Biological identity of orbital cavernous venous malformations

ABSTRACT Vascular anomalies comprise a wide spectrum of clinical manifestations related to disturbances in the blood or lymph vessels. They correspond to mainly tumors (especially hemangiomas), characterized by high mitotic activity and proliferation of the vascular endothelium, and malformations, endowed with normal mitotic activity and no hypercellularity or changes in the rate of cell turnover. However, the classifications of these lesions go beyond this dichotomy and consist various systems adapted for and by different clinical subgroups. Thus, the classifications have not reached a consensus and have historically caused confusion regarding the nomenclatures and definitions. Cavernous venous malformations of the orbit, previously called cavernous hemangiomas, are the most common benign vascular orbital lesions in adults. Herein, we have compiled and discussed the various evidences, including clinical, radiological, morphological, and molecular evidence that indicate the non-neoplastic nature of these lesions.

Modified facelift in severe plexiform neurofibromatosis associated with venous vascular malformation: Case report.

Neurofibromatosis is an autosomal dominant disorder characterized by tumors of the nervous system and skin. Plexiform neurofibromas are common complications of neurofibromatosis type 1 and can cause large facial deformities. Vascular anomalies are in turn a rare manifestation of neurofibromatosis. We present the case of a 48-year-old female patient with right hemifacial neurofibromatosis associated with venous vascular malformation, previously treated surgically and then with sclerosing agents, determining severe residual facial deformity. Her surgical approach using a modified facelift technique associated with partial tumor debulking and lipofilling seems to be a valid technical alternative for these highly complex cases that require a customized approach after exhaustive preoperative evaluation.

Blue Rubber Bleb Nevus Syndrome With Multiple Cavernoma-Like Lesions on MRI: A Familial Case Report and Literature Review.

Blue rubber bleb nevus syndrome (BRBNS), also called Bean's syndrome, is a rare disease associated with multiple venous malformations in the skin and gastrointestinal (GI) tract. Dermatological lesions, which are the first clinically visible manifestations, appear as skin-colored compressible protuberances or as dark-blue venous nodules, rubbery in consistency. Central nervous system (CNS) manifestations are rare, variable, non-specific, and tend to occur late in the disease, mainly reported as seizures and focal neurological deficits secondary to compression. Most cases occur sporadically, however, an autosomal dominant inheritance pattern has been reported. A 74-year-old male with history of focal epilepsy secondary to possible neurocysticercosis presented at the emergency department due to sudden onset of aphasia, left central facial paralysis, and dysphagia secondary to catastrophic intracerebral hemorrhage. Cerebral MRI showed multiple cerebral cavernous malformations (CCM)-like lesions and, on the general exploration, multiple dark-blue nodules, rubbery in consistency. One week later he died due to complicated pneumonia; a brain autopsy was performed showing multiple vascular malformations. His son had a history of focal epilepsy presumed to be related to neurocysticercosis. He had the same skin lesions and brain MRI pattern. Histological analysis of the skin lesions of the two cases showed venous vascular malformations. A non-systematic review was carried out, in which all case reports of blue nevus syndrome with neurological manifestations in adults were included.

Telangiectasia hemorrágica hereditaria Síndrome de Osler Weber Rendú y manejo con bevacizumab / Hereditary hemorrhagic telangiectasia Osler Weber Rendu syndrome and management with bevacizumab

Presentamos un caso clínico de una paciente con diagnóstico de telangiectasia hemorrágica hereditaria con múltiples manifestaciones sistémicas debidas a sangrados profusos, anemia severa y malformaciones arteriovenosas pulmonares, hepáticas y falla cardiaca de alto débito, con adecuada respuesta al uso de bevacizumab. (Acta Med Colomb 2015; 40: 66-68).

The case of a patient diagnosed with hereditary hemorrhagic telangiectasia with multiple systemic manifestations due to profuse bleeding, severe anemia and pulmonary arteriovenous malformations, liver and heart failure high debit adequate response to the use of bevacizumab, is presented. (Acta Med Colomb 2015; 40: 66-68).

Ethanol sclerotherapy of head and neck venous malformations / Escleroterapia com etanol em malformações venosas da cabeça e pescoço

Objective This retrospective study evaluated the results of sclerotherapy with low doses of ethanol for treatment of head and neck venous malformations. Methods We treated 51 patients, 37 females. Median age was 23 years. Patients were treated with percutaneous intralesional injection of alcohol every two weeks and followed up prospectively for a median period of 18 months. Most lesions affected the face and cosmetic disfigurement was the most frequent complaint. Results We performed a median of 7 sessions of sclerotherapy. Complete resolution or improvement was observed in 48 patients presented. Five cases of small skin ulceration, two cases of hyperpigmentation and two of paresthesia were documented; all of them were treated conservatively. Conclusion Percutaneous sclerotherapy with low doses of ethanol is a safe and effective treatment modality for venous malformations affecting the head and neck. .

Objetivo Estudo retrospectivo que analisou os resultados da escleroterapia, com doses baixas de etanol, realizada sob anestesia local para tratamento de malformações venosas na cabeça e pescoço. Métodos Foram tratados 51 pacientes, sendo 37 do gênero feminino. A mediana de idade foi de 23 anos. Os pacientes foram tratados com injeções alcoólicas nas lesões, por via percutânea, em sessões quinzenais, e acompanhados prospectivamente por um período mediano de 18 meses. A maioria das lesões acometia a face e a queixa principal mais frequente foi deformidade estética. Resultados Foram realizadas, em média, sete sessões de escleroterapia. Resolução completa ou melhora foi observada em 48 pacientes. Cinco pacientes apresentaram pequena úlcera cutânea, dois hiperpigmentação e dois parestesia, sendo todos tratados conservadoramente. Conclusão A escleroterapia percutânea com doses baixas de etanol é um método seguro e eficaz para tratar malformações venosas na cabeça e pescoço. .

Malformaciones arteriovenosas. Revisión y análisis estadístico de 52 MAVs tratadas durante el período de 2000-2010. Tercera parte / Arteriovenous malformations. Review and statistical analysis of 52 MAVs treated during the period 2000-2010. Third part

INTRODUCCIÓN: describir nuestra experiencia en el tratamiento de las MAVs analizando: procedimientos endovasculares utilizados, resultados postoperatorios y complicaciones asociadas, estadificación según escalas de Barthel y Rankin modificadas. MATERIAL Y MÉTODOS: la población de estudio se constituyó por 52 pacientes con MAVs la cual fue analizada y estudiada mediante examen neurológico pre y postoperatorio, TC cerebral, RM cerebral y arteriografía de 4 vasos de cuello, la totalidad fue tratada mediante cirugía convencional en nuestro Servicio durante el período comprendido entre los años 2000 a 2010. RESULTADOS: recibieron tratamiento endovascular previo a la cirugía 16 MAVs (30,76 %). Todas fueron operadas. Doce pacientes mejoraron en el postoperatorio (23,07 %), 30 pacientes (57,69%) no sufrieron modificaciones y 10 de ellos (19,23%) empeoraron durante el postoperatorio. La mortalidad fue de 7 casos (13,46 %). CONCLUSIÓN: consideramos a los procedimientos endovasculares y la radiocirugía una herramienta de indudable valor terapéutico. Creemos que el subgrupo de MAVs grados III, IV y V representa una entidad singular que las distingue del resto, como una subtipo que requiere más aun de una compleja toma de decisiones. Tuvimos las mayores complicaciones postoperatorias en MAVs grados III y IV. Nuestra mortalidad postoperatoria coincide con la bibliografía consultada

INTRODUCTION: to describe our experience in treating AVMs based on the endovascular procedures used, postoperative results and associated complications, staging according to Barthel Index and modified Rankin Scale. PATIENTS AND METHOD: we present 52 patients with AVMs which were analysed and studied by Pre and Post-Surgery Neurological exam, brain CT, Brain IRM and four Neck vessels arteriography. All the patients were treated by conventional Surgery at our Department of Neurosurgery for the 2000­2010 period. RESULTS: Sixteen patients with AVMs (30.76%) underwent endovascular treatment prior to surgery. 100% were operated. 12 patients (23.07%) improved their clinical condition in the postoperative period, 30 patients (57.69%) showed no changes, and 10 of them (19.23%) experienced deterioration during the postoperative period. There were 7 mortal cases (13.46%). CONCLUSION: we believe that endovascular procedures and radiosurgery are tools of immense therapeutic value. We also consider that the subgroup of AVMs grade III, IV and V have unique features that distinguish them among the rest as a subtype, thus requiring extreme care when making decisions. Most postoperative complications occurred with AVMs grade III and IV. The mortal cases in the postoperative period coincided with those mentioned in the bibliography consulted

Outpatient percutaneous treatment of deep venous malformations using pure ethanol at low doses under local anesthesia

INTRODUCTION: Venous malformations are the most frequent vascular malformation. Deep venous malformations are located in subcutaneous tissue or in the muscles. Percutaneous sclerotherapy is the treatment of choice, and the use of ethanol at low doses has not yet been described. OBJECTIVE: To analyze the results of treating Deep venous malformations patients with low doses of ethanol. METHODS: Thirty-nine patients treated between July 1995 and June 2007 were followed up prospectively over a median period of 18 months. Twenty-nine were female (74.4 percent) and 10 were male (25.6 percent), with ages ranging from 11 to 59 years (median of 24 years). All of the lesions affected limbs, and the main symptom reported was pain (97.4 percent). Each patient underwent fortnightly alcohol application sessions under local anesthesia on an outpatient basis. The lesions were classified into three groups according to size using nuclear magnetic resonance imaging: small, up to 3 cm (4 patients); medium, between 3 and 15 cm (27 patients); and large, greater than 15 cm (8 patients). RESULTS: The symptoms completely disappeared in 14 patients (35.9 percent) and improved in 24 (61.5 percent). The lesion size reduced to zero in 6 patients (15.4 percent) and decreased in 32 (82 percent). The median number of sessions was 7. There were no complications in 32 patients (82 percent), while 3 presented local paresthesia (7.7 percent), 2 superficial trombophlebites (5.1 percent), 1 skin ulcer (2.6 percent), and 1 case of hyperpigmentation (2.6 percent). CONCLUSION: Outpatient treatment for Deep venous malformations patients using ethanol at low doses was effective, with a low complication rate.