Cystic lymphatic malformations and their treatment]

Lymphangiomas are remarkably well amenable to treatment with direct aspiration then embolization with a sclerosing agent derived from maize: Ethibloc.     

Hemangiomas and superficial vascular malformations: classification]

Superficial vascular malformations of the face, trunk and limbs are better known today, and they can be divided up into simple and complex vascular malformations. Simple vascular malformations may form five major categories: immature hemangiomas of infants, port-wine stains, capillarovenous angiodysplasias, and arteriovenous fistulae and malformations. Complex angiodysplasias are systematized (Sturge-Weber and Bonnet-Dechaume-Blanc syndromes, Cobb's metameric angiomatosis, Klippel-Trenaunay and Parkes Weber's syndromes) or disseminated (Weber-Osler-Rendu disease and blue rubber-bleb nevus syndrome). Various modalities of treatment may be contemplated, according to the type of malformations, and an interdisciplinary consultation is essential to decide whether a watch-and-wait policy, a physical method (laser), embolization, fibrosing injections, vascular, maxillofacial or plastic surgery, or a successive combination of various techniques should be resorted to.     

Exploration strategy for superficial vascular malformations]

Transillumination of cystic lymphangiomas, MRI of venous vascular malformations, pulsed Doppler and arteriography of arteriovenous malformations, echo-Doppler and radiomeasurement of malformations in the lower limbs are key examinations in the exploration strategy for superficial vascular malformations.     

Congenital vascular malformations: general treatment principles

The era where surgical excision alone was the sole treatment of venous malformation (VM) is now over. A multidisciplinary approach that utilizes both traditional surgical therapy and endovascular therapy is now the standard of care. Endovascular therapy utilizing primarily both embolization therapy and sclerotherapy is the treatment of choice for surgically 'inaccessible' VM lesions. Surgical therapy of VM lesions has been shown to be more effective when combined with supplemental endovascular therapy.     

Cervico-cephalic hemangiomas and vascular malformations. Histopathological appearance and classification]

We present the histological and clinicopathological classification of hemangiomas and vascular malformations, such as was produced by the work of the study group on hemangiomas and vascular malformations of Lariboisière hospital. These lesions can first be classified according to the presence (phasic hemangioma of infants and pyogenic granuloma) or absence (malformations) of active cell proliferation. The malformations are classified according to their histological architecture and to the histological structure of the vessels they are composed of: capillary (hemangioma simplex, telangiectasias, etc.), capillary and/or venous (capillarovenous and cavernous malformations), arterial, venous and capillary (arteriovenous malformations). The classification of lymphatic malformations follows the same principles. The distinctive histological features of the various lesions are illustrated and briefly described.     

Late results in the treatment of vascular malformations

The data in literature for late results in treatment of vascular malformations are still rare and concern almost exclusively the peripheral angiodysplasias. In the present work, the late results in 700 of a total of 1036 patients are investigated, diagnosed, and treated over the last 33 years and followed for 10 years. The types, species, and morphological forms of vascular malformations, as well as the therapeutic tactics applied, were analyzed. The late results are separated into eight categories: excellent (115 cases), good (240 cases), improvement (294 cases), relapse (26 cases), no change (10 cases), worse (9 cases), amputation (5 cases), and exitus letalis (1 case). Analysis of the late results leads to the following conclusions: treatment must be performed in childhood, in many stages; must be active and causal; individual and unconventional; functionally radical, and frequently multidisciplinary, and combined. Experience shows that with good will, indispensable knowledge, and surgical creativity ars possibilis is probable.Presented at the 38th World Congress, International College of Angiology, Köln, Germany, June 1996.     

Venous capacity and venous drainage capacity of the legs in subjects with healthy vascular systems]

In altogether 102 probands (51 men and 51 woman) with an average of 35 and 27.5 years, respectively, on the two calves and the two ankles the venous capacity and the average inclincation of the tissue to swelling were determined in the deep positioning experiment. Apart from this, with the help of the venous drainage experiment we established the venous reflux speed in the region of the lower extremities. From the 2040 measured values received for each measuring place the mean values x and the standard deviation s were calculated. Besides, it was sought for statistically significant differences between the individual mesuring places as well as between men and women. With the help of these investigation methods we succeeded in establishing several parameters of the venous haemodynamics quantitatively and on an unbloody way and in making them usable for the recognition of disturbances of the venous circulation in the region of the legs.     

Vascular malformations of the limbs. Role of vascular and reconstructive surgery]

The progress made by noninvasive exploration for the assessment of vascular malformations now allows the therapeutic strategies to be better established. While observation and conservative methods are most often chosen, some venous or arteriovenous malformations lead to contemplating a palliative or curative treatment. Two therapeutic choices may thus be used: embolization through an arterial route or, most often, through direct puncture of the malformation, or exeresis surgery, sometimes associated to surgery for tissue reconstruction. These techniques may be used alone, but are most often associated within the scope of a multidisciplinary management of the condition.     

Angiogenesis and vascular malformations： Antiangiogenic drugs for treatment of gastrointestinal bleeding

Treatment of gastrointestinal bleeding in patients with angiodysplasias and Osler＇s disease （hereditary hemorrhagic teleangiectasia） is clinically challenging. Frequently, vascular malformations occur as multiple disseminated lesions, making local treatment an unfavorable choice or impossible. After local therapy, lesions often recur at other sites of the intestine. However, as there are few therapeutic alternatives, repeated endoscopic coagulations or surgical resections are still performed to prevent recurrent bleeding. Hormonal therapy has been employed for more than 50 years but has recently been shown to be ineffective. Therefore, new therapeutic strategies are required. Understanding of the pathophysiology of angiogenesis and vascular malformations has recently substantially increased. Currently, multiple inhibitors of angiogenesis are under development for treatment of malignant diseases. Experimental and clinical data suggest that antiangiogenic substances, which were originally developed for treatment of malignant diseases, may also represent long-awaited specific drugs for the treatment of vascular malformations. However, antiangiogenics display significantly different actions and side-effects. Although antiangiogenics like thalidomide seem to inhibit gastrointestinal bleeding, other substances like bevacizumab can cause mucosal bleeding. Therefore differential and cautious evaluation of this therapeutic strategy is necessary.     

Superficial and peripheral vascular malformations. Role of interventional radiology and of embolization]

Embolization plays a major role in the management of arteriovenous malformations and fistulae on one hand, and of venous malformations and cystic lymphangiomas on the other hand. The treatment of arteriovenous fistulae today resorts to a primarily endovascular technique including the insertion under controlled flow of a releasable balloon or of a metallic coil positioned in the area of the fistula. Of course, this is possible only if there is a gap between the arterial and venous pathways. When the vessels are in direct contact, surgery must be preferred. In cases of arteriovenous malformations, embolization currently plays a great role; either it is performed with particles in the immediate preoperative period, two or three days before surgery, or as a definitive curative treatment with a polymerizing substance applied in situ. The use of flexible microcatheters allows penetrating into most of these vascular malformations and scattering polymerizing material all over the shunting areas. This is possible for superficial malformations, as is now performed, for instance, for brain AVMs. This embolization obviously can be contemplated only after a decision to treat these malformations has been made, knowing that they may be silent or acquire an uncontrollable evolution potential. This therefore is a collegial decision. As far as venous hemangiomas and cystic lymphangiomas are concerned, the greatest basic therapeutic means today is direct puncture and the in situ injection of a fibrosing substance under angiographic monitoring: the use of Ethibloc or, failing this, of absolute alcohol, has dramatically transformed the prognosis of these malformations, for which the surgical difficulties are well known (easy rupture, blood that often fails to coagulate, life-long progressive evolution).(ABSTRACT TRUNCATED AT 250 WORDS)     

Febrile infections and their treatment]

Fever is a cardinal symptom of most infectious diseases; the febrile conditions not caused by infection are, however, not sufficiently taken into consideration in diagnosis and therapy. In 297 infections with a febrile course only in 26.26% of the cases a therapy with antibiotics was indicated. Antipyretic drugs were only exceptionally used under hospital conditions. Before hospitalisation, however, more than 75% of the 297 patients were treated with antibiotics and/or antipyretically. Virus infections stand in the first place of an unnecessary and, therefore, uncritical chemotherapy. With a casuistic report on the sequels of an uncritical chemotherapy is referred to the dangers for the patient and to the not insignificant economic burden for the society.     

Terminology and classification of congenital vascular malformations

Venous malformation (VM) is a congenital vascular malformation (CVM) that develops along the venous system through the various stages of embryogenesis. Older terminology and classification were often misleading and confusing. A newer classification system has emerged that is based on advances in the study of these conditions and is useful in contemporary management. The Hamburg classification was introduced after reappraisal of older terminology and has become the standard system for contemporary classification, which is based on anatomical, pathological and embryological criteria.     

The anatomic basis of vascular malformations

Basing themselves on anatomical and embryological considerations, the authors suggest a distinction between angiodysplasias and abnormalities. They define the characteristics proper to these varieties and establish the bases of therapeutic inferences. They briefly recall the lymphatic malformations. Practical and therapeutic inferences.     

Hemangioma and superficial arteriovenous malformations]

Haemangiomas are different from true superficial vascular malformations. The haemangiomas, mainly affecting the newborn and small babies, will, after a phase of progression, sometimes regress completely. Therapeutic abstention is the rule except in high risk angiomas when steroid therapy may be effective. Visceral involvement poses problems. Superficial vascular malformations, on the other hand, arise at all ages and may affect any blood vessel. Each type has a specific clinical presentation, complementary investigations and appropriate treatment. Some are slowly progressive, for example capillary, venous and lymphatic malformations. Others are haemodynamically active, such as the arteriovenous malformations. Capillary malformations are flat angiomas with aesthetic consequences, apart from the Sturge-Weber-Krabbe syndrome. Cold, blue venous malformations confirmed by ultrasonography and magnetic resonance imaging, when necessary, require treatment adapted to their site and size: compression, embolisation, surgery or abstention. Lymphatic malformations may be cystic or tissular: the cystic lymphangioma, a soft swelling of often healthy skin, with compartments separated by septa on ultrasound scan, is usually treated by ethibloc embolisation. Arteriovenous malformations, warm and pulsatile, demonstrated at arteriography, may progress rapidly and treatment by surgery or embolisation, when necessary, has to be complete. Finally, there are complex vascular malformations which pose very difficult problems of management.     

Arterial malformations and Takayashu disease]

The authors report a recent series of 5 cases with Takayashu's disease and discuss the common pathological presentation of malformations of the arterial trunk, caused by disorderd development of the arterial system or by later segmental arrested development of the large trunks. The disease, which presents in the young adult as a diffuse inflammatory process, is probably auto-immune, but has its foundations in embryonic and foetal development.