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Monobloc and facial bipartition combined with distraction osteogenesis (MFBDO) has gained popularity over the past several years as a treatment of syndromic craniosynostosis, in part because this surgical technique effectively removes many stigmatic clinical features associated with the syndromic face. The objective of this study is to detail the surgical planning used to achieve medialization of the orbits and describe the authors' experience using MFBDO to destigmatize the syndromic face. By using MFBDO, hypertelorism, vertical orbital dystopia, and downslanting of the palpebral fissure were surgically corrected in all patients, thereby destigmatizing the syndromic face.

Objective We aimed to better characterize the magnetic resonance imaging (MRI) findings of mild malformation of cortical development with oligodendroglial hyperplasia (MOGHE), a rare clinicopathological entity associated with pharmacoresistance recently described in patients with frontal lobe epilepsy. Methods We studied 12 patients who underwent epilepsy surgery and whose surgical specimens showed histopathological findings of MOGHE, characterized by preserved cortical lamination, blurred gray-white matter interface due to increased number of oligodendrocytes, and heterotopic neurons in the white matter. The age at MRI evaluation ranged from 11 to 58 years, except for one 4.5-year-old patient. Results Following a detailed MRI analysis using an in-house protocol, we found abnormalities in all cases. The lesion was circumscribed in the frontal lobe in six (50%) and in the temporal lobe in three (25%) patients. In the remaining three patients (25%), the lesion was multilobar (frontotemporal and temporoparieto-occipital). Cortical thickening was mild in all patients, except in the 4.5-year-old patient, who had pronounced cortical thickening and white matter blurring. We also identified cortical/subcortical hyperintense T2/fluid-attenuated inversion recovery signal associated with gray/white matter blurring in all but one patient. When present, cleft cortical dimple, and deep sulci aided in localizing the lesion. Overall, the MRI findings were like those in focal cortical dysplasia (FCD) Type IIa. Surgical outcome was excellent in five patients (Engel Class I in 25% and II in 17%). The remaining seven patients (58%) had worthwhile seizure reduction (Engle Class III). Incomplete lesion resection was significantly associated with worse outcomes. Significance MRI findings associated with MOGHE are similar to those described in FCD Type IIa. Although more frequent in the frontal lobe, MOGHE also occurred in the temporal lobe or involved multiple lobes. Multilobar or extensive MOGHE MRI lesions are associated with less favorable surgical outcomes. Because this is a rare condition, multicenter studies are necessary to characterize MOGHE further.

The most commonly occurring syndromic craniosynostoses are Apert syndrome, Crouzon syndrome, Pfeiffer syndrome, and Saethre-Chotzen syndrome. There is insufficient data regarding postoperative syndrome–related outcomes following the posterior vault distraction osteogenesis (PVDO) procedure, as well as data addressing whether or not additional procedures will be subsequently necessary to comprehensively treat children who undergo PVDO. Thus, the objective of this study is to describe and compare syndrome-related potential complications and outcomes associated with the PVDO procedure. An observational retrospective study was performed on consecutive patients (n=24) with Apert syndrome, Crouzon syndrome, Pfeiffer syndrome, or Saethre-Chotzen syndrome, respectively, who underwent PVDO between 2012 and 2019. Demographic data (patient gender and age when the PVDO procedure was performed), diagnosis, surgery-related data, and outcome data (perioperative and midterm complications and need for additional surgery) were verified. Total relative blood transfusion volumes per kilogram for the patients were as follows: 22.75 ± 9.30 ml for Apert syndrome, 10.73 ± 2.28 ml for Crouzon syndrome (Apert versus Crouzon, p

Craniofrontonasal dysplasia (CFND) is a rare congenital craniofacial syndrome characterized by single suture synostosis, hypertelorism, other clinical facial features, and abnormalities in the upper extremities. There are only a few studies in the applicable literature that address hypertelorism management for CFND patients and outcomes and complication rates. A retrospective study was performed on consecutive late presenting CFND patients referred to our hospital with substantially completed craniofacial skeleton growth, who underwent hypertelorism correction between 2007 and 2019 following intracranial pressure screening, and who received at least 1 year of follow-up care. None of the patients in this study underwent prior craniofacial surgery. Only those patients with a confirmed mutation of the EFNB1 gene were included in this study. All patients in this study underwent hypertelorism correction by facial bipartition or box osteotomy. A total of ten late presenting CFND patients (all female) were treated at our hospital during the study period. None of the patients presented signs of elevated intracranial pressure. The average patient age at hypertelorism correction was 13.4 ± 7.68 years of age. Major complications, defined as complications requiring a return to the operating room, were limited to infection of the frontal bone, which required partial bone removal, and cerebrospinal fluid (CSF) leak, which was completely resolved by insertion of a lumbar shunt for a 7-day period. The absence of elevated intracranial pressure enables hypertelorism correction in late presenting CFND patients via facial bipartition or box osteotomy without the need for additional operations that provide for cranial expansion.

OBJECTIVE The objective of this study was to evaluate the efficacy and safety of a modified surgical approach for the treatment of temporal lobe epilepsy secondary to hippocampal sclerosis (HS). This modified approach, called temporopolar amygdalohippocampectomy (TP-AH), includes a transsylvian resection of the temporal pole and subsequent amygdalohippocampectomy utilizing the limen insula as an anatomical landmark. METHODS A total of 61 patients who were diagnosed with HS and underwent TP-AH between 2013 and 2017 were enrolled. Patients performed pre- and postoperative diffusion tensor imaging and were classified according to Engel’s scale for seizure control. To evaluate the functional preservation of the temporal stem white-matter fiber tracts, the authors analyzed postoperative Humphrey perimetries and pre- and postoperative neurocognitive performance (Rey Auditory Verbal Learning Test [RAVLT], Weschler Memory Scale–Revised [WMS-R], intelligence quotient [IQ], Boston Naming Test [BNT], and semantic and phonemic fluency). Demographic data and surgical complications were also recorded and described. RESULTS After a median follow-up of 36 ± 16 months, 46 patients (75.4%) achieved Engel class I, of whom 37 (60.6%) were Engel class IA. No significant changes in either the inferior frontooccipital fasciculus and optic radiation tractography were observed postoperatively for both left- and right-side surgeries. Reliable perimetry was obtained in 40 patients (65.6%), of whom 27 (67.5%) did not present any visual field defects (VFDs) attributable to surgery, while 12 patients (30%) presented with quadrant VFD, and 1 patient (2.5%) presented with hemifield VFD. Despite a significant decline in verbal memory (p = 0.007 for WMS-R, p = 0.02 for RAVLT recognition), there were significant improvements in both IQ (p < 0.001) and visual memory (p = 0.007). Semantic and phonemic fluency, and scores on the BNT, did not change postoperatively. CONCLUSIONS TP-AH provided seizure control similar to historical temporal lobe approaches, with a tendency to preserve the temporal stem and a satisfactory incidence of VFD. Despite a significant decline in verbal memory, there were significant improvements in both IQ and visual memory, along with preservation of executive function. This approach can be considered a natural evolution of the selective transsylvian approach.

We present here the unique case of a patient with a Tessier 1-13 rare facial cleft accompanied by cleft lip and palate and Tessier grade 2 hypertelorism. The patient described in this article has a twin brother who shares a number of genetic traits and physical features but does not present facial cleft or hypertelorism. The 45-year follow-up in this case is believed to be the longest follow-up to date to have been reported in the literature, and sheds significant light on the importance of extended longitudinal follow-up to maximize patient outcomes.

Monobloc advancement is a complex procedure used to treat patients with syndromic craniosynostosis. Studies directly addressing the long-term stability of monobloc advancement with distraction osteogenesis are underreported in the literature. The objectives of this study were to assess 5-year midface bone stability following monobloc advancement performed on patients with syndromic craniosynostosis, identify risk factors for relapse, and present strategies for prevention and management of complications.An observational retrospective study was performed on consecutive patients with Apert, Crouzon, or Pfeiffer syndrome (n = 23) who underwent monobloc advancement using distraction osteogenesis between 1994 and 2013. A total of 130 lateral cephalograms were used to assess both long-term stability after monobloc advancement with distraction osteogenesis and risk factors for relapse. All serious complications were identified and recorded. The analysis of variance test was used to assess horizontal relapse of the midface and mandibular plane.Cephalometric analysis revealed long-term stability of frontofacial advancement using distraction osteogenesis, regardless of the presence of tested variables. Serious complications arising from monobloc advancement using distraction osteogenesis included cerebrospinal fluid leakage in six patients (26 percent), accompanying meningitis in two patients (8.7 percent), seizures in seven patients (30.4 percent), and impaired visual acuity in one eye of one patient (4.3 percent).Frontofacial monobloc advancement with distraction osteogenesis provides long-term midface bone stability.Therapeutic, IV.

Background: Despite the various treatment protocols available, survival evaluation is a fundamental criterion for the definition of surgical management; there are still many inconsistencies in the literature on this topic, especially in terms of the value of surgery and its morbidity in patients with very short survival. Objective: The objective was to analyze the association of clinical, oncological, and surgical factors in the survival of patients undergoing spinal surgery for spinal metastases (SM). Materials and Methods: A retrospective cohort of forty patients who were surgically treated at our institution for SM between 2010 and 2018 were included in the study. We applied the prognostic scales of Tomita and Tokuhashi in each patient and evaluated the systemic status using Karnofsky Performance Scale (KPS) and Eastern Cooperative Oncology Group Performance Scale. Survival rate in months was estimated using the Kaplan–Meier curve, with death considered as primary outcome and, for the evaluation of the association between the variables, the Chi-square test, Fisher's exact test, or Fisher–Freeman–Halton test was applied for better survival. The level of statistical significance was considered as 5% (P ≤≤ 0.05). Results: The mean survival was 8.4 months. Patients with KPS 70 had a mean survival of 14.48 months (P = 0.04). The mean survival of patients classified as ECOG 2 was 7.05 months (95% confidence interval [CI]: 3.4–10.7), and that of patients classified as ECOG 3 and 4 was 1.24 months (95% CI: 0.8–1.59). The mean survival rate among the patients with unresectable metastases in other organs was 6.3 months (95% CI: 3.9–8.9), while the survival rate of those who did not have metastases was 13.8 months (95% CI: 10.0–17.68; P = 0.022). Conclusion: Survival was associated with the preoperative functional status defined by the KPS and ECOG scales and with the presence of nonresectable visceral metastases.

BACKGROUND Presenting a wide clinical spectrum and large variety of clinical features, successful treatment of Apert syndrome necessitates performance of sequential multiple surgeries before a patient's facial skeleton growth is complete.The objective of this study is to compare forehead contour asymmetry and clinical outcomes between Apert patients who underwent either fronto-orbital advancement (FOA) or posterior vault distraction osteogenesis (PVDO). METHODS A retrospective study was performed on consecutive patients with Apert syndrome who underwent either FOA or PVDO between 2007 and 2019, and participated in at least 6 months of follow-up care. Forehead contour asymmetry and surgical outcomes for each of the included patients were verified through medical records, clinical photographs, and interviews with the parents of the patients. The need for additional craniofacial procedures based on the surgical outcomes of each patient was graded from I to IV utilizing the Whitaker outcome classification system. RESULTS Forehead contour asymmetry for all included patients was rated under the Whitaker grading scale as type II (n = 4) 44.4%, type III (n = 2) 22.2%, and type IV (n = 3) 33.3% for FOA, and type I (n = 5) 35.7%, type II (n = 7) 50%, and type III (n = 2) 14.3%, for PVDO (P

There are paucity of studies focused on the long-term assessment of the craniofacial changes after a pi-plasty procedure and self-reported quality of life outcomes. Thus, this study aimed to assess long-term morphologic changes and quality of life of patients with sagittal synostosis who underwent surgery with a modified pi-plasty.Consecutive patients with sagittal synostosis who underwent surgery and had more than 5 years of follow-up and standard preoperative and 1, 3, and 5 years right profile view photographs were included. Nasofrontal angle and angle of total facial convexity were evaluated using computerized photogrammetric measurements. Additionally, quality of life outcome was evaluated by the Quality of Life Scale Short Form.The total facial convexity angle and nasofrontal angle increased significantly (P 0.05), with a P-value of 0.013 and 0.012, respectively. Patients had quality of life scores80 in all of the 4 domains, with the highest scores being: physical health domain 80.0 ± 0, psychologic domain 85.0 ± 5, social relationships domain 86.6 ± 0, and environmental domain 98.13 ± 2.42.Facial angles significantly changed over 5 years of follow-up. Self-reported quality of life instrument showed that patients are satisfied with their own appearance, contributing positively to their quality of life.

This study assessed whether optic radiations (OR) microstructure after temporal lobe epilepsy (TLE) surgery correlated with visual field defects (VFD). Patients were subjected to diffusion tensor imaging (DTI) tractography of the OR and Humphrey perimetry after TLE surgery. We used Spearman’s test to verify correlations between tractographic parameters and perimetry mean deviation. Tractographic variables were compared between patients with VFD or intact perimetry. Multiple logistic regression was applied between DTI and perimetry values. DTI sensitivity and specificity were assessed with a receiver operating characteristic (ROC) curve to evaluate VFD. Thirty-nine patients had reliable perimetry and OR tractography. There was a significant correlation between (1) fractional anisotropy (FA) and both total (rho = 0.569, p = 0.0002) and quadrant (rho = 0.453, p = 0.0037) mean deviation and (2) radial diffusivity and total mean deviation (rho = − 0.350, p = 0.0286). There was no other significant correlation. Patients with VFD showed a significantly lower FA compared with patients with normal perimetry (p = 0.0055), and a 0.01 reduction in FA was associated with a 44% increase in presenting VFD after surgery (confidence interval, CI = 1.10–1.88; p = 0.0082). Using a FA of 0.457, DTI tractography showed a specificity of 95.2% and a sensitivity of 50% to detect VFD after surgery (area under the curve = 0.7619, CI = 0.6020–0.9218). The postoperative OR microstructure correlated with visual loss after epilepsy surgery. DTI postoperative OR tractography may be helpful in evaluating VFD.

Simultaneously advancing and medializing the orbital segments in a stable bone bloc constitutes a major advancement in craniofacial surgery. Monobloc bipartition enables destigmatization of the syndromic face by correcting the abnormal orbital axis and interorbital distance. The authors stratified this complex surgical approach into five major steps to facilitate a holistic understanding of the surgical sequence. The rationale for latency and activation periods and the advantages and disadvantages of this technique are described.

Epilepsy is a common disease presenting with recurrent seizures. Hippocampal sclerosis (HS) is the commonest histopathological alteration in patients with temporal lobe epilepsy (TLE) undergoing surgery. HS physiopathogenesis is debatable. We have recently studied, by using mass spectrometry-based proteomics, an experimental model of TLE induced by electrical stimulation. Specifically, protein expressions of both the beta subunit of mitochondrial ATP synthase (ATP5B) and of membrane ATPases were found to be reduced. Here, we investigated tissue distribution of ATP5B and sodium/potassium-transporting ATPase subunit alpha-3 (NKAα3), a protein associated with neuromuscular excitability disorders, in human hippocampi resected "en bloc" for HS treatment (n = 15). We used immunohistochemistry and the stained area was digitally evaluated (increase in binary contrast of microscopic fields) in the hippocampal sectors (CA1-CA4) and dentate gyrus. All HS samples were classified as Type 1, according to the International League Against Epilepsy (ILAE) 2013 Classification (predominant cell loss in CA1 and CA4). ATP5B was significantly decreased in all sectors and dentate gyrus of HS patients compared with individuals submitted to necropsy and without history of neurological alterations (n = 10). NKAα3 expression showed no difference. Moreover, we identified a negative correlation between frequency of pre-operative seizures and number of neurons in CA1. In conclusion, our data showed similarity between changes in protein expression in a model of TLE and individuals with HS. ATP5B reduction would be at least in part due to neuronal loss. Future investigations on ATP5B activity could provide insights into the process of such cell loss.

Crouzon syndrome is a rare form of syndromic craniosynostosis (SC) characterized by premature fusion of the cranial and facial sutures, elevated intracranial pressure, varying degrees of ocular exposure due to exorbitism, and airway compromise caused by midface retrusion. Craniolacunae and upper and lower extremity anomalies are not frequently found in Crouzon syndrome. We present a girl with Crouzon syndrome caused by c.1040 C > G, p.Ser347Cys, a pathogenic mutation in the FGFR2 gene with atypical characteristics, including craniolacunae resembling severe Swiss cheese type of bone formation, and upper and lower extremity anomalies which are more commonly associated with Pfeiffer syndrome patients. Distinguishing between severe Crouzon syndrome patients and patients who have mild and/or moderate Pfeiffer syndrome can be challenging even for an experienced craniofacial surgeon. An accurate genotype diagnosis is essential to distinguishing between these syndromes, as it provides predictors for neurosurgical complications and facilitates appropriate family counseling related to long-term outcomes.

OBJECTIVE This study's objective was to compare the transinsular (TI-AH), transuncus (TU-AH), and temporopolar (TP-AH) amygdalohippocampectomy approaches regarding seizure control, temporal stem (TS) damage, and neurocognitive decline. METHODS We included 114 consecutive patients with unilateral hippocampal sclerosis (HS) who underwent TI-AH, TU-AH, or TP-AH between 2002 and 2017. We evaluated seizure control using Engel classification. We used diffusion tensor imaging and postoperative Humphrey perimetry to assess the damage of the TS. We also performed pre- and postoperative memory performance and intelligence quotient (IQ). RESULTS There were no significant differences in the proportion of patients free of disabling seizures (Engel IA+IB) among the three surgical approaches in the survival analysis. However, more patients were free of disabling seizures (Engel IA+IB) at 2 years of postsurgical follow-up with TP-AH (69.5%) and TI-AH (76.7%) as compared to the TU-AH (43.5%) approach (p = .03). The number of fibers of the inferior fronto-occipital fasciculus postoperatively was reduced in the TI-AH group compared with the TU-AH and TP-AH groups (p = .001). The rate of visual field defects was significantly higher with TI-AH (14/19, 74%) in comparison to the TU-AH (5/15, 33%) and TP-AH (13/40, 32.5%) approaches (p = .008). Finally, there was a significant postoperative decline in verbal memory in left-sided surgeries (p = .019) and delayed recall for both sides (p

A male 3-year-old child was referred to our service due to increasing head size and mild headache. On physical examination, head circumference was 55 cm (bigger than 97th percentile), the child was conscious, alert and oriented to person, place, time and event, his pupils were isochoric, the light reflex was bilaterally positive, eye movements showed a slight palsy on the abduction of the left eye, and a partial vision loss on the left eye was detected. Gait was also impaired. MRI presented a cystic lesion in the suprasellar region suggesting arachnoid cyst, associated with hydrocephalus. An endoscopic ventricular approach was performed through a straight incision over Kocher’s point. The cyst was easily identified, coagulated with cautery and incised with endoscopic scissors. A ball-valve mechanism was well visualized. Prepontine cistern was also sharply opened and the whole cyst wall was coagulated. The patient was discharged on the second postoperative day showing a clinical improvement. Late head CT presented also a radiologic improvement.

Background:. Pfeiffer syndrome (PS) is a very rare condition with a wide clinical spectrum. There are only a few studies that address the classification and treatment of PS and take into account the most commonly presented clinical features. Thus, the objectives of this study are to propose an algorithm for PS management based on a modified severity scale and correlate PS severity with tracheostomy placement. Methods:. An observational retrospective study was performed on consecutive patients with PS (n = 12), who underwent surgery between 2008 and 2018. Clinical features and findings of all included patients with PS were classified as types A, B, and C, which guided treatment workflow. The Fisher test was used to correlate the severity of patients with PS with tracheostomy placement. Results:. There were 12 patients, classified as type A (n = 3), type B (n = 6), and type C (n = 3). All patients who received tracheostomies (n = 6) were stratified into the severe category (n = 9; types B and C) (P < 0.05). There were 4 minor complications, and 1 major complication according to a modified Clavien–Dindo surgical complication scale. Conclusion:. A treatment algorithm based on the 3 different Pfeiffer types was proposed. Severity of PS statistically correlates to tracheostomy placement.

BACKGROUND The purpose of this study is to review 10 years of surgical experience in the management of Apert syndrome, focusing on an updated algorithm which includes hand reconstruction and posterior vault distraction osteogenesis (PVDO). Additionally, the authors compare PVDO, which is currently used, with fronto-orbital advancement (FOA), which was utilized in a previous algorithm. METHODS An observational retrospective study was performed on consecutive patients with Apert syndrome who underwent upper and lower limb reconstruction and craniofacial surgery between 2007 and 2017. A modified Clavien-Dindo surgical complication scale was used to stratify complications between PVDO and FOA. Demographic, surgical, and outcome data was also recorded. The blood transfusion rate between PVDO and FOA was also assessed and compared utilizing the Student t test. RESULTS The present study included 69 patients with Apert syndrome (34 males and 35 females). Craniofacial surgeries were performed on a total of 38 patients. A total of 210 operations were performed on the respective upper and lower limbs of patients included in this study. A total of 18 patients underwent PVDO (n = 9) and FOA (n = 9). Posterior vault distraction osteogenesis required significantly less transfused blood volume than FOA (P

Visualizing a brain in its native space plays an essential role during neurosurgical planning because it allows the superficial cerebral veins and surrounding regions to be preserved. This paper describes the use of a visualization tool in which single gadolinium contrast-enhanced T1-weighted magnetic resonance imaging was applied in nondefective and nonresective skulls to promote visualization of important structures. A curvilinear reformatting tool was applied on the supratentorial compartment to peel the tissues to the depth of the dura mater and thereby revealing cortical and vascular spatial relationships. The major advantage of our proposed tool is that it does not require coregistration of anatomical and vascular volumes. The reliability of this technique was supported by comparisons between preoperative images and digital photographs of the brain cortical surface obtained after the dura mater was removed in 20 patients who underwent surgery in the Clinics Hospital of the University of Campinas from January 2017 to April 2018. Single fat-suppressed GAD contrast-enhanced T1-weighted magnetic resonance scans provide accurate preoperative 3D views of cortical and vascular relationships similar to neurosurgeons’ intraoperative views. In developing countries with limited access to state-of-the-art health technologies, this imaging approach may improve the safety of complex neurosurgeries.

Understand the variables that could interfere with diagnosis and prompt treatment in CNS childhood cancer in Brazil, a developing country with continental dimensions. From 2005 to 2010, we retrospectively evaluated factors, which could represent a negative influence on the time period elapsing from the onset of symptoms until the diagnosis of the central nervous system (CNS) neoplasia in children and adolescents attended in our service. Two hundred seventeen records were analyzed retrospectively. Factors of the households were evaluated, and this data was related to the time period elapsing from presentation of the first symptoms until the diagnosis of CNS neoplasia. The average time elapsed from the onset of the symptoms until seeking medical assistance was 96 days, and from medical assistance to patient referral to a reference service was 33 days. The symptoms which most contributed to a shorter delay in diagnosis were changes in gait and paresis, mother’s occupation, father’s education level, patient gender, and living in the state of Sao Paulo. Besides that, variables such as male gender, mother’s education level, and lower patient age were associated with an early diagnosis time. There is great difficulty in performing early diagnosis of CNS tumors, partly due to parent’s inability to recognize signs and symptoms, and in part due to an educational deficit among healthcare professionals. Identification of measures that can minimize these causes of delay is fundamental to increasing the chance of cure and survival of these patients.

Background Treacher Collins syndrome has a wide variation of clinical presentation, including airway obstruction and hearing loss. The objective of this study was to assess patient-rated quality of life in Treacher Collins syndrome, who received multidisciplinary treatment at a specialized craniofacial hospital, and to compare their quality of life with a control sample. Methods Treacher Collins patients under treatment in a multidisciplinary setting were assessed with the World Health Organization Quality of Life (WHOQOL-100) questionnaire. The Siviero scale was used to classify quality of life as unsatisfactory (scores below 25), intermediate (scores between 25 and 75), and satisfactory (scores above 75). Quality of life comparison between Treacher Collins patients and 2342 unaffected individuals from a previous database was performed using the analysis of variance test. Differences were considered significant at P Results Treacher Collins patients demonstrated an overall intermediate and satisfactory (score >60%) quality of life in almost all WHOQOL-100 facets and domains. The comparison of the scores between unaffected individuals and Treacher Collins patients demonstrated a significant difference (P Conclusion The highest functioning Treacher Collins syndrome patients under multidisciplinary treatment presented intermediate and satisfactory quality of life, consistent with quality of life scores of unaffected controls. In the environment domain, Treacher Collins patients presented even higher scores of quality of life than the control group. Clinical question/level of evidence Therapeutic, III.

Study design Retrospective cohort. Objective Evaluate the epidemiology of surgical patients with spinal metastases, identify the complications, and evaluate their neurological prognoses. Summary of background data The development of new oncological treatments and screening tests have increased the survival of oncologic patients, and consequently, the incidence of metastatic lesions of the spine. Methods Retrospective cohort of 40 patients surgically treated at the Hospital de Clinicas of UNICAMP for spinal metastases from January 2010 to September 2018, after diagnosis of symptomatic spinal cord compression and/or mechanical instability of the spine. Retrospectively analyzed patient charts applied the SINS score to evaluate the presence of mechanical instability. Neurological function was classified based on the Frankel index preoperative and postoperatively. To evaluate the association between variables, the Chi-square test, Fisher exact test, or Fisher-Freeman-Halton test was applied. For evaluating the improvement of neurological status between the Frankel scores before and after surgery, the McNemar test was applied for categorical and qualitative variables. In both the tests, variables with values of P > 0.05 were considered. Results Pain as the reason for the first visit presented an odds ratio (OR) = 2.44 (95% [CI]: 1.14-5.2) for instrumentation need (P = 0.024). A higher SINS score corresponded to the indication for instrumentation surgery due to the instability of the spine (P = 0.004). Within 30 days postoperative, five patients (11.1%) had complications. There was a statistically significant neurological improvement in patients who underwent surgery (P = 0.002). Conclusion Pain as the first symptom was related to mechanical instability of the spine and surgical instrumentation. Patients treated with surgery presented improvement of the neurological function in the postoperative period. Level of evidence 3.

OBJECTIVEMesial temporal lobe epilepsy (MTLE) is the most common type of focal epilepsy in adolescents and adults, and in 65% of cases, it is related to hippocampal sclerosis (HS). Selective surgical approaches to the treatment of MTLE have as their main goal resection of the amygdala and hippocampus with minimal damage to the neocortex, temporal stem, and optic radiations (ORs). The object of this study was to evaluate late postoperative imaging findings on the temporal lobe from a structural point of view.METHODSThe authors conducted a retrospective evaluation of all patients with refractory MTLE who had undergone transsylvian selective amygdalohippocampectomy (SAH) in the period from 2002 to 2015. A surgical group was compared to a control group (i.e., adults with refractory MTLE with an indication for surgical treatment of epilepsy but who did not undergo the surgical procedure). The inferior frontooccipital fasciculus (IFOF), uncinate fasciculus (UF), and ORs were evaluated on diffusion tensor imaging analysis. The temporal pole neocortex was evaluated using T2 relaxometry.RESULTSFor the IFOF and UF, there was a decrease in anisotropy, voxels, and fibers in the surgical group compared with those in the control group (p < 0.001). An increase in relaxometry time in the surgical group compared to that in the control group (p < 0.001) was documented, suggesting gliosis and neuronal loss in the temporal pole.CONCLUSIONSSAH techniques do not seem to totally preserve the temporal stem or even spare the neocortex of the temporal pole. Therefore, although the transsylvian approaches have been considered to be anatomically selective, there is evidence that the temporal pole neocortex suffers structural damage and potentially functional damage with these approaches.

Gliomas sao os tumores cerebrais mais comuns. Estas neoplasias sao originarias de celulas gliais e sua fisiopatogenese envolve alteracoes genicas e metabolicas. Recentemente, foram descritas mutacoes no gene da isoforma 1 da isocitrato desidrogenase (IDH-1) que alteram o funcionamento desta enzima. A forma mutante mais comum, IDH-1 R132H, leva a ganho de funcao, atraves da qual a IDH-1 mutante passa a sintetizar metabolitos oncogenicos, alem de tornar a celula mais suscetivel a acao oxidante. Apesar disso, observa-se que individuos portadores do IDH-1 R123H respondem melhor aos tratamentos e possuem maior sobrevida. Alem das alteracoes genicas referentes ao IDH-1 tambem sao descritas mutacoes no gene ATRX (do ingles, Alpha Thalassemia/Mental Retardation Syndrome X-linked), o qual sintetiza proteina que atua na remodelacao da cromatina e manutencao dos telomeros. No presente estudo, foi avaliada, atraves de imunoistoquimica, a distribuicao tecidual da IDH-1 mutante e ATRX em cortes histologicos de gliomas de baixo (n=25) e alto (n=64) grau de especimes cirurgicos obtidos de pacientes acompanhados em hospital universitario. Os dados obtidos apoiam o fato de que a imunopositividade para IDH-1 R132H possui relacao direta com uma maior sobrevida nos pacientes que possuem tal mutacao, enquanto que nao verificamos valor prognostico da mutacao do ATRX quando considerada isoladamente.

Objetivos: Compararar a eficácia do tratamento cirúrgico da lombalgia axial crônica associada à doença degenerativa discal com o tratamento conservador. Métodos: Efetuou-se pesquisa na base de dados PubMed de ensaios clínicos randomizados que comparassem o tratamento cirúrgico com o tratamento conservador da lombalgia crônica, sendo obtidos seis trabalhos. Foram excluídos estudos cuja técnica cirúrgica consistisse em procedimentos percutâneos, como denervação facetaria, ou intradiscais. Resultados: Cinco estudos utilizaram a fusão lombar e um a artroplastia lombar para comparação do tratamento cirúrgico com o conservador. Apenas um ensaio sugeriu que a fusão fosse mais eficaz na diminuição da dor e incapacidade após um ano de seguimento do que o tratamento não cirúrgico convencional. Quatro trabalhos reportaram que os resultados do tratamento conservador estruturado com exercícios físicos associados à terapia cognitiva foi semelhante ao tratamento cirúrgico. O estudo com artroplastia sugeriu que, embora a mesma fosse estatisticamente mais efetiva no tratamento da lombalgia do que tratamento estruturado não cirúrgico, tal diferença era clinicamente irrelevante. Conclusões: Os resultados, embora heterogêneos, não sugerem grandes benefícios no tratamento cirúrgico da lombalgia axial crônica em relação a dor e incapacidade quando comparado com o tratamento conservador estruturado. Desta forma, concluímos que a cirurgia, seja a fusão como a artroplastia, não deve ser recomendada de rotina no tratamento de pacientes com lombalgia axial crônica secundária a doença degenerativa discal.

A 27 years-old woman presenting with a giant cervical neurofibroma with spinal cord compression and cervical kyphosis is reported. We discuss the surgical nuances of the multiple surgeries required to achieve a total tumor resection and deformity correction. An algorithm used in our institution to treat cervical kyphosis is also presented.

We review the surgical anatomy of the thoraco-lumbar spine region located between the eleventh thoracic and the second lumbar vertebrae (T11-L2). Anatomical features of muscular, vascular and neural structures important to surgical approaches are described in details. We also discuss surgical nuances of the transthoracic retroperitoneal and the lateral retropleural approaches. We conclude that anatomical knowledge is important to improve the efficacy and safety of surgical procedures in the thoraco-lumbar spine region.

Pedicle subtraction osteotomy (PSO) of the cervical spine is a complex and challenging procedure used to treat severe and rigid deformities, especially those secondary to ankylosing spondylitis. In this paper, we discuss the indications of the cervical PSO in a patient with ankylosing spondylitis, as well as the surgical nuances of this technique, in a step-by-step fashion.

Made available in DSpace on 2018-12-11T16:55:02Z (GMT). No. of bitstreams: 0 Previous issue date: 2018-06-01 Background: The objective was to evaluate if there is an association of the spino-pelvic relationships and the global spinal alignment with the outcome of AO type A injuries treated nonsurgically. Methods: This is a retrospective case series. Patients treated nonsurgically for AOSpine type A fractures (T1-L5) with at least 1 year follow-up identified. A standing antero-posterior and lateral 36-inch radiographs and measures of spino-pelvic relationships and sagittal alignment were obtained, as well as clinical assessment using the visual analog scale, the Short-Form 36 (SF-36) questionnaire, the Oswestry Disability Index (ODI), and labor status. Results: Twenty-two patients with 33 fractures were included (L1 was the most injured level with 18.2%). There were 17 men (77.2%) and the mean age was 47.1 years. Follow-up ranged from 12 to 60 months (mean of 27.8 months). There were 22 type A1 (66.7%), 3 type A2 (9%), 6 type A3 (18%), and 2 type A4 (6%) fractures. The ODI ranged from 4% to 58%, with a mean of 24.4%. The SF-36 physical health score ranged from 23 to 82.25 (mean 49.59), and the mental health score ranged from 14.75 to 94.25 (mean 63.28). No association was identified between the spino-pelvic measurements, global alignment, and patient-reported outcomes. Conclusions: Type A fractures had a clinically relevant amount of long-term disability even when surgical treatment is not required. Spino-pelvic relationships and final global spinal alignment did not associate with outcome measurements. Department of Neurology Neurosurgical Division State University of Campinas-UNICAMP Department of Bioprocess and Biotechnology State University of São Paulo-UNESP Department of Orthopaedic Surgery The Rothman Institute at Thomas Jefferson University Department of Orthopedics Northwest University Department of Bioprocess and Biotechnology State University of São Paulo-UNESP

Introduction Spinal cord injury (SCI) is common in polytrauma patients. The standard exam for the initial evaluation is computed tomography (CT), due to its higher sensitivity and specificity when compared with plain radiographs. However, CT is insufficient for the management of some cases, especially to evaluate ligamentous and spinal cord injuries. The objective of the present study is to describe clinical scenarios in which the CT scan was insufficient to guide the treatment of SCIs.Methods We present the cases of four polytrauma patients with normal CT scans at admission and with unstable or surgically-treated lesions.Discussion The cases reported evidence the need for ongoing neurological surveillance with serial physical examination and magnetic resonance imaging (MRI) in cases of neurological injury not explained by CT or occult instability.Conclusion Computed tomography is not always sufficient to determine the management of SCIs. A comprehensive evaluation of the clinical data, CT findings and, occasionally, MRI findings is crucial in order to choose the best conduct.

Summary Introduction: Spinal tumors are rare in the pediatric population, presenting many specific peculiarities when compared to adults. We have performed a broad narrative review to describe the most common spinal tumors in children, discussing their main characteristics and management options. Method: The authors have performed an extensive review of the peer-reviewed literature addressing the aforementioned objectives. Results: Multimodality radiological studies (plain films, 3D computed tomography scan and magnetic resonance imaging) are necessary for proper evaluation and differential diagnosis of spinal tumors in children. In selected cases nuclear medicine imaging is used to improve the chances of a more accurate diagnosis. As a general rule, a fine needle biopsy is recommended after radiological evaluation to confirm the tumor's histology. Primary bone tumors can be divided into benign bone tumors, mostly represented by vertebral hemangiomas, osteoid osteomas, osteoblastomas, aneurismal bone cysts, and eosinophilic granulomas, and malign or aggressive tumors, such as Ewing's or osteogenic sarcomas. Secondary bone tumors (spinal metastases) comprise different tumor histologies, and treatment is mainly based on tumor's radiosensitivity. The characteristics and treatment options of the main spinal tumors are discussed in details. Conclusion: Spinal tumors in children are rare lesions that demand a thorough understanding of their main characteristics for their proper management. Understanding the nuances of spinal tumors in children is of paramount importance for improving outcomes and chances of cure. Resumo Introdução: Os tumores de coluna em crianças são raros, apresentando peculiaridades únicas quando comparados com os da população adulta. Método: Dada a escassez de trabalhos que avaliem o tema, realizou-se extensa revisão de literatura objetivando descrever os tumores de coluna que acometem a população pediátrica, discutindo características e opções de manejo. Resultados: A utilização de exames radiológicos combinados (radiografias, tomografia computadorizada com reconstrução em 3D e ressonância magnética) é necessária para avaliação adequada e diagnóstico diferencial dessas lesões. Em casos selecionados, exames de medicina nuclear aumentam as chances do diagnóstico preciso. Como regra geral, biópsia por agulha é recomendada para confirmação da histologia tumoral e tratamento subsequente. As lesões primárias de coluna podem ser benignas, representadas principalmente pelos hemangiomas, osteomas osteoides, osteoblastomas, cistos ósseos aneurismáticos e granulomas eosinofílicos, enquanto as lesões malignas são geralmente representadas por tumores agressivos, como o sarcoma de Ewing ou os sarcomas osteogênicos. Metástases de coluna podem ter diferentes etiologias, sendo o tratamento dependente principalmente da radiossensibilidade do tumor de origem. As opções de tratamento dessas lesões são descritas em detalhes. Conclusão: Tumores de coluna em crianças são raros e o seu manejo requer um conhecimento amplo e variado das diferentes possibilidades diagnósticas. Conhecer os nuances envolvidos no tratamento dessas lesões e os sintomas iniciais é fundamental para melhorar o prognóstico e as chances de cura.

Background Although craniofacial bone correction is the essential step in hypertelorbitism correction, the final result depends on the management of soft tissue deformities. The purpose of this study was to review our surgical strategies for soft tissue reconstruction in hypertelorbitism correction. Methods A retrospective study was performed of consecutive patients with hypertelorbitism, undergoing hypertelorbitism correction between 2007 and 2014. All aspects related to the craniofacial surgical procedures (number and type of procedures, outcomes, and complications) were verified through medical records, clinical photographs, and interviews with all patients. The surgical results were classified based on a previously published outcome grading scale I-IV on the need for additional surgery. Results The present study included 16 patients diagnosed with Crouzon syndrome (n = 2), Apert syndrome (n = 1), encephalocele (n = 1), frontonasal dysplasia (n = 2), craniofrontonasal dysplasia (n = 5), Tessier number 10 type (n = 1), and Tessier number 0 to 14 type (n = 4).The number and types (local flaps, medial canthopexy, Converse scalping flap, and/or the K stitch technique) of surgeries performed varied according to the facial soft tissue deformities of each patient. The overall rate of surgical results ranked according to the need for additional surgery was 1.56 ± 0.51 (between categories I and II). Conclusions As hypertelorbitism has been associated with a variety of congenital deformities, plastic surgeons who deal with these patients should have a broad surgical armamentarium tailored to each individual presentation.

Neurofibromatosis type II (NF2) is a rare autosomal dominant inherited disease caused by a mutation in chromosome 22q12 and associated with multiple central nervous system tumors. In this paper, we describe a rare case of cervicomedullary junction ependymoma associated with NF2 in a 25-year-old man who underwent surgical treatment with total resection and had a good clinical outcome. We discussed the nuances of the surgical resection and the literature concerning this rare form of presentation of NF2.

Patients with cervical disc herniation can be asymptomatic or present with radiculopathy or myelopathy symptoms. Generally, they are located anterior or antero-laterally in the epidural space. We present a case of an extruded cervical disc with a dorso-lateral migration, similar to a tumoral extradural lesion. Sequestered cervical disc herniation should be considered in the differential diagnosis of dorso-lateral lesions.

OBJECTIVEMany repair techniques have been proposed to treat large myelomeningocele (MMC), and although effective in many cases, some of these techniques can be complex and time consuming, with complications such as cerebrospinal fluid (CSF) leakage, flap loss, tip necrosis, and wound dehiscence. The purpose of this study was to analyze cases of large skin defects and the methods applied and to report the outcomes of the keystone design perforator island flap (KDPIF) technique for large MMC closure.METHODSThe authors performed a retrospective review of all neonatal patients who had undergone KDPIF for MMC closure in the period from 2013 to 2018. All patients had a diagnosis of lumbosacral MMC based on obstetric ultrasound. The neurosurgeons and plastic surgeons had selected the cases after concluding that primary closure would be unlikely. The design of the flap is based on the randomly located vascular perforators, creating two identical opposing flaps to fashion a double keystone flap. During wound closure, V-Y advancement of each end of the double flap in the longitudinal axis creates redundancy in the central portion of the flap and reduces the horizontal tension. After discharge, both the neurosurgery and plastic surgery teams followed up all patients, tracking the results with photography.RESULTSNo skin flap dehiscence or necrosis, infection, or CSF leakage was detected, proving the reliability of the flap. One of the patients required further surgery for the large skin defects after insufficient intrauterine closure of the MMC and successfully underwent KDPIF treatment. Another patient (14.3%) had severe neonatal sepsis, which ultimately led to death. A ventriculoperitoneal shunt was required after the skin defect repair in 5 (83.3%) of the 6 surviving patients. Exceptional aesthetic results were achieved for all patients during the follow-up.CONCLUSIONSThe KDPIF technique is based on well-known vascular perforators of the intercostal, lumbar, and gluteal regions. Wound tension is widely distributed by the flap and, as a consequence, relevant tissue bulk, reliable vascularity, and important geometrical versatility are provided. In addition, most of the muscles and fascia are preserved, which is another advantage in terms of minimizing secondary morbidity to local tissue rearrangement. The use of KDPIF closure was successfully shown to be a viable alternative for more complex MMCs that present with large skin defects.

Fat grafting has been described as an option to repair frontotemporal contour deformities (volumetric deficiency of bone and/or soft tissues) after neurosurgical/craniofacial surgical interventions. However, technical surgical descriptions have varied, with reports describing the bolus fat injection or the classical multilayer injection, but with no detailed descriptions concerning how and where the fat should be grafted. The purpose of this study was to assess the frontotemporal symmetry outcomes after a single fat-grafting procedure for postoperative frontotemporal contour deformity reconstructions using the anatomical fat-grafting approach.A prospective analysis was conducted of consecutive patients (n = 106) who underwent anatomical fat grafting (Coleman's structural fat grafting technique using anatomical facial subunit and fat compartment principles) to reconstruct frontotemporal contour deformities after neurosurgical/craniofacial surgical interventions. A subjective assessment by a panel of external surgical professionals and laypersons was obtained to grade the frontotemporal symmetry. Objective ultrasound symmetry assessment was blindly performed preoperatively and at 3- and 12-month follow-up.There were significant (all P 0.05) postoperative subjective and objective frontotemporal symmetry enhancements (preoperativepostoperative) after anatomical fat grafting, with no differences (all P0.05) between the 3- and 12-month postoperative comparisons. Thirty-seven percent of patients required an additional fat grafting session for residual asymmetry after 12 months of follow-up.Patients with frontotemporal contour deformities presented improved subjective and objective frontotemporal symmetry after an anatomical fat grafting session.

Central nervous system (CNS) tumor has a high incidence during childhood and represents the second most common neoplasm in the pediatric population, only behind leukemias that are the most frequent tumors in children under 15 years old. The most frequent CNS tumors in the pediatric population are midline neoplasms (pontine and hypothalamic gliomas and craniopharyngiomas), posterior fossa tumors, and embrionary tumors. Approximately, 50–60% of these tumors have their initial growth in the posterior fossa. Many of the signs and symptoms are present in common pathologies like gastroenteritis, migraine, and behavioral disorders and can cause misdiagnoses. We describe the most common pediatric tumors and their treatment and prognosis.

Tethered cord syndrome is a clinical condition characterized by signs and symptoms secondary to conus medullaris dysfunction and abnormally low spinal cord, resulting in functional alterations of motor and sensitive neurons due to conus or spinal cord traction. It usually presents as a neuro-orthopedic syndrome with neurological, orthopedic, and urological symptoms and signs. The main pathologies related to tethered cord syndrome are congenital due to spinal dysraphism. We present a didactic and simplified view of key embryological processes related and describe the most common causes of tethered cord syndrome and its management.