic children who present with simultaneous foramen magnum stenosis is not well understood. We sought to determine the percentage of symptomatic achondroplastic children with foramen magnum stenosis who had stabilization or improvement in ventriculomegaly following CMD. The authors retrospectively reviewed the records of pediatric patients at Cedars-Sinai Medical Center with achondroplasia and signs of progressive ventriculomegaly who underwent CMD for symptomatic foramen magnum stenosis between the years 2000 and 2018. Clinical outcomes included changes in fontanelle characteristics, head circumference (HC) percentile, and incidence of ventriculoperitoneal (VP) shunting. Radiographic outcomes measured included changes in Evans ratio. We excluded individuals who were shunted before CMD from our study. Sixteen children presented with symptomatic foramen magnum stenosis and full anterior fontanelle or jump in the HC percentiles. Two children underwent placement of a VP shunt before decompressive surgery and were excluded from further analysis. Of the remaining 14 children who underwent CMD, 13 (93%) showed softening or flattening of their fontanelles post-operatively. Ten of these 14 children had both pre- and post-operative HC percentile records available, with 8 showing increasing HC percentiles before surgery. Seven of those eight children (88%) showed a deceleration or stabilization of HC growth velocity following decompression of the foramen magnum. Among 10 children with available pre- and post-operative brain imaging, ventricular size improved in 5 (50%), stabilized in 2 (20%), and slightly increased in 3 (30%) children after decompression. Two children (14%) required a shunt despite decompression of the foramen magnum. A significant proportion of children with concomitant signs of raised intracranial pressure or findings of progressive ventriculomegaly and foramen magnum stenosis may have improvement or stabilization of these findings following CMD. CMD may decrease the need for VP shunting and its associated complications in the select group of hydrocephalic children with achondroplasia presenting with symptomatic foramen magnum stenosis.

Spontaneous, Intrasphenoidal Rupture of Ecchordosis Physaliphora with Pneumocephalus Captured During Serial Imaging and Clinical Follow-Up: Pathoanatomic Features and Management.

BACKGROUND: Ecchordosis physaliphora (EP) is a congenital, uniformly asymptomatic, hamartomatous lesion of the primitive notochord. Herein we report, to our knowledge, the first credible case report of unprovoked intrasphenoidal rupture resulting in recurrent pneumocephalus and cerebrospinal fluid leak, definitively captured over serial imaging during clinical and radiologic surveillance. CASE DESCRIPTION: A 68-year old woman with Marfan syndrome presented to the emergency department with the worst headache of her life. Imaging demonstrated extensive pneumocephalus and revealed a small, dorsal midline clival lesion consistent with EP and a transsphenoidal defect. Remote imaging encounters confirmed typical EP without pneumocephalus or cortical defect, and an uneventful clinical course years preceding presentation. Over the ensuing months during neurosurgical follow-up, the patient reported recurrent headaches, imbalance, and unprovoked clear rhinorrhea. Further imaging demonstrated an apparently enlarging transsphenoidal defect which was managed by endoscopic transnasal resection and nasoseptal flap. Pathologic evaluation confirmed the diagnosis of EP and chronic dural defect. CONCLUSIONS: This represents, to our knowledge, the first unambiguous example of spontaneous EP rupture and recurrent pneumocephalus captured over serial imaging. The case further underscores rare but potentially significant complications of EP and highlights management options.

The Role of MRI in Children With Congenital Limb Deficiencies With Associated Scoliosis.

BACKGROUND: The association of scoliosis and congenital limb deficiency has been well described. However, the incidence of neural axis abnormalities in this population is not known. The ability to assess the neural axis by physical examination may be limited in patients with a limb deficiency. Although mobility of the spine is important for all children, it can be especially so in children with a limb deficiency. As spinal fusion in children with limb deficiency potentially has more functional impact, detecting reversible forms of scoliosis seems particularly important. METHODS: Retrospective review of children treated at 1 institution between 1990 and 2017 with both a diagnosis of a congenital limb deficiency, upper or lower, and scoliosis. Children were excluded if they had any neurological difference on history or physical examination, if they had sacral agenesis or spina bifida, or if their limb deficiency was related to trauma or early amniotic rupture sequence. RESULTS: Twenty-four children were identified, 11 with lower extremity deficiency, 14 with upper extremity deficiency with 1 having both. Fifteen children demonstrated neural axis abnormalities, 6 (40%) required neurosurgery. Five (45%) of 11 lower extremity deficiency children had MRI findings, 3 of these needing neurosurgery. Of the 14 upper extremity deficiency children, 10 had MRI changes, and 3 required neurosurgery. Eight children with congenital scoliosis, 5 had MRI findings, with 4 children requiring neurosurgery. The other 16 children had scoliosis without vertebral abnormalities, 10 had MRI findings, and 2 required neurosurgery. CONCLUSIONS: There is a high incidence of neural axis abnormalities (63%) in children with congenital limb deficiencies and scoliosis. A large portion of these require neurosurgical intervention. MRI should be considered soon after presentation in this population of children. LEVEL OF EVIDENCE: Level IV. DESIGN: Retrospective cohort.

Developmental Retrocerebellar Cysts: A New Classification for Neuroendoscopic Management and Systematic Review.

OBJECTIVE: Posterior fossa cystic malformations are diversely classified with considerable overlap without therapeutic relevance. These cysts posterior to the cerebellum, presenting in children younger than 5 years, are labeled developmental retrocerebellar cysts (DRCCs) under a new classification in relation to neuroendoscopy. METHODS: DRCC was categorized as type 0 for asymptomatic enlarged cistern magna and was not treated. Among symptomatic cases, cysts with a compressed fourth ventricle were labeled type 1, whereas cysts in continuity with the fourth ventricle were termed type 2. They were further categorized as subtype B if hydrocephalus was greater relative to the cyst, or otherwise as subtype A. The literature was reviewed according to PRISMA guidelines. RESULTS: There were 13 children aged 3-48 months. Type 1A DRCC was noted in 5 patients, with onset before 6 months, 4 of whom (80%) had intracranial hypertension. All underwent suboccipital endoscopic deroofing and cisternostomy (SEDC), a new technique. Type 1B DRCC was seen in 2 patients, with onset at 8-9 months, who underwent endoscopic third ventriculostomy (ETV) + endoscopic ventriculocystostomy (EVC). Type 2A DRCC was observed in 4 patients, with onset at 5-47 months, who underwent SEDC. Type 2B DRCC was noted in 2 patients, with onset 6-8 months, who underwent ETV. With a mean follow-up of 32 months, all showed clinicoradiologic improvement. The application of our classification to other studies showed ETV/EVC to be successful in only 67% of type 1A DRCC and 72% of type 2A DRCC, compared with 100% efficacy of SEDC in our series. CONCLUSIONS: This is probably the first ever endoscopic classification of pediatric posterior fossa cyts, elucidating pathophysiology, presentation, and treatment. Patients with type 1 DRCC present early because of extraneous compression. Among patients with type 2 DRCC, posterior fossa compliance determines the degree of hydrocephalus. The newly described SEDC seems more appropriate for types 1A and 2A DRCC. ETV is adequate in type 2B DRCC and effective with EVC in type 1B.

Deciding on Appropriate Telemetric Intracranial Pressure Monitoring System.

BACKGROUND: The clinical advantage of telemetric intracranial pressure (ICP) monitoring has previously been limited by issues with inaccuracy and zero-drift. Today, 2 comparable telemetric ICP monitoring systems are available performing adequately in these parameters. The objective of this study is to identify appropriate uses of each system. METHODS: The 2 telemetric ICP monitoring systems from Raumedic (implant: Neurovent-P-tel) and Miethke (implant: Sensor Reservoir) are compared in terms of fundamental differences, sensor survival, monitoring possibilities, complications, and cost/benefit. Two illustrative cases are presented highlighting clinical advantages and disadvantages of each system. RESULTS: Both systems provide transdermal (telemetric) ICP measurements through external application of a reader unit cabled to a portable data sampler. Thereby, they allow several ICP monitoring sessions without multiple surgical insertions of a cabled ICP sensor. The Miethke implant has a high sampling frequency (40 Hz) and a long CE (Conformité Européenne) approval (3 years) but cannot be used for long-duration monitoring sessions. In comparison, the Raumedic implant has a lower sampling frequency (5 Hz) and shorter CE approval (90 days) but can be used for long-duration monitoring sessions. The standard 3-year cost for a patient with a Neurovent-P-tel is 17,380 €, and for the Sensor Reservoir it is 15,790 €. CONCLUSIONS: The Miethke system is useful in outpatient clinics where patients have sequential point measurements of ICP performed, whereas the Raumedic system is ideal for long-duration ICP monitoring outside the hospital. When choosing between the 2 systems, it must primarily be decided if the clinical situation requires long-duration monitoring sessions or continuous repeated ambulatory follow-up sessions.

Surgical Management of Brainstem Cavernous Malformation: Report of 67 Patients.

BACKGROUND: Brainstem cavernous malformations (CMs) are benign lesions, often show an acute onset, and result in a high rate of morbidity. Surgical resection could inhibit the progressive deterioration of neurologic function caused by repetitive hemorrhage. This study aimed to summarize timing, approaches, and techniques of surgery and to evaluate outcomes of treatment. METHODS: Between March 2011 and May 2013, 67 patients (32 male, 35 female; average age 40 years; range, 14-68 years) with brainstem CMs received surgical treatment. Clinical presentation, surgical approaches, and results of follow-up were retrospectively analyzed. RESULTS: Seven surgical approaches were used: orbitozygomatic approach (1 case), suboccipital transtentorial approach (Poppen approach; 3 cases), subtemporal transtentorial approach (32 cases), subtemporal transtentorial/anterior petrosectomy approach (9 cases), suboccipital retrosigmoid approach (3 cases), midline suboccipital approach (16 cases), and far lateral approach (3 cases). Total resection of the brainstem CM was achieved in all cases (100%). No operative mortality was encountered. Nine patients had new symptoms after surgery: 3 had diplopia, 3 had facial numbness, 1 had numbness of contralateral limbs, 1 had transient aphasia, and 1 had reduced muscle strength of contralateral limbs. Symptoms significantly improved in 23 patients (34.3%), symptoms were unchanged in 36 patients (53.7%), and new postoperative symptoms occurred in 9 patients (13.4%). CONCLUSIONS: Choosing a proper surgical approach and using appropriate techniques are fundamental for favorable outcomes of patients with brainstem CMs.

The Ebb and Flow of Neurosurgery in a Re-Emerging Sub-Saharan Center. Lessons from 1025 Consecutive Operated Cases and Progress Models. A Prospective Observational Cohort Study.

BACKGROUND: The evolution of neurosurgery in our center comprises 2 active phases separated by a decade-long latent phase. This study evaluates our experience in the renaissance phase using 1025 cases. We identify paradigms that predict progress under existing practice conditions. METHODS: A prospective observational study was undertaken of all freshly operated patients from 2009 to 2016. Ethical approval was obtained from the hospital institutional review board. Data of enrolled patients were acquired and analyzed using SPSS software for Windows version 21. Inferences were evaluated using a 95% level of significance. Major and intermediate cases were included, whereas bedside cases as well as patients operated on elsewhere were excluded. RESULTS: Of 5132 patients treated, 3033 had surgical lesions, 1129 (33.2 ± 2.7% confidence limit [CL]) were operated on, and 1025 satisfied the study criteria. The unmet surgical load (66.1%) was significant (χ2 = 10.9; degree of freedom = 1; P = 0.001). The most common diseases treated were neurotrauma in 438 patients, congenital malformations in 268, and tumors in 170. The mean annual operative rate was 125 ± 5.7 patients (95% CL). Cases showed significant variability using 1-way analysis of variance (F = 13.77; P = 0.03). Residency training was a positive influence on surgeons' operative rate (χ2 = 7.59; P = 0.02). The mean complication rate was 10.3% ± 2.9%, and the mortality was 5.5% ± 1.7%. CONCLUSIONS: We found a large unmet neurosurgical load. To resolve this situation, we recommend an increase in the number of training programs for neurosurgery residency. As neurosurgery units evolve, their progress may be evaluated using the NESCAPE (Neurosurgery Care Phase Evolution) paradigm.

Severe gyration and migration disorder in fetofetal transfusion syndrome: two case reports and a review of the literature on the neurological outcome of children with lesions on neuroimaging.

INTRODUCTION: Fetofetal transfusion syndrome is a dreaded cause of morbidity and mortality in monochorionic pregnancies. CASE REPORTS: We present two pairs of twins one of which we have followed for more than 6 years. The donors suffer from cerebral palsy, orofacial, and motor problems, and both are significantly smaller than their recipient twins. Interestingly, cranial MRI revealed medial frontal lobe polymicrogyria, ventriculomegaly, and decreased thickness in both parietal lobes in both donors. We suggest this as a possible feature of fetofetal transfusion syndrome. REVIEW: A minireview of the literature on neuroimaging and neurodevelopmental outcome in fetofetal transfusion syndrome is presented. CONCLUSION: While the close resemblance of the imaging features of both cases is likely incidental further study of a connection between migration and gyration disorders and fetofetal transfusion syndrome is warranted.

Malformación de Chiari Tipo I en adultos. Un enfoque médico-quirúrgico. Parte I / Type I Chiari malformation in adults. A medico-surgical approach. Part I

Se revisó la literatura existente sobre la Malformación de Chiari Tipo I en el adulto, haciendo énfasis en su diagnóstico imagenológico y tratamiento médico-quirúrgico. La malformación de Chiari es una enfermedad poco frecuente. Representa entre el 1 y el 4% de todas las patologías neuroquirúrgicas. El diagnóstico se realiza meses y hasta años después de comenzada la sintomatología y se confirma en el 100% de los casos con Resonancia magnética de cráneo. El tratamiento es quirúrgico en los pacientes sintomáticos, siendo controversial en aquellos oligosintomáticos o con diagnóstico casual. La cirugía siempre debe realizarse con el apoyo de monitoreo neurofisiológico, que puede ser determinante en la técnica quirúrgica a emplear. La mortalidad asociada a la cirugía es muy baja, oscilando entre el 0 y 0,5% según la mayoría de las series. Lo más importante es la selec­ción de los pacientes para el tratamiento quirúrgico. No debe asumirse una actitud expectante en espera de un deterioro neurológico que justifique la cirugía, cuando ésta se hace a tiempo los resultados son mejores y con un mínimo de complicaciones (AU).

The aim of this article is reviewing the existent literature on Type I Chiari malformation in adults and making emphasis in its imaging diagnosis and medico-surgical treatment. Chiari malformation is a few frequent diseases. It represents 1-4 % of all the neurosurgical diseases. The diagnosis is made months and even years after the beginning of the symptoms and it is confirmed by cranial magnetic resonance in the 100 % of the cases. The treatment is surgical in symptomatic patients, and it is controversial in the oligosymptomatic ones and in those with casual diagnosis. The surgery should be performed with the support of neurophysiological monitoring that might be determinant in the surgical technique to use. The mortality associated to the surgery is low, ranging from 0 and 0.5 % according to most of the series. The most important thing is the choice of the patients for the surgical treatment. The neurological deterioration should not be expected to justify the surgery; when the surgery is carried out on time the results are better and with the minimum of complications (AU).