Research advances in the diagnosis and treatment of hepatic vein occlusion-type Budd-Chiari syndrome / 临床肝胆病杂志

Budd-Chiari syndrome is a rare liver vascular disease and can be classified into hepatic vein occlusion type， inferior vena cava occlusion type， and mixed type according to the location of hepatic outflow tract obstruction. This article reviews the hepatic vein occlusion type from the aspects of epidemiology， subtypes， clinical diagnosis， and treatment strategies.

Descompresión de fosa posterior sin duroplastia expansiva en adulto con malformación de Chiari tipo 1-siringomielia

Introducción: La malformación de Chiari tipo 1 incluye un grupo heterogéneo de malformaciones congénitas, caracterizadas por el descenso caudal del cerebelo a través del foramen magno. En un 30-70 % de los casos tiene siringomielia asociada. Existen controversias en torno a la técnica quirúrgica ideal. Objetivo: Presentar un caso de MC-1 asociada a siringomielia en el que no se aplica una duroplastia expansiva. Presentación de caso: Paciente femenina de 43 años, con antecedentes de hipertensión arterial. Acudió a consulta neuroquirúrgica por dolor cervical irradiado al miembro superior derecho. Al examen neurológico mostró signos de afectación de primera y segunda motoneurona. La resonancia magnética confirmó el diagnóstico de MC-1. Se intervino quirúrgicamente mediante descompresión de fosa posterior sin duroplastia expansiva. La paciente evolucionó sin complicaciones y egresó a las 48 horas. Durante el seguimiento mejoraron las manifestaciones parestésicas; sin embargo, el examen neurológico se mantuvo igual. A los seis meses, la resonancia magnética indicó una marcada disminución de la siringomielia y la reconformación de la cisterna magna. Hasta los 18 meses del tratamiento, los síntomas no habían empeorado y la capacidad funcional resultaba aceptable (Karnofsky 90/100). Conclusiones: La descompresión de fosa posterior sin duroplastia expansiva, seguida de re-permeabilización microquirúrgica del foramen de Magendie, tuvo resultados favorables en este caso.

Introduction: Chiari malformation type 1 includes a heterogeneous group of congenital malformations, characterized by caudal descent of the cerebellum through the foramen magnum. It has associated syringomyelia in 30-70% of cases. Controversies exist regarding the ideal surgical technique. Objective: To present a case of MC-1 associated with syringomyelia in which an expansive duroplasty is not applied. Case report: Female patient, 43 years old, with history of arterial hypertension. She went to the neurosurgical consultation for cervical pain radiating to the right upper limb. On neurological examination, she showed signs of first and second motor neuron involvement. MRI confirmed the diagnosis of MC-1. She underwent surgery by decompression of the posterior fossa without expansive duroplasty. The patient evolved without complications and she was discharged after 48 hours. During the follow-up, the paresthetic manifestations improved; however, the neurological examination remained the same. At six months, MRI indicated a marked decrease in syringomyelia and reshaping of the cisterna magna. Until 18 months after treatment, symptoms had not worsened and functional capacity was acceptable (Karnofsky 90/100). Conclusions: Posterior fossa decompression without expansive duroplasty, followed by microsurgical re-permeabilization of Magendie's foramen, had favorable results in our case.

Rare Association of Budd-Chiari Syndrome with COVID-19: A Case Report

Introduction: Severe acute respiratory syndrome coronavirus type 2 (SARS-CoV-2) is a virus that can cause respiratory infections and pose a risk to patients' lives. While it primarily affects the airways, it can also lead to extrapulmonary clinical manifestations, such as hypercoagulable states, resulting in conditions like cerebrovascular disease, acute myocardial infarction, and, in rare cases, Budd-Chiari syndrome. Clinical case: This case involves a patient who was admitted to the emergency room with dyspnea on moderate exertion, progressive functional class deterioration, lower extremity edema, cough, fever, and weakness. The patient had a history of coronavirus disease 2019 (COVID-19) infection one month prior. Abdominal CT scan revealed subacute Budd-Chiari syndrome involving the middle sub-hepatic vein, along with right ventricle thrombosis, and a positive test for SARS-CoV-2. Conclusions: Budd-Chiari syndrome has multiple causes, primarily associated with hypercoagulable states or the presence of neoplasms that disrupt liver function or obstruct hepatic venous drainage. However, the understanding of the relationship between COVID-19 hypercoagulability and Budd-Chiari syndrome is still limited. Further research is needed to explore the heterogeneity of its pathogenesis in the context of the SARS-CoV-2 pandemic.

Introducción: el coronavirus del síndrome respiratorio agudo grave de tipo 2 (SARS-CoV-2) es un virus que puede causar infecciones respiratorias y poner en riesgo la vida de los pacientes. Aunque afecta principalmente las vías respiratorias, se pueden presentar manifestaciones clínicas extrapulmonares como estados de hipercoagulabilidad, lo que causa patologías como enfermedad cerebrovascular e infarto agudo de miocardio, y en casos menos frecuentes, síndrome de Budd-Chiari. Caso clínico: se presenta el caso de una paciente que ingresó al servicio de urgencias por disnea de medianos esfuerzos, deterioro progresivo de la clase funcional y edema en extremidades inferiores asociado con tos, fiebre y adinamia. Refirió el antecedente de infección por enfermedad por coronavirus de 2019 (COVID-19) un mes antes y la tomografía de abdomen contrastada reveló síndrome de Budd-Chiari subagudo de la vena subhepática media asociada a trombosis del ventrículo derecho con prueba positiva para SARS-CoV-2. Conclusiones: El síndrome de Budd-Chiari es de origen multicausal, principalmente por estados de hipercoagulabilidad o presencia de neoplasias que ocasionan disrupción de la función del hígado o causan la ocupación de espacio sobre el drenaje venoso hepático; sin embargo, en el contexto de la pandemia de SARS CoV-2, aún se conoce muy poco la heterogeneidad de su patogénesis, como es la hipercoagulabilidad de la COVID-19 y el síndrome de Budd-Chiari, por lo que inferimos que están altamente relacionados.

Malformación de Arnold-Chiari tipo I con siringomielia

Introducción: Las malformaciones de Arnold-Chiari, son un grupo heterogéneo de desórdenes neurológicos caracterizados por alteraciones dentro de las regiones del cerebelo, tallo cerebral y unión cráneo-cervical; todas resultan en un desplazamiento inferior del cerebelo hacia el canal espinal por el foramen magno, sea en conjunto con la médula inferior o no. Objetivo: Describir los hallazgos clínicos, estudios complementarios, criterios diagnósticos, conducta terapéutica y evolución, en un caso con malformación de Chiari tipo I con siringomielia. Presentación de caso: Se presenta a una paciente con cefalea de curso insidioso a la que se le diagnostica malformación de Chiari tipo I con siringomielia, que requirió tratamiento neuroquirúrgico. La evolución posoperatoria fue favorable. Conclusiones: La malformación de Arnold-Chiari es una entidad rara; la aparición de la resonancia magnética nuclear ha permitido que su diagnóstico aumente y por tanto, su incidencia. El caso presentado tuvo un curso clínico clásico.

Introduction: Arnold-Chiari malformations are a heterogeneous group of neurological disorders characterized by alterations within the regions of the cerebellum, brain stem and craniocervical junction; all result in inferior displacement of the cerebellum into the spinal canal through the foramen magnum, whether in conjunction with the inferior cord or not. Objective: To describe the clinical findings, complementary studies, diagnostic criteria, therapeutic approach and evolution, in a case with type I Chiari malformation with syringomyelia. Case report: A patient with an insidious course of headache is reported. She was diagnosed with type I Chiari malformation with syringomyelia, which required neurosurgical treatment. The postoperative evolution was favorable. Conclusions: Conclusions: Arnold-Chiari malformation is a rare entity. the appearance of nuclear magnetic resonance has allowed it to be diagnosed and therefore its incidence has increased. The reported case had a classic clinical course.

Experiencia en patología cervical alta en el Hospital Guillermo Almenara Irigoyen 2016 - 2021

Introducción : El complejo C0-C1-C2 es responsable de la transición de la carga axial, con función biomecánica única, siendo afectada por múltiples patologías, que por lo general la literatura no las considera como un solo ítem, sino que lo desarrolla según su etiología, pero en nuestro estudio se ha considerado en 5 grupos: traumática, congénita, inflamatoria reumática, neoplásica y degenerativa. Objetivo : Determinar las características epidemiológicas, clínicas y del tratamiento en la patología cervical alta. Materiales y métodos : Se incluyeron a todos los pacientes con diagnóstico clínico radiológico de alguna patología cervical alta que hayan sido sometidos a tratamiento quirúrgico entre 2016 y 2021 en el Hospital Almenara. Se usó el test "t" de student y de chi cuadrado. Se dividió a los pacientes en alguno de los 5 grupos antes mencionados. Resultados : Se consideraron 31 pacientes, con una edad media de 51.16 años. La patología cervical alta más frecuente fue la traumática con el 35.48%. El déficit motor se presentó en el 51.61% y el déficit sensitivo se presentó en el 54.84%. La cirugía más frecuente fue la fijación cervical alta con el 43.89%. La tasa de complicaciones fue del 16.13% con una mortalidad del 0%. Conclusiones : La patología cervical alta es rara, siendo la del tipo traumática la más frecuente, pero un manejo oportuno y adecuado permite un mejor pronóstico funcional del paciente.

Introduction : The C0-C1-C2 complex is responsible of axial load transition, and its biomechanical function is unique, it is affected by multiple pathological conditions; and generally speaking, the literature does not consider these conditions as a single item, it describes them according to etiology. For our study we considered five groups: trauma-related, congenital, rheumatic-inflammatory, neoplastic, and degenerative. Objective : To determine epidemiological, clinical, and therapy-related characteristics in upper cervical pathological conditions. Materials and methods : All patients with a clinical-radiological diagnosis of any upper cervical pathological condition that had undergone surgery between 2016 and 2021 in Guillermo Almenara Hospital were included. Student's t test and chi square methods were used. patients were divided into one of the five aforementioned groups. Results : Thirty-one patients were included in the study; their mean age was 51.16 years. The most frequent upper cervical pathological condition was trauma-related, with 35.48%. Motor deficit occurred in 51.61% of all patients, and sensitive deficit occurred in 54.84%. The most frequently surgical procedure performed was upper cervical fixation, in 43.89% of all patients. Complication rate was 16.13%, and mortality was 0%. Conclusions : Upper cervical pathological conditions are rare, trauma-related conditions are most frequent, but timely and adequate management allow us to achieve better functional prognosis for these patients.

Malformación de Chiari tipo I en una embarazada. Reporte de caso / Chiari malformation type I in pregnant women, case report

Resumen ANTECEDENTES: La malformación de Chiari es un padecimiento neurológico, congénito o adquirido, infrecuente. Se caracteriza por el desplazamiento de las estructuras rombencefálicas hacia el canal espinal, por debajo del foramen magno. Se desconoce la frecuencia y evolución que puede tener durante el embarazo porque existen cambios durante el trabajo de parto que pueden predisponer a la herniación del tronco encefálico con compresión medular. Esta situación plantea un desafío terapéutico porque no se dispone de recomendaciones unificadas acerca del momento del parto, vía del nacimiento y mejor técnica anestésica en estas pacientes. CASO CLÍNICO: Paciente primípara, de 24 años, con 34 semanas de embarazo. Acudió a Urgencias del Hospital Universitario de la Samaritana debido a un cuadro clínico de cefalea con signos de alarma. En la resonancia magnética cerebral se encontró una malformación de Chiari tipo I. Con el objetivo de limitar las maniobras de Vasalva durante el trabajo de parto se decidió la cesárea, con anestesia neuroaxial, sin complicaciones y con recién nacido sano. CONCLUSIÓN: La malformación de Chiari tipo I durante el embarazo es infrecuente. Las pacientes con diagnóstico previo al embarazo condicionan un seguimiento en el que se valora la indicación de tratamiento neuroquirúrgico antes de la concepción. Durante el embarazo no puede recomendarse un único método de finalización o anestesia y la atención médica debe estar a cargo un equipo multidisciplinario que formule un plan de atención individualizado para mejorar el desenlace materno y fetal.

Abstract BACKGROUND: Chiari malformation is a rare congenital or acquired neurological disorder, characterized by the displacement of the rhombencephalic structures towards the spinal canal below the level of the foramen magnum; The frequency and progression that can occur during pregnancy are unknown, since there are changes during labor that can predispose to brainstem herniation with spinal cord compression; which poses a therapeutic challenge since there are no unified recommendations about the moment of delivery, delivery route and the best anesthetic technique in these patients CLINICAL CASE: A 24-year-old patient, G1P0 with a 34-week pregnancy, consulted the emergency room of the Hospital Universitario de la Samaritana, due to a clinical profile of headache with warning signs; to the study of cerebral magnetic resonance with a finding of Chiari type I malformation. In order to limit the Valsalva maneuvers during labor, a caesarean section was performed at term under neuraxial anesthesia without complications with a healthy newborn. CONCLUSION: Chiari malformation type I during pregnancy is rare. Patients diagnosed prior to pregnancy condition a follow-up in which the indication for neurosurgical treatment prior to conception is assessed. During pregnancy, no single method of termination or anaesthesia can be recommended and medical care should be provided by a multidisciplinary team formulating an individualized care plan to improve maternal and fetal outcome.

Juvenile scoliosis as the first manifestation of syringomyelia associated with Chiari malformation type I / Escoliosis juvenil como primera manifestación de siringomielia asociada con malformación de Chiari tipo I

Abstract Background: Scoliosis is a spinal deformity that usually manifests as a structural curve determining a variable deformity of the trunk. According to some published series, 4 to 26% of scoliosis initially classified as idiopathic show neurological alterations when studied with nuclear magnetic resonance, such as syringomyelia and Chiari malformation, among the most frequent. Chiari malformations are characterized by descending herniation of the cerebellar tonsils, brainstem, and IV ventricle into the spinal canal. Case report: We present the case of a patient whose first symptomatic manifestation was early-onset scoliosis. The overlapping of some physical examination signs, such as postural lateralization and scoliosis, reinforced the active suspicion of neuroaxis alterations. Conclusions: Early childhood-onset scoliosis should raise a high degree of suspicion for association with neuro-spinal diseases. Although the frequency of Chiari malformation is low, its early approach could reduce the progression of associated comorbidities. An early detection could change the prognosis of the disease.

Resumen Introducción: La escoliosis es una deformidad de la columna que usualmente se manifiesta como una curva estructural que determina una deformidad variable del tronco. Las malformaciones de Chiari se caracterizan por la herniación descendente de las amígdalas cerebelosas, tronco encefálico y IV ventrículo hacia el canal espinal. Según algunas series publicadas, del 4 al 26% de las escoliosis inicialmente catalogadas como idiopáticas muestran alteraciones neurológicas al ser estudiadas con resonancia magnética nuclear, como la siringomielia y malformación de Chiari, dentro de las más frecuentes. Caso clínico: Se presenta el caso de un paciente cuya primera manifestación sintomática fue escoliosis de comienzo temprano. El solapamiento de algunos signos del examen físico, como la lateralización de la postura y la escoliosis, reforzaron la sospecha activa de alteraciones del neuroeje. Conclusiones: La escoliosis de inicio temprano en la infancia debe despertar un alto grado de sospecha de asociación con enfermedades neuro-espinales. Dentro de este contexto, aunque malformación Chiari es de baja frecuencia, su abordaje precoz permitiría disminuir la progresión de comorbilidades asociadas. La detección temprana podría cambiar el pronóstico de la enfermedad.

Research Progress on the Application of Goel Technique in Craniovertebral Junction Anomalies / 中国医学科学院学报

Craniovertebral junction anomalies are a group of diseases characterized by the pathological changes of occipital bone,atlantoaxial bone,cerebellar tonsil,surrounding soft tissue,and nervous system,which are caused by a variety of factors.Chiari malformation is a common type of craniovertebral junction anomalies,the conventional surgical therapy of which is posterior fossa decompression.Currently,scholars represented by Goel have proposed a new theory on the classification,pathogenesis,and treatment of Chiari malformation based on posterior atlantoaxial fixation (Goel technique).This article introduces the progress in Goel technique,aiming to provide reference for the clinical work.

Transabdominal pericardial anastomosis of suprahepatic vena cava and right atrium in liver transplantation for Budd-Chiari syndrome complicated with liver cancer: a case report with surgical video / 器官移植

Objective To summarize clinical experience of transabdominal pericardial anastomosis of suprahepatic vena cava of the donor and right atrium of the recipient in liver transplantation for Budd-Chiari syndrome (BCS) complicated with liver cancer. Methods Clinical data of a BCS patient complicated with liver cancer undergoing transabdominal pericardial anastomosis of suprahepatic vena cava and right atrium in liver transplantation were retrospectively analyzed. Results The hepatic vein and suprahepatic vena cava were partially occluded in the patient. Liver transplantation was completed by transabdominal pericardial anastomosis of suprahepatic vena cava and right atrium with beating-heart. In addition, due to pathological changes of the recipient's hepatic artery, splenic artery of the recipient was cut off, distal ligation was performed, and the proximal end was reversed and anastomosed with the common hepatic artery of the donor liver, and the reconstruction of hepatic artery was completed. The surgery was successfully performed. At approximately postoperative 1 week, the function of the liver allograft was gradually restored to normal, and no major complications occurred. The patient was discharged at postoperative 25 d. No signs of BCS recurrence was reported after 8-month follow-up. Conclusions It is safe and feasible to treat BCS by liver transplantation with transabdominal pericardial anastomosis of suprahepatic vena cava and right atrium. BCS patients complicated with liver cancer obtain favorable prognosis.

Research progress of Chiari malformation type I and atlantoaxial instability / 中华骨科杂志

Chiari malformation (CM) is a group of congenital cerebellar tonsillar hernia malformations involving the craniocervical junction. Chiari malformation type I (CMI) is the most common in clinic, however its pathogenesis is still unclear, and there is no consensus on the surgical treatment standard of CMI. At present, the most widely accepted is the theory of posterior fossa incompatibility, so doctors at home and abroad use posterior fossa decompression (PFD) and posterior fossa compression with duraplasty (PFDD) as the gold standard for surgical treatment, and have their own experience and technical improvement. However, the volume of the posterior cranial fossa in some patients is no different from that in healthy people, and about 30% of the patients with CMI have poor results after posterior cranial fossa decompression. As a result, this operation cannot treat all patients with CMI. In recent years, with the development of imaging, the progress of diagnostic technology and the deepening of understanding of CM, some studies have shown that CMI may be related to atlantoaxial instability, and proposed that CMI is the secondary factor of atlantoaxial instability, and atlantoaxial fusion is the standard of surgical treatment, which has caused great controversy in academic circles. Different clinical research results of scholars support or oppose this theory: some studies have shown that the clinical symptom relief rate of patients with CMI treated with atlantoaxial fusion is 96.9%; another study showed that 70% of patients with CMI underwent atlantoaxial fusion had improved neurological function, but the overall postoperative effect was not satisfactory. In short, CMI is related to many diseases and its clinical manifestations are complex. Therefore, individualized management and treatment should be carried out in combination with the clinical manifestations and auxiliary examination results of patients.

Discussion of issues related to Budd-Chiari syndrome / 国际外科学杂志

Budd-Chiari syndrome (B-CS) is a rare disease caused by hepatic vein outflow obstruction, and its etiology is complex and inconclusive. Current studies suggest that vascular dysplasia, gut microbiota and trace element imbalance may be related to the pathogenesis of B-CS, and the development of high-throughput sequencing technology may help to clarify the exact pathogenesis of B-CS. The symptoms of B-CS are not specific and rely mainly on imaging methods to establish the diagnosis, so there is an urgent need to find new noninvasive biological diagnostic markers. In addition, there are many pathological types and different criteria of B-CS, which mostly can′t fully reflect the pathophysiological changes of B-CS patients and guide clinical treatment. Therefore, we recommend pathophysiological classification according to the hemodynamic changes and collateral circulation compensation of B-CS, and then develop personalized treatment strategies for stratified management different from the traditional early diagnosis and treatment protocols. This article summarizes and discusses the above contents.

Alternativas de manejo intervencionista en el síndrome de Budd-Chiari. Serie de casos y revisión de la literatura / Interventional management alternatives in Budd-Chiari syndrome. Series of cases and review of the literature

La obstrucción en el tracto de salida del flujo venoso hepático, también conocida como síndrome de Budd-Chiari, es una condición infrecuente que causa congestión hepática, hipertensión portal, ne-crosis de los hepatocitos y, eventualmente, falla hepática aguda o crónica. Actualmente, el manejo de esta condición representa un reto para el médico, quien debe estar preparado para determinar la mejor alternativa entre las diferentes opciones terapéuticas disponibles. Este artículo pretende ilus-trar las alternativas del manejo intervencionista de esta enfermedad, a través de una serie de casos de pacientes tratados en el servicio de Radiología Intervencionista de un hospital de referencia de la ciudad de Medellín, entre 2011 y 2017.

Hepatic venous outflow tract obstruction, also known as Budd-Chiari syndrome, is a rare condition that causes hepatic congestion, portal hypertension, hepatocyte necrosis and eventually acute or chronic liver failure. Currently, the management of this condition represents a challenge for the physi-cian, who must be prepared to determine the best alternative among the different therapeutic options available. This article aims to illustrate the alternatives of interventional management of this disease, through a series of cases of patients treated in the Interventional Radiology service of a referral hos-pital in the city of Medellin, between 2011 and 2017.

Síndrome de Budd-Chiari secundario asociado a hepatitis alcohólica grave: reporte de caso / Secondary Budd-Chiari syndrome associated with severe alcoholic hepatitis: A case report

Resumen La hepatitis alcohólica es la inflamación aguda del hígado secundaria al consumo de alcohol en cantidades hepatotóxicas, su fisiopatología está influida por diversos factores asociados. El diagnóstico tiene tres escenarios: la hepatitis alcohólica probable, posible y definitiva. La hepatitis alcohólica probable se basa en el cumplimiento de criterios diagnósticos clínicos y laboratoriales; la hepatitis alcohólica posible corresponde a casos de cumplimiento de estos criterios, pero con presencia de factores potencialmente confusores del diagnóstico, y la definitiva se sustenta sobre una base histopatológica. El síndrome de Budd-Chiari es una entidad poco frecuente, heterogénea y potencialmente letal, caracterizada por la presencia de trombos a nivel de las venas suprahepáticas, así como de la vena cava inferior. El diagnóstico definitivo está basado en pruebas de imagen. Por su parte, el síndrome de Budd-Chiari secundario es una entidad aún más infrecuente, poco estudiada, cuyo diagnóstico es difícil debido a su gran similitud al síndrome de Budd-Chiari primario, tanto clínica como en imágenes diagnósticas, por lo que se tiene que acudir a métodos diagnósticos de mayor complejidad e incluso invasivos. A continuación, se presenta un caso inusual de un paciente con consumo crónico de alcohol que presenta síndrome de Budd-Chiari secundario asociado a hepatitis alcohólica grave confirmada mediante biopsia hepática y sometido a múltiples estudios de imagen que descartaron la presencia de trombosis a nivel de las venas suprahepáticas.

Abstract Alcoholic hepatitis is an acute inflammation of the liver secondary to the consumption of alcohol in hepatotoxic amounts; various associated factors influence its pathophysiology. The diagnosis has three scenarios: probable, possible, and definite alcoholic hepatitis. Probable alcoholic hepatitis is based on compliance with clinical and laboratory diagnostic criteria; possible alcoholic hepatitis corresponds to cases that meet these criteria but with potentially confounding factors in the diagnosis, and the definite one is based on histopathology. Budd-Chiari syndrome is a rare, heterogeneous, potentially lethal entity characterized by thrombi in the suprahepatic veins and the inferior vena cava. The final diagnosis relies on imaging tests. Moreover, secondary Budd-Chiari syndrome is an even rarer entity, little studied, whose diagnosis is difficult due to its remarkable similarity to primary Budd-Chiari syndrome, both clinically and in diagnostic images, for which more complex and even invasive diagnostic methods must be used. Then, we describe an unusual case of a male patient with chronic alcohol consumption presenting with secondary Budd-Chiari syndrome associated with severe alcoholic hepatitis confirmed by liver biopsy and subjected to multiple imaging studies that ruled out thrombosis in the suprahepatic veins.

Chiari Type 1 malformation: A case report

Type I Chiari malformation (CM) is an acquired or congenital disorder characterized by herniation of the cerebellar tonsils through the foramen magnum and into the spinal canal. The resulting anatomic abnormality impairs the flow of cerebral spinal fluid and results in the Chiari symptoms and signs. The purpose of this study was to report a case of successfully treated CM Type 1 that was discovered accidentally during childhood. A 9-year-old female presented to the emergency department complaining of dizziness and headache. Before her admission, she lost balance and collapsed to the ground fainting. A magnetic resonance imaging (MRI) scan of the brain disclosed herniation of cerebellar tonsils through the foramen magnum at 13 mm below the spinal canal. It is very difficult to determine the true frequency of this disorder early in childhood. A definitive diagnosis is generally made after a MRI, where the abnormal protrusion of the cerebellum toward the spinal cord can be seen

Lesão medular por traumatismo raquimedular e malformação de Chiari tipo I: uma comparação de características clínicas / Spinal cord injury and Chiari type I malformation: a comparison of clinical features

Foram comparadas características clínicas e epidemiológicas de pacientes com tetraplegia traumática e Malformação de Chiari tipo I, atendidos em centro de neurorreabilitação através de estudo observacional analítico e retrospectivo, realizado por meio de análise de prontuário eletrônico. No grupo de pacientes com Malformação de Chiari tipo I predominaram mulheres, com marcha e que apresentavam transtornos de ansiedade e a presença de siringomielia. No grupo com tetraplegia traumática predominaram os homens, com locomoção em cadeira de rodas e maior presença de espasticidade e disfunções neurogênicas do intestino e do trato urinário inferior. Os pacientes com Malformação de Chiari tipo I representam um grupo com diferentes características clínicas e epidemiológicas em relação aos pacientes com sequelas de traumatismo raquimedular, tradicionalmente o principal grupo atendido dentro dos programas de neurorreabilitação. Desta forma, os pacientes com Malformação de Chiari tipo I necessitam de uma abordagem diferenciada que contemplem suas necessidades nos programas de neurorreabilitação.

Clinical and epidemiological characteristics of patients with traumatic tetraplegia and Chiari malformation type I treated at a neurorehabilitation center were compared through an analytical and retrospective observational study, carried out through electronic medical record analysis. In the group of patients with Chiari malformation type I predominated female gender, with gait, the presence of syringomyelia and anxiety disorders. In the group with traumatic tetraplegia predominated male gender, use of wheelchair and presence of spasticity and neurogenic dysfunctions of the bowel and lower urinary tract. Patients with Chiari malformation type I represent a group with different clinical and epidemiological characteristics in relation to patients with sequelae of spinal cord injury, traditionally the main group treated within neurorehabilitation. Thus, patients with Chiari malformation type I need a differentiated approach that addresses their needs in neurorehabilitation.

A case report of Budd-Chiari syndrome with cardiac complications and low SPO2 successfully managed by an integrated-approach

Budd-Chiari syndrome [BCS] is a rare, potentially life-threatening condition characterized by the triad of abdominal pain, ascites, and hepatomegaly (with or without jaundice). There will be an underlying disorder in most cases. The diagnosis, as well as management of the case, requires a multidisciplinary approach. The treatment should aim at reducing the presenting symptoms as well as removing the underlying pathology. Here we explain a case report of a 21-year-old male patient in the subacute stage of BCS with its complications and considered as a candidate for liver transplantation. His liver enzymes, PT/INR, D- dimer, and homocysteine values were above normal levels. The arterial oxygen saturation level was subnormal, and he was on supportive oxygen supplement. Inferior venacava [IVC] Doppler revealed a non-obstructive intrahepatic thrombus. The patient was treated with Homoeopathic medicine Arsenicum album and Arnica montana, given as an adjuvant to conventional treatment.

Síndrome de Budd Chiari secundario al síndrome antifosfolipídico y lupus eritematoso sistémico en una adolescente: reporte de un caso

El síndrome de Budd-Chiari es causado por una obstrucción del flujo sanguíneo del hígado. Los casos publicados del síndrome antifosfolípido asociado a SBC son limitados en la población pediátrica. Reportamos el caso de una adolescente de 15 años que presentó fiebre, ascitis y hepatoesplenomegalia. En la ecografía Doppler hepática se observó ausencia de flujo en la vena hepática derecha y media, y en la vena cava inferior. En la tomografía abdominal se observó una extensa trombosis de la vena cava inferior. Durante la hospitalización se le diagnosticó SAF y lupus eritematoso sistémico. Se le administró tratamiento con heparina no fraccionada, heparina de bajo peso molecular y anticoagulantes. El síndrome de Budd-Chiari secundario al síndrome antifosfolípido es una enfermedad potencialmente mortal. El diagnóstico y el tratamiento oportunos permiten mejorar la calidad de vida del paciente.

Budd-Chiari syndrome is caused by an obstruction of blood flow to the liver. Published cases of the antiphospholipid syndrome associated with BCS are limited in the pediatric population. We report a 15-year-old adolescent who presented with fever, ascites, and hepatosplenomegaly. Hepatic Doppler ultrasound revealed no flow in the right and middle hepatic veins and in the inferior vena cava. Abdominal tomography revealed extensive thrombosis of the inferior vena cava. During hospitalization, she was diagnosed with antiphospholipid syndrome and systemic lupus erythematosus. She was given treatment with unfractionated heparin, low molecular weight heparin, and anticoagulants. Budd-Chiari syndrome secondary to the antiphospholipid syndrome is a life-threatening disease. Timely diagnosis and treatment improve the quality of life of the patient.

Post-COVID Pediatric Budd-Chiari Syndrome

Introduction: Budd-Chiari syndrome (BCS) is rare in children. Underlying etiologies, natural history and management differ in children and adults. Treatment options like liver transplantation and transjugular intrahepatic portosystemic shunt have also been less well-studied in children. Presented here is the case of a male child with BCS after coronavirus disease 2019 (COVID-19) infection. Case presentation: A 3-year-old male child presented with complaints of gradually increasing abdominal distension, constipation, decreased urine output, visible veins over abdomen and swelling in lower limbs and periorbital edema since last 15 days. He was diagnosed as BCS after multiple imaging investigations, including triple phase contrast-enhanced computed tomography (CECT) abdomen. His COVID antibody tested positive. Conclusion: Practice guidelines for children with BCS should be formulated, expert group recommendations should be reviewed and a consensus statement should be issued. Underlying etiology remains obscure despite extensive work-up in most of the children. Association of COVID-19 with BCS may be incidental but should be studied further as COVID is known to cause thrombotic complications

Platelet-albumin-bilirubin score in assessing short-term prognosis of patients with Budd-Chiari syndrome presenting with upper gastrointestinal bleeding / 中华肝胆外科杂志

Objective:To study the factors influencing short-term prognosis of patients with Budd-Chiari syndrome (B-CS) presenting with upper gastrointestinal bleeding and to assess the predictive value of platelet-albumin-bilirubin score (PALBI) on death within 30 d in these patients.Methods:A retrospective study was conducted on 74 patients with B-CS who presented with upper gastrointestinal bleeding and were treated at the First Affiliated Hospital of Zhengzhou University from January 2014 to February 2020. There were 51 males and 23 females, with age of (46.5±11.1) years old. These patients were divided into the survival group ( n=58) and the death group ( n=16) according to the disease outcomes up to 30 d of follow-up. Factors influencing short-term deaths of these patients were analyzed, and the predictive values of PALBI, ALBI, CTP and MELD scores on short-term prognosis of the patients were assessed. The receiver operating characteristic (ROC) curves were plotted, and the areas under the curve (AUC) were calculated and compared. Results:The differences between patients in the survival and death groups for white blood cell, platelet, PALBI score, PALBI classification, ALBI score, CTP score, MELD score, and presence or absence of hepatic encephalopathy were significantly different (all P<0.05). Multivariate logistic regression analysis showed that CTP score≥10 or CTP grade C ( OR=1.669, 95% CI: 1.048-2.661), and PALBI score >-2.09 or PALBI grade 3 ( OR=5.245, 95% CI: 2.128-12.924) were independent risk factors for predicting death within 30 days. The areas under the ROC curves for PALBI, ALBI, CTP and MELD score were 0.89, 0.72, 0.77 and 0.76, with the cut-off values of -1.92, -1.60, 8.50 and 13.60, respectively. The differences between the PALBI score and ALBI, CTP scores were significantly different ( P<0.05). Conclusion:The PALBI score showed a positive predictive value on short-term prognostic assessment of patients with B-CS presenting with upper gastrointestinal bleeding. It was comparable to the effect of the MELD score but was significantly better than the ALBI and CTP scores.

Study on the mechanism of mTOR/HIF-1α signaling pathway in Budd-Chiari syndrome liver fibrosis / 国际外科学杂志

Objective:To explore the mechanism of mTOR/HIF-1α signaling pathway in Budd-Chiari syndrome (B-CS) liver fibrosis.Methods:Twenty male C57 mice were randomly divided into Sham operation group (Sham), sham operation+ rapamycin (Sham+ Ra) group, B-CS group, B-CS+ rapamycin (B-CS+ Ra) Group, 5 in each group. The B-CS mouse model was constructed by partial ligation of the inferior vena cava(IVC) at the posterior segment of the liver; IVC was not ligated in the Sham group. Mice in Sham+ Ra and B-CS+ Ra groups were intraperitoneally injected with rapamycin (2 mg/kg, 5% DMSO solution preparation) every other day, Sham group and B-CS group were injected with the same dose of 5% DMSO solution.After 6 weeks, samples were taken, and part of the liver tissue was used to make paraffin sections for hematoxylin-eosin (HE) and Sirus Red staining to observe the pathological changes, and immunohistochemical staining to detect the expression of α-SMA and Fibrinogen in liver tissues; Protein and RNA were extracted from fresh liver tissue, and Western-blot was used to detect α-SMA, Fibrinogen, p-mTOR, mTOR, HIF-1α, Collagen I, and VEGF protein levels. Real-time fluorescent quantitative PCR was used to detect mTOR, HIF-1α, CollagenⅠ, VEGF mRNA levels.Measurement data were expressed as mean±standard deviation ( ± s), and the comparison between groups was performed by one-way ANOVA test. Results:The results of pathological staining showed that in the B-CS group, there was severe congestion around the central vein of the liver and sinusoids, widening of the sinus space, and increased collagen deposition, indicating that this study successfully established a mouse B-CS liver fibrosis model. The expression levels of fibrosis indicators α-SMA and Collagen I protein, mTOR pathway related indicators p-mTOR and HIF-1α protein, and microthrombus indicator Fibrinogen protein in the Sham group were 0.027±0.012, 0.337±0.008, 0.138±0.024, 0.296±0.113, 0.733±0.192; B-CS group were 0.986±0.001, 0.927±0.055, 0.936±0.044, 1.693±0.443, 1.612±0.068, and the differences were statistically significant ( P<0.05). The expression levels of B-CS+ Ra group were 0.707±0.078, 0.311±0.024, 0.332±0.094, 0.254±0.117, 0.569±0.075, which were statistically significant compared with B-CS group ( P<0.05). Conclusions:The mTOR/HIF-1α signaling pathway is significantly activated in mouse B-CS liver fibrosis. This pathway may participate in the development of liver fibrosis by regulating microthrombosis.