ANGIOMATOSIS (HAMARTOMATOUS HEM-LYMPHANGIOMATOSIS)

PEDIATRICS ◽  
1961 ◽  
Vol 28 (1) ◽  
pp. 65-76
Author(s):  
Peter J. Koblenzer ◽  
Martin J. Bukowski

A case is described of a diffuse, possibly generalized, abnormality of a hamartomatous nature of the peripheral vascular system. A number of cases from the literature, which also appear to belong in this category, are summarized. Histologic examination shows that lymph and blood vessels may both be involved, though this may be essentially an abnormality of the lymphatic system in which extensive venolymphatic communications occur. The clinical manifestations vary according to the site of involvement and the extent of dissemination and also according to whether the lesions are predominantly hemangiomatous or lymphangiomatous. The main features are osteolytic lesions, visceromegaly, cutaneous hemangiomas or lymphangiomas and massive effusions into any body cavity. The effusions are usually chylous, sanguineous or a mixture of both. Any or all of these features may be present in any one case. The disease frequently has its onset in childhood or adolescence and tends to be progressive. If the lesions are widely disseminated or an effusion into a body cavity is present the outlook is grave. Treatment so far has been unsuccessful. Surgery may occasionally have a place. The term angiomatosis is employed to denote this condition not only to underline its potentially extensive nature but also to avoid debate as to whether it is essentially hemangiomatosis or lymphangiomatosis.

This memoir contains a detailed description, with illustrations, of the intracranial blood-vessels of the Tuatara, of which no account has hitherto been published. The description is belived to be more complete than any hithero given for any reptile, and a considerable number of vessels are described which have not hithero been noted in Lacertilia. This comparative completeness of detail is largely due to the employment of a special method of investigation. By this method the entire contents of the cranial cavity are fixed and hardened in situ , and are then in excellent condition either for dissection or for histological purposes. The brain does not occupy nearly the whole of the cranial cavity, there being a very large subdural space (especially above the brain), across which many of the blood-vessels run, together with delicate strands of connective tissue which connect the dura mater with pia. The eyeballs are removed and an incision is made on each side in the cartilaginous wall which separates the cranial cavity from the orbit. Acetic bichromate of potash (made up according to the formula given by Bolles Lee) is injected in to the cranial cavity through these incision, and the entire animal, after opening the body cavity, is suspended in a large volume of the same fluid for about five days, and then graded up to 70 per cent. Alcohol. When the cranial cavity is now opened up the cerebral vessels are seen with extraordinary distinctness, although they have not been artifically injected. Futher details were made out by means of serial sections, both transverse and longitudinal, and both of the adult and of advanced embroyes (Stage S). In most respect the arrangement of the intracranical blood-vessels agrees with found in the Lacertilia, so far as these have been investigated, but there is an important difference in the fact that the posterior cephalic vein leaves the cranial cavity through the foramen jugulare and not through the foramen magnum, while a slightly more primitive condition is shown in the less complete union of the right and left halves of the basilar artery. Sphenodon makes some approach to the condition of the Chelonia in this latter respect, but differs conspicuously from this group in the fact that the circle of Willis is not completed anteriorly, as well as in the fact that no branch of the posterior cephalic vein leaves the cranial cavity through the foreman magnum. A very characteristic features of Sphenodon is the development of large transverse sinues resembling those of the crocodile, but these communicate with the extracranial vascular system in quite a different manner from that described by Rathke in the latter animal.


2021 ◽  
Vol 9 (12) ◽  
pp. 516-524
Author(s):  
Nawaf M.O.S Ali ◽  
◽  
Mohammad H.AAA Alsaffar ◽  

Over the past two decades, the field of vascular surgery has experienced tremendous advances in technique and technology. After the description of vascular anastomosis by Dr. Alexis Carrel almost one hundred years ago, the subsequent years were slowly crowned by the differentiation of general and vascular surgeons (Friedman 2016). Notably, surgeons were able to distinguish themselves by developing and acquiring techniques that are specific to operations in vascular surgery. This type of surgery involves diseases associated with the vascular system, which specifically includes the arteries, veins, and the lymphatic structure. Thus, vascular surgery provides treatment for diseases associated with the veins and arteries and is used in cases where less invasive methods cannot be incorporated (Reis and Roever 2017). Importantly, vascular diseases arise from damaged vessels or the presence of inflammations or blood clots leading to the occurrence of illnesses like peripheral vascular disease or peripheral arterial disease (PAD) that is occlusive in nature. Notably, vascular occlusion can be defined as the blockage of blood vessels due to the growth of an abnormality or the accumulation of fat or calcium in the inner lining of the venous structure. Occlusion is a common characteristic of PAD where the diameter of the blood vessels narrows considerably (Rotzinger, Lu, Kawkabani, Marques-Vidal, Fetz and Qanadli 2020). Another good example of peripheral vascular occlusion arises from atherosclerosis. This is an occlusive disease where the arteries harden due to fat or calcium, leading to the narrowing of the internal diameter of the vessels (Rotzinger et al. 2020). Over time, this diameter narrows greatly resulting in the formation of clots that restrict the supply of blood to the entire area past the blockage, leading to the development of PVD/PAD.


2019 ◽  
Author(s):  
Mohammad Toliyat ◽  
Anish Patel ◽  
Ali Alian ◽  
Patrick Sutphin ◽  
Sanjeeva Kalva

2020 ◽  
Vol 5 (5) ◽  

Background and Objective: Rosai-Dorfman disease (RDD) are usually misdiagnosed because of rarity and nonspecific clinical and radiological features. The aim of our study is to explore the clinical and imaging characteristics of RDD to improve diagnostic accuracy. Methods: Clinical and imaging data in 10 patients with RDD were retrospectively analyzed. 7 patients were underwent CT scanning and 3 patients were underwent MR examination. Results: 8 (8/10) patients presented with painless enlarged lymph nodes (LNs) or mass. 3 cases were involved with LNs, 5 cases were involved with extra-nodal tissues, and the remaining 2 cases were involved with LNs and extra-nodal tissue simultaneously. In enhanced CT images, enlarged LNs displayed mild or moderate enhancement, and 2 cases showed heterogeneous ring-enhancement. MR features of 3 patients with extra-nodal RDD, 2 cases showed a mass located in the subcutaneous and anterior abdominal wall respectively, and 1 case showed an intracranial mass. Besides, all lesions showed high signal foci on DWI images, and were characterized by marked heterogeneous enhancement with blurred edge. The dural/fascia tail sign and dilated blood vessels could be seen around all the lesions on enhanced MRI. Radiological features of 2 cases with LN and extranodal tissue involved, one case presented with the swelling and thickening of pharyngeal lymphoid ring and nasopharynx, meanwhile with enlarged LNs in bilateral submandibular area, neck and abdominal cavity, and also companied with osteolytic lesion in right proximal humerus. All these LNs displayed mild and moderate enhancement on CT images. Another case showed enlarged LNs in bilateral neck accompanied with soft tissue mass in the sinuses. Conclusions: RDD occurred commonly in young and middle-aged men and presented with painless enlarged LNs or mass.RDD had a huge diversity of imaging findings, which varied with different location. The radiological features, such as small patches of high signal foci in the masses on DWI images, heterogeneous enhancement and blood vessels around the masses, are helpful in diagnosis of extranodal RDD.


2016 ◽  
Vol 11 (2) ◽  
pp. 210-217 ◽  
Author(s):  
A.T. Akhmetov ◽  
A.A. Valiev ◽  
A.A. Rakhimov ◽  
S.P. Sametov ◽  
R.R. Habibullina

It is mentioned in the paper that hydrodynamic conditions of a flow in blood vessels with the stenosis are abnormal in relation to the total hemodynamic conditions of blood flow in a vascular system of a human body. A microfluidic device developed with a stepped narrowing for studying of the blood flow at abnormal conditions allowed to reveal blood structure in microchannels simulating the stenosis. Microstructure change is observed during the flow of both native and diluted blood through the narrowing. The study of hemorheological properties allowed us to determine an increasing contribution of the hydraulic resistance of the healthy part of the vessel during the stenosis formation.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
G Rossitto ◽  
S Mary ◽  
C McAllister ◽  
K.B Neves ◽  
L Haddow ◽  
...  

Abstract Background Coronary and skeletal muscle microvascular dysfunction have been proposed as main factors in the pathogenesis of Heart Failure with Preserved Ejection Fraction (HFpEF). However, assessment of systemic arterial function has only been indirect thus far; most importantly, no direct link between systemic microvasculature and congestion, one of the core characteristics of the syndrome, has yet been investigated. Purpose To provide direct functional and anatomical characterisation of the systemic microvasculature and to explore in vivo parameters of capillary fluid extravasation and lymphatic clearance in HFpEF. Methods In 16 patients with HFpEF and 16 age- and sex-matched healthy controls (72±6 and 68±5 years, respectively) we determined peripheral microvascular filtration coefficient (proportional to vascular permeability and area) and isovolumetric pressure (above which lymphatic drainage cannot compensate for fluid extravasation) by venous occlusion plethysmography and collected a skin biopsy for vascular immunohistochemistry and gene expression analysis (TaqMan). Additionally, we measured brachial flow-mediated dilatation (FMD) and assessed by wire myography the vascular function of resistance arteries isolated from gluteal subcutaneous fat biopsies. Results Skin biopsies in patients with HFpEF showed rarefaction of small blood vessels (82±31 vs 112±21 vessels/mm2; p=0.003) and in ex-vivo analysis (n=6/group) we found defective relaxation of peripheral resistance arteries (p<0.001). Accordingly, post-ischaemic hyperaemic response (fold-change vs baseline, 4.6±1.6 vs 6.7±1.7; p=0.002) and FMD (3.9±2.1 vs 5.6±1.5%; p=0.014) were found to be reduced in patients with HFpEF compared to controls. In the skin of patients with HFpEF we also observed a reduced number (85±27 vs 130±60 vessels/mm2; p=0.012) but larger average diameter of lymphatic vessels (42±19 vs 26±9 μm2; p=0.007) compared to control subjects. These changes were paralleled by reduced expression of LYVE1 (p<0.05) and PROX1 (p<0.001), key determinants of lymphatic differentiation and function. Whilst patients with HFpEF had reduced peripheral capillary fluid extravasation compared to controls (microvascular filtration coefficient, leg 33.1±13.3 vs 48.4±15.2, p<0.01; trend for arm 49.9±20.5 vs 66.3±30.1, p=0.09), they had lower lymphatic clearance (isovolumetric pressure: leg 22±4 vs 16±4 mmHg, p<0.005; arm 25±5 vs 17±4 mmHg, p<0.001). Conclusions We provide direct evidence of systemic dysfunction and rarefaction of small blood vessels in patients with HFpEF. Despite a reduced microvascular filtration coefficient, which is in keeping with microvascular rarefaction, the clearance of extravasated fluid in HFpEF is limited by an anatomically and functionally defective lymphatic system. Funding Acknowledgement Type of funding source: Foundation. Main funding source(s): British Heart Foundation Centre of Research Excellence Award


Sensors ◽  
2021 ◽  
Vol 21 (3) ◽  
pp. 836
Author(s):  
Thi Thao Mai ◽  
Manh-Cuong Vo ◽  
Tan-Huy Chu ◽  
Jin Young Kim ◽  
Chulhong Kim ◽  
...  

Carfilzomib is mainly used to treat multiple myeloma. Several side effects have been reported in patients treated with carfilzomib, especially those associated with cardiovascular events, such as hypertension, congestive heart failure, and coronary artery disease. However, the side effects, especially the manifestation of cardiovascular events through capillaries, have not been fully investigated. Here, we performed a pilot experiment to monitor peripheral vascular dynamics in a mouse ear under the effects of carfilzomib using a quantitative photoacoustic vascular evaluation method. Before and after injecting the carfilzomib, bortezomib, and PBS solutions, we acquired high-resolution three-dimensional PAM data of the peripheral vasculature of the mouse ear during each experiment for 10 h. Then, the PAM maximum amplitude projection (MAP) images and five quantitative vascular parameters, i.e., photoacoustic (PA) signal, diameter, density, length fraction, and fractal dimension, were estimated. Quantitative results showed that carfilzomib induces a strong effect on the peripheral vascular system through a significant increase in all vascular parameters up to 50%, especially during the first 30 min after injection. Meanwhile, bortezomib and PBS do not have much impact on the peripheral vascular system. This pilot study verified PAM as a comprehensive method to investigate peripheral vasculature, along with the effects of carfilzomib. Therefore, we expect that PAM may be useful to predict cardiovascular events caused by carfilzomib.


Circulation ◽  
1984 ◽  
Vol 69 (5) ◽  
pp. 963-972 ◽  
Author(s):  
P B Kurnik ◽  
A J Tiefenbrunn ◽  
P A Ludbrook

1868 ◽  
Vol 16 ◽  
pp. 230-231

To explain the true nature of the phenomenon of drops of blood propelled in rapid succession, as if from the caudal heart, along the caudal vein,—to prove thereby that the caudal heart belongs, not to the blood-vascular system, but to the lymphatic system,—and to inquire into the influence which the force of the lymph-stream from the caudal heart exerts in accelerating and promoting the flow of blood in the caudal vein, constitute the object of this paper. The great caudal vein of the eel is formed by the junction of two trunks, a larger and a smaller. It is into the smaller trunk, near its junction with the larger, that the caudal heart opens. At the opening, there is a valve which prevents regurgitation of the lymph back from the vein into the heart.


Sign in / Sign up

Export Citation Format

Share Document