Angiodysplasia Combined with Thymic Aplasia in an Infant: A Rare Clinical Case

Background. The prevalence of congenital malformations of the vessels or angiodysplasia ranges from 1:50,000 to 1:5,000,000. Congenital angiodysplasia is a consequence of impaired formation and development of the vascular system in embryogenesis. The aetiology of angiodysplasia remains poorly studied, and the diagnosis involves significant difficulties in some cases. Clinical Case Description. The observation of a rare case of a combined malformation of vessels and thymic aplasia in a female infant is presented. Angiodysplasia included the syndrome of congenital generalised phlebectasia (synonym: congenital telangiectatic marbled skin) combined with multiple vascular malformations with predominant vascular lesions of the brain, lungs, heart, kidneys, and mesentery. Clinically, the disease was characterised by a generalised change in the skin in the form of livedo reticularis accompanied by the development of severe pneumonia, persistent urinary syndrome, neurological symptoms (convulsive seizures, motor disorders), and progressive heart failure. The diagnosis was confirmed in the course of a pathological study. Conclusion. The presented case allows expanding the notion of the variety of clinical manifestations of congenital angiodysplasia, as well as its possible combinations with other malformations.

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Clinical case of semilobar holoprosencephaly

Лечащий врач ◽

10.51793/os.2021.24.9.005 ◽

2021 ◽

Author(s):

А.В. Серёжкина ◽

И.Г. Хмелевская ◽

Н.С. Разинькова ◽

Т.А. Миненкова ◽

И.И. Жизневская ◽

...

Keyword(s):

Clinical Case ◽

Fatal Outcome ◽

Clinical Manifestations ◽

Specific Treatment ◽

Stable Condition ◽

Horseshoe Kidney ◽

Structural Defects ◽

Surgical Interventions ◽

Ultrasound Diagnostics ◽

The Brain

Поскольку непосредственные этиологические факторы аномалии развития не известны, в работе выделены основные группы причин, способствующих формированию пороков развития головного мозга. Большое значение отводится ранней диагностике пороков развития, которая позволяет своевременно решить вопрос о возможности пролонгирования беременности, что определяется видом порока, совместимостью с жизнью и прогнозом в отношении постнатального развития. В исследовании рассматриваемого порока развития большую роль играют такие современные методы, как пренатальная ультразвуковая диагностика, нейросонография, рентгеновская компьютерная и магнитно-резонансная томография головного мозга, имеющие достаточно высокую информативность. Указаны сроки гестации, позволяющие выявить структурные дефекты головного мозга. Медико-генетическое консультирование помогает выявить риск появления больного потомства. Проведена дифференциальная диагностика семилобарной с другими формами голопрозэнцефалии. Также отмечены возможные клинические проявления рассматриваемой нозологии. В данной статье представлен клинический случай семилобарной голопрозэнцефалии, диагностированной у мальчика в возрасте 1 месяц. При поступлении мать предъявляла жалобы на срыгивания и периодическое беспокойство сына. Объем и результаты обследования ребенка изложены ниже. Выявлена сопутствующая патология в виде пупочной грыжи, врожденной аномалии развития мочевой системы: подковообразная почка; водянки яичек и головчатой формы гипоспадии. После проведения курса поддерживающей терапии пациент был выписан в стабильном состоянии. В настоящее время специфическое лечение голопрозэнцефалии отсутствует. Оперативные вмешательства на головном мозге проводятся редко ввиду тяжести состояния больных, в связи с чем лечение данной патологии возможно только с помощью хирургической коррекции симптомов. Длительная дыхательная и кардиоваскулярная дисфункция предопределяет летальный исход заболевания. Since the direct etiological factors of developmental abnormalities are not known, the main groups of causes contributing to the formation of brain malformations are identified in the work. Great importance is given to the early diagnosis of malformations, which allows us to timely resolve the issue of the possibility of prolonging pregnancy, which is determined by the type of defect, compatibility with life and the prognosis for postnatal development. In the study of the considered malformation, such modern methods as prenatal ultrasound diagnostics, neurosonography, X-ray computer and magnetic resonance imaging of the brain, which have a sufficiently high informative value, play an important role. The terms of gestation are indicated, which make it possible to identify structural defects of the brain. Medical and genetic counseling helps to identify the risk of the appearance of sick offspring. The differential diagnosis of semilobar with other forms of holoprosencephaly was carried out. Possible clinical manifestations of the nosology under consideration are also noted. This article presents a clinical case of semilobar holoprosencephaly diagnosed in a boy at the age of 1 month. Upon admission, the mother complained of regurgitation and periodic anxiety of her son. The scope and results of the child's examination are set out below. Concomitant pathology was revealed in the form of an umbilical hernia, a congenital abnormality of the development of the urinary system: a horseshoe kidney; dropsy of the testicles and a cephalic form of hypospadias. After a course of maintenance therapy, the patient was discharged in a stable condition. Currently, there is no specific treatment for holoprosencephaly. Surgical interventions on the brain are rarely performed due to the severity of the patients ' condition, and therefore, the treatment of this pathology is possible only with the help of surgical correction of symptoms. Prolonged respiratory and cardiovascular dysfunction determines the fatal outcome of the disease.

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Rapidly progressive course of nonspecific aortoarteritis: a clinical case

Clinical Practice ◽

10.17816/clinpract54587 ◽

2020 ◽

Vol 11 (4) ◽

pp. 83-89

Author(s):

Marina V. Grushina ◽

Ilya Sergeevich Grekov ◽

Kseniya D. Arkhypova

Keyword(s):

Cerebral Ischemia ◽

Arterial Hypertension ◽

Clinical Case ◽

Carotid Arteries ◽

Correct Diagnosis ◽

Clinical Manifestations ◽

Practical Interest ◽

Diagnostic Errors ◽

Vascular Lesions ◽

First Year

Background. Nonspecific aortoarteritis, or Takayasus disease, is one of the most complex and rare pathologies in modern clinical practice. It is the orphan nature of the disease, along with non-specific clinical manifestations, that causes a large number of clinical and diagnostic errors that lead to an unfavorable prognosis and early disability of patients. Despite the development of modern methods of treatment of nonspecific aortoarteritis, in some cases it is not possible to achieve a stable remission, which leads to a steady progression of the pathological process. Clinical case description. The article presents a case of a rapidly progressing course of Takayasu's disease in a young woman with multiple arterial vascular lesions that developed during the first year after the onset of arterial hypertension, while the narrowing of the carotid arteries (7585%) was not accompanied by signs of cerebral ischemia. The follow-up period was 10 years. Conclusion. Given the peculiarities of this nosology, each identified case of Takayasus disease is of great clinical and practical interest. The disease peculiarity in thise patient is that during the first year from the onset of arterial hypertension, the main occlusive lesions of the aorta and arterial vessels were identified. At the same time, the narrowing of the carotid arteries (7585%) was not accompanied by signs of cerebral ischemia. It should be noted that often the symptoms of non-specific aortoarteritis appear under the masks of other diseases, which requires a careful differential search. A correct diagnosis and timely treatment can prevent the development of complications and slow the progression of the disease.

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The Histopathological Criteria of Vascular Disturbances in the Brain.∗

Journal of Mental Science ◽

10.1192/bjp.83.346.509 ◽

1937 ◽

Vol 83 (346) ◽

pp. 509-509 ◽

Cited By ~ 2

Author(s):

Alfred Meyer

Keyword(s):

Blood Vessels ◽

Cardiac Disease ◽

Vascular System ◽

Head Injuries ◽

Common Factor ◽

Mental Deficiency ◽

Vascular Lesions ◽

Organic Lesion ◽

Histological Appearance ◽

The Brain

Vascular lesions, identical in appearance with the sequelae of organic diseases of the blood-vessels, have been found in a great variety of conditions (for instance in epilepsy, hypertension and cardiac disease, psychoses associated with infectious and toxic conditions, head injuries, certain groups of mental deficiency) in which no organic lesion of the blood-vessels themselves could be demonstrated. The theory has been put forward (Ricker, Spielmeyer and others) that these lesions owe their origin to functional disorder of the vascular system, and that this common factor accounts for the identity in histological appearance in spite of the profound ætiological differences.

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Vascular dementia

New Oxford Textbook of Psychiatry ◽

10.1093/med/9780199696758.003.0048 ◽

2012 ◽

pp. 375-384

Author(s):

Timo Erkinjuntti

Keyword(s):

Cognitive Impairment ◽

Vascular Dementia ◽

Cerebrovascular Disorders ◽

Clinical Manifestations ◽

Executive Dysfunction ◽

Vascular Lesions ◽

Different Types ◽

Dementia Research ◽

Definition Of ◽

The Brain

Vascular dementia is the second most frequent cause of dementia. Because vascular causes of cognitive impairment are common, may be preventable, and the patients could benefit from therapy, early detection, and accurate diagnosis of vascular dementia is desirable. Vascular dementia is not only multi-infarct dementia, but is related to other vascular mechanisms and pathological changes in the brain, and has other causes and clinical manifestations. Vascular dementia is not a disease, but a syndrome. The origin of this syndrome reflects complex interactions between vascular aetiologies (cerebrovascular disorders and vascular risk factors), changes in the brain (infarcts, white-matter lesions, atrophy), host factors (age, education), and cognition. Conceptual issues related to of vascular dementia include the definition of the cognitive syndrome (type, extent, and combination of impairments in different cognitive domains), and the vascular causes (vascular aetiologies and changes in the brain). Variations in these definitions has led to different estimates of point prevalence, to different groups of patients, and to reports of different types and distribution of brain lesions. The cognitive syndrome of vascular dementia is characterized by predominate executive dysfunction rather than deficits in memory and language function. Although the course of cognitive decline may be stepwise, it is often slowly progressive, and may include periods of stability or even some improvement. The relationship between vascular lesions in the brain and cognitive impairment is important, but which type, extent, side, site, and tempo of vascular lesions in the brain relates to different types of vascular dementia is not established in detail. Current criteria for vascular dementia are based on the concept of cerebral infarcts. For example the widely used NINDS-AIREN criteria include dementia, cerebrovascular disease, and a relationship between these two disorders. The main tools for the diagnosis include detailed history, neurological examination, mental state examination, relevant laboratory examinations, and preferably magnetic resonance imaging of the brain. Vascular dementia research, until recently overshadowed by that into Alzheimer's disease, is now developing rapidly. There is great promise for intervention. Developments in classification, diagnosis, and treatment are likely.

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Paraneoplastic Vasculitis in a Patient with Astrocytoma of the Brain

The Russian Archives of Internal Medicine ◽

10.20514/2226-6704-2021-11-1-67-71 ◽

2021 ◽

Vol 11 (1) ◽

pp. 67-71

Author(s):

A. V. Yagoda ◽

A. V. Rybas ◽

E. N. Danilova ◽

Yu. V. Gromova

Keyword(s):

Clinical Case ◽

Leukocytoclastic Vasculitis ◽

Clinical Manifestations ◽

Necrotizing Vasculitis ◽

Rare Type ◽

Pulmonary Hypertension Syndrome ◽

Significant Stenosis ◽

Idiopathic Pulmonary Hypertension ◽

Breast And Prostate Cancer ◽

The Brain

Clinical case of paraneoplastic vasculitis аssociated a brain tumor was presented. Paraneoplastic vasculitis is a rare type of paraneoplastic syndrome. The frequency of detection of paraneoplastic vasculitis in cancer patients is 0.01-5%. In 70% of cases, the manifestation of vasculitis is observed long before the clinical manifestations of the tumor. Most studies report so-called leukocytoclastic vasculitis (allergic) or allergic angiitis. Vasculitis is usually accompanied by slowly progressing tumors such as breast and prostate cancer. It also develops with of stomach cancer, lung cancer, kidney adenocarcinoma, epithelioma, sarcoma, cholangiocarcinoma, other solid tumors, multiple myeloma, non-Hodgkin’s lymphoma. The nosological forms of paraneoplastic vasculitis include called polyarteritis nodosa, hemorrhagic vasculitis, Wegener’s granulomatosis, non-specific aortoarteritis, idiopathic pulmonary hypertension syndrome, thrombovasculitis, allergic hemorrhagic vasculitis, cutaneous vasculitis, systemic necrotizing vasculitis with increased ANCA titer. The patient suffered from paraneoplastic vasculitis with the development of amyloidosis of vascular tissues and arterial hypertension. The progression of the vascular process led to damage of the arteries of the brain and heart, the development of ischemic stroke and hemodynamically significant stenosis of the coronary arteries, the development of acute myocardial infarction complicated by acute heart failure, which caused death. The clinical significance of the case lies in the fact that paraneoplastic vasculitis, which was developed due to a brain astrocytoma with the formation of amyloidosis was firstly described.

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345. Dermatological Manifestations in Patients with COVID-19 Pneumonia in Veracruz, Mexico

Open Forum Infectious Diseases ◽

10.1093/ofid/ofab466.546 ◽

2021 ◽

Vol 8 (Supplement_1) ◽

pp. S276-S277

Author(s):

Luis Del Carpio-Orantes ◽

Sergio García-Méndez ◽

Jesús Salvador Sánchez-Díaz ◽

Andrés aguilar-silva ◽

Elisa Estefania Aparicio-Sánchez ◽

...

Keyword(s):

Viral Infections ◽

Clinical Manifestations ◽

Adaptive Immune System ◽

Severe Pneumonia ◽

Acute Stage ◽

Androgenetic Alopecia ◽

Vascular Lesions ◽

Cutaneous Manifestations ◽

Low Incidence ◽

The One

Abstract Background A large number of viral infections are characterized by the presence of cutaneous manifestations. Multiple dermatological manifestations have been observed in patients with COVID-19. Dermatological lesions in patients infected by SARS-CoV-2 such as livedo reticularis, rash and vascular lesions may represent manifestations of secondary phenomena such as paraviral rashes or by participation of the innate or adaptive immune system that cause vasodilation, vascular leakage or procoagulant effects Methods Descriptive and observational study, adult patients with COVID-19 pneumonia were selected, confirmed by RT-PCR and chest CT. General symptoms, hematic cytometry results, pneumonia severity, prognosis as well as dermatological manifestations are characterized. Results 100 patients were entered into the study, with an average age of 49.4 years, 54% male. The general symptoms with the highest incidence were: fever, cough and dyspnea characteristic of SARS-CoV-2 infection, followed by chest pain, headache, anosmia and dysgeusia. The main alteration of the hemogram was lymphopenia, no leukopenia or plaquetopenia was demonstrated. 54% of those affected had mild pneumonia, the rest severe pneumonia. 75% progressed towards improvement and 25% died. Among the dermatological manifestations identified, all occurred in cases with severe pneumonia, the one with the highest incidence was the morbilliform viral exanthema in 18%, the presence of diffuse partial alopecia in 7% as well as manifestations of lividity and maceration in 1%. Regarding alopecia, in 6% it was reversible androgenetic alopecia, having manifested during the acute stage of pneumonia (all men), in 1% it presented alopecia areata (male) that has been persistent beyond the acute phase and in frank recovery Demographic and clinical variables Clinical manifestations Conclusion The incidence of dermatological manifestations is low in this study population, the most frequent being the morbilliform viral exanthema expected in a virus, however they present manifestations of low incidence such as reversible androgenetic alopecia associated with severity of the disease, a finding that has been documented recently as a manifestation associated with COVID-19 Disclosures All Authors: No reported disclosures

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A clinical case of metastatic osteosarcoma in the heart cavity

Patologiya krovoobrashcheniya i kardiokhirurgiya ◽

10.21688/1681-3472-2018-1-55-60 ◽

2018 ◽

Vol 22 (1) ◽

pp. 55

Author(s):

E. I. Iofe ◽

V. G. Konov ◽

E. B. Solovyev ◽

I. I. Bridun

Keyword(s):

Conflict Of Interest ◽

Right Atrium ◽

Clinical Case ◽

Vascular System ◽

Heart Diseases ◽

Clinical Manifestations ◽

Pericardial Patch ◽

Metastatic Osteosarcoma ◽

Malignant Lesions ◽

The Right

Tumors of the heart are catalogued as a poorly studied area of clinical oncology because of an extreme rarity of this pathology, absence of pathognomonic symptoms, a large variety of clinical manifestations, and diagnostic complexity. In order to achieve diagnostic and therapeutic adequacy, clinicians need to be aware of cancer heart diseases and their frequently atypical clinical manifestations. The report presents a clinical case of tumor spreading through the vascular system and malignant lesions of the right atrium of the heart in a patient with osteosarcoma of the humerus, where osteosarcoma metastases are resected and plasty of the right atrium is performed by using a pericardial patch.Received 22 August 2017. Revised 7 December 2017. Accepted 11 December 2017.Funding: The study did not have sponsorship.Conflict of interest: Authors declare no conflict of interest.

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Automatic Brain Segmentation in Time-of-Flight MRA Images

Methods of Information in Medicine ◽

10.3414/me9237 ◽

2009 ◽

Vol 48 (05) ◽

pp. 399-407 ◽

Cited By ~ 25

Author(s):

D. Säring ◽

J. Fiehler ◽

T. Illies ◽

D. Möller ◽

H. Handels ◽

...

Keyword(s):

Brain Tissue ◽

Vascular System ◽

Vascular Malformations ◽

Automatic Segmentation ◽

Time Of Flight ◽

Volume Visualization ◽

Brain Segmentation ◽

The Individual ◽

The Brain ◽

Cerebral Vascular

Summary Objectives: Cerebral vascular malformations might, caused by ruptures, lead to strokes. The rupture risk depends to a great extent on the individual anatomy of the vasculature. The 3D Time-of-Flight (TOF) MRA technique is one of the most commonly used non-invasive imaging techniques to obtain knowledge about the individual vascular anatomy. Unfortunately TOF images exhibit drawbacks for segmentation and direct volume visualization of the vasculature. To overcome these drawbacks an initial segmentation of the brain tissue is required. Methods: After preprocessing of the data is applied the low-intensity tissues surrounding the brain are segmented using region growing. In a following step this segmentation is used to extract supporting points at the border of the brain for a graph-based contour extraction. Finally a consistency check is performed to identify local outliers which are corrected using non-linear registration. Results: A quantitative validation of the method proposed was performed on 18 clinical datasets based on manual segmentations. A mean Dice coefficient of 0.989 was achieved while in average 99.56% of all vessel voxels were included by the brain segmentation. A comparison to the results yielded by three commonly used tools for brain segmentation revealed that the method described achieves better results, using TOF images as input, which are within the inter-observer variability. Conclusion: The method suggested allows a robust and automatic segmentation of brain tissue in TOF images. It is especially helpful to improve the automatic segmentation or direct volume rendering of the cerebral vascular system.

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Vascular Lesions with Insanity

Journal of Mental Science ◽

10.1017/s0368315x00000578 ◽

1877 ◽

Vol 23 (101) ◽

pp. 76-85

Author(s):

G. Hunter Mackenzie

Keyword(s):

Nerve Cell ◽

Blood Supply ◽

Functional Activity ◽

Vascular System ◽

Vascular Lesions ◽

Structural Elements ◽

Cause And Effect ◽

Normal Functional ◽

The Brain

The determining causes of the pathological states met with in the cerebral cells may be due to morbid activity of the cells themselves (Virchow), or to abnormal conditions of the vascular system influencing their nutrition. These may be—(1) inefficiency of the vis-a-tergo; (2) diminution of the nutritive properties of the blood; or, (3) obstruction in the vessels to the blood-supply; and their frequent association with insanity affords every reason for regarding them, in numerous instances, as direct cause and effect, and for viewing many of the changes in the brains of the insane as of a secondary nature—the results of defective nourishment. Mal-nutrition of a nerve-cell must invariably induce abrogation or diminution of its normal functional activity, with atrophy of its structural elements. The condition of fatty and granular degeneration of the cellular elements of the brain is so universally met with, as to fail to account for the great diversity of clinical phenomena, and hence the various morbid states of the vascular system in the insane become of considerable primary etiological importance. As results of this disordered circulation, there is induced an asthenic irritability, and subsequently the destruction of the normal excitability of the cerebrum, associated with the characteristic physical appearances of wasting and decay.

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Imaging Spectrum of Hemangioma and Vascular Malformations of the Head and Neck in Children and Adolescents

Journal of Clinical Imaging Science ◽

10.4103/2156-7514.135179 ◽

2014 ◽

Vol 4 ◽

pp. 31 ◽

Cited By ~ 17

Author(s):

Venkatraman Bhat ◽

Paul C Salins ◽

Varun Bhat

Keyword(s):

Head And Neck ◽

Vascular System ◽

Vascular Malformations ◽

Imaging Techniques ◽

Management Strategies ◽

Image Data ◽

Neck Region ◽

Vascular Lesion ◽

Vascular Lesions ◽

Vascular Tumors

Vascular lesions of the head and neck region in children constitute an interesting group of lesions that benefit immensely from imaging techniques. Imaging is essential for identification, characterization, and delineation of the extent of lesion and subsequent follow-up. Infantile hemangiomas, which are vascular tumors with a specific evolution pattern, constitute a large majority of these lesions. On the other hand, there are vascular malformations, which are anomalies of the vascular system, consisting of a range of vascular tissues associated with various flow patterns. When diagnosis is clinically evident, imaging should utilize non-radiation techniques and address the issues necessary for management. Timing and interpretation of imaging methods employed in assessing childhood vascular lesion should also take into consideration the natural history so that imaging is performed to address a specific question. This review highlights the typical appearance of a hemangioma and a group of vascular malformations of the head and neck. For descriptive purpose, an attempt has been made to group lesions into specific subsites, with each one having specific clinical significance. Cases included illustrate the spectrum of the disease ranging from classical form in young children to slightly differing manifestations of the disease in adolescents and adults. The illustrations also provide a novel way of presenting image data using volume-rendering techniques of 3D data. Multi-modality team interaction and management strategies of these complex lesions are also emphasized.

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