Shunt portosistémico, una causa poco frecuente de hepatitis colestásica en neonatología. A propósito de un caso / Portosystemic shunt, a rare cause of neonatal cholestatic hepatitis. A case report

El shunt portosistémico congénito es una anomalía vascular venosa que comunica circulación portal y sistémica, por la que se deriva el flujo sanguíneo, salteando el paso hepático. Es una entidad poco frecuente, cuya incidencia varía entre 1/30 000 y 1/50 000 recién nacidos. Puede cursar de forma asintomática o presentarse con complicaciones en la edad pediátrica o, menos frecuente, en la edad neonatal. Ante el diagnóstico, se deberá definir la necesidad de intervención quirúrgica o intravascular para el cierre. Esta decisión depende de las características anatómicas de la malformación, de las manifestaciones clínicas y complicaciones presentes. Se presenta el caso de un paciente de un mes de vida derivado a nuestro centro para estudio de hepatitis colestásica neonatal, con diagnóstico de shunt portosistémico extrahepático. Se realizó cierre intravascular de la lesión con mejoría significativa posterior.

Congenital portosystemic shunt is a venous vascular abnormality that connects portal and systemic circulation, resulting in diversion of the blood flow, bypassing the hepatic passage. It is a rare malformation; its incidence varies from 1:30 000 to 1:50 000 newborns. It may be asymptomatic or present with complications in the pediatric age or, less frequently, in the neonatal age. Upon diagnosis, the need for a surgical or an intravascular intervention for closure should be defined. This decision depends on the malformation anatomical characteristics, clinical manifestations, and complications. We present the case of a 1-month-old patient referred to our center for the study of neonatal cholestatic hepatitis, with a diagnosis of extrahepatic portosystemic shunt. Intravascular closure of the defect was performed with significant subsequent improvement.

Asociación del complejo ring-sling con pig bronchus: a propósito de un caso pediátrico / Ring-sling complex association with pig bronchus: a pediatric case report

El complejo ring-sling es una asociación entre el sling de la arteria pulmonar y la estenosis traqueal congénita por anillos traqueales completos. El sling de la arteria pulmonar es una forma rara de anillo vascular dentro de las cardiopatías congénitas. Se presenta el caso clínico de un niño con estridor laríngeo asociado a dificultad respiratoria evaluado en otro centro, donde se realizó endoscopia respiratoria y se observó compresión traqueal extrínseca. Ante la sospecha clínica de anillo vascular, se solicitó angiotomografía computada (angioTC) y se confirmó diagnóstico de sling de arteria pulmonar. Fue derivado a nuestro hospital y durante la intervención quirúrgica se realizó nueva endoscopia respiratoria y traqueobroncografía. Se llegó al diagnóstico de estenosis traqueal congénita con bronquio derecho accesorio (pig bronchus) y anillos vasculares completos, ambos reparados en el mismo acto quirúrgico.

The ring-sling complex is an association between pulmonary artery sling and congenital tracheal stenosis. Pulmonary artery sling is a rare form of vascular ring in congenital heart disease. The clinical case of a child with inspiratory laryngeal stridor associated with respiratory distress is presented, evaluated in another center where respiratory endoscopy was performed, observing extrinsic tracheal compression. Given the clinical suspicion of a vascular ring, CT angiography was requested, confirming the diagnosis of pulmonary artery sling. He was referred to our hospital and during the surgical intervention a new respiratory endoscopy and tracheobronchography were performed, reaching the diagnosis of congenital tracheal stenosis, right accessory bronchus (pig bronchus) and complete vascular rings, both repaired in the same surgical act.

Agenesia de segmento infra-hepático de veia cava inferior associada a trombose venosa profunda de repetição: relato de caso / Agenesis of the infra-hepatic segment of the inferior vena cava associated with recurrent deep venous thrombosis: case report

Resumo A agenesia de veia cava inferior é descrita em menos de 1% da população, de ocorrência rara e devido a alterações embrionárias. Sua correlação com a trombose venosa profunda certamente é subestimada, visto que tal alteração é de difícil detecção pela ultrassonografia. O objetivo deste artigo foi relatar o caso de uma paciente de 41 anos com dor e edema até a raiz de coxa direita após cirurgia plástica. Foi realizado dúplex venoso de membros inferiores e evidenciada trombose venosa profunda ilíaco-femoro-poplíteo e distal bilateralmente. Solicitado angiotomografia venosa devido a não visualização de veia cava inferior no ultrassom, evidenciando trombose de plexo lombar direito e segmento ilíaco-femoral bilateral, além de agenesia de segmento infra-hepático de veia cava inferior, com ectasia e tortuosidade compensatória de veias paravertebrais, sistema ázigos e hemiázigos, com varizes pélvicas bilateralmente. Foi realizada anticoagulação sistêmica e oral, com boa evolução clínica.

Abstract Agenesis of the inferior vena cava (IVC) has been described in less than 1% of the population; a rare occurrence caused by embryonic abnormalities. Its correlation with deep vein thrombosis (DVT) is certainly underestimated, since this change is hard to detect using ultrasound. The aim of the article is to report the case of a 41-year-old female patient with pain and edema up to the top of the right thigh after plastic surgery. Bilateral venous duplex ultrasound revealed bilateral DVT involving iliac-femoral-popliteal and distal segments. Venous angiotomography was requested because the IVC was not visible on ultrasound, revealing thrombosis of the right lumbar plexus and iliofemoral segment bilaterally and agenesis of the infrahepatic segment of the inferior vena cava, with ectasia and compensatory tortuosity of paravertebral veins and the azygos-hemiazygos system, and bilateral pelvic varices. Systemic and oral anticoagulation were administered, with a satisfactory clinical response.

Endovascular Treatment of Ruptured Vertebral Artery Dissecting Aneurysm in Fibromuscular Dysplasia

Background Fibromuscular dysplasia (FMD) affects predominantly the cervical and renal arteries and may cause the classical angiographic pattern of string-of-beads. The diagnosis is increasing with the advances of imaging techniques. Case Report A 37-year-old man presenting with subarachnoid hemorrhage due to a dissecting aneurysm of the vertebral artery was treated by angioplasty with stent, with good outcome. All of the cervical and renal arteries were diseased and showed dysplasia and/or ectasias. Conclusions There are no guidelines or protocols to treat patients with FMD.

Malformación de Abernethy: causa inusual de cianosis central en pediatría / Abernethy malformation: unusual cause of central cyanosis in pediatrics

El shunt porto sistémico congénito es una causa rara de hipoxemia y una patología muy poco frecuente con complicaciones severas si no es tratada. Fue descrito por primera vez por John Abernethy en 1793. Existen dos tipos: tipo I (shunt termino lateral) en el que hay ausencia total del flujo portal intrahepático y tipo II (shunt latero lateral) con flujo portal parcialmente conservado. Se presenta el caso de una niña de 6 años de edad con antecedente de hipoxemia crónica desde los 4 años y medio de vida, acompañado de disnea progresiva, quien fue referida a la unidad de neumología pediátrica con diagnóstico de cianosis central. Entre los estudios diagnósticos considerados se realizó ecografía doppler del sistema venoso portal, evidenciándose ausencia de vena porta principal; además se realizó angiotomografía del sistema arterio-venoso portal y mesentérico, confirmándose la agenesia de vena porta. Se completó el estudio con una porto-esplenografía que confirmó el diagnóstico de malformación de Abernethy tipo I b. La malformación de Abernethy tipo I es más frecuente en el sexo femenino, tiene varias formas de presentación y el tratamiento es el trasplante hepático. En la malformación de Abernethy tipo II la circulación portal es variable y tiene mejor pronóstico que la de tipo I. La disnea al ejercicio y la cianosis central es una forma de presentación que debemos tener en cuenta en el diagnóstico diferencial de la patología cardiorrespiratoria en la edad pediátrica.

The congenital portosystemic shunt is an uncommon disease with severe complications if not treated. This rare cause of hypoxemia was first described by John Abernethy in 1973. There are two types: type I (termino-lateral shunt), in which there is total absence of the intrahepatic portal flow, and type II (latero-lateral shunt), in which the portal flow is partially preserved. We present the case of a 6-year-old girl with chronic hypoxemia history since 4 and a half years of age, showing progressive dyspnea, who was referred to the Pediatric Pulmonary Division with the diagnosis of central cyanosis. An Echo-Doppler in the portal venous system was performed, reporting agenesis of the principal portal vein. This finding was corroborated by an angiography of the portal and mesenteric arteriovenous system. The study was completed with a portosplenography, which confirmed the diagnosis of type Ib Abernethy malformation. The type I Abernethy malformation is more common in females, shows up in different ways and is treated with liver transplantation. On the other hand, type II Abernethy malformation shows a variable portal circulation and has a better prognosis than type I. Dyspnea when exercising and central cyanosis should be considered to make a differential diagnosis of cardiorespiratory disease at a pediatric age.

Anillos vasculares en pediatria: presentación de 2 casos / Pediatric vascular rings: case reports

Vascular rings are uncommon congenital anomalies that result from anomalous embryological development of the mediastinum main arteries (aortic arch and its branches, pulmonary artery). Such anomalous vascular structures may produce compression of the trachea and / or esophagus, with consequent obstructive respiratory and digestive symptoms. Vascular rings can be complete or incomplete. They can be asymptomatic or manifest with persistent or recurrent respiratory and / or digestive symptoms. The study should include chest X-ray, barium swallow, flow-volume loop, fiberoptic bronchoscopy, chest computed tomography, angiotomography or magnetic resonance. Symptomatic patients often need surgery

Los anillos vasculares son el resultado de anomalías del desarrollo embrionario de grandes troncos vasculares del mediastino, tanto del cayado aórtico y sus ramas, como de la arteria pulmonar. Dichas estructuras vasculares anómalas pueden producir compresión de la tráquea y/o del esófago, con la consiguiente sintomatología obstructiva respiratoria y digestiva. En algunas ocasiones forman un anillo completo y en otras incompleto. El diagnóstico puede ser casual cuando son silentes o resultado del estudio de síntomas respiratorios y/o digestivos persistentes o recurrentes. El estudio debe incluir radiografía de tórax, esofagograma, curva flujo/volumen, fibrobroncoscopía, angiotomografía computada o angioresonancia magnética de tórax. En pacientes sintomáticos se debe recurrir a la cirugía, en cambio los asintomáticos u oligosintomáticos deben ser observados

Malformación vascular venosa intestinal: una rara causa de hemorragia digestiva en pediatría. Presentación de un caso / Intestinal venous vascular malformation: Unusual etiology of gastrointestinal bleeding in pediatrics. Case report

Las malformaciones vasculares intestinales, en especial las del colon derecho, son causa frecuente de sangrado digestivo en adultos mayores, pero son raras en pediatría. Se pueden manifestar como hemorragia aguda, obstrucción intestinal o como anemia crónica de etiología incierta; esta es la presentación más frecuente, pero también la de mayor dificultad diagnóstica y terapéutica. Se presenta un niño de 11 años, que ingresó por Guardia con vómitos, dolor abdominal y descompensación hemodinámica, que requirió expansión y transfusión sanguínea. Entre sus antecedentes, figuraban episodios recurrentes de hemorragia intestinal baja desde los primeros años de vida, con estudios endoscópicos y centellograma Tc99 normales e historia de anemia persistente, a pesar del tratamiento. En la última internación, con la videocolonoscopía, se logró identificar una malformación venosa intestinal en la zona ileocecal. La angiografía digital y la enterotomografía multislice resultaron de suma utilidad en el proceso diagnóstico y en la elección de la conducta quirúrgica.

Intestinal vascular malformations, especially those in the right colon, are a frequent cause of lower gastrointestinal bleeding in adults, but they are a very rare condition in children. Symptoms include acute hemorrhage, intestinal obstruction, or chronic anemia of uncertain etiology, which is the most frequent form of presentation but the most difficult to diagnose and thus properly treat. We report the case of an 11 year old boy admitted to the Emergency Room with abdominal pain, vomits, hemodynamic decompensation, who required expansion and blood transfusion. With history ofrecurrent bloody stools since infancy with repeated normal endoscopies and Tc99 scintigraphy with chronic anemia and no improvement despite adequate treatment. In the last admission, the videocolonoscopy detected a venous vascular malformation in the ileocecal region. The angiography and the entero multislice computer tomography scanner were valuable tools to confirm the diagnosis and to select the appropriate surgical procedure for this rare condition.

Malformação venosa associada a hiperelasticidade cutânea e atrofia do tecido subcutâneo / Venous malformation associated with skin hyperelasticity and subcutaneous atrophy

A rigidez da parede venosa pode aumentar em síndromes em que há uma redução da quantidade de elastina, ocasionando malformações venosas mesmo em indivíduos que possuem mosaicismo para tais síndromes. Casos com apresentação de afecções colagenosas em áreas delimitadas não foram descritos na literatura. O paciente apresentava lesão bem delimitada em região anteromedial da coxa com aumento de elasticidade e presença de vasos tortuosos apenas no local da lesão, não apresentando nenhuma síndrome colagenosa. Foi realizada uma biópsia que evidenciou alterações em relação ao padrão das fibras elásticas e proliferação de vasos sanguíneos. A malformação venosa foi tratada satisfatoriamente com embolização. As doenças do colágeno causam hiperextensibilidade cutânea, o que provoca flacidez e propicia traumas. As colagenoses bem delimitadas são raras, pois geralmente esse grupo de doenças envolve acometimento sistêmico. As malformações vasculares podem ocorrer em diversas doenças do colágeno, mas de forma generalizada e não localizada, e uma explicação para isso seria o mosaicismo genético.

In syndromes that involve reduced quantities of elastin, the rigidity of vein walls may be increased, causing venous malformations, even in people who have mosaicism for these syndromes. There are no previous descriptions in the literature of collagen diseases presenting in specific, delimited areas. The patient described here presented with a lesion restricted to a well-defined area of the anteromedial thigh, in which elasticity was increased and vessels were tortuous, in the area of the lesion only, and with no other signs of collagen syndromes. A biopsy was conducted and the findings included changes to the normal arrangement of the elastic fibers and proliferation of blood vessels. The venous malformation was treated satisfactorily by embolization. Collagen diseases can cause cutaneous hyperextensibility, provoking flaccidity and a propensity to traumatisms. Connective tissue diseases restricted to well-delimited areas are rare, since this group of diseases usually has systemic involvement. Vascular malformations can be seen in many different collagen diseases, but with generalized rather than localized presentation. One possible explanation for the case described here is genetic mosaicism.

Aberrant Origin of Vertebral Artery and its Clinical Implications

Abstract Aberrant origin of vertebral artery is rare. The anatomical features and clinical significance of this lesion remain to be clarified. A comprehensive collection of the pertinent literature resulted in a cohort of 1286 cases involving 955 patients and 331 cadavers. There were more left than right and more unilateral than bilateral aberrant vertebral arteries. Patients with aberrant origin of vertebral artery were often asymptomatic and in only 5.5% of the patients their symptoms were probably related to the aberrant origin of vertebral artery. The acquired cardiovascular lesions were present in 9.5% of the patients, 20.9% of which were vertebral artery-associated lesions. Eight (0.8%) patients had a vertebral artery dissection. Logistic regression analysis showed significant regressions between bovine trunk and left vertebral artery (P=0.000), between the dual origins of vertebral artery and cerebral infarct/thrombus (P=0.041), between associated alternative congenital vascular variants and cervical/aortic dissection/atherosclerosis (P=0.008). Multiple logistic regression demonstrated that side of the aberrant origin of vertebral artery (left vertebral artery) (P=0.014), arch branch pattern (direct arch origin) (P=0.019), presence of the common trunk (P=0.019), associated acquired vascular disorder (P=0.034) and the patients who warranted management (P=0.000) were significant risk predictors for neurological sequelea. The patients with neurological symptoms and those for neck and chest operations/ interventions should be carefully screened for the possibility of an aberrant origin of vertebral artery. The results from the cadaver metrology study are very helpful in the design of the aortic stent. The arch branch pattern has to be taken into consideration before any maneuver in the local region so as to avoid unexpected events in relation to aberrant vertebral artery.

Double aortic arch as a source of airway obstruction in a child.

Double aortic arch (DAA) is a congenital vascular anomaly. The diagnosis was difficult till the child was symptomatic, and other causes were ruled out. We present the interesting images of a child of respiratory distress because of tracheal compression from DAA.

Síndrome de CLOVES / CLOVES syndrome

El síndrome de CLOVES fue recientemente descrito en el 2007 por Sapp et al. y se caracteriza por la presencia de un sobre crecimiento lipomatoso de predominio troncal, malformaciones vasculares, nevo epidérmico y malformaciones esqueléticas, como escoliosis y variables alteraciones espinales. Estos fenómenos determinan su nombre, CLOVES, Congenital, Lipomatous, Overgrowth, Vascular malformations, Epidermal nevi,Skeletal/Scoliosis/Spinal abnormalities. Se produce por una alteración de origen genético, esporádico, pero no necesariamente heredable, secundario a una mutación somática en el periodo embrionario. Se describe un caso clínico de paciente portador de esta patología.

CLOVES syndrome was recently described in 2007 by Sapp et al. and is characterized by the presence of lipomatous growth mainly on trunk, vascular malformations, epidermal nevi, and skeletal malformations such as scoliosis and variables spinal abnormalities. These phenomena determine its name, CLOVES, congenital lipomatous overgrowth, Vascular malformations, Epidermal nevi, Skeletal / Scoliosis / Spinal abnormalities. It is produced by a genetic alteration, sporadic, but not necessarily heritable, CLOVES secondary to a somatic mutation in embryonic period. We described a clinical case of a patient with this condition.

Multi-Detector Row Computed Tomographic Evaluation of a Rare Type of Complete Vascular Ring: Double Aortic Arch with Atretic Left Arch Distal to the Origin of Left Subclavian Artery

Double aortic arch with an atretic left arch distal to the origin of left subclavian artery was diagnosed with multi-detector row computed tomography (MDCT) in two children with dysphagia. This rare type of complete vascular ring is clinically important because it may be confused with right aortic arch in mirror imaging. Anatomic details of this rare type of complete vascular ring demonstrated on MDCT facilitated appropriate surgical treatment.

Evaluation of Cardiovascular Anomalies in Patients with Asymptomatic Turner Syndrome Using Multidetector Computed Tomography

Turner syndrome is well known to be associated with significant cardiovascular abnormalities. This paper studied the incidence of cardiovascular abnormalities in asymptomatic adolescent patients with Turner syndrome using multidetector computed tomography (MDCT) instead of echocardiography. Twenty subjects diagnosed with Turner syndrome who had no cardiac symptoms were included. Blood pressure and electrocardiography (ECG) was checked. Cardiovascular abnormalities were checked by MDCT. According to the ECG results, 11 had a prolonged QTc interval, 5 had a posterior fascicular block, 3 had a ventricular conduction disorder. MDCT revealed vascular abnormalities in 13 patients (65%). Three patients had an aberrant right subclavian artery, 2 had dilatation of left subclavian artery, and others had an aortic root dilatation, aortic diverticulum, and abnormal left vertebral artery. As for venous abnormalities, 3 patients had partial anomalous pulmonary venous return and 2 had a persistent left superior vena cava. This study found cardiovascular abnormalities in 65% of asymptomatic Turner syndrome patients using MDCT. Even though, there are no cardiac symptoms in Turner syndrome patients, a complete evaluation of the heart with echocardiography or MDCT at transition period to adults must be performed.

Presentación de un caso: trombosis venosa profunda bilateral de etiología infrecuente / Case report: rare bilateral deep venous trombosis

La agenesia de vena cava inferior (VCI) es una patología poco frecuente que suele diagnosticarse a raíz de una trombosis venosa secundaria sintomática de las venas ilíacas. Presentamos el caso de un varón de 28 años que acude al centro de salud por dolor en miembros inferiores y datos de insuficiencia venosa. Se visualiza mediante ecografía trombosis venosa profunda (TVP) bilateral...

Malformación arteriovenosa del íleon medio, identificada por cápsula endoscópica: reporte de caso / Ileal vascular malformation, identified by capsule endoscopy: case report

Forty-nine year-old-man, diabetes mellitus Type 2, bone marrow transplantation 10 years ago due to chronic myelocytic leukemia, deep vein thrombosis and placement of cava vein filter 6 years ago, chronic use of Aspirin (100 mg). He presents with melena and abdominal pain. Upper endoscopy, colonoscopy, angio- CT and angiography were negative. Capsule endoscopy (PillCamTMSB) shows two segments of vascular malformation with active bleeding from the second segment in the middle-ileum. Laparotomy was performed founding at the level of the middle-ileum two vascular lesions of variceal type in the intestinal wall. Surgical resection of 15 cm of middle ileum and a primary anastomosis was performed. Histopathology reports, submucosal arteriovenous vascular malformation (AVM).

Hombre de 49 años, con antecedentes de diabetes mellitus tipo 2, trasplante de médula ósea hace 10 años por leucemia mieloide crónica, trombosis venosa profunda e instalación de filtro de vena cava inferior hace 6 años, usuario crónico de Aspirina® 100 mg/día. Presenta melena y dolor abdominal difuso. Es transfundido y se realizan estudios de endoscopia alta, colonoscopia, AngioTC y angiografía sin hallazgos del sitio de sangrado. Paciente ingiere cápsula endoscópica (PillCamTMSB) que muestra dos segmentos en intestino delgado, íleon medio y medio distal con malformaciones vasculares tipo várices, identificando sangrado activo en el segundo segmento. Se realiza laparotomía y a nivel del íleon medio se observan dos paquetes varicosos en la pared intestinal. Se realiza resección quirúrgica de 15 cm de íleon medio, y anastomosis primaria término-terminal. El paciente evolucionó satisfactoriamente sin recurrencia de sangrado. El estudio histopatológico e histoquímico con tinción de Van Gieson-elástica reporta malformación vascular arteriovenosa (AVM) submucosa de la pared del intestino delgado.

Malformaciones vasculares: manejo mediante técnicas de radiología intervencional y satisfacción de los pacientes / Vascular malformations: interventional radiology management and assesment of patient satisfaction

Background: Vascular anomalies are prevalent in the general population and may produce significant impairment in quality of life. In recent years, minimally invasive interventional radiology techniques have become an efficient alternative for the treatment of these patients. The aim of this study was to present our experience in the management of vascular malformations with such techniques at Clínica Alemana in Santiago, Chile, and assess the satisfaction of patients with this management. Material and Methods: Evaluation of the follow-up of patients with vascular malformations treated in the Interventional Radiology Unit at our center since June - 2006 to June - 2009. Patients were classified according to the hemodynamics of the lesions (high vs low-flow). Complications and patient satisfaction with the treatment were assessed. Results: Eighty-seven patients (253 procedures) were included in the study, 36 men and 51 women, all with complete follow-up during the period of the study. Their average age was 20.6 years. In over 50 percent of the patients the vascular anomaly was located on the face or the lower extremities. Twenty-eight percent (25 patients) had a high-flow malformation (pure or mixed) and 62 (72 percent) had a low-flow lesion (vascular malformation, lymphangioma or mixed). On average, each patient underwent 2.9 procedures (1-10). There were 4 major complications (1.65 percent), one of which was predominantly cosmetic with necrosis in the area of the lesion. The average satisfaction rate was 7.95 (on a rating scale of 1 to 10). Satisfaction was significantly higher in patients with low-flow malformations (8.27) than in those with high-flow lesions (7.16). (p < 0.05) Discussion: Treatment of vascular malformations using interventional radiology techniques is an effective alternative, with good prospects of clinical improvement, patient satisfaction and low complication rates.

Introducción: Las anomalías vasculares son una patología frecuente en la población general, y son causa de deterioro de la calidad de vida de los pacientes que las padecen. En los últimos años, las técnicas mínimamente invasivas de radiología intervencional se han convertido en una alternativa eficaz para el tratamiento de estos pacientes. El objetivo del presente estudio es mostrar nuestra experiencia en el manejo de malformaciones vasculares con estas técnicas en la Clínica Alemana de Santiago y evaluar la satisfacción de los pacientes con este manejo. Material y Métodos: Evaluación del seguimiento de todos los pacientes con malformaciones vasculares tratados en la Unidad de Radiología Intervencional desde el año 2006 hasta junio del año 2009. Los pacientes fueron clasificados en base a la hemodinamia de la malformación vascular (alto flujo vs. bajo flujo). Se evaluaron las complicaciones, y se realizó una encuesta sobre la satisfacción de los pacientes con el tratamiento. Resultados: Ochenta y siete pacientes (253 procedimientos) fueron incluidos en el estudio, 36 hombres y 51 mujeres, todos con seguimiento completo en este periodo. La edad promedio de ellos fue de 20,6 años. En más del 50 por ciento de los pacientes la anomalía vascular se ubicó en la cara o extremidades inferiores. Un 28 por ciento (25 pacientes) presentó malformación de alto flujo (pura o mixta) y 72 por ciento (62 pacientes) de bajo flujo (malformaciones vasculares, linfangiomas o mixtas). En promedio, cada paciente se realizó 2,9 procedimientos (1-10). Del total de procedimientos realizados, hubo 4 complicaciones mayores (1,65 por ciento), una de ellas con secuelas estéticas producto de necrosis de la zona. El promedio de satisfacción fue de un 7,95 en una escala de 1 a 10, siendo significativamente mayor en pacientes con malformaciones de bajo flujo (8,27) que en los de alto flujo (7,16) (p < 0.05)...

Aneurisma verdadero en arteria ciática persistente: caso clínico / Persistent sciatic artery aneurysm: a case report

We report the case of a 71 years old female patient, who had a partially thrombosed true aneurysm in a persistent sciatic artery (PSA), treated in the Surgery Department of Hospital Dr. Eduardo Pereira of Valparaiso. The sciatic artery arises from the umbilical artery and during early embryological state is the main blood supply of the lower limbs. Later, the superficial femoral artery appears with the subsequent progressive involution of the sciatic artery. PSA is a rare congenital vascular anomaly that occurs when sciatic artery fails to regress during fetal development. This is associated with superficial femoral artery hipoplasia and the PSA becomes the dominant arterial inflow to the lower limb. This anatomic abnormality may be bilateral and can remain asymptomatic for many years, however, it has been described aneurysmal degeneration, like in this case, symptoms of sciatic nerve compression, aneurysm thrombosis and distal embolization.

Se reporta el caso de una paciente de 71 años, que presenta un aneurisma verdadero, parcialmente trombosado, en una Arteria Ciática Persistente (ACP), tratada en el Servicio de Cirugía del Hospital Dr. Eduardo Pereira de Valparaíso. La arteria ciática se origina en la arteria umbilical y durante las primeras fases embriológicas es el principal aporte sanguíneo de la extremidad inferior. Posteriormente aparece la arteria femoral superficial y se produce la progresiva involución de la arteria ciática. La ACP constituye una rara anomalía y ocurre por la falta de desarrollo de la arteria femoral superficial. La ACP se origina en el adulto en la arteria hipogástrica, transcurre hacia la región glútea a través de la escotadura ciática y sigue hacia distal por la parte posterior del muslo continuándose después con la arteria poplítea. Se acompaña de un variable menor desarrollo del eje arterial anterior de la arteria ilíaca externa y femoral superficial. Esta anomalía anatómica puede ser bilateral y puede permanecer asintomática durante muchos años, sin embargo, se ha descrito la degeneración aneurismática, como en este caso, y síntomas por compresión del nervio ciático, trombosis del aneurisma y embolización distal.

Congenital hypoplasia of the internal carotid artery.

A newborn presented at 14 hrs of age with right sided clonic seizures and shrill cry. Magnetic Resonance Imaging of the brain showed left cerebral hemiatrophy with cystic changes in left fronto-parietal lobe and parasylvian region. The Magnetic Resonance Angiography revealed hypoplasia of left supraclinoid Internal Carotid Artery and hypoplasia and irregularity of vessel wall affecting the left Middle Cerebral Artery. Such an early presentation of this rare disorder has not been reported previously. Recognition of this anomaly has important implications during carotid and trans-sphenoidal surgery, in thromboembolic disease, and in the surveillance and detection of associated cerebral aneurysms.

Hematoma subdural crónico calcificado: reporte de un caso / Calcified chronic subdural hematoma: a case report

El hematoma subdural crónico constituye una enfermedad frecuente en la práctica neuroquirúrgica cotidiana. Su diagnóstico se realiza actualmente de forma temprana en la mayoría de los pacientes, a partir de la introducción de las modernas técnicas de imagenología. Se presenta el caso de un enfermo de 84 años de edad con historia de cefalea, mutismo acinético y cuadriparesia, cuyo examen tomográfico mostró una colección extraxial supratentorial hemisférica derecha con calcificación masiva; se practicó la exéresis mediante una craneotomía y el estudio histopatológico confirmó el diagnóstico de hematoma subdural crónico. Se concluye que, al constituir la calcificación masiva del hematoma subdural crónico una rara forma de presentación imagenológica en la actualidad, deben contemplarse otras entidades en el diagnóstico diferencial como neoplasias y malformaciones vasculares intracraneales; por otro lado, aumenta la complejidad quirúrgica ya que es imprescindible la práctica de una craneotomía para la evacuación de la lesión.

The chronic subdural hematoma constitutes a frequent disease in daily neurosurgical practice. Currently its diagnosis of premature form is performed in the majority of patients, since the introduction of imaging's modern techniques. An 84 years old patient with history of cephalea, akinetic mutism and cuadriparesis, whose tomographic exam showed a right hemispheric supratentorial extraxial collection with massive calcification; the exeresis was performed by means of a craniotomy and the histopathological study confirmed the diagnosis of chronic subdural hematoma. It was concluded that, when constitute the massive calcification of the chronic subdural hematoma a rare form of imaging presentation at present, should contemplate another entities in the differential diagnostic like neoplasias and intracranial vascular malformations; In addition, increases the surgical complexity since it is essential the practice of a craniotomy for the evacuation of the lesion.

Cutaneous vascular anomalies pattern in neonates and infants

To analyze the patterns of cutaneous vascular anomalies in neonates and infants. To identify the complications arising in various vascular lesions. A descriptive hospital based observational study. Dermatology and pediatric department of DHQ and Allied Hospital PMC, Faisalabad From 1 Feb 2005 to 31 January 2006. 72 consecutive newborns and infants of either sex having any vascular anomaly on cutaneous areas were included in the study. The information regarding size, site, type of lesions, sex of patients, with family history and any complication were recorded on a proforma prepared for this study. Out of 72 infants 25 were male [35%] and 47[65%] were females. Family history was present in 12 cases [17%]. The commonly involved sites were head and neck 45 [62.5%], trunk 18[25%] and upper limb 9[12.5%]. In 59 children [82%] it was single and in 13[18%] the lesions were multiple. The complications observed were ulceration in 13[18%], infection in 8[11%], haemorrhage in 3[4%] and visual impairment in 4[5%], while 56[78%] had no complications