Deep learning for intracranial aneurysm segmentation using CT angiography.

OBJECTIVE: This study aimed to employ a two-stage deep learning method to accurately detect small aneurysms (4-10 mm in size) in computed tomography angiography (CTA) images. Methods: This study included 956 patients from 6 hospitals and a public dataset obtained with 6 CT scanners from different manufacturers. The proposed method consists of two components: a lightweight and fast head region selection (HRS) algorithm and an adaptive 3D nnU-Net network, which is used as the main architecture for segmenting aneurysms. Segments generated by the deep neural network were compared with expert-generated manual segmentation results and assessed using Dice scores. Results: The area under the curve (AUC) exceeded 79% across all datasets. In particular, the precision and AUC reached 85.2% and 87.6%, respectively, on certain datasets. The experimental results demonstrated the promising performance of this approach, which reduced the inference time by more than 50% compared to direct inference without HRS. Conclusion: Compared with a model without HRS, the deep learning approach we developed can accurately segment aneurysms by automatically localizing brain regions and can accelerate aneurysm inference by more than 50%. Keywords: Deep Learning, CT Angiography, Aneurisms.

Early anti IL-1 treatment replaces steroids in refractory Kawasaki disease: clinical experience from two case reports.

Refractory Kawasaki disease (KD) is related to a major risk of coronary arteries abnormalities and its treatment is not standardized. In this regard, anakinra (ANA), an interleukin (IL)-1 receptor antagonist, represents an emerging therapeutic option. We report two cases of children, diagnosed with KD, nonresponsive to two doses of intravenous immunoglobulins, successfully treated with ANA, without a prior use of steroids. Patient 2 developed a coronary dilatation, that improved significantly after ANA therapy. Our experience highlights IL-1 blockade effectiveness in reducing KD inflammation and suggests ANA adoption as second-line therapy, with a timesaving and steroid-sparing strategy. Our results, combined with the evidence of the IL-1 key role in KD and coronary arteritis pathogenesis and to the recent clinical evidence reported by the KAWAKINRA trial, encourage an earlier recourse to ANA in patients with refractory KD, in order to fight inflammation, and to treat and prevent the development of coronary artery aneurysms. Further studies are needed to better define the place of IL-1 blockade in KD step-up treatment.

Surgical myocardial revascularization outcomes in Kawasaki disease: systematic review and meta-analysis.

BACKGROUND: Kawasaki disease (KD) is a systemic inflammatory condition occurring predominantly in children. Coronary artery bypass grafting (CABG) is performed in the presence of inflammation and aneurysms of the coronary arteries. The objectives of our study were to assess which CABG strategy provides better graft patency and early and long-term outcomes. METHODS: A systematic review using Medline, Cochrane, and Scopus databases was performed in February 2020, incorporating a network meta-analysis, performed by random-effect model within a Bayesian framework, and pooled prevalence of adverse outcomes. Hazard ratios (HR) and corresponding 95% credible intervals (CI) were calculated by Markov chain Monte Carlo methods. RESULTS: Among 581 published reports, 32 studies were selected, including 1,191 patients undergoing CABG for KD. Graft patency of internal thoracic arteries (ITAs), saphenous veins (SV), and other arteries (gastroepiploic artery and radial artery) was compared. ITAs demonstrated the best patency rates at long-term follow-up (HR 0.33, 95% CI: 0.17-0.66). Pooled prevalence of early mortality after CABG was 0.28% (95% CI: 0.00-0.73%, I 2 = 0%, tau2 = 0), with 63/1,108 and 56/1,108 patients, undergoing interventional procedures and surgical re-interventions during follow-up, respectively. Pooled prevalence was 3.97% (95% CI: 1.91-6.02%, I 2 = 60%, tau2 = 0.0008) for interventional procedures and 3.47% (95% CI: 2.26-4.68%, I 2 = 5%, tau2 <0.0001) for surgical re-interventions. Patients treated with arterial, venous, and mixed (arterial plus second venous graft) CABG were compared to assess long-term mortality. Mixed CABG (HR 0.03, 95% CI: 0.00-0.30) and arterial CABG (HR 0.13, 95% CI: 0.00-1.78) showed reduced long-term mortality compared with venous CABG. CONCLUSIONS: CABG in KD is a safe procedure. The use of arterial conduits provides better patency rates and lower mortality at long-term follow-up.

Cyclophosphamide use in treatment of refractory Kawasaki disease with coronary artery aneurysms.

BACKGROUND: Despite timely administration of IVIG, some patients with Kawasaki disease (KD) develop rapidly progressive or giant coronary artery aneurysms (CAA). CASE PRESENTATION: We describe our experience using cyclophosphamide (CYC) for the treatment of such cases as well as a review of the literature on the use of CYC in KD. Through a retrospective chart review of our KD population, we identified ten children treated for KD with intravenous CYC (10 mg/kg/dose) for one or two doses. Seven patients were male, the median age was 2.0 years (range 4 months - 5 years). All patients received initial IVIG between day 4-10 of illness. Other anti-inflammatory treatments administered before CYC included second IVIG (n = 9), corticosteroids (n = 10), infliximab (n = 4), cyclosporine (n = 2), and anakinra (n = 1). Median illness day at administration of the first CYC dose was 22.5 days (range:10-36 days). The primary indication for treatment with CYC for all patients was large or giant CAA and/or rapid progression of CAA. Three patients received a second dose of CYC (10 mg/kg) for progressively enlarging CAA. CAA did not progress after final CYC treatment. One patient with a history of neutropenia in infancy developed severe neutropenia 9 days after treatment with CYC, which recovered without intervention or complications. No patient developed infections or other serious toxicity from CYC. CONCLUSION: In KD patients with severe and progressive enlargement of CAA despite anti-inflammatory therapy, CYC seemed to arrest further dilation and was well-tolerated. Future multicenter studies are needed to confirm our findings in this subgroup of KD patients.

Enfermedad de Kawasaki en lactantes bajo un año de edad: un reto para el diagnóstico y tratamiento de los pacientes. Experiencia en un centro hospitalario en México / Kawasaki disease in infants under one year of age: a challenge for the diagnosis and treatment of patients. Experience in a hospital center in Mexico

Resumen Antecedentes: La enfermedad de Kawasaki (EK) en lactantes bajo un año de edad es poco frecuente en la mayoría de los países. Este grupo de pacientes tienen mayor riesgo de desarrollar complicaciones cardiacas. Objetivo: Evaluar el comportamiento clínico, tratamiento empleado y pronóstico cardiaco en lactantes bajo un año de edad atendidos por EK en un hospital pediátrico de tercer nivel en la Ciudad de México. Pacientes y Métodos: Estudio retrospectivo, descriptivo, de pacientes con diagnóstico de EK desde agosto de 1995 a agosto de 2019. Se estudió la presentación clínica, los exámenes de laboratorio, el tratamiento administrado y el desarrollo de lesiones coronarias en pacientes bajo un año de edad y se comparó con pacientes mayores. Resultados: Se estudiaron 687 pacientes, 152 de ellos eran lactantes bajo un año de edad (22,1%). Hubo un mayor tiempo al diagnóstico de la EK en los lactantes menores, con un incremento de presentaciones clínicas incompletas; este grupo de pacientes desarrolló en forma más frecuente lesiones coronarias en comparación con los pacientes mayores y también tuvo un mayor porcentaje de aneurismas coronarios gigantes. Hubo dos fallecimientos en los lactantes menores, secundarios a infarto al miocardio. Conclusiones: El diagnóstico de EK en pacientes bajo un año de edad es un reto diagnóstico con presentaciones clínicas incompletas y mayor riesgo de desarrollar complicaciones cardiacas graves.

Abstract Background: Frequency of Kawasaki disease (KD) in infants is low in almost all countries. These patients are at higher risk of developing cardiac complications. Aim: To evaluate the clinical features, treatment used and cardiac outcome in infants under one year of age attending for KD in a third level pediatric hospital in Mexico City, Mexico. Methods: A cross-sectional study was conducted in our hospital from August 1995 to August 2019. We analyzed the clinical features, laboratory results, treatment used and cardiac outcomes in infants younger than one year of age and compared them with older patients. Results: We included 687 patients, 152 were younger than one year of age (22.1%). There was a delayed diagnosis in younger patients with an increased frequency of incomplete clinical presentations. Coronary artery abnormalities were most common in younger infants who also had an increased frequency of giant coronary artery aneurysms. Two patients in the younger group died in the acute phase of KD of myocardial infarction. Conclusions: Diagnosis of KD in infants younger than 1 year of age is a clinical challenge with an increased rate of incomplete clinical presentations and also an increased risk of development of severe cardiac complications.

Association of autosomal dominant polycystic kidney disease, asymptomatic multiple giant coronary arteries aneurysms and abdominal aortic aneurysm: a case report.

Autosomal dominant polycystic kidney disease (ADPKD) is a systemic disease characterized by the formation of multiple cysts in several organs. The formation of aneurysms accompanying this disease is being increasingly reported in the literature, and mutations in PKD-1 and PKD-2 are suspected in this etiology. Although the association between ADPKD and multiple coronary arteries aneurysms (CAA) was reported several times, we are presenting a case with the combination of ADPKD, multiple giant CAAs, abdominal aortic aneurysms and a suspected intracranial aneurysm, which has never been reported. The asymptomatic presentation of these multiple aneurysms might support the recommendations for further diagnostic investigations in these patients.

Hughes Stovin: Sustained remission and regression of pulmonary aneurysms with anti-tumor necrosis factor treatment.

We report the first case of Hughes Stovin Syndrome successfully treated with long-lasting tumor necrosis factor α (TNF-α) blocker (infliximab) treatment. Because of the failure of the standard therapeutic regimen with steroids and cyclophosphamide, infliximab was started achieving a stable disease remission and a complete resolution of pulmonary aneurysms. Hughes Stovin Syndrome, although rare, is a life-threatening condition that needs to be timely identified and treated aggressively. Our report underlines the importance of TNF-α blocker treatment in Hughes Stovin Syndrome, suggesting its use as long-term safe and useful.

Síndrome de Ehlers-Danlos vascular: complicaciones vasculares graves en enfermos con hipermovilidad articular / Vascular Ehlers-Danlos syndrome: severe vascular complications in patients with articular hypermobility

El Síndrome de Ehlers-Danlos Vascular es una de las Alteraciones Hereditarias del Tejido Conectivo. Es poco frecuente, pero es de alta morbilidad y mortalidad. Se debe a una mutación del gen COL3A1, que da debilidad del tejido colágeno, por lo que se pueden producir complicaciones graves afectando a la piel, tubo digestivo, hígado, pulmón y sistema vascular. Se pueden producir rupturas intestinales, neumotórax espontáneo, dilatación o ruptura aortica y anuerismas, lo que puede llevar a la muerte súbita del paciente.

The Vascular Ehlers-Danlos is one of Hereditary Diseases of Connective Tissue. Has low prevalence, but high morbidity and mortality. It is due to a COL3A1 gene mutation, producing tissue fragility, which can produce serious complications in the skin, digestive tract, liver, lungs and in the vascular system. It is frequently associated to intestinal ruptures, spontaneous pneumothorax, dilation or rupture of the aorta anuerism, which can lead to sudden death.

Abordaje fronto-orbito-cigomático en dos piezas: indicaciones y técnica quirúrgica / Fronto-orbito-zygomatic two-piece approach: indications and surgical technique

Objetivo: Resaltar las indicaciones y detalles técnicos en el abordaje fronto-órbito-cigomático en dos piezas en base a nuestra experiencia quirúrgica. Material y método: se realizó un estudio descriptivo retrospectivo analizando las historias clínicas de 18 pacientes intervenidos quirúrgicamente a través de un abordaje fronto-orbito-cigomático en dos piezas, entre junio de 2010 y junio de 2013, en nuestro servicio. Se obtuvieron datos epidemiológicos y los relacionados a las diferentes patologías alcanzadas mediante esta vía. Resultados: durante dicho período se realizaron 18 craneotomías fronto-orbito-cigomática en dos piezas. Fueron intervenidos 11 pacientes con aneurismas cerebrales, de los cuales 4 fueron aneurismas del segmento oftálmico de la arteria carótida interna, 3 de bifurcación alta de arteria carótida interna, 2 de la arteria comunicante anterior con orientación cefálica y 2 de la bifurcación de la arteria basilar. Así mismo, se logró la exéresis de 7 lesiones tumorales, de los cuales 4 fueron adenomas de hipófisis, 2 craneofaringioma y 1 glioma hipotalámico. Se estandarizaron los siguientes pasos para la realización del abordaje fronto-órbito-cigomático en dos piezas, compuesto por una craneotomía fronto-temporo-esfenoidal y una segunda pieza compuesta por techo orbitario y arcada cigomática. Conclusiones: dicho abordaje representa una importante vía de acceso para lesiones que comprometen región selar, paraselar y patología vascular del polígono de Willis con menor retracción cerebral y mayor exposición.

Purpose: To highlight the indications and surgical details in the two pieces fronto-orbito-zigomatic approach based on our surgical experience.Material and Methods: a descriptive retrospective study was done by the analysis of 18 medical histories of patients operated by a two pieces fronto-orbito-zigomatic approach, between June 2010-2013 in our sevice. Epidemiological facts and those related to the different pathology approaches were obtainedResults: 18 two pieces fronto-orbito-zigomatic craniotomies were performed during the analized period. 11 patients had cerebral aneurisms, 4 of them arised from the oftalmic segment of the internal carotid artery, 3 from a high bifurcation of the internal carotid artery, 2 from the basilar artery´s bifurcation. We also performed the resection of 7 tumors: 4 pituitary adenomas, 2 craniopharyngiomas and 1 hipotalamic glioma. The following steps were standarized to perform the two pieces fronto-orbito-zigomatic approach: first a fronto-temporo-esphenoidal craniotomy and then a second piece composed by the orbital roof and the zigomatic arc.Conclusions: this approach represents an important route of access to lesions that involve both sellar and parasellar regions and vascular pathology from the Circle of Willis with less brain retraction and more exposure.

Endovascular treatment of aneurisms: pre, intra and post operative management.

The most frequent risk in endovascular aneurysm treatment is thromboembolic complications. Thus adjuvant pharmaceuticals are largely focused on preventing and treating these latter. Additionally symptomatic treatment of subarachnoid hemorrhage (SAH) and treatments to avoid vasospasm will enter into play in cases of ruptured aneurisms. Consensus exists in the literature neither for the necessity of heparin or antiplatelets nor for the doses to be administered. The principles and rationale of the use of these medications are reviewed with a discussion of protocols according with clinical situations and technical choices.

Aneurismas periféricos postraumáticos: casos clínicos y su tratamiento quirúrgico / Post-traumatic peripheral aneurisms: clinical costs and surgical treatment

Los aneurismas distales tienen pocos reportes en la literatura por su escasa presencia en la clínica médica. Su etiología es diversa y su tratamiento difiere en cada caso individualmente. Se presentan tres pacientes portadores de aneurismas verdaderos, postraumáticos y de localización periférica. Los mismos fueron diagnosticados y tratados en la República de Yemen, en el período de 2006 a 2009, durante el transcurso de la colaboración médica cubana en este país. Todos obtuvieron un resultado terapéutico satisfactorio.

There are few reports on distal aneurisms in the literature because of their scarce presence in the medical clinic. Their etiology is diverse and their treatment differs individually in each case. We present three patients carrying truly, post-traumatic, peripheral aneurisms. They were diagnosed and treated in the Republic of Yemen, in the period 2006-2009, during the Cuban medical collaboration in that country. In all of them the therapeutic results were satisfactory.

Aneurismas en la aorta abdominal: presentación de un caso / Aneurisms in the abdominal aorta: presentation of a case

El aneurisma de la aorta abdominal ha sido reconocido como un importante problema de salud en la última década. Las anomalías del árbol vascular son comunes. Los aneurismas aislados de la arteria aorta son de observación bastante frecuentes. Las estadísticas asociadas con esta patología son de gran preocupación y se prevé que su incidencia se incremente en los próximos años, debido al aumento en la expectativa de vida de la población. El calibre de la aorta abdominal no excede de 3 cm de diámetro y se va afilando progresivamente a medida que discurre distalmente. El riesgo de ruptura depende del tamaño del aneurisma. Se reportó un caso femenino, de 61 años de edad, que asistió a consulta por presentar dolor, acompañado de hipotensión una anemia aguda, fatigas con sudoraciones profusas, pulsaciones en epigastrio. Se le realizó un ultrasonido de abdomen, observándose ligera dilatación de la aorta abdominal de 3,7 cm que se extendía hasta las ramas iliacas. Se le indicó tomografía axial computarizada de abdomen, donde se halló afectación de la aorta abdominal.

The aneurism of the abdominal aorta has been recognized as an important health problem in the last decade. The anomalies of the vascular tree are common. The isolated aneurisms of the aorta artery are observed quite frequently. The statistics associated to this disease provoke great preoccupation and it is foreseen that its incidence increases in the subsequent years, due to the increase of the population’s life expectancy. The caliber of the abdominal aorta does not exceed 3 cm of diameter and it gets thinner progressively at the time it wanders distally. The rupture risk depends on the aneurism size. We reported the case of a woman aged 61 years, who assisted the consultation for having pain, accompanied by hypotension, an acute anemia, fatigue with profuse perspiration and pulsations in the epigastrium. An abdominal ultrasound was made, finding a slight distension of the abdominal aorta of 3,7 cm, extended up to the iliac branches. We indicated an abdominal computed axial tomography discovering the damage of the abdominal aorta.

Muerte súbita de origen neuropatológicos: (II parte) / Sudden death of neuropathological origin: (Part II)

En la primera parte del presente artículo, en el número anterior de esta Revista, se discutió ampliamente el concepto de muerte súbita de origen neuropatológico. Tomando en cuenta el mismo, se realizó este análisis de las autopsias y reportes de neuropatología de 1998 al 2006 de la Sección de Patología Forense del Departamento de Medicina Legal para establecer las causas de muerte más frecuentes y el perfil epidemiológico de los fallecidos. Esto para recomendar aspectos útiles para el manejo este tipo de casos para el personal directamente involucrado. Después de revisar las causas de muerte de 23099 autopsias que se efectuaron en el período en estudio, 338 casos coincidieron con la definición propuesta de muerte súbita de origen neuropatológico y en términos generales hubo concordancia entre lo que se anota en la literatura mundial y lo que se ha presentado en nuestro país en el intervalo analizado.

In the first section of these report, on the previous edition of these magazine, it was discussed widely the concept of the neuropathological origen of the sudden death. Taking that preview this analysis was made from the autopsies and the neuropathological reports from 1998 to 2006 from the "Sección de Patología Forense" of costarican "Departamento de Medicina Legal" to establish the most frequent causes of death and the epidemiologic profile of the deceased. These to advice usefull aspects for the management of those cases by the involved staff. After reviewing the causes of death of 23099 autopsies made on the chosen period, 338 cases achived the definition of neuropathological sudden death proposed. In general there was found agreement between the findings on worldwide literature and the findings of our country during these period.

Morphological aspects of mural thrombi deposition residual lumen route in infrarenal abdominal aorta aneurisms / Morfologia da deposição de trombos murais: trajeto da luz residual em aneurismas de aorta abdominal infra-renal

PURPOSE: To assess the most frequent deposition site of mural thrombi in infrarenal abdominal aorta aneurisms, as well as the route of the residual lumen. METHODS: Assessment of CT scan images from 100 patients presenting asymptomatic abdominal aorta aneurism, and followed at HC-FMRP-USP. RESULTS: In 53 percent of the cases the mural thrombus was deposited on the anterior wall; from these, in 22 percent, the residual lumen described a predominantly right sided route; in 22 percent, a left sided route; on the mid line in 5 percent; and crossing over the mid line in 1 percent. In 23 percent, the deposition of thrombi was concentric. In 11 percent it occured on the posterior wall; from these, in 5 percent, the route of the residual anterior lumen was predominantly right sided; in 5 percent, left sided; and crossed over the mid line in 1 percent. In 13 percent complex morfological deposition patterns were found. CONCLUSION: Mural thrombi formation was predominantly found on the anterior wall of the aneurismatic mass, with the route of the residual lumen projecting towards the posterior wall.

OBJETIVOS: Avaliar o local mais freqüente de deposição do trombo mural em aneurismas de aorta abdominal infrarenal, bem como o trajeto da luz residual. MÉTODOS: Avaliação de tomografias de 100 pacientes do HC-FMRP-USP apresentando aneurisma de aorta abdominal assintomático. RESULTADOS: O trombo mural se deposita na parede anterior em 53 por cento dos casos, sendo que a luz residual posterior descreveu um trajeto predominantemente à direita em 22 por cento dos casos, à esquerda em 22 por cento, na linha mediana em 5 por cento e cruzando da direita para a esquerda em 4 por cento. 23 por cento dos casos apresentaram deposição concêntrica do trombo e 11 por cento apresentaram deposição na parede posterior, sendo o trajeto da luz residual anterior predominante a direita em 5 por cento dos casos, a esquerda em 5 por cento e cruzando a linha mediana em 1 por cento. Padrões morfológicos complexos de deposição do trombo foram encontrados em 13 por cento dos casos. CONCLUSÃO: A formação do trombo mural predominou na parede anterior da massa aneurismática, com o trajeto da luz residual se projetando para a parede posterior.