Racemose hemangioma presenting as a vitreous hemorrhage: case report / Hemangioma racemoso apresentando-se como hemorragia vítrea: relato de caso

ABSTRACT This report describes a case of retinal racemose hemangioma that first presented as a vitreous hemorrhage. The authors present the case of a 47-year-old woman with a sudden 5-day painless visual loss in her left eye. At the first visit, the best-correct visual acuities were 20/20 in the right eye and hand motions in the left eyes. Ultrasonography showed an attached retina and a massive vitreous hemorrhage. Pars plana vitrectomy was performed and a dilatation of large vessels was detected bulging from the optic disc. The best-correct visual acuities on day 30 postoperatively was 20/25 in the left eye. Fundus angiography and spectral-domain optical coherence tomography angiography showed anomalous arteriovenous communications with no intervening capillaries. The diagnosis was racemose hemangioma, an arteriovenous malformation of group 2 retina based on the Archer classification.

RESUMO Este relato descreve um caso de hemangioma racemoso da retina que se apresentou inicialmente como hemorragia vítrea. Os autores apresentam o caso de uma mulher de 47 anos com perda visual súbita e indolor 5 dias antes no olho esquerdo. Na primeira visita, a melhor acuidade visual corrigida foi de 20/20 no olho direito e movimentos das mãos no olho esquerdo. A ultrassonografia mostrou uma retina aderida e uma hemorragia vítrea maciça. Foi realizada vitrectomia pars plana, sendo detectada proliferação de grandes vasos salientes do disco óptico. A acuidade visual no dia 30 de pós-operatório foi de 20/25 no olho esquerdo. A angiografia de retina e a angiotomografia de coerência óptica de domínio espectral mostraram comunicações arteriovenosas anômalas sem capilares intermediários. O diagnóstico foi hemangioma racemoso, uma malformação arteriovenosa da retina do grupo 2 com base na classificação de Archer.

Malformaciones arteriovenosas pulmonares: presentación de un caso clínico pediátrico y revisión de la literatura / Pulmonary arteriovenous malformations: presentation of a pediatric case and review of the literature

Las malformaciones arteriovenosas pulmonares (MAVP) consisten en comunicaciones directas entre el sistema arterial y el sistema venoso pulmonar, sin paso de la sangre por el lecho capilar, produciéndose un cortocircuito de derecha a izquierda extracardíaco, pueden ser congénitas o adquiridas. Algunos casos pueden ser asintomáticos, en cambio en otros pueden ocasionar diversas manifestaciones clínicas y se pueden asociar a complicaciones severas. En niños se observa una baja incidencia y son más frecuentes las formas congénitas. La MAVP se debe sospechar por las manifestaciones clínicas y las imágenes de la radiografía de tórax (RxTx) y su confirmación se realiza mediante una AngioTomografía Computada (TC) de tórax. La embolización endovascular es actualmente el tratamiento de elección, con excelentes resultados, aunque requiere de un seguimiento posterior y de un operador experimentado. Reportamos el caso de una niña que ingresó con clínica muy sugerente, incluyendo: disnea, acropaquia, cianosis periférica, e hipoxemia refractaria. Sin embargo, inicialmente el cuadro clínico fue confundido con una crisis asmática. La Angio-TC de tórax confirmó el diagnóstico y el tratamiento mediante embolización endovascular resultó exitoso.

Pulmonary arteriovenous malformations (PAVM) are communications between the arterial and the pulmonary venous system, without passage of blood through the capillary bed, causing a left to right extracardiac shunt. Some cases may be asymptomatic, while others may cause various clinical manifestations and may be associated with severe complications. In children a low incidence is observed, and congenital forms are more frequent. PAVM should be suspected by clinical manifestations and chest x-ray imaging and confirmed by chest Computed Tomography Angiography (CTA). Endovascular embolization is currently the treatment of choice, with excellent results, although it requires subsequent follow-up. We report a patient who was admitted with a very suggestive clinical history, including: dyspnea, clubbing, peripheral cyanosis, and severe hypoxemia, refractory to oxygen therapy. However, initially the clinical picture was confounded with an asthmatic crisis. CTA confirmed the diagnosis and treatment by endovascular embolization was successful.

O papel da ultrassonografia na doença trofoblástica gestacional / The role of ultrasonography in gestational trophoblastic disease

A doença trofoblástica gestacional (DTG) agrupa um conjunto de anomalias do desenvolvimento trofoblástico, que incluem formas clínicas benignas como a mola hidatiforme completa e parcial, o nódulo do sítio placentário atípico e o sítio trofoblástico exagerado, e malignas, caracterizando a neoplasia trofoblástica gestacional (NTG). De modo geral, seu diagnóstico precoce antecipa complicações clínicas que podem estar associadas a near miss obstétrico. Diante da suspeição clínica, é a ultrassonografia (US) precoce o exame de escolha pa ra o diagnóstico, associado à dosagem sérica de gonadotrofina coriônica humana, capaz de minimizar a ocorrência de complicações clínicas associadas à gravidez molar. Nos casos de NTG, é a US também de grande valia para estadiamento, avaliação de prognóstico e acompanhamento da mulher tratada para DTG. Este estudo faz uma revisão sobre o papel da US na DTG, sendo importante para familiarizar os tocoginecologistas com essa doença e salientar o papel da US consoante as melhores práticas clínicas.(AU)

Gestational trophoblastic disease (GTD) includes a set of trophoblastic developmental anomalies, which include benign forms such as complete and partial hydatidiform mole, atypical placental site nodule and exaggerated trophoblastic site, and malignant forms, characterizing gestational trophoblastic neoplasia (GTN). In general, its early diagnosis anticipates clinical complications that could be associated with obstetric near miss. In view of clinical suspicion, early ultrasonography (US) and serum levels of human chorionic gonadotropin are the best diagnostic screening techniques, able to minimizing the occurrence of medical complications associated with molar pregnancy. In cases of GTN, US is also of great value for staging, assessment of prognosis and follow-up of women treated for GTN. This study reviews the role of US in GTD, being important to familiarize tocogynecologists with this disease and highlight the role of US according to best clinical practices to minimize the morbidity of these patients and maximize the remission rates of this disease.(AU)

What do Cochrane systematic reviews say about congenital vascular anomalies and hemangiomas? A narrative review

ABSTRACT BACKGROUND: Congenital vascular anomalies and hemangiomas (CVAH) such as infantile hemangiomas, port-wine stains and brain arteriovenous malformations (AVMs) impair patients' lives and may require treatment if complications occur. However, a great variety of treatments for those conditions exist and the best interventions remain under discussion. OBJECTIVE: To summarize Cochrane systematic review (SR) evidence on treatments for CVAH. DESIGN AND SETTING: Review of SRs conducted in the Division of Vascular and Endovascular Surgery of Universidade Federal de São Paulo, Brazil. METHODS: A broad search was conducted on March 9, 2021, in the Cochrane Database of Systematic Reviews to retrieve any Cochrane SRs that assessed treatments for CVAH. The key characteristics and results of all SRs included were summarized and discussed. RESULTS: A total of three SRs fulfilled the inclusion criteria and were presented as a qualitative synthesis. One SR reported a significant clinical reduction of skin redness by at least 20%, with more pain, among 103 participants with port-wine stains. One SR reported that propranolol improved the likelihood of clearance 13 to 16-fold among 312 children with hemangiomas. One SR reported that the relative risk of death or dependence was 2.53 times greater in the intervention arm than with conservative management, among 218 participants with brain AVMs. CONCLUSION: Cochrane reviews suggest that treatment of port-wine stains with pulsed-dye laser improves redness; propranolol remains the best option for infantile hemangiomas; and conservative management seems to be superior to surgical intervention for treating brain AVMs.

Sangramento gastrointestinal em paciente portador de síndrome de Rendu-Osler-Weber: relato de caso / Gastrointestinal bleeding in a patient with Osler-Weber-Rendu syndrome: a case report / Sangrado gastrointestinal en paciente con síndrome de Rendu-Osler-Weber: reporte de caso

A síndrome de Rendu-Osler-Weber, ou telangiectasia hemorrágica hereditária (THH), é uma doença autossômica dominante de penetrância variável que altera a camada muscular e elástica da parede dos vasos sanguíneos, causando neles grande fragilidade e dilatação e tornando-os suscetíveis a sangramentos secundários, a traumatismos e malformações arteriovenosas (MAV) ­ sendo elas as mais comuns no cérebro, nos pulmões e no trato gastrointestinal e hepático. Este relato de caso tem como objetivo realizar uma revisão sobre essa síndrome, bem como a respeito da melhor terapia e do melhor manejo para sangramentos gastrointestinais por angiectasias. Trata-se de uma paciente de 62 anos de idade, com episódio de hematêmese e melena recorrentes, que foi admitida instável hemodinamicamente e com queda importante dos níveis de hemoglobina, necessitando de múltiplas transfusões sanguíneas. Após entrevista clínica, que chamou a atenção para o histórico familiar de quadros similares, epistaxes recorrentes associados ao exame físico e angiectasias gástricas à endoscopia digestiva alta, foi atribuído o diagnóstico de síndrome de Rendu-Osler-Weber. Angiectasias foram erradicadas com terapia endoscópica usando plasma de argônio. A THH é uma doença subdiagnosticada que merece atenção especial por conta do seu risco de causar hemorragias graves com expressão fenotípica mais aflorada com o avançar da idade. Por seu cunho hereditário, faz-se necessário um acompanhamento médico linear com a família para evitar complicações secundárias da doença, bem como para o manejo adequado de suas apresentações.

Osler-Weber-Rendu syndrome or hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease that changes the muscular and elastic layer of the blood vessel wall, making them fragile and more susceptible to bleeding secondary to trauma and arteriovenous malformations (AVM) ­ a condition that commonly affects the brain, lungs, gastrointestinal tract, and liver. This study aims to perform a literature review on this syndrome, as well as on the best treatment and management for gastrointestinal bleeding from angioctasia. To this end, it reports the case of a 62-year-old female patient with recurrent episodes of hematemesis and melena, who was admitted to hospital with hemodynamic instability and low hemoglobin levels, requiring multiple blood transfusions. After clinical interview, which indicated family history of recurrent epistaxis, physical examination, and upper digestive endoscopy showing gastric angiectasias, the patient was diagnosed with Osler-Weber-Rendu syndrome. Angiectasias were treated with endoscopic argon plasma. Considering the risk of severe hemorrhages and its more pronounced phenotypic expression with advancing age, HHT is an underdiagnosed disease that deserves special attention. Due to its hereditary nature, a linear medical follow-up with the family is necessary to avoid secondary complications of the disease, as well as the proper management of its presentations.

El síndrome de Rendu-Osler-Weber o telangiectasia hemorrágica hereditaria (THH) es una enfermedad autosómica dominante de penetrancia variable, que modifica la capa muscular y elástica de la pared de los vasos sanguíneos, provocando una gran fragilidad y dilatación de estas, haciéndolas susceptibles a hemorragias secundarias a trauma y malformaciones arteriovenosas (MAV), que son las más comunes en el cerebro, pulmones, tracto gastrointestinal e hígado. Este estudio tiene como objetivo realizar una revisión de este síndrome, así como la mejor terapia y manejo del sangrado gastrointestinal por angioctasia en esta patología. Se trata de un paciente de 62 años, con episodio de hematemesis recurrente y melena, que ingresa hemodinámicamente inestable y con descenso significativo de los niveles de hemoglobina, requiriendo múltiples transfusiones sanguíneas. Tras entrevista clínica, destacando los antecedentes familiares de patologías similares, epistaxis recurrente, exploración física y angiectasias gástricas a endoscopia digestiva alta, se asignó el diagnóstico de síndrome de Rendu-Osler-Weber. Las angiectasias se erradicaron con terapia endoscópica con plasma de argón. La HHT es una enfermedad infradiagnosticada que merece una atención especial por su riesgo de hemorragias severas, con una expresión fenotípica más pronunciada con el avance de la edad. Además de su carácter hereditario, es necesario un seguimiento médico lineal con la familia para evitar complicaciones secundarias de la enfermedad, así como el adecuado manejo de sus presentaciones.

Malformaciones vasculares cerebrales de alto flujo en pediatría: experiencia en un hospital pediátrico de alta complejidad / High-flow vascular malformations of the brain in pediatrics: Experience in a tertiary care children's hospital

Introducción. Las malformaciones vasculares cerebrales de alto flujo son poco comunes en la edad pediátrica. El objetivo del trabajo es diferenciar y agrupar estas enfermedades según edad de debut, manifestaciones clínicas y angioarquitectura.Población y método. Se realizó un estudio retrospectivo y observacional. Se analizaron las historias clínicas, los estudios por imágenes y los protocolos de procedimientos de pacientes del Hospital J. P. Garrahan con diagnóstico de malformaciones vasculares cerebrales desde enero de 2010 hasta enero de 2020.Resultados. Ciento ochenta y tres pacientes cumplieron los criterios de inclusión. Se identificaron 131 pacientes con malformaciones arteriovenosas con nido (MAV) y 52 con fístulas directas (sin nido), entre los que se hallaron 19 malformaciones aneurismáticas de vena de Galeno, 23 fístulas piales y 10 fístulas durales. La edad promedio fue de 105 meses para las MAV, 1,7 meses para las malformaciones aneurismáticas de vena de Galeno, 60,5 meses para fístulas piales y 41 meses para fístulas durales.Conclusión. Según su angioarquitectura, las malformaciones vasculares cerebrales de alto flujo tuvieron nido (MAV) o fueron fístulas directas (malformaciones aneurismáticas de vena de Galeno, fístulas piales y fístulas durales). Las MAV se manifestaron a partir de la primera infancia, sobre todo, por hemorragia intracraneana. Las fístulas directas se expresaron en la primera etapa de la vida, frecuentemente, con insuficiencia cardíaca.

Introduction. High-flow vascular malformations of the brain are uncommon in pediatrics. The objective of this study is to establish the differences among these pathologies and group them by age at onset, clinical manifestations, and angioarchitecture.Population and method. This was a retrospective and observational study. The medical records, imaging studies, and procedure protocols of patients seen at Hospital J. P. Garrahan diagnosed with vascular malformations of the brain between January 2010 and January 2020 were analyzed.Results. A total of 183 patients met the inclusion criteria. It was possible to identify 131 patients with arteriovenous malformations with a nidus (AVMs) and 52 with direct fistulas (without a nidus), including 19 vein of Galen aneurysmal malformations, 23 pial fistulas, and 10 dural fistulas. The average age of patients was 105 months for AVMs, 1.7 months for vein of Galen aneurysmal malformations, 60.5 months for pial fistulas, and 41 months for dural fistulas.Conclusion. Based on their angioarchitecture, high-flow vascular malformations of the brain presented a nidus (AVMs) or direct fistulas (vein of Galen aneurysmal malformations, pial fistulas, and dural fistulas). AVMs were observed in early childhood, especially due to intracranial hemorrhage. Direct fistulas occurred in the first stage of life, commonly with heart failure.

Uterine rescue in high-risk gestational trophoblastic neoplasia treated with ema-co by uterine arteries embolization due to arteriovenous malformations / Resgate uterino em neoplasia trofoblástica gestacional de alto risco tratada com EMA-CO por embolização de artérias uterinas devido a malformações arteriovenosas

Abstract Complete hydatidiform mole (CHM) is a rare type of pregnancy, in which 15 to 20% of the cases may develop into gestational trophoblastic neoplasia (GTN). The diagnostic of GTN must be done as early as possible through weekly surveillance of serum hCG after uterine evacuation.We report the case of 23-year-old primigravida, with CHM but without surveillance of hCG after uterine evacuation. Two months later, the patient presented to the emergency with vaginal bleeding and was referred to the Centro de Doenças Trofoblásticas do Hospital São Paulo. She was diagnosed with high risk GTN stage/score III:7 as per The International Federation of Gynecology and Obstetrics/World Health Organization (FIGO/WHO). The sonographic examination revealed enlarged uterus with a heterogeneous mass constituted of multiple large vessels invading and causing disarrangement of the myometrium. The patient evolved with progressive worsening of vaginal bleeding after chemotherapy with etoposide, methotrexate, actinomycin D, cyclophosphamide and vincristine (EMA-CO) regimen. She underwent blood transfusion and embolization of uterine arteries due to severe vaginal hemorrhage episodes, with complete control of bleeding. The hCG reached a negative value after the third cycle, and there was a complete regression of the anomalous vascularization of the uterus as well as full recovery of the uterine anatomy. The treatment in a reference center was essential for the appropriate management, especially regarding the uterine arteries embolization trough percutaneous femoral

Resumo Mola hidatiforme completa (MHC) é um tipo raro de gravidez, na qual 15 a 20% dos casos podem desenvolver neoplasia trofoblástica gestacional (NTG). O diagnóstico de NTG deve ser feito o mais cedo possível, pelo monitoramento semanal do hCG sérico após esvaziamento uterino. Relatamos o caso de uma paciente primigesta, de 23 anos de idade, com MHC, sem vigilância de hCG após esvaziamento uterino. Dois meses depois, a paciente compareceu na emergência com sangramento vaginal, sendo encaminhada ao Centro de Doenças Trofoblásticas do Hospital São Paulo, onde foi diagnosticada com NTG de alto risco, estádio e score de risco III:7 de acordo com a The International Federation of Gynecology and Obstetrics/Organização Mundial de Saúde (FIGO/OMS). O exame ultrassonográfico revelou útero aumentado com uma massa heterogênea constituída pormúltiplos vasos volumosos invadindo e desestruturando o miométrio. A paciente evoluiu com piora progressiva do sangramento vaginal após quimioterapia com o regime etoposide, methotrexate, actinomycin D, cyclophosphamide and vincristine (EMA-CO). Ela foi submetida a transfusão de sangue e embolização das artérias uterinas devido aos episódios graves de hemorragia vaginal, com completo controle do sangramento. O hCG atingiu valor negativo após o terceiro ciclo, havendo regressão completa da vascularização uterina anômala, assim como recuperação da anatomia uterina. O tratamento em um centro de referência permitiu o manejo adequado, principalmente no que se refere à embolização das artérias uterinas através da punção percutânea da artéria femoral, que foi crucial para evitar a histerectomia, permitindo a cura da NTG e a manutenção da vida reprodutiva.

Sudden convulsion with multiple pulmonary nodules in a girl aged 15 years / 中国当代儿科杂志

A girl, aged 15 years, was admitted due to sudden convulsion once and multiple pulmonary nodules on lung CT. Acrocyanosis or acropachy/toe deformity was not observed. Laboratory examinations showed an increase in hemoglobin (162 g/L) and a reduction in arterial partial pressure of oxygen (61.5 mm Hg). Lung CT showed irregular slightly high-density nodules in the middle lobe of the right lung, and contrast-enhanced CT scan showed obvious enhancement with thick vascular shadow locally. An investigation of medical history revealed that the girl's mother had a history of epistaxis and resection of pulmonary mass and the girl presented with tongue telangiectasia. The girl was diagnosed with hereditary hemorrhagic telangiectasia and pulmonary arteriovenous malformation. she was given interventional embolization therapy. Transcutaneous oxygen saturation reached 98% without oxygen inhalation on the day after surgery. Pulmonary angiography at 3 months after surgery showed the recurrence of pulmonary vascular malformation, and embolization of pulmonary arterial fistula was not performed since the guide wire could not enter the branch artery. There was still a need for long-term follow-up.

Prenatal Diagnosis of Vein of Galen Aneurysmal Malformation Allows Early Transumbilical Endovascular Treatment

Neonates with vein of Galen aneurysmal malformation (VGAM) presenting with severe cardiac failure and pulmonary hypertension represent a challenge for endovascular therapy.When early treatment is required, the small femoral arteries in this population are usually difficult to cannulate. Alternatively, the umbilical vessels offer a natural pathway to reach the lesion. Therefore, prenatal diagnosis of VGAM allows for delivery planning, perinatal management, and embolization through umbilical approach, thus leading to better outcomes.

Fertility preserving surgical approach to uterine arteriovenous malformation / Philippine Journal of Obstetrics and Gynecology

@#Arteriovenous malformations (AVM) are vascular disorders with a mixture of arterial, venous and small capillary-like channels with fistulous connections. Uterine arteriovenous malformations are rare cause of abnormal uterine bleeding with only a few reported cases. They may arise from pregnancy, miscarriage, previous cesarean section or other uterine surgery and gestational trophoblastic disease. Diagnosis can be made through angiography or doppler ultrasonography. Traditionally, uterine AVMs are treated with hysterectomy but with the advances in technology, minimally invasive conservative approaches such as radiologic arterial embolization or laparoscopic uterine artery ligation have become available. We present a case of a 29-year-old, G2P1 (1011) who had a three- month history of heavy, intermittent vaginal bleeding from uterine arteriovenous malformation after a miscarriage. Laparoscopic bilateral uterine artery occlusion, offered a minimally invasive treatment with high symptomatic effectiveness.

Síndrome de Osler-Weber-Rendu: presentación de un caso clínico y revisión de la literatura / Osler-Weber-Rendu syndrome: a case report and literature review

El síndrome de Osler-Weber-Rendu, también conocido como telangiectasia hemorrágica hereditaria, es una enfermedad de herencia autosómica dominante de baja prevalencia, que se caracteriza por unas paredes delgadas de los vasos sanguíneos que conducen a malformaciones arteriovenosas, principalmente en cerebro, pulmón e hígado, pero que pueden potencialmente afectar cualquier otro órgano. La principal manifestación clínica son las epistaxis recurrentes; sin embargo, también pueden aparecer otras manifestaciones como hemoptisis o sangrado gastrointestinal, y telangiectasias mucocutáneas, entre otras. Su expresión clínica varía de un paciente a otro y el diagnóstico usualmente se basa en los criterios de Curazao. Se describe el caso de una paciente con historia de 12 años de epistaxis recurrente, a quien se le diagnosticó síndrome de Osler-Weber-Rendu hace dos años. Fue referida al servicio de Hepatología por presentar compromiso hepático, además de malformaciones arteriovenosas cerebrales y pulmonares. El manejo óptimo de la telangiectasia hemorrágica en esta paciente por parte de los diferentes especialistas, apoyado en los estudios de imagenología, ha permitido que la paciente tenga una buena calidad de vida hasta el momento. Se presenta el caso de esta paciente de 46 años y se realiza una revisión corta de la literatura.

Osler-Weber-Rendu syndrome, also known as hereditary hemorrhagic telangiectasia, is an autosomal dominant low-prevalence disease, characterized by thin blood vessel walls, leading to arteriovenous malformations, mainly in the brain, lung and liver, but can potentially affect any other organ. The main clinical manifestation is recurrent epistaxis; however, other manifestations may also appear, such as hemoptysis or gastrointestinal bleeding, and mucocutaneous telangiectasias, among others. Its clinical expression varies from patient to patient and the diagnosis is usually based on Curacao criteria. We present the case of a patient with a 12-year history of recurrent epistaxis, who was diagnosed with Osler-Weber-Rendu syndrome two years ago. She was referred to the Hepatology service due to liver findings, in addition to brain and pulmonary arteriovenous malformations. The optimal management of hemorrhagic telangiectasia in this patient by the different specialists, supported by imaging studies, has allowed the patient to have a good quality of life. The case of this 46-year-old patient is presented, and a brief literature review is also provided.

Abnormalities of the hallux skin and nail in the course of very rare arteriovenous malformation / Anormalidades de unha e pele do hálux no decorrer de malformação arteriovenosa muito rara

Abstract Arteriovenous malformations (AVMs) are usually found in the pelvic area and the brain. These vascular anomalies are rarely reported in the toes. AVMs in the toes may be asymptomatic, but can also cause atypical symptoms. Congenital AVMs can expand as patients age and manifest in adulthood. They may be provoked by injury. Acquired AVM might be caused by iatrogenic factors, venous or arterial catheterization, percutaneous invasive vascular procedures, surgery, or degenerative vascular disorders. An AVM can damage surrounding tissues and can cause destruction of skin, nails and bones. The course of the disease is often unpredictable and diagnosis is usually delayed as a result.

Resumo As malformações arteriovenosas (MAVs) são geralmente encontradas na região pélvica e no cérebro.. Essas anomalias vasculares raramente são relatadas nos dedos dos pés. A MAV nesse local pode ser assintomática ou apresentar sintomas atípicos. MAVs congênitas podem evoluir com a idade e se manifestar na idade adulta. O fator provocante pode ser uma lesão traumática. Uma MAV adquirida pode ser causada por fatores iatrogênicos, cateterismo venoso e arterial, procedimentos percutâneos vasculares invasivos, cirurgias e alterações degenerativas vasculares. A MAV pode danificar tecidos adjacentes e pode causar destruição de pele, unhas e ossos. O curso da doença é muitas vezes imprevisível, e como resultado, atrasar o diagnóstico.

Rare occurrence of uterine arteriovenous malformation clinically mimicking a malignant growth: A critical reminder for pathologists

Arteriovenous malformation (AVM) is a rare lesion in the uterus, which can lead to abnormal uterine bleeding. While AVM has been described in other organs in the literature, there is a paucity of pathology reports of the AVM in uterus. On gross examination, the uterus was markedly enlarged and partly distorted with a pedunculated solid mass, which on the cut surface showed multiple well-circumscribed hemorrhagic cysts ranging from 0.1 to 4.0 cm in size. Microscopically, they were malformed dilated vascular structures containing organized thrombi. We present this case of uterine AVM with gross and microscopic findings, which can serve as a crucial reminder for pathologists to keep in the differential diagnoses as a potential cause of abnormal uterine bleeding.

Cranial tomographic angiographic evaluation of suspected intracranial vascular abnormalities among a Nigerian cohort

Background: Lately, there has been an increased utilization of computed tomography angiography (CTA) as the preferred first-line modality for the evaluation and diagnosis of most cerebral vascular lesions.Objective: The objective of this study was to evaluate suspected intracranial vascular cases, using CTA at a major referral tertiary hospital in South West Nigeria.Materials and Methods: This was a hospital-based retrospective study of suspected intracranial vascular cases in all ages and both sexes that had CTA from January 2011 to December 2018. Data were analyzed with IBM SPSS version 23.0, and P < 0.005 was considered statistically significant.Results: A total of 128 patients were studied, the mean age was 44.1 ± 17.7 years, and male: female ratio was 1:1.06. The leading clinical diagnoses were as follows: intracranial aneurysms (34/128), subarachnoid hemorrhage (27/128), intracranial vascular tumors (26/128), brain hemorrhage from vascular abnormality (19/128), and arteriovenous malformations (AVMs) (10/128). At CTA, 61 patients had vascular abnormalities: intracranial aneurysm was seen in 63.9% with a peak age range of 41­60 years, and the leading location of aneurysms was posterior cerebral artery (18.8%), followed by posterior communicating artery (16.7%) and the cavernous segment of the internal carotid artery (16.7%). AVMs were more common in patients aged 40 years and below (91.7%) in males (66.7%) and in the parietal lobe. Intracranial aneurysms were 3.25 times as common as brain AVMs.Conclusion: Intracranial aneurysms are the predominant vascular lesions, occurring mostly in the older age group. AVMs occurred mostly in younger people, more in males, and predominantly in the parietal lobes. The hospital incidence of aneurysms to AVMs was 3.25:1

Utilidad de las Escalas de Gradación en el Tratamiento Quirúrgico de Malformaciones Arteriovenosas Cerebrales / Usefulness of the Gradation Scales in the Surgical Treatment of Cerebral Arteriovenous Malformations

Introducción: Las malformaciones arteriovenosas (MAVs) cerebrales comprenden una compleja patología responsable de hasta el 38% de las hemorragias en pacientes de entre 15-45 años, acarreando cada episodio de sangrado un 25-50% de morbilidad y un 10-20% de mortalidad. La decisión terapéutica en un paciente con una MAV debe tener en cuenta la comparación entre los riesgos propios de la intervención y los de la historia natural de esta enfermedad. Objetivo: Evaluar la utilidad de predecir riesgo quirúrgico de diferentes escalas de gradación de MAV cerebrales según nuestra experiencia en una serie de casos. Material y Métodos: Se realizó un análisis bibliográfico de escalas de gradación de riesgo quirúrgico de MAV cerebrales utilizando como motor de búsqueda Pubmed incluyendo como palabras clave "malformación arteriovenosa cerebral" y "escala de gradación" (brain arteriovenous and malformation grading scale). Se analizaron de forma retrospectiva aquellos pacientes intervenidos quirúrgicamente por MAV en este hospital público, se las clasificó acorde a las escalas analizadas y se compararon los resultados obtenidos con los previstos en ellas. Resultados: Se analizaron 90 pacientes intervenidos quirúrgicamente por MAV, sin tratamiento coadyuvante. De forma retrospectiva se los agrupó acorde a las escalas de Spetzler Martin (SM), Spetzler-Ponce (SP) y suplementaria de Lawton. Las MAV grado 3 se subclasificaron según las escalas de Lawton y de de Oliveira. Considerando buenos resultados aquellos con Rankin modificado (mRs) igual o menor a 2. Con un rango de seguimiento de 12 a 48 meses, encontramos buenos resultados en el 100% de MAV SM grado 1, 91.7% de las grado 2, 80% en grado 3 y 42.9% en grado 4. Utilizando la escala SP, 93.7% de buenos resultados en tipo A, 80% en tipo B y 42.9% en tipo C. Subclasifican-do las MAV SM 3 acorde a las escalas de de Oliveira y Lawton, 84% de buenos resultados en el tipo 3A, 71.3% en las 3B, 92% en MAV tipo 3-, 72.1% en el tipo 3+, 60% en tipo 3. Utilizando la escala suplementaria de Lawton combinada con SM, buen resultado en 100% grados II y III, 85,7% grado IV, 87,6 grado V, 80% grado VI, 75% grado VII y 66,6% grado VIII. Conclusión: Reafirmamos en esta serie, la utilidad de estimar riesgo quirúrgico con las escalas SM, SP, y la subclasificación de las MAV grado 3 propuesta por Lawton. Y principalmente el utilizar la escala suplementaria de Lawton-Young al considerar el tratamiento quirúrgico de los pacientes con MAV que sangraron.

Introduction: Brain arteriovenous malformations (AVM) are a complex disease responsible for up to 38% of hemorrhages in patients between 15-45 years old, carrying every bleeding episode a 25-50% risk of morbidity and a 10-20% of mortality. The therapeutic decision in a patient with an AVM needs to consider both the risks of the intervention and the risks of the natural evolution of the disease. Objective: To assess the effectiveness of different AVM grading scales in predicting surgical risks according to our experience in a case series. Material and Method: a literature review of the AVM grading scales was made, through Pubmed including as key words "brain arteriovenous malformations" and "grading scale". A retrospective analysis was made of patients with AVM who were operated in our institution, they were classified according to the scales and their results were compared. Results: 90 patients were operated in our institution with AVM. Retrospectively, they were classified according to the Spetzler-Martin (SM), Spetzler-Ponce (SP), Lawton supplementary, and the sub-classifications in AVM grade 3, from Lawton and de Oliveira. Good outcome were considered when modified Rankin Scale (mRs) was equal or less than 2. The follow-up ranged from 12-48 months, having good outcome in 100% of AVM SM grade I, 91,7% grade II, 80% in grade III and 42,9% in grade IV. Using the SP scale, 93,7% of good outcome in grade A, 80% in grade B and 42,9% in grade C. In the sub-classification of AVM SM 3, we found 84% of good outcome in type 3A de Oliveira and 71,3% in type 3B. According to the Lawton scale, good outcome were found in 92% in type 3-, 72,1% in type 3+ and 60% in type 3. Using Lawton supplementary scale combined with SM, there were 100% of good outcome in grades II and III, 85,7% in grade IV, 87,6% in grade V, 80% in grade VI, 75% in grade VII, 66,6% in grade VIII. Conclusion: In our series, we reaffirm the effectiveness to predict surgical risk of the following scales: SM, SP and the Lawton's sub-classification of AVM grade 3. Specially, the use of the supplementary Lawton-Young scale in the surgical treatment of bleeding AVMs.