Beyond image defined risk factors (IDRFs): a delphi survey highlighting definition of the surgical complexity index (SCI) in neuroblastoma.

PURPOSE: Preoperative evaluation of Image Defined Risk Factors (IDRFs) in neuroblastoma (NB) is crucial for determining suitability for upfront resection or tumor biopsy. IDRFs do not all carry the same weighting in predicting tumor complexity and surgical risk. In this study we aimed to assess and categorize a surgical complexity (Surgical Complexity Index, SCI) in NB resection. METHODS: A panel of 15 surgeons was involved in an electronic Delphi consensus survey to identify and score a set of shared items predictive and/or indicative of surgical complexity, including the number of preoperative IDRFs. A shared agreement included the achievement of at least 75% consensus focused on a single or two close risk categories. RESULTS: After 3 Delphi rounds, agreement was established on 25/27 items (92.6%). A severity score was established for each item ranging from 0 to 3 with an overall SCI range varying from a minimum score of zero to a maximum score of 29 points for any given patient. CONCLUSIONS: A consensus on a SCI to stratify the risks related to neuroblastoma tumor resection was established by the panel experts. This index will now be deployed to critically assign a better severity score to IDRFs involved in NB surgery.

Frontal lobe growth is impaired in fetuses with congenital heart disease.

OBJECTIVES: The primary objective of this study was to assess whether fetuses with congenital heart disease (CHD) have smaller frontal brain areas compared with normal controls. The secondary objective was to evaluate whether there are any differences in frontal brain area between cases with different types of CHD, grouped according to their impact on hemodynamics. METHODS: This was a retrospective cross-sectional study, including 421 normal fetuses and 101 fetuses with isolated CHD evaluated between 20 and 39 gestational weeks at our fetal medicine and surgery unit in the period January 2016-December 2019. The study group was subdivided, according to the CHD hemodynamics, as follows: (1) hypoplastic left heart syndrome and other forms of functionally univentricular heart defect; (2) transposition of the great arteries; (3) conotruncal defects and other CHDs with large shunts; (4) right ventricular outflow tract obstruction, without a hypoplastic right ventricle; (5) left outflow tract obstruction; (6) others. The transventricular axial view of the fetal head was used as the reference view, on which the frontal lobe anteroposterior diameter (FAPD) and the occipitofrontal diameter (OFD) were measured, assuming the former to be representative of the area of the frontal lobes. The FAPD/OFD ratio was then calculated as FAPD/OFD × 100. These two variables (FAPD and FAPD/OFD ratio) were then evaluated and compared between the study and control groups. Adjustment for gestational age, both via multiple linear regression and by using a-posteriori matching based on the propensity score, was employed. RESULTS: In normal fetuses, FAPD showed a linear positive correlation with gestational age. In fetuses with CHD, the FAPD was shorter than in normal fetuses from the 20th gestational week onwards, with the difference increasing after 30 gestational weeks. FAPD/OFD ratio was significantly smaller in fetuses with CHD than in normal fetuses (P < 0.0001) at all gestational ages, with no apparent differences among the various CHD categories, all of which had smaller FAPD/OFD ratio compared with controls. CONCLUSIONS: Fetuses with CHD have a shorter FAPD and a smaller FAPD/OFD ratio compared with normal fetuses. This impaired growth of the frontal area of the brain seems to occur in all types of CHD, regardless of their impact on hemodynamics. © 2021 International Society of Ultrasound in Obstetrics and Gynecology.

Hindbrain morphometry and choroid plexus position in differential diagnosis of posterior fossa cystic malformations.

OBJECTIVE: To assess the differential diagnostic significance of a series of quantitative and qualitative variables of the cerebellar vermis in fetuses with posterior fossa cystic malformation, including Dandy-Walker malformation (DWM), vermian hypoplasia (VH) and Blake's pouch cyst (BPC). METHODS: This was a retrospective study of confirmed cases of DWM, VH and BPC, diagnosed at the Fetal Medicine and Surgery Unit of the Federico II University between January 2005 and June 2013 or the Fetal Medicine and Surgery Unit of G. Gaslini Hospital between July 2013 and September 2017. All included cases had good-quality three-dimensional (3D) volume datasets of the posterior fossa, acquired by transvaginal ultrasound through the posterior fontanelle. The midsagittal view of the posterior fossa was the reference view for the study. We assessed brainstem-tentorium angle and brainstem-vermis angle (BVA), as well as craniocaudal (CCVD) and anteroposterior (APVD) vermian diameters and vermian area (VA), which were normalized by biparietal diameter (BPD) to take into account gestational age (CCVD/BPD × 100, APVD/BPD × 100 and VA/BPD × 100, respectively). Finally, the position of the fourth ventricular choroid plexus (4VCP) was defined as normal ('up') or abnormal ('down'), relative to the roof/cyst inlet of the fourth ventricle. RESULTS: We analyzed 67 fetuses with posterior fossa malformations (24 cases of DWM, 13 of VH and 30 of BPC). The mean gestational age at diagnosis was 23.6 weeks. Regardless of gestational age, the BVA differed significantly between the three groups, and the VA/BPD was able to differentiate between VH and BPC. In differentiating between VH and BPC, the greatest areas under the receiver-operating characteristics curve were those for VA/BPD ratio. The 4VCP position was down in all cases of DWM and VH, while it was up in all cases of BPC. CONCLUSIONS: Our data support the concept that VA/BPD ratio and 4VCP position may be used to differentiate between DWM, VH and BPC in the fetus. In our series, the position of the 4VCP had the highest accuracy, but a larger number of VH cases should be evaluated to confirm that an up position of the 4VCP indicates BPC while a down position indicates DWM or VH. Copyright © 2018 ISUOG. Published by John Wiley & Sons Ltd.

Childhood infectious diseases and risk of multiple myeloma: an analysis of the Italian multicentre case-control study.

Common childhood infectious diseases have been associated with a reduced risk of following haematopoietic malignancies, but investigations on multiple myeloma (MM) are scarce. Information about 213 MM cases and 1128 healthy controls were obtained from a multicentre population-based Italian case-control study. The association between chickenpox, measles, mumps, pertussis and rubella and the MM risk was estimated by unconditional logistic regression, adjusting for age, gender and residence area. No association was found between MM risk and any considered infectious disease. The number of infections was slightly inversely associated with the risk of MM, but statistical significance was not reached (OR 0.87, 95% CI 0.55-1.4 for 1-2 diseases vs. none and OR 0.68, 95% CI 0.41-1.1 for 3-5 diseases, respectively, P = 0.131). We did not find a clear evidence that common infections during childhood are associated with the subsequent risk of developing MM.

Restricted ROC curves are useful tools to evaluate the performance of tumour markers.

In Clinical Epidemiology, receiver operating characteristic (ROC) analysis is a standard approach for the evaluation of the performance of diagnostic tests for binary classification based on a tumour marker distribution. The area under a ROC curve is a popular indicator of test accuracy, but its use has been questioned when the curve is asymmetric. This situation often happens when the marker concentrations overlap in the two groups under study in the range of low specificity, corresponding to a subset of values useless for classification purposes (non-informative values). The partial area under the curve at a high specificity threshold has been proposed as an alternative, but a method to identify an optimal cut-off that separates informative from non-informative values is not yet available. In this study, a new statistical approach is proposed to perform this task. Furthermore, a statistical test associated with the area under a ROC curve corresponding to informative values only (restricted ROC curve) is provided and its properties are explored by extensive simulations. Finally, the proposed method is applied to a real data set containing peripheral blood levels of six tumour markers proposed for the diagnosis of neuroblastoma. A new approach to combine couples of markers for classification purposes is also illustrated.

Influence of segmental chromosome abnormalities on survival in children over the age of 12 months with unresectable localised peripheral neuroblastic tumours without MYCN amplification.

BACKGROUND: The prognostic impact of segmental chromosome alterations (SCAs) in children older than 1 year, diagnosed with localised unresectable neuroblastoma (NB) without MYCN amplification enrolled in the European Unresectable Neuroblastoma (EUNB) protocol is still to be clarified, while, for other group of patients, the presence of SCAs is associated with poor prognosis. METHODS: To understand the role of SCAs we performed multilocus/pangenomic analysis of 98 tumour samples from patients enrolled in the EUNB protocol. RESULTS: Age at diagnosis was categorised into two groups using 18 months as the age cutoff. Significant difference in the presence of SCAs was seen in tumours of patients between 12 and 18 months and over 18 months of age at diagnosis, respectively (P=0.04). A significant correlation (P=0.03) was observed between number of SCAs per tumour and age. Event-free (EFS) and overall survival (OS) were calculated in both age groups, according to both the presence and number of SCAs. In older patients, a poorer survival was associated with the presence of SCAs (EFS=46% vs 75%, P=0.023; OS=66.8% vs 100%, P=0.003). Moreover, OS of older patients inversely correlated with number of SCAs (P=0.002). Finally, SCAs provided additional prognostic information beyond histoprognosis, as their presence was associated with poorer OS in patients over 18 months with unfavourable International Neuroblastoma Pathology Classification (INPC) histopathology (P=0.018). CONCLUSIONS: The presence of SCAs is a negative prognostic marker that impairs outcome of patients over the age of 18 months with localised unresectable NB without MYCN amplification, especially when more than one SCA is present. Moreover, in older patients with unfavourable INPC tumour histoprognosis, the presence of SCAs significantly affects OS.

Risk of non-Hodgkin's lymphoma and residential exposure to air pollution in an industrial area in northern Italy: a case-control study.

The aim of this study was to evaluate the risk of non-Hodgkin's lymphoma (NHL) in an adult population residing in an area in northern Italy exposed to industrial air pollution from a big power plant, a coke oven, 2 chemical factories, and some minor plants. The design was a population-based case-control study and information about residential history and the main risk factors for NHL was obtained interviewing 133 cases and 279 controls using a structured questionnaire. Three exposure categories (heavy, moderate, and slight) were defined on the basis of the location of the major facilities with respect to the subject residence. NHL risk was not associated either with location or duration of residence in the heavily polluted area. However, the unavoidable limitations of this study prevent us from drawing definitive conclusions.

Treatment of children over the age of one year with unresectable localised neuroblastoma without MYCN amplification: results of the SIOPEN study.

BACKGROUND: In children older than 1 year with localised unresectable neuroblastoma (NB), treatment strategies are heterogeneous according to the national groups. The objective of this phase III non-randomised study was to evaluate the efficacy of conventional chemotherapy followed by surgery. PATIENTS AND METHODS: In the presence of surgical risk factors (SRF), six courses of chemotherapy alternating Carboplatin-Etoposide and Vincristin-Cyclophosphamide-Doxorubicin were given, and surgical resection was attempted after four. Survival analyses were performed using an intention-to-treat approach. The main objective was to achieve a 5-year survival over 80%. RESULTS: Out of 191 registered children, 160 were evaluable. There were 62.5% older than 18 months and 52.5% had unfavourable histology according to International Neuroblastoma Pathology Classification (INPC). Chemotherapy reduced the number of SRFs by one third. Delayed surgery was attempted in 86.3% of patients and was complete or nearly complete in 74%. The 5-year EFS and OS were 76.4% and 87.6% respectively, with significant better results for patients younger than 18 months or with favourable histology. CONCLUSION: This strategy provides encouraging results in children older than 1 year or 12 months with localised unresectable NB without MYCN amplification. However, in children older than 18 months and with unfavourable histology, additional treatment is recommended.

Role of angiogenesis inhibitors in colorectal cancer: sensitive and insensitive tumors.

Angiogenesis is a key factor in the carcinogenesis process. In oncological practice, angiogenesis inhibition, mainly through the blockade of the VEGF family and its receptors, has been robustly demonstrated to produce clinical benefits and, in specific disease subsets such as colorectal cancer, to extend the overall survival of treated patients. VEGF is a multifunctional growth factor that mediates its functions through cognate receptors on endothelial cells and it has been discovered for its capability to induce macromolecule hyperpermeability in veins and venules. Several approaches have been taken to target angiogenesis in cancer: drugs that target one or more soluble ligands of the VEGF family, drugs that selectively inhibit one or more receptors of the VEGF receptor family, and drugs that inhibit VEGF receptor(s) among other, non VEGF-related targets. At present, two compounds have shown significant clinical activity, bevacizumab, Avastin® and aflibercept, Zaltrap®, and only one of these (bevacizumab) has so far been registered for use in clinical practice. In the present review, we explore and summarize the main features of the angiogenetic process, concerning in particular a common and potentially lethal disease as colorectal cancer. We overview the molecular pathways that characterize angiogenesis, focusing on VEGF family, the current applications and limitations of its blockade in oncology, and the hypothetical future perspectives of anti-angiogenic therapy.

Patient-tailored treatments with anti-EGFR monoclonal antibodies in advanced colorectal cancer: KRAS and beyond.

Personalized medicine emphasizes the practice of considering individual patient characteristics as opposed to that centered on standards derived from epidemiological studies which, by definition, do not take into account the variability of individuals within a given population. When applied to oncology, personalized medicine is an even more complex concept because it extends the variability beyond the individual patient to the individual tumor. Indeed, the great genotypic and phenotypic variability (both in primary and metastatic sites of cancer) the development of targeted therapies, and the growing availability of biological assays complicate the scenario of personalized medicine in the oncological field. In this paper we review the results of anti-epidermal growth factor receptor (EGFR) monoclonal antibody (mAb) therapy in metastatic colorectal cancer (mCRC) in the context of tumor biology, delineating the future prospects of patient-tailored medicine in this area. In particular, we deal with EGFR inhibition by Cetuximab, a chimeric mouse human IgG1 mAb, and panitumumab, a fully human IgG2 mAb. We discuss the clinical impact of anti-EGFR mAbs on wild-type (WT) KRAS mCRC, also taking into account the feasibility of novel multi-marker approaches to treatment decision-making, aimed at increasing the predictive power of pre-therapy biomarkers. Experimental topics and fields of ongoing research, such as targeting microRNAs (miRNAs) with novel anticancer drugs and epigenetics in CRC are also addressed.

A multi-scale approach to colorectal cancer: from a biochemical- interaction signaling-network level, to multi-cellular dynamics of malignant transformation. Interplay with mutations and onco-protein inhibitor drugs.

This review article is part of a special Current Cancer Drug Targets issue devoted to colorectal cancer and molecularly targeted treatments. In our paper we made an attempt to connect more basic aspects with preclinical, pharmacological / therapeutic and clinical aspects. Reconstruction of a Molecular Interaction Map (MIM) comprising an important part of the G0 - G1 - S cell cycle transition, was a major component of our review. Such a MIM serves also as a convenient / organized database of a large set of important molecular events. The frequency of mutated / altered signaling-proteins indicates the importance of this signaling-network region. We have considered problems at different scale levels. Our MIM works at a biochemical-interaction level. We have also touched the multi-cellular dynamics of normal and aberrant colon crypts. Until recently, dynamic simulations at a biochemical or multi-cellular scale level were considered as a sort of esoteric approach. We tried to convince the reader, also on the basis of a rapidly growing literature, mostly published in high quality journals, that suspicion towards simulations should dissipate, as the limitations and advantages of their application are better appreciated, opening the door to their permanent adoption in everyday research. What is really required is a more interdisciplinary mentality and an interdisciplinary approach. The prize is a level of understanding going beyond mere intuition.

Structural comparison of the interaction of tubulin with various ligands affecting microtubule dynamics.

Microtubules (MTs), which are highly dynamic assemblies of the protein tubulin, play important and diverse roles in eukaryotic cells. MT dynamics are regulated during the cell cycle by interacting with a large number of endogenous cellular regulators. In addition, many anti-tumour drugs and natural ligands that interact directly with tubulin are able to either stabilise or destabilise MTs and to disrupt the normal dynamics. Herein, we compare the structures of tubulin when complexed with different ligands in order to analyse: (i) various binding-sites of the protein and different positions of ligands within the microtubule (ii) the diverse effect on the microtubule dynamics. The structures and data given are essential for understanding tubulin-ligand interactions and their influence on the regulation of the microtubule system.

Tratamiento quirúrgico de fractura de cabeza femoral mediante luxación controlada de cadera: resultados a mediano plazo / Surgical treatment through controlled hip dislocation for femoral head fractures: mid term results

Femoral head fractures associated to hip dislocations are uncommon lesions. Most reports present only short-term results. Objectives: To show functional outcome after a mid term follow up of patients with Pipkin II femoral head fractures treated operatively. Materials and Methods: A retrospective and descriptive evaluation of a 4 consecutive case series (four men, mean age of 36.5 years) treated operatively with an 89-month average follow up (range form 72-108 month). Controlled femoral head dislocation was performed in all patients and two 2.4 mm screws were used for fixation. At final follow up, the Harris Hip Score (HHS) and complications were assessed. Results: No immediate or late complications were reported. The mean HHS was of 95 points (range from 89 to 100 points).No signs of postraumatic arthritis or femoral head osteonecrosis were seen at the last radiographic assessment. Conclusion: in this series of patients with Pipkin II femoral head fractures mid-term functional results are good and no postraumatic arthritis or femoral head osteonecrosis were observed at final follow-up.

Las fracturas de cabeza femoral, asociada a luxación de cadera, corresponden a lesiones infrecuentes. La mayoría de los estudios reportan resultados a corto plazo. Objetivo: Describir el resultado funcional de pacientes con fractura Pipkin II operados con seguimiento a mediano plazo. Material y Métodos: Evaluación retrospectiva y descriptiva de una serie consecutiva de 4 pacientes (cuatro hombres, mediana de edad 36,5 años) operados con un seguimiento de mediana de 89 meses (72-108 meses). En todos los casos se realizó luxación controlada de la cabeza femoral y se utilizaron 2 tornillos de 2,4 mm. Se evaluaron complicaciones y Harris Hip Score (HHS) al último control clínico. Resultados: No se presentaron complicaciones tempranas ni tardías. El HHS promedio fue de 95 puntos (89-100 puntos). La última evaluación radiográfica no demostró signos de artrosis postraumática ni signos de necrosis avascular de cabeza femoral. Conclusión: En este grupo de pacientes con fractura de Pipkin tipo II los resultados funcionales a mediano plazo son buenos, no registrándose en el seguimiento artrosis de cadera postraumática ni necrosis avascular de la cabeza femoral.

Cirugía de revisión acetabular utilizando un cotilo modular con superficie de metal trabecular de tantalio: resultados a corto plazo / Acetabular revision surgery using a modular acetabular cup coated with trabecular metal of tantalum: short term results

Objectives: To evaluate short-term clinical results of patients performed acetabular revision surgery using a Trabecular Tantalum coated modular acetabular cups. Materials and Methods: This is a retrospective and descriptive analysis of a serie of 32 consecutive patients (34 hips) with acetabular revision surgery (23 women, 9 men, mean age 70.7 years), mean follow-up 32 month (12-60). The Paprosky and the AAOS acetabular defects classifications were assessed. According to Paprosky acetabular defects classification, there were eleven type I, two IIA, three IIB, nine IIC, seven IIIA and two IIIB. According to AAOS acetabular defects classification there were thirteen patients with type 1, nine patients with type 2, ten type 3 and two type 4. The percentage of own acetabular bone in contact with the new cup was recorded for each patient at the time of surgery and was 45 percent (range from 20 to 80 percent), with 23 patients presenting less than 50 percent of bone contact (mean 35 percent). Postoperatively, osseous integration was radiographically assessed. Functional results were evaluated according to the Harris Hip Score (HSS). The complications related to the implant were also recorded. Results: In all radiographic assessments performed at 3, 6 and 12 month postoperatively an integrated cup without signs of migration or osteolysis was observed. Four patients (11.8 percent) presented recurrent hip dislocation that required a revision surgery without further cup revision. The final clinical assessment was performed at a mean of 26 month after surgery (12-60) with a mean HHS of 91.8 points. Conclusion: Trabecular tantalum coated modular acetabular cups in acetabular revision surgery shows good short term clinical results even with acetabular defects greater than 50 percent. No cup loosening was observed in these series.

Objetivo: Evaluar los resultados clínicos a corto plazo de pacientes sometidos a cirugía de revisión acetabular utilizando cotilos modulares con superficie de metal trabecular de tantalio. Material y Métodos: Estudio retrospectivo-descriptivo de una serie consecutiva de 32 pacientes (34 caderas) sometidos a cirugía de revisión acetabular (23 mujeres, 9 hombres, edad promedio 70,7 años), seguimiento promedio 32 meses (12-60). Se clasifican los defectos acetabulares de acuerdo ala clasificación de Paprosky y de la AAOS. Según la clasificación de Paprosky de los defectos acetabulares, 11 pacientes tenían categoría I, 2 categoría IIA, 3 categoría IIB, 9 categoría IIC, 7 categoría IIIA y 2 categoría IIIB. Según la clasificación de la AAOS, 13 pacientes correspondieron a nivel 1, 9 pacientes a nivel 2, 10 a nivel 3 y 2 a nivel 4. Se registra el porcentaje de hueso propio acetabular en contacto con el nuevo cotilo de cada paciente que fue en promedio 45 por ciento (20-80 por ciento), presentándose en 23 caderas un porcentaje < 50 por ciento, el cual fue en promedio 35 por ciento. En el postoperatorio se evalúa la integración ósea mediante radiografías de pelvis. Se evalúan los resultados funcionales mediante el HHS (Harris Hip Score) y las complicaciones postoperatorias relacionadas con el implante. Resultados: En todas las radiografías postoperatorias realizadas a los 3, 6 y 12 meses se evidenciaba el cotilo integrado, sin signos de migración ni osteolisis. Cuatro pacientes (11,8 por ciento) presentaron luxaciones recidivantes de cadera, las cuales requirieron de una cirugía de revisión sin recambio del cotilo. La última evaluación clínica fue en promedio 26 meses tras la cirugía (12-60 meses) constatándose un HHS promedio de 91,8 puntos. Conclusión: El uso de cotilos modulares con revestimiento de metal trabecular de tantalio en cirugías de revisión acetabular presenta buenos resultados clínicos a corto plazo incluso en defectos óseos acetabulares superiores...

Dynamic simulations of pathways downstream of ERBB-family, including mutations and treatments: concordance with experimental results.

The pathways downstream of ErbB-family proteins are very important in BC, especially when considering treatment with onco-protein inhibitors. We studied and implemented dynamic simulations of four downstream pathways and described the fragment of the signaling network we evaluated as a Molecular Interaction Map. Our simulations, enacted using Ordinary Differential Equations, involved 242 modified species and complexes, 279 reversible reactions and 111 catalytic reactions. Mutations within a single pathway tended to be mutually exclusive; only inhibitors acting at, or downstream (not upstream), of a given mutation were active. A double alteration along two distinct pathways required the inhibition of both pathways. We started an analysis of sensitivity/robustness of our network, and we systematically introduced several individual fluctuations of total concentrations of independent molecular species. Only very few cases showed significant sensitivity. We transduced the ErbB2 over-expressing BC line, BT474, with the HRAS (V12) mutant, then treated it with ErbB-family and phosphorylated MEK (MEKPP) inhibitors, Lapatinib and U0126, respectively. Experimental and simulation results were highly concordant, showing statistical significance for both pathways and for two respective endpoints, i.e. phosphorylated active forms of ERK and Akt, p one tailed = .0072 and = .0022, respectively. Working with a complex 39 basic species signaling network region, this technology facilitates both comprehension and effective, efficient and accurate modeling and data interpretation. Dynamic network simulations we performed proved to be both practical and valuable for a posteriori comprehension of biological networks and signaling, thereby greatly facilitating handling, and thus complete exploitation, of biological data.

Congenital central hypoventilation syndrome: genotype-phenotype correlation in parents of affected children carrying a PHOX2B expansion mutation.

Congenital Central Hypoventilation Syndrome (CCHS) is a rare genetic disorder. Although most CCHS associated PHOX2B mutations occur de novo, about 10% of the cases are inherited from apparently asymptomatic parents, thus confirming variable expressivity and incomplete penetrance of PHOX2B mutations. Three asymptomatic parents of children affected with CCHS, and found to carry the same PHOX2B expansion mutations as their siblings, were studied by overnight polysomnography and somatic mosaicism analysis. In one case, significant sleep breathing control anomalies were detected, while the other two resulted in normal. In tissue-specific allele studies, mosaicism with a comparatively low mutant allele proportion was showed in the two unaffected adult carriers. Accurate polysomnography and assessment of the degree of somatic mosaicism should be conducted in asymptomatic carriers of PHOX2B mutations, as they may unmask subclinical but significant anomalies.

Técnica quirúrgica: resección artroscópica del resalte o giba femoral en el pinzamiento femoroacetabular tipo leva / Surgical technique: arthroscopic resection of femoral bump in cam-type femoroacetabular impingement

Hip arthroscopy is a procedure which has significantly increased its frequency in recent years, especially due to the surgical treatment of femoroacetabular impingment. Cam type femoroacetabular impingement. Cam type femoroacetabular impingement results from abnormal head-neck junction, where a prominent anterior extension of the femoral head at the expected step off to the adjacent femoral neck can be seen. This femoral “bump” is typically located lateral to the physeal scar, causing damage of the acetabular rim, cartilage and labrum. The surgical treatment, also called osteochondroplasty of the femoral head-neck junction, consist of the complete resection of this femoral “bump”, in order to reduce pain, prevent further bone impaction and progressive articular damage. Prior to the arthroscopic resection of the femoral “bump”, it is recommended to identify important anatomic landmarks in the peripheral joint compartment (medial limit: medial synovial fold, central limit: acetabular labrum, lateral limit: synovial folds and retinacular vessels in the lateral limit of the peripheral joint compartment, a secure lateral bump resection can be performed, without risk of injuring these vessels. In this paper, we describe the detailed operative technique of an effective and secure arthroscopic femoral “bump” resection.

La artroscopía de cadera ha aumentado significativamente su frecuencia en los últimos años, especialmente debido al tratamiento quirúrgico del pinzamiento femoroacetabular (PFA). En el PFA tipo leva existe una anesfericidad de la cabeza femoral con la formación de una prominencia, resalte o giba ósea en la cara anterior de la unión cabeza-cuello femoral. El tratamiento quirúrgico consiste en la completa resección de esta giba para reducir el impacto óseo sobre el reborde y cartílago acetabular, prevenir un deterioro articular progresivo y lograr el alivio sintomático de los pacientes. Para realizar la completa resección artroscópica de la giba femoral se recomienda la identificación de los reparos anatómicos en el compartimiento articular periférico previo a la resección (límite medial: pliegue sinovial medial; límite central: labrum acetabular; límite lateral: pliegues sinoviales y vasos retinaculares; límite periférico y profundidad: cuello femoral). Más allá de la completa resección ésta debe ser segura sin lesionar los vasos retinaculares en la región lateral recomendándose la identificación de esos vasos previo a la resección de la giba en la región lateral. Se describen en este trabajo detalles de la técnica quirúrgica para una efectiva y segura resección artroscópica de la giba femoral en el pinzamiento femoroacetabular tipo leva.

Long-term follow-up of neuroblastoma-associated opsoclonus-myoclonus-ataxia syndrome.

OBJECTIVE: The aim of this study is to describe the long-term neurological, neuropsychological and neuroradiological sequelae and to determine prognostic factors for neurological outcome in children with neuroblastoma-associated opsoclonus-myoclonus-ataxia (OMA) syndrome. METHODS: Data on medical history were collected for the study patients. Examinations with grading of neurological signs, neuropsychological tests and brain magnetic resonance imaging with spectroscopy were performed during a follow-up clinic. RESULTS: Fourteen subjects entered the study. All had localized neuroblastoma and they were evaluated after a median of 7.8 years. Patients with a chronic/multiphasic neurological course received steroids combined with intravenous immunoglobulins in the majority of cases. 71% presented neurological sequelae and 62% had a full-scale IQ below the normal range. All patients showed at least some deficit in the neuropsychological functions assessed (language, visual-motor integration, memory, attention and motor ability). Long-term deficits were more frequently detected in patients with an interval of more than 2 months between OMA onset and its diagnosis, even if in most comparisons statistical significance was not reached. Cerebellar atrophy, observed in 36% of patients, was not associated with the neurological outcome. CONCLUSIONS: Persisting disability is present in most children with neuroblastoma-associated OMA. However, our results support the role of an early diagnosis of OMA in reducing sequelae and encourage the use of new immunosuppressive therapies.

Artroplastia bilateral de cadera en un tiempo quirúrgico: evaluación de complicaciones y grado de satisfacción de pacientes / Bilateral total hip arthroplasty

Objective: To assess the complication rate in bilateral total hip arthroplasty (THA) performed in one stage, and to evaluate the patient satisfaction after the surgery. Methods: We analyzed the pen-operative data from 40 patients who underwent bilateral total hip arthroplasty in one stage, between November 2002 and December 2008 in our institution. We registered the operative risk, based on the American Society of Anesthesiologists (ASA) risk score classification system, the type of anesthesia and postoperative complications. All patients completed a questionnaire by telephone regarding the level of satisfaction after the surgery. Results: Forty patients (80 THAs), 26 females and 14 males were evaluated. The average age was 50.7 years (19-76 years). Twenty-five patients (62.5 percent) had ASA 1 operative risk, 12 (30 percent ) ASA 2 and 3 patients (7.5 percent) had ASA 3. Thirty-eight (95 percent) patients received combined anesthesia and 25 patients (62.5 percent) hypotensive anesthesia. Twelve patients (30 percent) received blood transfusion during or after the surgery. The average length of stay was 7.8 days. One patient (2.5 percent) had an intraoperative incomplete calcar fracture and another patient (2.5 percent) had local wound infection. No other complications were reported. Thirty-nine (97.5 percent) patients answered the telephonic questionnaire on July 2009, after a mean follow up of 33.6 months (6-78 months). Thirty-eight (97.4 percent) of them were very satisfied after the surgery. Conclusion: A bilateral total hip arthroplasty performed in one stage is a controlled low risk procedure in ASA 1 and 2 patients, with a high level of patient satisfaction after a short term follow up.

Objetivo: Evaluar la incidencia de complicaciones al realizar una artroplastia bilateral de cadera en un tiempo quirúrgico. Evaluar además el grado de satisfacción de los pacientes a corto plazo. Métodos: Análisis retrospectivo de 40 pacientes (26 mujeres, 14 hombres, edad promedio 50,7 años) operados de una artroplastia bilateral de cadera en un tiempo quirúrgico entre noviembre de 2002 y diciembre de 2008. Se revisaron los protocolos operatorios, las fichas clínicas realizándose además una encuesta telefónica para evaluar grado de satisfacción de los pacientes operados. Resultados: De los 40 pacientes, en relación al riesgo quirúrgico 25 pacientes (62,5 por ciento) correspondieron a ASA 1, 12 (30 por ciento) a ASA 2y3 (7,5 por ciento) a ASA 3. Veinticinco pacientes (62,5 por ciento) recibieron anestesia hipotensiva. Doce pacientes (30 por ciento) requirieron transfusión de glóbulos rojos durante la cirugía o en el postoperatorio. El tiempo de estadía hospitalaria fue de promedio 7,8 días. Un paciente (2,5 por ciento) presentó como complicación intraoperatoria una fractura incompleta del calcar y 1 (2,5 por ciento) como complicación local una infección superficial de herida operatoria. Ninguno presentó alguna complicación sistémica. Treinta y nueve pacientes se contactaron vía telefónica en junio de 2009 (tiempo de evolución 6-78 meses), 38 de estos (97,4 por ciento) manifestaron una alta satisfacción con la cirugía. Conclusión: La artroplastia bilateral de cadera en un tiempo quirúrgico es un procedimiento de bajo riesgo en pacientes seleccionados (ASA 1 y 2) y con un alto grado de satisfacción por parte de éstos a corto plazo.