Biomedical subjects
Publications and source records attributed to O Enjolras.
Diagnosis relies first on clinical evaluation of the patients. Subsequently, modern diagnostic imaging techniques, such as CT scan, MRI and Echo-Doppler, confirm the clinical approach and define the type of vascular anomaly. Angiographic analysis is not performed for all cases: indications are carefully thought out.
Immature haemangiomas affect only infants and children and follow a peculiar course: first an expansion phase, then an involution phase which ends in complete cure in the majority of cases. Because of this favorable outcome, all inopportune treatments must be avoided, and abstention is the best attitude. However, early corticosteroid therapy must be prescribed when the lesion is located in a dangerous area, such as the eyelids or the lips. Specific treatments must be instituted only in very special cases, such as visceral or giant cutaneous haemangiomas or Kasabach-Merritt syndrome.
Mature vascular malformations of the venous compartment are haemodynamically inactive and persist throughout the patient's life. They have no systemic repercussions, except in patients with disorders of coagulation (e.g. disseminated intravascular coagulation), but they have local, cosmetic and functional repercussions. Percutaneous embolization, notably with Ethibloc, has modified their prognosis favourably. This simple and effective procedure is mainly used for superficial venous malformations of the face.
Arteriovenous malformations (AVM) are the most dangerous of vascular malformations, being haemodynamically active. These malformations proceed in two phases: a phase of quiescence which may last throughout life, and an unpredictable phase of activity sometimes triggered by a trauma or by hormonal variations (puberty, pregnancy, oral contraceptives). Treatment must be careful usually limited, and considered in the phase of activity. A multidisciplinary consensus is necessary.
Diffuse complex vascular malformations are sporadic or hereditary. They usually concern one type of vessel: capillary, venous or arterial (with AV fistula). They involve skin, mucosal membranes and internal organs. They lead to a rich and heterogeneous symptomatology, depending on the lesional localizations. Five out of them are described: cutis marmorata, Rendu Osler disease, Bean syndrome, Maffucci syndrome, and Proteus syndrome.
Various types of systematized vascular syndromes are described, either in the head or in the limbs. The Sturge-Weber syndrome includes a facial portwine stain, ocular anomalies, and a pial vascular malformation. Complex combined vascular malformations with limb overgrowth need careful investigations to clearly define the vascular component and the management.
Cystic lymphatic malformations are hemodynamically inactive vascular malformations of the lymphatic compartment. Over a 9 years period, 52 patients with superficial cystic lymphatic malformations of the head and neck underwent embolization by direct injections of Ethibloc (Ethicon, Ethnon Laboratories, Neuilly/France) under fluoroscopic control. Results were graded as excellent or good in 57% of patients after embolization alone. Treatment was completed by surgery in 19% of patients. Embolization was considered a failure in 24% (12 patients), although 10 of these patients showed regression of the cystic component after embolization. This safe, efficacious percutaneous technique has emerged as the first treatment to be considered for cystic lymphatic malformations.
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.
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.
The use of Ethibloc must be contemplated as a first-intention treatment for venous malformations of the face. This sclerosing agent has transformed the functional, esthetic and psychological prognosis for these vascular malformations.
Lymphangiomas are remarkably well amenable to treatment with direct aspiration then embolization with a sclerosing agent derived from maize: Ethibloc.
Seven cases of congenital Langerhans' cell histiocytosis (LH) are reported, with emphasis on clinical and immunohistochemical features. This is a polymorphic disease at birth. In 4/7 cases, the diffuse, generalized rash could be classified as cutaneous Letterer-Siwe disease (LSD); 3/4 remained purely cutaneous and healed in less than 3 months; whereas the fourth-one persisted, pulmonary lesions appeared, and the infant died on his 40th day. In 3/7 cases, the clinical diagnosis at birth was either a Blueberry Muffin Baby (BMB) or Hashimoto-Pritzker type LH (HPLH); the lesions healed rapidly, although one cas was contradictory: typical BMB at birth, histology mimicking a monoblastic cutaneous leukemia, no T.O.R.C.H. syndrome, normal bone marrow, immunophenotyping of LH, auto-involution; 2/3 were MZ twins, both with few lesions. We would like to stress the fact that the clinical spectrum of LH should include BMB, which, however, in most cases must be considered a differential diagnosis. Regarding cutaneous congenital LH, an eponymic classification (LSD, HPLH) is difficult to follow strictly, because overlapping pictures are observed. There is a wide spectrum of cutaneous congenital LH. The main problem at birth is the lack of prognostic criteria. Neither the presence of the rash at birth, nor its type and extension, is necessarily evidence of risk of systemic disease. Cases of HPLH involute, as also do cases of cutaneous LSD, and the "Blueberry Muffin" type of LH; overlapping clinical aspects exist. Histopathological data, electron microscopy or immunohistochemistry, define LH, but they do not enable the outcome to be predicted.(ABSTRACT TRUNCATED AT 250 WORDS)
Most of psoriatic patients require topical therapy. Dermal application of drugs may be the unique treatment; but it can also combined with oral drugs, or phototherapy. Patients are treated at home or in day-care centers; some require hospitalization. Topical corticosteroids are widely used: abuses are frequently observed, therefore skin and systemic side-effects may occur to varying degrees. Tars are still useful. Short-contact anthralin is active and well tolerated, and compliance is better than with conventional tar therapy, especially in children. Topical mechlorethamine clears the plaques but contact dermatitis may occur. New therapeutic approaches include Vitamin D3 analogues.
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A simple and precise classification has been established during a ten-year international cooperation. There are two major groups: hemangiomas, always regressive; and vascular malformations, which never regress; they grow, throughout life, to varying degrees. Management of vascular malformations has been clearly defined. Capillary malformations do not require examination, unless they are associated with other anomalies, such as hypertrophic underlying bone, or the leptomeningeal vascular anomaly of the Sturge-Weber syndrome. Venous type malformations are diffuse, and consist of entirely anomalous channels, with low flow. CT scan and MRI clearly demonstrate the extent of tissue involvement in venous malformations; for these slow-flow malformations the evaluation is performed without resorting to invasive diagnostic techniques: phlebography is rarely performed, and arteriography is unnecessary. Arteriovenous malformations are dangerous high-flow vascular malformations, leading to skin ischemic necrosis, and congestive cardiac failure. Arteriography shows enlarged tortuous arteries, with arteriovenous shunting, and early venous drainage. CT scan and MRI show the deep components of the lesions. Doppler ultrasound evaluation is used to follow the course of the disease.
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During the past 10 years, 25 infants with alarming hemangiomas--lesions that impaired important functions and were life threatening, especially when there was visceral involvement--have been treated. A vascular mark was present at birth in 68% of these infants. Visceral hemangiomas were associated with bulky cervicocephalic hemangiomas or with small hemangiomas scattered over the body. Among the 25 infants, 12 had laryngeal hemangiomas, 3 had hepatic hemangiomas, and 1 had gastrointestinal hemangiomatosis. Ocular sequelae, malocclusion, and cutaneous distortion were the most important functional problems. Corticosteroid treatment was used for 23 of 25 infants with alarming hemangiomas. There was a varied treatment response: total failure (30% of the patients); excellent, dramatic, rapid improvement (30% of the patients); and moderate, doubtful response, with the natural course of the disease remaining unaltered (40% of the infants). Arterial embolization, used in 6 infants, gave inconstant results. Cardiac failure, frequently associated with large cutaneous hemangiomas and always seen with hepatic multinodular hemangiomas, required digitalization. In some cases arterial embolization reduced the increased cardiac output. Liver hemangiomas had a high mortality; all 3 infants with hepatic involvement died.