Immune Response and Risk of Decompensation following SARS-CoV-2 Infection in Outpatients with Advanced Chronic Liver Disease.

Advanced chronic liver disease (ACLD) is associated with a wide spectrum of immune dysfunction. The clinical impact of SARS-CoV-2 on the development of decompensation and immune response in unvaccinated outpatients has not as yet been clearly defined. This study aimed to evaluate the clinical and immunological impact of SARS-CoV-2 on outpatients with ACLD. This is an observational case-control study, in which ACLD outpatients were included prospectively and consecutively and classified into two groups: SARS-CoV-2 infected and non-infected. Patients' baseline characteristics and infection data were collected and analyzed. Immunoglobulin G (IgG) levels against Spike 1 were evaluated. The primary endpoint was risk of liver decompensation during follow-up, assessed after propensity score matching and adjusted by Cox regression. Between October 2020 and July 2021, ACLD outpatients (n = 580) were identified, and 174 patients with clinical follow-up were included. SARS-CoV-2 infection incidence was 7.6% (n = 44). Risk of liver decompensation was significantly higher after infection (HR = 2.43 [1.01-5.86], p = 0.048) vs. non-infection. The time of IgG evaluation was similar in all patients (n = 74); IgG concentrations were significantly higher in compensated vs. decompensated patients (1.02 ± 0.35 pg/mL vs. 0.34 ± 0.16 pg/mL, p < 0.0001) and correlated with hemoglobin levels. The dysregulation of the innate immune response in patients with decompensated liver disease increased the risk of further decompensation following SARS-CoV-2, mainly due to a worsening of ascites.

Surgical Outcomes in Chiari 1 and Chiari 1.5 Malformation Treated by Posterior Fossa Reconstruction: A Comprehensive Analysis of 110 Pediatric Cases and Literature Review.

Background/Objectives: The management of Chiari malformations (CMs) remains a clinical challenge and a topic of great controversy. Results may vary between children and adults. The purpose of the current single-center study is to critically assess the one-year surgical outcomes of a cohort of 110 children with CM-1 or CM-1.5 who were treated using "posterior fossa reconstruction" (PFR), a surgical technique described in 1994 that has since been used in both adults and children. We also review the literature and discuss the possible causes of the drawbacks and pitfalls in children in whom PFR was ineffective in controlling the disease. Methods: The present cohort was selected from a prospective registry of adults and children with CMs collected since 2006. Patients included in this study were selected from a group of children with CMs who were operated on in our Pediatric Neurosurgical Unit between 1 January 2007 and 31 November 2023. Surgical outcome was defined based on clinical and neuroradiological results as very good, good, or bad. Results: The mean age of our child cohort was 9.9 ± 4.7 years, with 54 girls (49%) and 56 boys (51%). Sixty-six children had CM-1 (60%) while forty-four had CM-1.5 (40%). Following surgery, there was no neurological worsening or death among the children. Most children (70%) had an uneventful recovery and were discharged home on average one week after surgery. However, in 33 children (30%), we recorded at least one postoperative adverse event. Aseptic meningitis syndrome was the most frequent adverse event (n = 25, 22.7%). The final surgical outcome was evaluated one year after PFR by using both clinical and neuroradiological results. The one-year surgical outcome was excellent in 101 children (91.9%), good in 5 (4.5%), and bad in 4 (3.6%). Conclusions: PFR significantly enlarges the volume of the posterior fossa and recreates a CSF environment that generates buoyancy of the cerebellum, with a high percentage of excellent and good clinical results evaluated one year post-surgery.

Performance of glial fibrillary acidic protein (GFAP) and ubiquitin carboxy-terminal hydrolase L1 (UCH-L1) biomarkers in predicting CT scan results and neurological outcomes in children with traumatic brain injury (BRAINI-2 paediatric study): protocol of a European prospective multicentre study.

INTRODUCTION: In light of the burden of traumatic brain injury (TBI) in children and the excessive number of unnecessary CT scans still being performed, new strategies are needed to limit their use while minimising the risk of delayed diagnosis of intracranial lesions (ICLs). Identifying children at higher risk of poor outcomes would enable them to be better monitored. The use of the blood-based brain biomarkers glial fibrillar acidic protein (GFAP) and ubiquitin carboxy-terminal hydrolase-L1 (UCH-L1) could help clinicians in this decision. The overall aim of this study is to provide new knowledge regarding GFAP and UCH-L1 in order to improve TBI management in the paediatric population. METHODS AND ANALYSIS: We will conduct a European, prospective, multicentre study, the BRAINI-2 paediatric study, in 20 centres in France, Spain and Switzerland with an inclusion period of 30 months for a total of 2880 children and adolescents included. To assess the performance of GFAP and UCH-L1 used separately and in combination to predict ICLs on CT scans (primary objective), 630 children less than 18 years of age with mild TBI, defined by a Glasgow Coma Scale score of 13-15 and with a CT scan will be recruited. To evaluate the potential of GFAP and UCH-L1 in predicting the prognosis after TBI (secondary objective), a further 1720 children with mild TBI but no CT scan as well as 130 children with moderate or severe TBI will be recruited. Finally, to establish age-specific reference values for GFAP and UCH-L1 (secondary objective), we will include 400 children and adolescents with no history of TBI. ETHICS AND DISSEMINATION: This study has received ethics approval in all participating countries. Results from our study will be disseminated in international peer-reviewed journals. All procedures were developed in order to assure data protection and confidentiality. TRIAL REGISTRATION NUMBER: NCT05413499.

Home exercise, branched-chain amino acids, and probiotics improve frailty in cirrhosis: A randomized clinical trial.

BACKGROUND: Frailty is a predictive factor of hospitalization, falls, and mortality in patients with cirrhosis, regardless of the degree of liver failure. The aim was to analyze whether a multifactorial intervention consisting of home-based exercise, branched-chain amino acids, and a multistrain probiotic can improve frailty in these patients. METHODS: Outpatients with cirrhosis were classified according to the Liver Frailty Index (LFI). Prefrail and frail patients were randomized into 2 groups. The intervention group was assigned to a multifactorial intervention consisting of exercise at home, branched-chain amino acid supplements, and a multistrain probiotic for 12 months. The control group received standard care. All patients were prospectively followed up every 3 months for 1 year to determine LFI, incidence of falls, emergency room visits, hospitalizations, and mortality. RESULTS: Thirty-two patients were included: 17 patients were assigned to the intervention group and 15 to the control group. In the intervention group, the baseline LFI decreased at 3, 6, 9, and 12 months (p = 0.019 for overall change with respect to the control group). The change in LFI (ΔLFI) at 12 months was -0.71 ± 0.24 in the intervention group and -0.09 ± 0.32 in the control group (p<0.001). During follow-up, patients in the intervention group had a lower 1-year probability of falls (6% vs. 47%, p = 0.03) and emergency room visits (10% vs. 44%, p = 0.04) than patients in the control group. CONCLUSIONS: A long-term multifactorial intervention that included exercise at home, branched-chain amino acids, and a multistrain probiotic improved frailty in outpatients with cirrhosis and was associated with a decrease in the incidence of clinical events such as falls and emergency room visits.

Influence of further decompensation on survival across clinical stages of decompensated cirrhosis: The role of portal hypertension and HVPG changes.

BACKGROUND AND AIMS: Decompensated-cirrhosis encompasses several stages with different prognosis, such as bleeding, ascites and bleeding-plus-ascites. Development of further-decompensation worsens survival, while non-selective ß-blockers (NSBBs) can modify the risk. However, how this applies to each stage is uncertain. We aimed to investigate, in each stage of decompensated-cirrhosis, the influence of further-decompensation on mortality and whether changes in portal-pressure (HVPG) under NSBBs influence these outcomes. METHODS: Patients with variceal bleeding were consecutively included differentiating those with bleeding-alone from those who also had ascites. Patients with ascites and high-risk varices referred for primary-prophylaxis were also investigated. A baseline haemodynamic study was performed and was repeated after 1-3-months under NSBBs. Outcomes were investigated by competing-risk. RESULTS: Totally 103 patients had bleeding-alone, 186 bleeding-plus-ascites and 187 ascites-alone. Mean follow-up was 32-months (IQR, 12-60). Patients with bleeding-plus-ascites had higher HVPG and were more hyperdynamic than patients with ascites-alone and these than those with bleeding-alone. At each stage, the mortality risk was more than twice in patients developing further-decompensation vs. those without (p < .001). In each stage, HVPG-decrease under NSBBs showed better discrimination to predict further-decompensation than the baseline MELD, Child-Pugh or HVPG, by time-dependent ROC-curves (c-statistic >70%). At each stage, patients without HVPG-decreases, either ≥10% or ≥20% from the baseline, had higher risk of further-decompensation (sHR from 2.43 to 6.73, p < .01) and worse survival. CONCLUSIONS: In each stage of decompensated cirrhosis, mortality risk significantly and very markedly increase with further-decompensation. HVPG-non-response to NSBBs may adequately stratify the risk of further decompensation and death, in each stage. This suggests potential benefit with pre-emptive therapies in HVPG-non-responders at each-stage.

An artificial intelligence-generated model predicts 90-day survival in alcohol-associated hepatitis: A global cohort study.

BACKGROUND AND AIMS: Alcohol-associated hepatitis (AH) poses significant short-term mortality. Existing prognostic models lack precision for 90-day mortality. Utilizing artificial intelligence in a global cohort, we sought to derive and validate an enhanced prognostic model. APPROACH AND RESULTS: The Global AlcHep initiative, a retrospective study across 23 centers in 12 countries, enrolled patients with AH per National Institute for Alcohol Abuse and Alcoholism criteria. Centers were partitioned into derivation (11 centers, 860 patients) and validation cohorts (12 centers, 859 patients). Focusing on 30 and 90-day postadmission mortality, 3 artificial intelligence algorithms (Random Forest, Gradient Boosting Machines, and eXtreme Gradient Boosting) informed an ensemble model, subsequently refined through Bayesian updating, integrating the derivation cohort's average 90-day mortality with each center's approximate mortality rate to produce posttest probabilities. The ALCoholic Hepatitis Artificial INtelligence Ensemble score integrated age, gender, cirrhosis, and 9 laboratory values, with center-specific mortality rates. Mortality was 18.7% (30 d) and 27.9% (90 d) in the derivation cohort versus 21.7% and 32.5% in the validation cohort. Validation cohort 30 and 90-day AUCs were 0.811 (0.779-0.844) and 0.799 (0.769-0.830), significantly surpassing legacy models like Maddrey's Discriminant Function, Model for End-Stage Liver Disease variations, age-serum bilirubin-international normalized ratio-serum Creatinine score, Glasgow, and modified Glasgow Scores ( p < 0.001). ALCoholic Hepatitis Artificial INtelligence Ensemble score also showcased superior calibration against MELD and its variants. Steroid use improved 30-day survival for those with an ALCoholic Hepatitis Artificial INtelligence Ensemble score > 0.20 in both derivation and validation cohorts. CONCLUSIONS: Harnessing artificial intelligence within a global consortium, we pioneered a scoring system excelling over traditional models for 30 and 90-day AH mortality predictions. Beneficial for clinical trials, steroid therapy, and transplant indications, it's accessible at: https://aihepatology.shinyapps.io/ALCHAIN/ .

The biochemical pattern defines MASLD phenotypes linked to distinct histology and prognosis.

BACKGROUND: MASLD can manifest as hepatocellular damage, which can result in mild elevation of aminotransferases. However, in some patients, MASLD presents with cholestatic pattern. OBJECTIVE: To assess the impact of the biochemical pattern on the natural course of MASLD, including liver damage in histology, the accuracy of non-invasive tests(NITs), and prognosis. METHODS: Multicenter study enrolling 2156 patients with biopsy-proven MASLD, who were classified based on their[ALT/ULN)]/[(ALP/ULN)] levels at the time of biopsy: (a) hepatocellular pattern(H), > 5; (b) mixed pattern(M),2-5; (c) cholestatic pattern(C), < 2. OUTCOMES: (a) histological evaluation of the single components of NAS, MASH, and fibrosis; (b) NITs and transient elastography assessing advanced fibrosis; (c) prognosis determined by the appearance of decompensated cirrhosis and death. RESULTS: Out of the 2156 patients, 22.9% exhibited the H-pattern, whilst 31.7% exhibited the C-pattern. Severe steatosis, ballooning, lobular inflammation, and MASH (56.4% H vs. 41.9% M vs. 31.9% C) were more common in H-pattern (p = 0.0001),whilst C-pattern was linked to cirrhosis (5.8% H vs. 5.6% M vs. 10.9% C; p = 0.0001). FIB-4(0.74(95% CI 0.69-0.79) vs. 0.83 (95% CI 0.80-0.85); p = 0.005) and Hepamet Fibrosis Score(0.77 (95% CI 0.69-0.85) vs. 0.84 (95% CI 0.80-0.87); p = 0.044)exhibited lower AUROCs in the H-pattern. The C-pattern[HR 2.37 (95% CI 1.12-5.02); p = 0.024], along with age, diabetes, and cirrhosis were independently associated with mortality. Most patients maintained their initial biochemical pattern during the second evaluation. CONCLUSIONS: The H-pattern exhibited greater necro-inflammation in the histology than the C-pattern, whereas the latter showed more cirrhosis. The accuracy of NITs in detecting fibrosis was decreased in H-pattern. The occurrence of decompensated events and mortality was predominant in C-pattern. Therefore, identifying MASLD phenotypes based on the biochemical presentation could be relevant for clinical practice.

Consensus-based recommendations for diagnosis and surgical management of cranioplasty and post-traumatic hydrocephalus from a European panel.

Introduction: Planning cranioplasty (CPL) in patients with suspected or proven post-traumatic hydrocephalus (PTH) poses a significant management challenge due to a lack of clear guidance. Research question: This project aims to create a European document to improve adherence and adapt to local protocols based on available resources and national health systems. Methods: After a thorough non-systematic review, a steering committee (SC) formed a European expert panel (EP) for a two-round questionnaire using the Delphi method. The questionnaire employed a 9-point Likert scale to assess the appropriateness of statements inherent to two sections: "Diagnostic criteria for PTH" and "Surgical strategies for PTH and cranial reconstruction." Results: The panel reached a consensus on 29 statements. In the "Diagnostic criteria for PTH" section, five statements were deemed "appropriate" (consensus 74.2-90.3 %), two were labeled "inappropriate," and seven were marked as "uncertain."In the "Surgical strategies for PTH and cranial reconstruction" section, four statements were considered "appropriate" (consensus 74.2-90.4 %), six were "inappropriate," and five were "uncertain." Discussion and conclusion: Planning a cranioplasty alongside hydrocephalus remains a significant challenge in neurosurgery. Our consensus conference suggests that, in patients with cranial decompression and suspected hydrocephalus, the most suitable diagnostic approach involves a combination of evolving clinical conditions and neuroradiological imaging. The recommended management sequence prioritizes cranial reconstruction, with the option of a ventriculoperitoneal shunt when needed, preferably with a programmable valve. We strongly recommend to adopt local protocols based on expert consensus, such as this, to guide patient care.

Impact of the COVID-19 pandemic on the incidence and type of infections in hospitalized patients with cirrhosis: a retrospective study.

Infections are a major cause of morbidity and mortality in cirrhosis, especially those caused by multi-drug resistant bacteria. During the COVID-19 pandemic, the incidence and type of infection in these patients may have been influenced by the restrictive measures implemented. We aimed to compare the infections in patients with cirrhosis hospitalized before the COVID-19 pandemic versus those hospitalized during the pandemic. We retrospectively compared infections in patients with cirrhosis hospitalized in the hepatology unit during the pre-pandemic period (3/2019-2/2020) with infections in patients hospitalized during the pandemic (3/2020-2/2021). Baseline characteristics, type of infections, type of bacteria, antimicrobial resistance and mortality were evaluated. There were 251 hospitalizations in 170 patients during the pre-pandemic period and 169 hospitalizations in 114 patients during the pandemic period. One or more infections were identified in 40.6% of hospitalizations during the pre-pandemic period and 43.8% of hospitalizations during the pandemic, P = 0.52. We found 131 infections in the pre-pandemic period and 75 infections during the pandemic. The percentage of nosocomial infections decreased in the pandemic period (25.3% vs. 37.4% in the pre-pandemic period, P = 0.06). We found a non-significant trend to a higher incidence of infections by multi-drug resistant organisms (MDRO) in the pandemic period than in the pre-pandemic period (6.5% vs. 4%). The incidence of infections was similar in both periods. However, during the pandemic, we observed a trend to a lower incidence of nosocomial infections with a higher incidence of MDRO infections.

Migració de la vàlvula de derivació ventriculoperitoneal a l’escrot / Migración de la válvula de derivación ventriculoperitoneal al escroto / Ventriculoperitoneal shunt valve migration into the scrotum

Introducció. La migració de la vàlvula de derivació ventriculoperitoneal (VDVP) és una complicació infreqüent, i quan es produeix a l’escrot és excepcional. Es presenta un cas de migració escrotal de la VDVP. Cas clínic. Lactant de sexe masculí amb antecedents de prematuritat i acondroplàsia, a qui es va col·locar una VDVP quan tenia un mes de vida. Al cap d’un mes de la cirurgia, acudeix a urgències per irritabilitat i augment de volum escrotal dret. Se li fa una radiografia que objectiva el catèter distal de la VDVP a la zona inguinal, i es confirma la migració escrotal del catèter. No presenta simptomatologia de disfunció valvular. Als tres mesos d’edat, mitjançant abordatge laparoscòpic, es tanca la persistència del conducte peritoneovaginal de manera percutània. Posteriorment no presenta complicacions inguinoescrotals durant un seguiment de 13 mesos. Comentari. En un pacient pediàtric prematur portador de VDVP, cal sospitar la persistència del conducte peritoneovaginal. Les troballes més freqüents són l’hidrocele i l’hèrnia inguinal, però també s’han descrit casos de migració escrotal de la VDVP. La migració escrotal es diagnostica per la troballa radiològica del catèter a la zona inguinal, i el tractament és el tancament del conducte peritoneovaginal. (AU)

Introducción. La migración de la válvula de derivación ventriculoperitoneal (VDVP) es una complicación infrecuente, y cuando se produce en el escroto es excepcional. Se presenta un caso de migración escrotal de la VDVP. Caso clínico. Lactante varón con antecedentes de prematuridad y acondroplasia, a quien se colocó una VDVP cuando tenía un mes de vida. Al mes de la cirugía acude a urgencias por irritabilidad y aumento de tamaño escrotal derecho. Se realiza una radiografía que objetiva el catéter de la VDVP a nivel inguinal, confirmando la migración escrotal del catéter. No presenta sintomatología de disfunción valvular. A los tres meses de edad, mediante abordaje laparoscópico, se realiza el cierre de la persistencia del conducto peritoneovaginal de manera percutánea. Posteriormente no presenta complicaciones inguinoescrotales durante un seguimiento de 13 meses. Comentario. En un paciente pediátrico portador de VDVP, se debe sospechar la persistencia del conducto peritoneovaginal. Los hallazgos más frecuentes son el hidrocele y la hernia inguinal, pero también se han descrito casos de migración escrotal de la VDVP. La migración escrotal se diagnostica por el hallazgo radiológico del catéter a nivel inguinal, y su tratamiento es el cierre del conducto peritoneovaginal. (AU)

Introduction. Ventriculoperitoneal shunt (VPS) valve migration is an infrequent complication, and when it occurs to the scrotum is exceptional. A case of scrotal migration of the VPS valve is presented. Case report. A male infant with a history of prematurity and achondroplasia underwent VPS placement at one month of age. One month after the surgery, he was seen in the Emergency Room due to irritability and increased right scrotal size. An X-ray showed the tip of the VPS catheter at the inguinal level, confirming the scrotal migration. Symptoms of valve dysfunction were not present. At three months of age, percutaneously closure of the persistent peritoneovaginal duct was performed laparoscopically, with no additional complications recorded at a 13-month follow-up. Comments. The persistence of the peritoneovaginal duct must always be considered in children with VPS. The most frequent findings are hydrocele and inguinal hernia, but cases of scrotal migration have also been described. Scrotal migration of the VPS can be diagnosed by the radiological finding of the tip of the catheter at the inguinal level, and treatment is the closure of the peritoneovaginal duct. (AU)

Documento de posicionamiento de la «Societat Catalana de Digestologia» sobre elastografía hepática 2022 / Clinical practice guidelines of the Catalan Society of Gastroenterology about hepatic elastography 2022

Introducción: Después de casi 20 años utilizando la elastografía de transición para el diagnóstico no invasivo de la fibrosis hepática, su uso se ha extendido al cribado poblacional, la evaluación de la esteatosis y las complicaciones de la cirrosis. Por ello, la «Societat Catalana de Digestologia» encargó a un grupo de expertos actualizar el primer Documento realizado en 2011. Material y métodos: El grupo de trabajo (8 médicos y 4 enfermeras) elaboró un panel de preguntas en base a la encuesta online «Elastografía Hepática en Cataluña 2022» siguiendo la estructura PICO y el método Delphi. Resultados: Las respuestas se presentan con el nivel de evidencia, el grado de recomendación y el consenso final tras ser evaluadas por 2 revisores externos. Conclusión: La elastografía de transición utiliza el método elastográfico más sencillo y fiable para cuantificar la fibrosis hepática, evaluar la esteatosis y conocer el riesgo de complicaciones en pacientes con cirrosis. El documento ha sido avalado por la «Societat Catalana de Digestologia» y el «Col legi Oficial d’Infermeres i Infermers de Barcelona». (AU)

Introduction: After almost 20 years using transient elastography (TE) for the non-invasive diagnosis of liver fibrosis, its use has been extended to population screening, evaluation of steatosis and complications of cirrhosis. For this reason, the «Catalan Society of Gastroenterology» commissioned a group of experts to update the first document carried out in 2011. Material and methods: The working group (8 doctors and 4 nurses) prepared a panel of questions based on the online survey «Hepatic Elastography in Catalonia 2022» following the PICO structure and the Delphi method. Results: The answers are presented with the level of evidence, the degree of recommendation and the final consensus after being evaluated by two external reviewers. Conclusion: Transient elastography uses the simplest and most reliable elastographic method to quantify liver fibrosis, assess steatosis, and determine the risk of complications in patients with cirrhosis. The document has been endorsed by the “Catalan Society of Gastroenterology” and the “Col·legi Oficial d’Infermeres i Infermers de Barcelona”. (AU)

Abordaje laparoscópico de la acalasia. Resultados clínicos, de calidad de vida y funcionales a largo plazo / Laparoscopic approach of achalasia. Long-term clinical and functional results and quality of life

INTRODUCCIÓN: La miotomía laparoscópica se ha convertido en el tratamiento de elección en los pacientes con acalasia. Este estudio evalúa a largo plazo el estado clínico, de calidad de vida y los resultados funcionales posteriores a la miotomía de Heller laparoscópica. MATERIAL Y MÉTODOS: Se analizó a los pacientes diagnosticados de acalasia primaria e intervenidos mediante una miotomía de Heller laparoscópica con un procedimiento antirreflujo desde octubre de 1998 hasta diciembre de 2010. Previamente a la intervención, se evaluó la sintomatología de los pacientes mediante un cuestionario específico (escala DeMeester) y desde el 2002 se evaluó la calidad de vida mediante un test validado (GIQLI). En el año 2011 se reevaluó el estado clínico y de calidad de vida, y además se realizó una manometría y una pHmetría de 24h. En función del tiempo de seguimiento, los pacientes fueron divididos en 3 grupos (grupo 1: seguimiento entre 6 y 47 meses; grupo 2: seguimiento entre 48 y 119 meses y grupo 3, seguimiento superior a 120 meses). Además, 27 pacientes de la serie fueron evaluados con el mismo protocolo en 2003 y reevaluados en 2011. RESULTADOS: Noventa y cinco pacientes fueron incluidos en el estudio. El 80% (n = 76) estuvieron disponibles para realizar el seguimiento. El tiempo de seguimiento medio fue de 56 meses (rango 6-143 meses). La mejoría global de la disfagia fue del 89%. Los valores estudiados mediante la escala de DeMeester descendieron significativamente en los 3 grupos y los índices de calidad de vida se normalizaron posteriormente a la cirugía. Las determinaciones manométricas mostraron la normalización mantenida en la presión del esfínter esofágico inferior en todos los grupos. Se detectó una incidencia de reflujo gastroesofágico del 10%. En el subgrupo de pacientes analizados prospectivamente en 2003 y 2011, no se demostró empeoramiento de los resultados a largo plazo. CONCLUSIONES: La miotomía de Heller laparoscópica mejora la clínica y la calidad de vida de los pacientes con acalasia primaria manteniendo las presiones del esfínter esofágico inferior normales a largo plazo, con una baja tasa de incidencia de reflujo gastroesofágico

INTRODUCTION: Laparoscopic Heller myotomy has become the gold standard procedure for patients with achalasia. This study evaluates the clinical status, quality of life, and functional outcomes after laparoscopic Heller myotomy. MATERIAL AND METHODS: We analyzed patients who underwent laparoscopic Heller myotomy with an associated anti-reflux procedure from October 1998 to December 2010. Before surgery, we administered a clinical questionnaire and as of 2002, we also evaluated quality of life using a specific questionnaire (GIQLI). In 2011, we performed a follow up for all available patients. We administered the same clinical questionnaire and quality of life test as before surgery and performed manometry and 24-hour pH monitoring. According to the length of follow up, patients were divided into 3 groups. Group 1 with a follow-up between 6 and 47 months; group 2 follow-up between 48 and 119 months, and group 3 with a follow-up of more than 120 months). Moreover, 27 patients had already been evaluated with this same protocol in 2003. Pre- and postoperative data were compared for the 3 groups and for patients who completed follow up in 2003 and 2011. RESULTS: Ninety-five patients underwent laparoscopic Heller myotomy. Seventy-six (80%) were available for follow-up. Mean follow-up was 56 months (range 6-143). Global improvement in dysphagia was 89%. Total DeMeester score decreased in the 3 groups. GIQLI scores improved after surgery, reaching normal values. Manometric determinations showed normal LES pressures after myotomy in the 3 groups. Ten percent of overall 24-hour pH monitoring was abnormal. The group of patients followed up in 2003 and in 2011 showed no impairment in the variables studied in the long term. CONCLUSIONS: Long-term follow up of the laparoscopic approach to achalasia showed good results concerning clinical status and quality of life, with normal sphincteric pressures and a low incidence of gastroesophageal reflux

Potenciales evocados auditivos del tronco cerebral y somatosensoriales en los pacientes con malformación de Chiari / Brainstem auditory evoked potentials and somatosensory evoked potentials in Chiari malformation

Introducción: La malformación de Chiari (MC) incluye una serie de anomalías congénitas que tienen como común denominador la ectopia de las amígdalas del cerebelo por debajo del foramen magno, lo que puede condicionar fenómenos compresivos del troncoencéfalo, la médula espinal alta y los nervios craneales, alterando las respuestas de los potenciales evocados auditivos del tronco cerebral (PEATC) y de los potenciales evocados somatosensoriales (PESS). Sin embargo, las indicaciones de ambas exploraciones en las MC han sido motivo de estudio en un número limitado de publicaciones, centradas en series cortas y heterogéneas de pacientes. Objetivo: Revisar los hallazgos de los PEATC y los PESS en los estudios publicados en pacientes con MC tipo 1 (MC-1) o tipo 2 (MC-2), y su indicación en el diagnóstico, tratamiento y seguimiento, especialmente en la MC-1. Desarrollo: Es un estudio de revisión realizado mediante análisis de los estudios publicados en Medline desde 1966, localizados mediante PubMed, utilizando combinaciones de las palabras clave 'Chiari malformation', 'Arnold-Chiari malformation', 'Chiari type 1 malformation', 'Arnold-Chiari type 1 malformation', 'evoked potentials', 'brainstem auditory evoked potentials' y 'somatosensory evoked potentials', así como información de pacientes con MC-1 valorados en los servicios de neurocirugía y neurofisiología clínica del Hospital Universitari Vall d'Hebron. Conclusiones: Los hallazgos más comunes de los PESS son la reducción en la amplitud cortical para el nervio tibial posterior, la reducción o ausencia del potencial cervical del nervio mediano y el aumento del intervalo N13-N20. En el caso de los PEATC, los hallazgos más frecuentes descritos son el aumento del intervalo I-V y la alteración periférica o coclear

Introduction: Chiari malformations (CM) include a series of congenital anomalies involving the descent of the cerebellar tonsils below the foramen magnum, which may be associated with compression in the brainstem, upper spinal cord, and cranial nerves, consequently altering the responses of brainstem auditory evoked potentials (BAEP) and somatosensory evoked potentials (SSEP). However, only a small number of authors have described the indications of these tests in CM, and study groups to date have been small and heterogeneous. Aim: To review the results of BAEPs and SSEPs in published studies of patients with Chiari type 1 malformation (CM-1) or Chiari type 2 malformation (CM-2) as well as the indications of both tests in the diagnosis, treatment, and monitoring of both diseases, especially CM-1. Development: We present a review article analyzing data from all published studies in Medline starting in 1966, located through PubMed, using combinations of the following keywords: 'Chiari malformation', 'Arnold-Chiari malformation', "Chiari type 1 malformation", "Arnold-Chiari type 1 malformation", 'evoked potentials', 'brainstem auditory evoked Potentials' and 'somatosensory evoked potentials' as well as records of patients with CM-1 from the neurosurgery and neurophysiology departments at the Hospital Universitari Vall d'Hebron. Conclusions: Common findings of SSEP are a reduction in cortical amplitude from the posterior tibial nerve, a reduction or absence of cervical median nerve potential, and an increased N13-N20 interval. In BAEP, the most frequent findings are an increased I-V interval and a peripheral or cochlear auditory disturbance

Evaluación de la calidad de vida en los pacientes con una malformación de Chiari tipo I. Estudio piloto en una cohorte de 67 pacientes / Evaluation of the quality of life of patients with a Chiari type I malformation. A pilot study in a cohort of 67 patients

Introducción. La malformación de Chiari tipo I (MC-I) es una entidad de baja prevalencia, cuya clínica es muy variada dependiendo del cortejo malformativo asociado, y en la que pueden existir síntomas ansiosodepresivos y una pérdida nodefinida de la calidad de vida de los pacientes. El objetivo principal de este estudio es determinar la repercusión de la MC-I en la calidad de vida, así como la presencia de ansiedad y depresión en estos pacientes. Pacientes y métodos. Estudio prospectivo de una cohorte de 67 pacientes afectos de MC-I en los que se valoraron la calidadde vida, la presencia de ansiedad y síntomas depresivos mediante las escalas Sickness Impact Profile, inventario de ansiedad estado-rasgo e inventario de depresión de Beck, respectivamente. En cada paciente se registró la presencia de siringomielia o hidrocefalia, así como el grado de ectopia de las amígdalas del cerebelo. Resultados. El impacto de la MC-I en la calidad de vida fue nulo en seis pacientes (9%), leve en 36 (53,7%), moderado en 17 (25,4%) y grave en ocho (11,9%). El área de actividad más afectada fue el trabajo. El 86,6% de los pacientes presentóun nivel de ansiedad moderado o elevado. En el 25,4% de los pacientes también se constataron síntomas depresivos moderados o graves. Conclusiones. La gran mayoría de los pacientes con una MC-I considera que su enfermedad implica una pérdida de calidadde vida que, en muchos casos, se asocia a una alta ansiedad y a síntomas depresivos (AU)

Introduction. The Chiari type I malformation (CM-I) is a low prevalence disorder whose manifestations vary highly, depending on the associated malformative complex. The people with a CM-I can suffer anxiety, depression symptoms and an undefined loss of quality of life. The main purpose of this study is to establish the impact of CM-I on quality of life, as well as the presence of anxiety and depression in these patients. Patients and methods. Prospective study of a cohort of 67 patients suffering from CM-I who undergo an evaluation bymeans of the SIP scale (Sickness Impact Profile), STAI (State-Trait Anxiety Inventory) and BDI (Beck’s Depression Inventory) of their quality of life and of the presence of anxiety and depressive symptoms respectively. For every patient the degree of cerebellar tonsillar ectopia and the presence of syringomyelia and/or hydrocephalus were registered.Results. The impact of the CM-I on the quality of life was none for 6 patients (9%), mild for 36 (53.7%), moderate for 17 (25.4%) and severe for 8 (11.9%). The most affected area of activity was work. A total of 86.6% of the patients presented a moderate or high anxiety level. In 25.4% of the patients moderate or severe depressive symptoms were also acknowledged. Conclusions. The great majority of patients with a CM-I consider that their disorder implies a loss of their quality of lifewhich, in many cases, is associated with high anxiety and depressive symptoms (AU)

Déficit neuropsicológicos en la hidrocefalia crónica del adulto: definición y recomendaciones para su diagnóstico / Neuropsychological impairments in adult chronic hydrocephalus: definition and some suggestions for assessment

En 1965, Hakim y Adams publican la descripción clínica de un nuevo síndrome, la hidrocefalia "normotensiva" o hidrocefalia crónica del adulto (HCA), caracterizado por alteración de la marcha acompañada de deterioro cognitivo y de trastorno en el control de los esfínteres, junto con la presencia de dilatación ventricular. Los pacientes con HCA presentan un patrón de deterioro neuropsicológico fronto-subcortical. La presencia de signos corticales sugiere la existencia de una demencia añadida. Esta situación puede explicar la amplia variación existente en la gravedad y tipo de déficit cognitivos presentados por los pacientes con HCA antes y después del tratamiento quirúrgico. El tratamiento quirúrgico de la HCA puede revertir o al menos reducir la sintomatología que presentan estos pacientes. Sin embargo, hay una gran heterogeneidad en los resultados tras el tratamiento que ha sido principalmente atribuida a los criterios de selección de los pacientes. Por este motivo y por los posibles riesgos que acompañan a la cirugía, existe abundante literatura que pretende encontrar o mejorar los instrumentos diagnósticos. En este artículo se hace una revisión de la investigación existente en torno a los déficit neuropsicológicos que presentan los pacientes con una HCA y sus métodos diagnósticos (AU)