Vascular malformations of the spinal cord: intrathecal perimedullary arteriovenous fistulas fed by medullary arteries.

We studied eleven patients with intrathecal extramedullary arteriovenous fistulas identified by selective angiography and myelography. The fistulas were located on the surface of the cord, fed by medullary arteries. Angiography distinguished three separate types that gave identical clinical disorders. Therapeutic results of surgery and embolization allowed specific indications to be defined for treatment of each type of fistula.

Facial port-wine stains and Sturge-Weber syndrome.

A retrospective study was made of 106 cases of facial port-wine stains. It was concluded that only patients with lesions located in the ophthalmic (or V1 trigeminal) cutaneous area are at risk for associated neuro-ocular symptoms. It is proposed that Sturge-Weber syndrome results from a dysmorphogenesis of cephalic neuroectoderm.

Management of alarming hemangiomas in infancy: a review of 25 cases.

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.

Embolization of spinal cord vascular malformations via the anterior spinal artery.

During a 3 year period, 21 patients with spinal cord vascular malformations involving the anterior spinal artery were treated by embolization through this artery. Of four cases of extramedullary arteriovenous fistula, two patients embolized at an early stage showed excellent results, whereas results were mediocre in two patients who were paraplegic at embolization. Of 17 cases of intramedullary arteriovenous malformation (AVM), nine patients showed clinical improvement either with or without radiologic evidence of residual malformation; three patients had complications that led to clinical worsening; in the other five patients, results were mixed or inconclusive. Angiomyelotomography is recommended to diagnose extramedullary arteriovenous fistulae involving the anterior spinal artery. Pretherapeutic evaluation of intramedullary AVMs must consider the spinal cord level at which the malformation occurs. Angiotomography and temporary occlusion tests permit identification of collateral anastomoses before embolization. Respective criteria for surgery and embolization are defined. In cases where embolization is indicated, short-term results can generally be predicted.

Giant extramedullary arteriovenous fistula supplied by the anterior spinal artery: treatment by detachable balloons.

A case of a large extramedullary arteriovenous fistula in a young man, which was deemed inoperable, is described. The fistula was mainly supplied by the anterior spinal artery and was responsible for a progressive myeloradicular syndrome. Successful treatment with detachable balloons was carried out. Along with this case report, a review is made of the different types of extramedullary arteriovenous fistulas and their treatment based upon 11 cases seen at Lariboisière Hospital: type 1 is a plain small fistula where embolization is contraindicated; type 2 shows an enlarged anterior spinal artery with dysplastic vein and may be embolized with solid particles; type 3 is a very large multipediculated fistula where the best treatment seems to be detachable balloons. The indications for using detachable balloons for temporary occlusion and for definitive treatment are reviewed. Special emphasis is placed on the possibility of detaching balloons without the need for a coaxial catheter.

MR imaging of spinal cord arteriovenous malformations at 0.5 T: study of 34 cases.

Thirty-four patients with angiographically proved arteriovenous malformations of the spinal cord were studied between May 1986 and July 1987. Examinations were performed on a CGR 5000 Magniscan 0.5-T scanner with a surface coil in all cases, and multislices in both T1- and T2-weighted sequences were obtained in sagittal and axial planes. The results showed that MR can accurately diagnose all cases of intramedullary arteriovenous malformations, since transverse images were able to precisely locate the nidus within the spinal cord. Other types of spinal cord arteriovenous malformations were also studied (perimedullary fistulae and dural arteriovenous fistula), and the findings confirmed the value of MR in the characterization of these lesions as well.

Place of embolization in the treatment of severe epistaxis.

On the basis of long-term study on embolization for severe epistaxis, the authors show the different indications and results of this relatively new method; and 54 cases are presented including Rendu-Osler diseases, primary and traumatic epistaxis, or those due to vascular malformation and benign or malignant tumors. Embolization can prove a very effective method in most cases.

Vascular malformations of the mandible (intraosseous haemangiomas). The importance of preoperative embolization. A study of 9 cases.

Haemangiomas of the mandible are relatively rare arteriovenous malformations: 85 cases have been described since 1849. The severe risk of bleeding during tooth extraction or biopsy led us to describe the radiological and angiographic features of this condition. The afferent vessels are the inferior dental, the lingual, and the facial arteries. Preoperative embolization reduces the risk of bleeding at operation. Surgical treatment must assure the complete removal of the lesion. Radiotherapy and vascular ligatures are useless and dangerous.

The treatment of capillary-venous malformations using a new fibrosing agent.

We present our early experience in the treatment of cutaneous capillary-venous malformations using a new fibrosing agent called Ethibloc. Superselective angiography was used to delineate the vascular anomaly and effect temporary occlusion. Whenever possible, compression of venous outflow also was used, while the sclerosing agent was injected directly into the malformation by percutaneous puncture. Sixteen patients with capillary-venous malformations have been treated with this agent since 1979. A mild local inflammatory reaction was noted in some cases; there were no instances of skin necrosis. In 10 patients, the fibrosed vascular malformation was excised, and in 6 patients, the area of vascular anomaly decompressed to such a degree that resection was unnecessary.

Arteriovenous fistulae of the internal iliac vessels.

Seven patients with arteriovenous fistulae of the internal iliac artery are reported. Ages varied from 6 to 50 years (mean 32 years). Cutaneous angiomata , leg oedema and pain was present in 4 cases each, bleeding in 2 cases whilst only one was asymptomatic. Only 1 patient had cardiac insufficiency. In six patients the lesion was a congenital malformation but in one it was posttraumatic. Arteriography both global and selective, intravenous pyelography and cardiac output are routine. Recently, pelvic computerised tomography has been most helpful. Embolization is recommended in all cases with surgery within 24 hours unless the lesion is very extensive and considered to be inoperable. Repeat embolization is used for recurrence or very extensive lesions. Results were good in five patients; in two patients the results were clinically good but control angiograms showed a recurrence.

Percutaneous embolization with Ethibloc of lymphatic cystic malformations with a review of the experience in 70 patients.

Cystic lymphatic malformations (CLM) are superficial vascular hemodynamically inactive malformations of the lymphatic compartment. We propose a new approach which uses a sclerosing agent as an alternative to surgical resection. In the past nine years we treated 70 patients with CLM. Fifty-five percent were younger than five years of age with a male preponderance and most (80%) of the CLM were located in the maxillofacial region. They usually presented with functional impairment from the mass effect; others had infections, bleeding, or inflammation. The CLM were injected under fluoroscopic control with a sclerosing agent, Ethibloc, which dries up the pockets and reduces the mass. On follow-up the results were good in 62%, unchanged in 5%, and continued progression in 20%. Fifteen percent underwent surgery failures (24%) occurred in mixed forms of cystic and cellular lymphangiomas. Complications were minors. Percutaneous embolization is useful for CLM, with minimal risk, absence of scar, and it avoids surgery. It should be the first line of treatment for these lesions.

Classification of spinal cord vascular malformations.

Through the aid of developments in the neuroradiological sciences, great attention has been given to the diagnosis and treatment of vascular malformations of the spinal cord. Some of these are congenital, such as intramedullary vascular malformations and arteriovenous fistulae supplied by the spinal cord. Others are rather frequent and appear in old age. Due to their poor prognosis when not treated, precise determinations and sufficient knowledge are of the utmost importance.

The use of detachable balloons in neuroradiology.

The development of the detachable balloon allows treatment of many neurological lesions, both congenital and acquired. We describe here a device that may be used easily. Complications and main indications are discussed on the basis of our experience with 114 cases.

[Pseudo-Kaposi syndromes of vascular origin]. / Les pseudo-syndromes de Kaposi "d'origine vasculaire".

Pseudo-Kaposi's Sarcoma with vascular disease concern mainly acroangiodermatitis described by Mali--(with chronic venous insufficiency)--, arteriovenous malformations with angiodermatitis described by Stewart and Bluefarb, and pseudo-Kaposi's Sarcoma occurring after placement of arteriovenous shunt for hemodialysis. Search for relation ships between classical Kaposi's Sarcoma and new AIDS Kaposi's Sarcoma explains new interest devoted to Pseudo-Kaposi's Sarcoma.

[Disabling calf pain in the context of venous angiodysplasia: Contribution of dynamic exploration]. / Douleur du mollet invalidante dans le cadre d'une angiodysplasie veineuse: apport des explorations dynamiques.

We report the case of a 25-year old woman presenting with disabling pain of the left calf, associated with lower left limb angiodysplasia. Dynamic testing (duplex scan and phlebography) showed a popliteal vein ectasia, with reflux into an incompetent lesser saphenous vein: the venous drainage of this ectasia was an embryonic femoropopliteal vein going to the profunda femoris vein. As long as patency and competence of deep veins and of the superficial femoral vein were confirmed by dynamic testing with compressions, curative surgery was considered: en-bloc resection of lesser saphenous vein and of embryonic femoropopliteal vein combined with lateral suture of the popliteal vein. One-year follow-up confirms the absence of recurrent symptoms. Dynamic testing allows safe surgical therapy of persisting embryonic abnormalities associated with incompetent veins, in cases where such testing clearly demonstrates that a sufficient venous drainage of the affected limb will be provided after surgery.

[Embolization of arteriovenous malformations using compression. Apropos of 11 cases]. / L'embolisation des malformations artério-veineuses sous compression. A propos de 11 cas.

External compression of both arterial feeder and venous return of an MAV allows embolization material to be stabilized into the area of the shunts. 11 patients were treated according to this method, either by an external compression method (4 cases) or by tourniquets (4 cases) or by a balloon compressing the vena cava (3 cases). The material of embolization was in all cases Isobutyl-2-Cyano-Acrylate. 5 patients were successfully operated after the procedure and 7 patients had considerable improvement after embolization, 2 of them being considered as cured.

[Treatment of diabetic arteriopathy. Importance of transluminal angioplasty]. / Le traitement des artériopathies diabétiques. Intérêt de l'angioplastie transluminale.

Results are reported of a retrospective analysis of transluminal angioplasty (TLA) interventions in 20 diabetic patients, 16 men and 4 women, mean age 56 years (range 32 to 82 years), with 24 dilated lesions, 16 patients having insulin-dependent diabetes. In 12 cases the lesions were at the intermittent claudication stage, trophic lesions being present in 8 cases. Stenotic lesions were iliac (12 cases), superficial femoral (2 cases), popliteal (6 cases) and tibial (4 cases). One patient developed an acute occlusion following popliteal-anterior tibial recanalization, the only direct complication of the angioplasty. Angiography showed immediate satisfactory results in 22 of the 24 dilated lesions. Functional and hemodynamic improvement was a constant finding in patients with intermittent claudication, trophic lesions being healed in 4 cases (50%) the other patient showing either no change or requiring an unavoidable amputation (2 cases). These overall findings suggest that at the intermittent claudication stage no differences exist in the results of TLA when compared with a non diabetic population; inversely, in the presence of trophic disorders, the local conditions (distal bed, infection, gangrene) interfere considerably in the course of the dilatation. Transluminal angioplasty should therefore be carried out as early as possible in diabetics; arteriography should be performed as soon as even minimal claudication appears and, a fortiori, even at the onset of a trophic lesion.

[Hemangiomas and superficial vascular malformations: classification]. / Hémangiomes et malformations vasculaires superficielles: 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]. / Stratégie d'exploration des malformations vasculaires superficielles.

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.

[Venous vascular malformations and their treatment with Ethibloc]. / Les malformations vasculaires veineuses et leur traitement par Ethibloc.

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.