Hallazgos por imagen de malformación de Abernethy tipo II en paciente de 12 años / Image finding of Abernethy type II malformation in 12 year old patient

Las malformaciones del sistema venoso abdominal son alteraciones vasculares raras. La incidencia de esta afección se estima en uno de cada 30,000 nacimientos y se asocian con malformaciones gas- trointestinal, genitourinaria, ósea y cardiovascular. En el 2018 se ha registrado en la literatura mundial 39 casos de Abernethy tipo I y 22 casos de Abernethy tipo II. CASO CLÍNICO paciente femenino de 12 años con antecedente de hipertensión portal tratada hace 2 años, con historia de malestar general e ic- tericia, acudió a centro privado para realizarse estudios complementarios. Un ultrasonido Doppler por- tal evidenció una lesión isoecogénica al parénquima hepático en el aspecto inferior del lóbulo derecho. Se continuó la evaluación realizando una tomografía en la cual se observó: configuración anómala del sistema venoso portal; la vena esplénica y mesentérica superior se encuentran dilatadas, además se evidenció confluencia portoesplénica elongada, en la cual derivan dos trayectos portales, uno de ellos drenando la lobulación hepática antes descrita y la segundo se comunica con el sistema venoso portal hepático derecho, demostrando tortuosidad de su trayecto, con estenosis de su porción proximal. Los hallazgos antes descritos sugieren malformación vascular del sistema venoso portal-esplácnico, que causa derivación porto-sistémica en relación a malformación de Abernethy tipo II. En conclusión se recomienda el diagnóstico precoz. El examen preferente es el ecodoppler con posterior confirmación mediante angiotac abdominal. El tratamiento es sumamente importante pues su retraso puede devenir en lesiones irreparables hasta la insuficiencia hepática y muerte...(AU)

Variz Intra-Óssea ­ um achado raro diagnosticado pela ressonância magnética / Intraosseous Varicose Vein ­ A rare finding diagnosed by magnetic resonance imaging

Introdução: As varizes são veias que após submetidas a períodos de pressão aumentada no sistema venoso periférico se tornam dilatadas, tortuosas e alongadas. Pacientes acometidos irão referir dor no membro, além de sinais de insuficiência venosa. O exame de imagem comumente utilizado é o ultrassom com Doppler, no entanto, na presença de anomalias de drenagem venosa intraóssea, deve-se preferir a ressonância magnética. Objetivo: Relatar um caso e revisar a literatura acerca desta lesão incomum. Materiais e Métodos: Revisão do prontuário do paciente no Hospital América, registro fotográfico do método diagnóstico e revisão da literatura. Resultados: Homem de 46 anos com dor e queimação constantes, além de edema na perna e no pé esquerdos há dois meses Ao exame físico apresenta edema da perna, tornozelo e pé esquerdos, com discreto aumento de temperatura ao toque, sem alteração da coloração da pele. A ressonância magnética demonstra varicosidades das veias tibiais posteriores com comunicação através de veia perfurante com varicosidade da veia intraóssea posterior da tíbia ­ variz intraóssea. Conclusão: Este relato demonstra a dificuldade de diagnosticar a variz intra-óssea que, além de ser uma lesão rara, é diagnosticada apenas pela ressonância magnética.

Introduction: Varicose veins are veins that after being submitted to high pressure on the peripheral venous system become dilated, tortuous and elongated. Patients affected will report pain and venous insufficiency signs in the area. The imaging exam commonly used is the Doppler ultrasound, however, when in front of an intraosseous venous drainage anomaly ­ intraosseous varix, magnetic resonance should be preferred. Objective: Report a case and review the literature about this uncommon lesion. Materials and Methods: We carried out a review of medical records at Hospital América, a photographic record of diagnostic methods, and a review from the literature. Results: A 46-year-old man with constant pain and burning, as well as edema in his left leg and foot for two months. On physical examination, he had edema in his left leg, ankle, and foot, with a slight increase in temperature at the touch, without changing the skin color. Magnetic resonance imaging shows varicosities of the posterior tibial veins with communication through a perforating vein with varicosity of the posterior tibial intraosseous vein - intraosseous varicose vein. Conclusion: This report demonstrates the difficulty of diagnosing intraosseous varices, which, in addition to being a rare lesion, are diagnosed only by magnetic resonance imaging.

Five veins at the deep inguinal ring: can they reduce the chances of indirect inguinal hernia and increase the chances of varicocele? / Cinco venas en el anillo inguinal profundo. ¿pueden reducir las probabilidades de una hernia inguinal indirecta y aumentar las posibilidades de varicocele?

Complete duplication of testicular veins is a rare phenomenon. However, a few cases of duplication of gonadal veins have been reported. Here, I report a case of unusual formation and termination of the right testicular vein in an adult male cadaver. Five veins arose from the pampinniform plexus and entered the abdomen through the deep inguinal ring. The most medial among the five was large (3 mm in diameter) and it continued as a testicular vein and opened into the right edge of the inferior vena cava, 1 cm above the union of the common iliac veins. The other four veins were about 1 mm in diameter and they united to form two veins in front of the lower part of the right psoas and iliacus muscles (about 2 cm above the deep inguinal ring) and the two veins united to form upper testicular vein, 4 cm above the deep inguinal ring. This testicular vein was 3 mm in diameter and it opened into the inferior vena cava, 4 cm above the union of common iliac veins. Having five veins at deep inguinal ring might increase the chances of varicocele and decrease the chances of indirect inguinal hernia.

La duplicación completa de las venas testiculares es un fenómeno raro. Sin embargo, se han reportado algunos casos de duplicación de venas gonadales. En el presente trabajo se informa un caso de formación y terminación inusual de la vena testicular derecha en un cadáver de un hombre adulto. Cinco venas surgieron del plexo pampiniforme y penetraron en el abdomen a través del anillo inguinal profundo. El más medial entre los cinco fue de gran tamaño (3 mm de diámetro) y continuó como una vena testicular y se abrió hacia el margen derecho de la vena cava inferior, 1 cm por encima de la unión de las venas ilíacas comunes. Las cuatro venas restantes eran de 1 mm de diámetro aproximadamente, y se unieron para formar dos venas frente a la parte inferior de los músculos psoas e ilíaco derechos (aproximadamente 2 cm por encima del anillo inguinal profundo). Se unieron dos venas para formar la vena testicular superior, la cual medía 3 mm de diámetro y se abría hacia la vena cava inferior, 4 cm por encima de la unión de las venas ilíacas comunes. Cinco venas en el anillo inguinal profundo podrían aumentar las posibilidades de varicocele y disminuir las posibilidades de una hernia inguinal indirecta.

Un caso de síndrome de Adams-Oliver con venas congénitas, dilatadas y persistentes en el cuero cabelludo / A case of Adams-Oliver syndrome with congenital and persistent dilated veins in the scalp

El síndrome de Adams-Oliver es un desorden congénito raro, caracterizado por la presencia de aplasia cutis congénita y defectos terminales transversos de los miembros. Comunicamos el caso de una niña de 4 años de edad con síndrome de Adams-Oliver que presenta venas congénitas, tortuosas y dilatadas en el cuero cabelludo, aplasia cutis congénita con defectos parciales del hueso craneal subyacente, calcificaciones intracraneales y anomalías leves de los pies.

Adams-Oliver syndrome is a rare congenital disorder characterized by aplasia cutis congenita and terminal transverse limb defects. We report a case of a 4-year-old girl with Adams-Oliver syndrome with congenital dilated and tortuous scalp veins, aplasia cutis congenita with partial underlying skull defects, intracranial calcifications, and mild foot anomalies.

Anesthetic implications of total anomalous systemic venous connection to left atrium with left isomerism.

Total anomalous systemic venous connection (TASVC) to the left atrium (LA) is a rare congenital anomaly. An 11-year-old girl presented with complaints of palpitations and cyanosis. TASVC with left isomerism and noncompaction of LV was diagnosed after contrast echocardiogram and computed tomography angiogram. The knowledge of anatomy and pathophysiology is essential for the successful management of these cases. Anesthetic concerns in this case were polycythemia, paradoxical embolism and rhythm abnormalities. The patient was successfully operated by rerouting the systemic venous connection to the right atrium.

Tratamento a laser das malformações vasculares venosas / Laser treatment of venous malformations

OBJETIVO: Avaliar se o tratamento das malformações vasculares venosas congênitas realizado com o emprego da tecnologia laser oferece clareamento ou desaparecimento das lesões com elevado grau de satisfação informado pelos pacientes e observado pelo médico. MÉTODOS: O estudo retrospectivo reuniu 26 pacientes, portadores de malformação vascular venosa, dos quais 73,07 por cento eram do sexo feminino e que foram tratados com o aparelho PhotoDerm®. O tratamento das malformações vasculares necessitou uma média de 6,43 sessões, com intervalos de seis a oito semanas. Os pacientes incluídos neste estudo apresentavam pele tipo II (57,40 por cento) e tipo III (42,30 por cento), conforme classificação de Fitzpatric. A idade média variou de 14 a 61 anos, com média de 36,70 anos. Os dados foram obtidos através do preenchimento de protocolo informado pelo paciente. RESULTADOS: Foi informado elevado grau de satisfação (96,16 por cento) e apenas um caso (3,84 por cento) parcialmente satisfeito, considerando o clareamento ou desaparecimento das lesões. Houve desaparecimento das lesões avaliado pelo profissional médico em 80,76 por cento dos casos e em 19,24 por cento apenas clareamento. CONCLUSÃO: O tratamento das malformações vasculares venosas com o Photo-Derm® é seguro e eficiente tendo proporcionado elevado grau de satisfação dos pacientes assim como bons resultados em relação ao desaparecimento das lesões.

OBJECTIVE: To evaluate whether treatment of venous congenital vascular malformations with the use of laser technology provides lightening or disappearance of lesions with a high degree of satisfaction reported by patients and observed by the physician. METHODS: A retrospective study gathered 26 patients suffering from vascular malformation, of which 73.07 percent were female and were treated with the PhotoDerm® device. The treatment of vascular malformations needed an average of 6.43 sessions, with intervals of six to eight weeks. Patients included in this study had skin type II (57.40 percent) and type III (42.30 percent), according to the Fitzpatric's classification. The mean age ranged from 14 to 61 years, averaging 36.70 years. Data were obtained through the completion of an informed protocol by the patient. RESULTS: High satisfaction rates were reported (96.16 percent) and only one case (3.84 percent) was partially satisfied when considering lightening or disappearance of lesions. When evaluated by medical professionals, lesions disappeared in 80.76 percent and became lighter in 19.24 percent. CONCLUSION: The treatment of venous vascular malformations with Photo-Derm® is safe and effective as it offered a high degree of patient satisfaction and good results in the disappearance of the lesions.

Ultrasonografía Doppler-color en el diagnóstico de las anomalías vasculares de las partes blandas superficiales en pediatría / Color Doppler ultrasonography in the diagnosis of vascular anomalies of the superficial soft tissue in children

Las anomalías vasculares de las partes blandas superficiales son frecuentes en el niño y se han clasificado en hemangiomas y malformaciones vasculares, según sus manifestaciones clínicas y hallazgos histológicos. La mayoría corresponde a hemangiomas, se presenta en el recién nacido, desaparece espontáneamente con el tiempo y sólo algunos de ellos requerirán de evaluación médica. Es importante especificar el tipo de lesión vascular para definir el tratamiento, lo que en la mayor parte de los casos es posible con los hallazgos clínicos. Sin embargo, a veces es necesario recurrir a estudios de imágenes para precisar el diagnóstico y los métodos más utilizados son la Ultrasonografía (US) Doppler-color; Resonancia magnética (RM) y Angiografía. La US Doppler-color es un método sencillo, inocuo, no invasivo, que permite diferenciar las lesiones superficiales de aquellas profundas y además permite evaluar la vascularización de la lesión y, de esta manera, lograr una adecuada aproximación diagnóstica. También permite seleccionar aquellos casos que requerirán de métodos más sofisticados, como RM o angiografía. El objetivo de esta revisión es mostrar la utilidad de la US Doppler en el estudio de las anomalías vasculares de las partes blandas superficiales en el niño.

Vascular anomalies of superficial soft tissues are frequent in children and have been classified in hemangiomas and vascular malformations, depending on clinical and histological findings. Most correspond to hemangiomas, present in the newborn period, which disappear spontaneously with time and only some may require an imaging evaluation. It is important to specify the type of vascular anomaly in order to define management, which in most cases is possible with just clinical findings. Nevertheless, some cases may require imaging studies to define diagnosis, and the most utilized methods are Doppler ultrasonography (US), magnetic resonance Imaging (MRI) and angiography. Doppler US is a non invasive, harmless and simple method which allows to differentiate between deep and superficial lesions. It also permits to evaluate vascularization and in this manner; achieve an adequate diagnosis and select those cases that will require more sophisticated methods like MRI or angiography. The main objective of this review is to show the role of Doppler US in the study of superficial soft tissue vascular anomalies in children.

Malformaciones Vasculares / Vascular malformation

Las malformaciones vasculares son anomalías presentes siempre desde el nacimiento que, al contrario de los hemangiomas, nunca desaparecen; pueden crecer durante toda la vida por hipertrofia. Según la clasificación de la ISSVA, las malformaciones vasculares se dividen en función del vaso afectado en capilares o venulares, venosas, linfáticas, arterio-venosas y combinadas o complejas. Cada una de ellas, con unas peculiaridades clínicas y hemodinámicas definitorias.

Vascular malformations are anomalies always present at birth that, contrary to hemangiomas, never regress; and may hypertrophy during lifetime. According to the ISSVA classification, vascular malformations are divided, depending on the affected vessel, into capillary or venular, venous, lymphatic, arteriovenous and combined or complex; each with certain defining clinical and haemodynamic peculiarities.

[Experience of a multidisciplinary comittee in the management of superficial venous malformations: 99 cases]

Vascular malformations are heterogenous angiodysplasias. The aim of this work is to study the epidemiological and clinical features of venous malformations [VM] as well as their multidisciplinary management. It is a retrospective study of patients having simple VM, seen at the multidisciplinary consultation of angiodysplasias of our hospital, over a 10 year period. This study included 99 patients having VM. A female predominance was noticed. Average age was 18.5 years. VM had mainly cephalic location. All VM were treated with percutaneous sclerotherapy. Aesthetic results were excellent in the cases of small VM size. VM are most frequent in cephalic region and cause aesthetic and functional handicaps. Percutaneous sclerotherapy is the treatment of choice for such a venous malformations, especially for small VM. VM are anomalies which can have serious complications. Their management has to be multidisciplinary in order to better precise the diagnosis and to take the adequate therapeutic decision

An unusual venous drainage of face--a case report.

Facial region is drained by the maxillary, facial and superficial temporal veins. In the present case, retromandibular venous junction divided into anterior and posterior divisions immediately after its formation and a common venous channel was formed between internal jugular and anterior jugular veins where facial, lingual and submental veins drained.

Unusual venous drainage of face: a case report.

Facial region has enormous blood supply. The maxillary vein, facial vein and superficial temporal vein are chief venous draining channels. There are numerous reports of unusual venous system of face, in the present case, retromandibular vein divides into anterior and posterior division soon after its formation, external carotid artery lying lateral to retromandibular vein, formation of common venous channel between internal jugular vein and anterior jugular vein where facial, lingual and submental vein drain.

Obstrucción ureteral parcial crónica por vena ovárica: Descripción de un caso y revisión de la literatura / Chronic partial uretheral obstruction by the ovarian vein: A case report and literature review

Se presenta el caso de una paciente con dolor lumbar crónico secundario a obstrucción ureteral parcial por la vena ovárica en la que se corrobora la efectividad del tratamiento quirúrgico.

We present a female patient with chronic loin pain caused by partial obstruction of the urether secondary to ovarian vein crossing. We highlight the benefit derived from surgical treatment.

Anatomia das veias e troncos gastrocnêmios em cadáveres humanos adultos / The anatomy of the gastrocnemial veins and trunks in adults human cadavers

Objetivo: realizar, através de dissecção, um estudo anatômico das veias gastrocnêmias em cadáveres humanos adultos.Métodos: foram dissecadas 80 cabeças de músculo gastrocnêmio em 20 cadáveres do sexo masculino, fixados e mantidos em uma solução de formol a 10 por cento. Todas as veias gastrocnêmias foram dissecadas desde a emergência das cabeças do músculo gastrocnêmio até o seulocal de desembocadura. Para a tomada das medidas, foi utilizado um paquímetro digital. Foram obtidas estatísticas descritivas para as variáveis do estudo.Resultados: em 80 cabeças de músculos gastrocnêmios dissecadas, foram encontradas 438 veias gastrocnêmias, sendo 233 na perna direita e 205 na perna esquerda. Foram identificados 95 troncos gastrocnêmios principais, 51 na perna direita e 44 na esquerda. A extensão dos troncos variou de 0,5 cm a 7,8 cm, com uma amplitude expressiva no conjunto das observações de 7,3 cm. Do total de troncosgastrocnêmios principais, 83 desembocavam na veia poplítea e 12, em outras veias.Conclusão: a quantidade de veias e troncos gastrocnêmios foi maior na perna direita, e a extensão média do tronco gastrocnêmio principalfoi semelhante em ambas às pernas. A maioria desses troncos desembocava na veia poplítea.

Left costocervical vein malformation with anomalous ramification of aortic arch in a dog

This report describes coexistence of anomalous branches of the aortic arch and the costocervical vein malformation in a German shepherd dog. The first branch of the aortic arch was a bicarotid trunk that divided into the left and right common carotid arteries. The next branch to leave the aortic arch was a common trunk for the right and left subclavian arteries, a bisubclavian trunk, which was immediately bifurcated. The right subclavian artery passed over the esophagus forming a deep groove, so-called incomplete vascular ring on the dorsal wall of the esophagus. Although the esophagus was constricted by the right subclavian artery dorsally and by the trachea ventrally, no clinical symptoms of esophageal obstruction and dysphagia were observed. The left costocervical vein coursed caudoventrally, passed over the aortic arch, and entered the left ventricle. This vessel was much smaller than the right costocervical vein and was partially occluded at its origin.

Venous malformation of the cheek: treatment selection based on C.T. scan evaluation

Venous malformations represent a great cosmetic and functional problem to the patients; especially those lesions occurring in the face. Although a variety of treatments of this condition exist in the field of plastic surgery, yet the selection of the appropriate modality is not clear in the literature. In the current paper, 15 patients -suffering from venous malformation of the cheek area- were selected from a large pool of patients presented to the plastic surgery department of Ain-Shams University. They were classified into two groups [localized and diffuse] according to the clinical examination and the CT. scan findings. The first group was treated by surgical excision; while the second group was subjected to Nd.:YAG laser photocoagulation. The results, of the classification and the different treatments were presented and discussed

Radiologic-Pathologic Correlation of Unusual Lingual Masses:Part I: Congenital Lesions

Because the tongue is superficially located and the intial manifestation of most diseases occurring there is mucosal change, lingual these lesions can be easily accessed and diagnosed without imaging analysis. Most congenital lesions of the tongue, however, can manifest as a submucosal bulge and be located in a deep portion of that organ such as its base; their true characteristics and extent may be recognized only on cross-sectional images such as those obtained by CT or MRI. In addition, because it is usually difficult to differentiate congenital lesions from other submucosal neoplasms on the basis of imaging findings alone, clinical history and physical examination should always be taken into consideration when interpretating CT and MR images of the tongue. Although the radiologic findings for congenital lesions are nonspecific, CT and MR imaging can play an important role in the diagnostic work-up of these unusual lesions. Delineation of the extent of the tumor, and recognition and understanding of the spectrum of imaging and the pathologic features of these lesions, often help narrow the differential diagnosis.

Bilateral coronary arteriovenous fistula coexistent with atrial septal defect and pulmonary stenosis

A coronary arteriovenous fistula consists of a communication between a coronary artery and a cardiac chamber, a great artery or the vena cava. It is the most common anomaly that can affect coronary perfusion. Bilateral involvement of coronary fistula, however, constitutes an uncommon subgroup of coronary arteriovenous fistulas. We report a case which shows a rare occurrence of bilateral coronary arteriovenous fistula coexistent with atrial septal defect and pulmonic stenosis.