RF catheter ablation of AVNRT in a patient with interrupted inferior vena cava anomaly with hemiazygos continuity with persistent left superior vena cava.

Intrahepatic interruption of the inferior vena cava (IVC) with continued hemizygous is a very rare abnormality and sometimes it may be accompanied by other cardiovascular abnormalities. Continuation of the hemizygous vein draining into the right atrium through the left superior vena cava (LSVC) is much rarer. In this paper, we have presented a patient who had simultaneous IVC interrupted with persistent LSVC and suffered from Atrioventricular nodal reentrant tachycardia (AVNRT). Finally, radiofrequencies (RF) catheter ablation for AVNRT was successfully performed through a left subclavian vein access.

Levopositioned caudal vena cava and circumcaval left ureter with subsequent hydronephrosis and hydroureter in a dog.

The presentation and investigation of a levopositioned caudal vena cava, a rare congenital vascular abnormality seen mainly in Bernese Mountain Dogs, which resulted in ureteral compression, hydronephrosis and hydroureter. Surgical transection, transposition and anastomosis were performed to reposition the circumcaval ureter. A 19-month-old male neutered Bernese Mountain Dog was presented with a 13-month history of vague signs including intermittent abdominal pain, inappetence and diarrhoea, which were poorly responsive to medical management. Abdominal ultrasound revealed left-sided hydronephrosis and hydroureter. Further investigation included abdominal computed tomography and pre- and post-intravenous iodinated contrast, which revealed a levopositioned caudal vena cava with associated ureteral displacement to a circumcaval position, resulting in ureteral compression and proximal dilation affecting both the proximal ureter and the left kidney. The patient was also found to have concomitant gall bladder agenesis. Surgery was performed to transect, transpose and anastomose the ureter in a normal anatomical position, and the patient made an excellent clinical recovery. All gastrointestinal signs resolved within 2 weeks of surgery and remained resolved 12 months later. Follow-up ultrasound and pyelography were performed at 4-month post-surgery, revealing a mild improvement in the hydronephrosis and hydroureter. Although rare, circumcaval ureter should be considered a differential in dogs presenting with hydroureter and hydronephrosis, especially Bernese Mountain Dogs.

Kidney Transplant for a Patient With Inferior Vena Cava and Abdominal Aorta Abnormalities.

Here, we describe an interesting case of a patient with the duplication of inferior vena cava, high-positioned bifurcation of the abdominal aorta with transposition of iliac arteries, and right renal aplasia associated with end-stage renal disease who underwent kidney transplant. In this case, the patient with anorectal malformations with a vaginal fistula was prepared and underwent a kidney transplant. During the surgery, we discovered duplicated inferior vena cava and transposed iliac arteries. After the surgery, computed tomography angiography revealed the inferior vena cava duplication with the 2 connections between the right and left inferior vena cava with the formation of an anomalous circle, high-positioned bifurcation of the abdominal aorta at the level of the L2 vertebral body, and transposition of right and left iliac arteries. Also, we observed the right kidney aplasia and absence of blood circulation in the left native kidney. In our case, a delayed diagnosis of pyelonephritis resulted in the progression to end-stage renal disease that necessitated a kidney transplant, during which we found these anomalies. We confirmed the asymptomatic course of these anomalies, diagnosed only during radiological imaging or surgical intervention. Patients with congenital anomalies of the kidney and urinary tract should undergo complete investigations before surgical decisions. Diagnosis of this pathology in the preoperative period, especially in transplant patients, will alert the surgery team in advance of the operation and allow preparation for the intraoperative difficulties that are typically associated with anomalies such as inferior vena cava transposition or aplasia.

A cadaver study evaluating intraluminal anomalies of the left common iliac vein.

OBJECTIVE: Intraluminal anomalies within the left common iliac vein, characteristic of iliac vein compression syndrome, are thought to result from compression by and pulsation of the overlying right common iliac artery. This cadaver study was designed to expand on the existing literature by surveying and photographing these spurs in addition to exploring whether certain factors, inherent to the cadaver, are associated with spur presence. METHODS: Dissection to expose the aorta, inferior vena cava, and common iliac arteries and veins was performed in 51 cadavers. The spinal level at which the iliac vein confluence occurred was noted. The point at which the right common iliac artery crossed the left common iliac vein was examined for plaque presence. The overlying arterial structures were then transected to expose the venous system. The inferior vena cava was incised to facilitate observation into the mouth and full extent of the left common iliac vein. Spurs were photographed and documented. Statistical analysis was conducted to determine whether sex, body mass index (BMI), plaque presence, or level of the iliac vein confluence are associated with spur presence. RESULTS: Spurs within the left common iliac vein were observed in 16 of 51 cadavers (31.4%). All spurs were located at the point that the right common iliac artery crossed the left common iliac vein. Using1 the classification system established by McMurrich, 67% of spurs (n = 10) were marginal and triangular; 25% (n = 4) were columnar. One marginal, linear spur (6%) and one partially obstructed spur with multiple synechiae (6%) were observed. Among this population, males were 73% less likely to have a spur (odds ratio, 0.269; P = .041). No significant relationship was found between plaque presence and spur presence (odds ratio, 0.933; P = .824) and no significant differences were noted between BMI and spur presence (χ2 = 1.752, P = .625). Last, a significantly greater percent of spurs was found within cadavers with an iliac vein confluence located at the L5/S1 disc space (χ2 = 9.650; P = .002). CONCLUSIONS: Study findings show that spurs are more common when the confluence of the common iliac veins occurs at a lower spinal level. The level of the iliac vein confluence may be important in identifying patients at increased risk of venous disease. The findings also suggest that plaque within the right common iliac artery and BMI display no distinct relationship with spur presence. Further investigation is needed to understand exactly what factors lead to spur formation.

Interrupted inferior vena cava draining into the coronary sinus associated with circumflex artery to coronary sinus fistula.

In an adult patient, coronary artery fistula involving the circumflex artery (CX) connected to the coronary sinus caused aneurysm of the left main and CX associated with ectasia of the intermediate branch. The patient had posterolateral infarction with severe ischemic mitral regurgitation and moderate tricuspid regurgitation. A rare venous return anomaly was also present. The inferior vena cava, which was interrupted at the level of the liver, continued with the hemiazygos to drain into a persistent left superior vena cava, which in turn drained into the coronary sinus. Surgery included CX closure and mitral and tricuspid repair. The strategy had to be adapted to the anatomy. The fistula was dissected and snared for cardioplegia delivery, and venous return was achieved by cannulation of the superior vena cava and femoral vein. The procedure was uneventful, and 4 years later the patient is asymptomatic.

Conduta obstétrica em caso de hipoplasia materna da veia cava inferior / Obstetric management in case of the maternal inferior vena cava hypoplasia

A hipoplasia da veia cava inferior é uma patologia rara que integra o conjunto de anomalias do desenvolvimento da veia cava inferior. A sua incidência situa-se entre 0,3%-0,5% na população saudável e 5% nos adultos jovens sem fatores de risco para trombose venosa profunda, sendo considerada um importante fator de risco para o desenvolvimento de trombose dos membros inferiores. O principal objetivo deste trabalho é reportar a conduta obstétrica de um caso clínico de uma grávida diagnosticada com hipoplasia da veia cava inferior, prévia à gravidez. Trata-se de um caso clínico, de uma grávida, primigesta, com 37 anos, com hipoplasia da veia cava inferior e heterozigotia para o gene MTHFR677 diagnosticadas, na sequência de uma trombose venosa bilateral dos membros inferiores e do segmento infrarrenal da veia cava inferior. A gravidez foi seguida em consulta hospitalar na nossa instituição, tendo a grávida sido medicada com enoxaparina em dose profilática e ácido acetilsalicílico, com um período pré natal que decorreu sem intercorrências. Às 37 semanas e 6 dias de gestação, deu entrada no Serviço de Urgência de Obstetrícia por rotura prematura de membranas. Intraparto foram utilizadas meias de compressão pneumática intermitente, tendo o parto ocorrido às 38 semanas de gestação por via vaginal (parto eutócico), do qual nasceu um recém-nascido do sexo feminino, com 2620g e índice de Apgar 9/10/10. O presente caso clínico demonstra que em situações de hipoplasia da veia cava inferior com um seguimento obstétrico adequado é possível a realização de um parto vaginal, possibilitando um desfecho obstétrico favorável (AU).

Hypoplasia of the inferior vena cava is a rare condition that belongs to the group of developmental anomalies of the inferior vena cava. It has an incidence between 0.3% and 0.5% in the healthy population and 5% in young adults without risk factors for deep venous thrombosis, being considered an important risk factor for the development of lower limb thrombosis. This study aims to report the obstetric conduct of a clinical case of a pregnant woman diagnosed with hypoplasia of the inferior vena cava prior to pregnancy. This is a clinical case of a pregnant woman, primigravid 37 years old, with hypoplasia of the inferior vena cava and heterozygosity for MTHFR677, diagnosed following a bilateral venous thrombosis of the lower limbs and the infrarenal segment of the inferior vena cava. The pregnancy was followed up in our institution. The pregnant woman was medicated with a prophylatic dose of low molecular weight heparin and acetylsalicylic acid with an uneventful prenatal period. At 37 weeks and 6 days of gestation, she was admitted to the Obstetrics Emergency Service due to premature rupture of membranes. Intermittent pneumatic compression sockings were used intrapartum, and at 38 weeks of gestation, a female newborn was vaginally delivered (eutocic delivery) with 2620g and an Apgar score of 9/10/10. The present clinical case demonstrates that in situations of hypoplasia of the inferior vena cava with an adequate obstetric follow-up, it is possible to perform a vaginal delivery, enabling a favourable obstetric outcome (AU).

Laparoscopic para-aortic lymphadenectomy for metastatic colon cancer in a patient with left-sided inferior vena cava: a case report.

Transposition of inferior vena cava, or, left-sided inferior vena cava (LS-IVC) is a rare clinical entity, in which the inferior vena cava ascends along the left side of the abdominal aorta. Literature contains mainly clinical case reports. Although it is usually not associated with clinical symptomatology, this anomaly should be detected during preoperative planning to avoid iatrogenic injuries intraoperatively. We present a case of left-sided inferior vena cava encountered during laparoscopic lymphadenectomy in a 45-year-old man with previous laparoscopic hemicolectomy due to colon adenocarcinoma. Preoperative CT abdomen revealed the left-sided location of infrarenal IVC and laparoscopic trans-peritoneal aortic lymphadenectomy was decided. Intraoperatively, transposition of inferior vena cava was confirmed in accordance with the CT findings. Resection of lymph node block was conducted with no complications and with minimal blood loss. The postoperative course was uneventful, and the patient was discharged from the hospital the day following surgery. In conclusion, transposition of the inferior vena cava, although rare, constitutes an anatomical variant that should be identified preoperatively to decrease intraoperative risks. Several anatomical variants have been associated with left-sided inferior vena cava.

Rare case of concomitant juxtarenal aortic aneurysm and type 1 Inferior Vena Cava duplication: -intraoperative challenges to avoid catastrophe / Caso raro de aneurisma de aorta justarrenal e duplicação do tipo 1 concomitantes na veia cava inferior - desafios intraoperatórios para evitar catástrofes

Abstract Duplication of the inferior vena cava is a rare congenital anomaly, with an incidence of 0.2-3%. Despite being asymptomatic, anomalies of IVC are important in aortoiliac and retroperitoneal surgeries. Preoperative CT imaging is essential to identify any IVC anomaly and to prevent unexpected hemorrhage during surgery. Here, we report a case of a juxtarenal abdominal aortic aneurysm in which we encountered a type I IVC duplication anomaly intraoperatively while performing transperitoneal aneurysmorrhaphy and took precautions to avoid any iatrogenic injuries to either of the two trunks or the pre-aortic trunk of the anomalous duplicate IVC.

Resumo A duplicação da veia cava inferior (VCI) é uma anomalia congênita rara com incidência de 0,2 a 3%. Apesar de assintomáticas, anomalias da VCI são importantes em cirurgias aortoilíacas e retroperitoneais. A imagem da tomografia pré-operatória é essencial para identificar qualquer anomalia de VCI e para evitar hemorragia inesperada durante a cirurgia. Relatamos um caso de aneurisma de aorta abdominal justarrenal, no qual encontramos uma anomalia de duplicação de VCI do tipo 1 intraoperatório enquanto realizávamos correção cirúrgica de aneurisma transperitoneal. Por isso, tomamos a precaução para evitar qualquer lesão iatrogênica nos dois troncos e no tronco pré-aórtico de VCI duplicada anômala.

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.

Tratamento endovascular de síndrome congestiva venosa pélvica em paciente com duplicação de veia cava inferior e anatomia venosa pélvica incomum: revisão bibliográfica / Endovascular treatment of pelvic venous congestion syndrome in a patient with duplication of the inferior vena cava and unusual pelvic venous anatomy: literature review

Resumo A dor pélvica crônica afeta aproximadamente 1/3 de todas as mulheres e é responsável por cerca de 20% de todas as consultas ginecológicas. Os autores relatam um raro caso de congestão venosa pélvica sintomática na presença de duplicação de veia cava inferior e comunicação interilíaca através de veia hipogástrica direita tratado com abordagem endovascular, por embolização das veias varicosas pélvicas e revisão da literatura publicada.

Abstract Chronic pelvic pain affects approximately one-third of all women and is responsible for about 20% of all gynecological consultations. The authors report a rare case of symptomatic pelvic venous congestion in the presence of duplication of the inferior vena cava and inter-iliac communication through the right hypogastric vein that was treated via an endovascular approach with embolization of varicose pelvic veins. The published literature is reviewed.

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.

Ascitis fetal como forma de presentación de agenesia de la vena cava inferior / Fetal ascites as clinical presentation of inferior vena cava agenesis

La vena cava inferior (VCI) está constituida por tres segmentos de diferente origen embriológico. De su mala fusión, surge un amplio espectro de anomalías. La prevalencia de anomalías de la VCI es de 0,07-8,7% de la población. Generalmente, se diagnostica como hallazgo incidental en la vida adulta. Representa el 5-9,5% de las trombosis venosas profundas idiopáticas en menores de 30 años sin factores de riesgo asociados. Se presenta a una recién nacida a término con diagnóstico prenatal de ascitis en la semana 20 de gestación. Se diagnosticó, mediante angiotomografía abdominal, la agenesia de VCI. El tratamiento de pacientes con agenesia de la VCI se basa en el manejo de las complicaciones. Debido al mayor riesgo que presentan de sufrir un evento trombótico, se debe considerar la profilaxis antitrombótica a largo plazo. Se recomienda iniciar profilaxis anticoagulante en la pubertad.

Inferior Vena Cava (IVC) is composed of three segments from different embryological origin. Its lack of fusion originates a wide spectrum of anomalies of the IVC. These malformations are present in 0.07-8.7% of the population. It is generally diagnosed as an incidental finding in adult life. It represents between 5 and 9.5% of idiopathic deep vein thrombosis in patients younger than 30 years old without associated risk factors. We present a case of a term newborn with prenatal diagnosis of ascites during the 20th week of gestation. IVC Agenesis was diagnosed with the use of abdominal angiotomography. The treatment of patients with IVC Agenesis is based on the management of its complications. Due to the increased thrombotic risk of these patients, we should consider lifelong anticoagulation. We suggest initiating it during puberty.

Interrupcion de vena cava inferior: diagnostico prenatal de sus variantes / Inferior vena cava interruption: prenatal diagnosis of its variants

La anomalía más frecuente de la vena cava inferior es su interrupción. En estos casos, el drenaje al corazón se realizará por la vena ácigos y menos frecuentemente por la vena hemiácigos. Se considera un marcador ecográfico prenatal de isomerismos y/o cardiopatías por lo que su hallazgo obliga a descartarlos realizando una ecografía detallada del corazón y del situs fetal. Además, probablemente sea de las anomalías venosas que más implicaciones clínicas pueden tener en la edad adulta por su asociación a trombosis venosa profunda y es útil conocer la anatomía para posibles futuros procedimientos quirúrgicos o cateterismos cardiacos. Por lo tanto, es interesante realizar el diagnóstico prenatal, aunque se presente de forma aislada, siendo su diagnóstico ecográfico sencillo si somos sistemáticos en la práctica de la ecografía morfológica. El signo característico ecográfico es el de «doble vaso¼ o «doble burbuja¼ en un corte axial abdominal o torácico. Presentamos los esquemas ecográficos de diagnóstico de los dos tipos de drenaje principales de la interrupción de la vena cava inferior para poder realizar el correcto diagnóstico prenatal. Para ello tomamos de referencia dos casos clínicos que no presentan cardiopatías ni isomerismos asociados.

The most frequent anomaly of the inferior vena cava is its interruption. In these cases, drains into the heart by the azygos vein and less frequently by the hemiazygos vein. It is considered a prenatal ultrasound marker of isomerisms and/or heart disease, therefore, its finding requires to discard them by performing a detailed ultrasound of the fetal heart and situs. Probably it is one of the venous anomalies with more clinical implications in adulthood due to their association with deep venous thrombosis, and it is useful to know the anatomy for possible future surgical or cardiac catheterization procedures. Therefore, it is interesting to perform the prenatal diagnosis, although isolated. In this sense, the ultrasound diagnosis of the interruption is simple if we are systematic in the practice of morphological ultrasound. The characteristic ultrasound sign of the aforementioned interruption is the "double vessel" or "double bubble" in an axial abdominal or thoracic section. We present the ultrasound diagnosis diagrams of the two main types of drainage of the inferior vena cava interruption in order to perform the correct prenatal diagnosis. For these purposes, we refer two cases with no heart diseases or isomerisms associated.

Double inferior vena cava detected by CT venography and confirmed by magnetic resonance venography: embryogenesis and literature review / Doble vena cava inferior detectada a través de venografía por tomografía computada y confirmada con venografía por resonancia magnética: embriogénesis y revisión de la literatura

A hospital based prospective study was conducted from July 2001 to July 2015 at the Department of Radiology, Jordan University Hospital, Amman, Jordan. During that period, five cases of double inferior vena cava (DIVC) were discovered among a cohort of 7722 patients (3861 men and 3861 women, 49.5±16.9 years, range 16­78 years). Cases were diagnosed by contrast-enhanced Spiral CT venography (CTV) and confirmed by turbo three-dimensional (3D) time-of-flight contrast-enhanced MR venography. The majority of patients 3166 (41 %) were referred for staging and follow-up of malignancy, postoperative complications 1777 (23 %), non-specific abdominal pain 1467 (19 %), preoperative assessment 849 (11 %) and trauma 463 (6 %). Magnetic resonance venography showed higher sensitivity, diagnostic accuracy and noninvasive modality for assessment of IVC map. MRV is a more useful, noninvasive modality for assessment of IVC map. DIVC is a common anomaly, its incidence in our study found to be 0.064 %. The incidence, literature review, embryogenesis, and importance of this anomaly are discussed. In addition, sample figures of relevant cases are provided.

En el Departamento de Radiología del Hospital de la Universidad de Jordania, Amman, Jordania, se llevó a cabo un estudio prospectivo entre el mes de julio de 2001 al mes de julio del 2015. Durante ese período se descubrieron cinco casos de vena cava inferior doble (VCID) en una cohorte de 7722 pacientes (3861 hombres y mujeres 3861, de 49,5 ± 16,9 años, con un rango de edad de 16-78 años). Los casos fueron diagnosticados por medio de venografía por tomografía computada espiral con contraste (TCV) y confirmados por medio de venografía por estudio tridimensional turbo. La mayoría de los pacientes (3166, 41 %) fueron remitidos para estadificación y seguimiento de tumores malignos. Se presentaron complicaciones postoperatorias en 1777 pacientes (23 %), dolor abdominal no especificado en 1467 (19 %), evaluación preoperatoria en 849 (11 %) y traumatismo en 463 pacientes (6 %). La venografía por resonancia magnética (RMV) mostró una mayor sensibilidad, precisión diagnóstica, y resultando no invasiva para la evaluación de la vena cava inferior (VCI). RMV es una modalidad más útil, no invasiva para la evaluación de la VCI. VCID es una anomalía frecuente, encontrándose en nuestro estudio una incidencia de 0,064 %. Además se realizó una revisión de la literatura, la embriogénesis, y la importancia de esta anomalía. También, se proporcionaron cifras de muestras de los casos relevantes.

Ausencia congénita de la vena porta hepática asociada a malrotación intestinal y a vena cava inferior izquierda: reporte de caso / Congenital absence of the hepatic portal vein associated to intestinal malrotation and left-sided inferior vena cava: a case report

La ausencia congénita de la vena porta hepática es una malformación en extremo rara, fue descrita por primera vez en 1793 por John Abernethy y a la fecha se han reportado sólo 101 casos. Afecta con mayor frecuencia a mujeres y determina que el drenaje venoso intestinal sea derivado hacia el territorio de las venas sistémicas. Es también conocida como derivación porto-sistémica extra hepática congénita (CEPS), por su sigla en inglés, y se asocia a otras alteraciones congénitas,incluyendo anomalías cardíacas, de las vías biliares, enfermedades metabólicas y retardo mental. En este trabajo presentamos el hallazgo de esta malformación en el cadáver de un niño de dos años de edad, donde la vena porta seguía un trayecto anómalo y se unía a la vena renal derecha. El confluente venoso "mesentérico-esplénico-renal" así formado presentaba un trayecto descendente, recibía a la vena gonadal derecha, y desembocando en la confluencia de las venas ilíacas comunes. Esto se asociaba a mal rotación intestinal, arteria hepática aberrante y a vena cava inferior izquierda, situación descrita sólo una vez en la literatura. El hallazgo de estas anomalías anatómicas en los cadáveres disecados con fines docentes en nuestro Departamento de Anatomía, tiene un valor formativo indiscutible para nuestros alumnos de pre y postgrado, quienes pueden valorar las implicancias de este conocimiento anatómico en la clínica diaria.

Congenital absence of the hepatic portal vein is an extremely rare malformation that was first described by John Abernethy in 1793. Only 101 cases had been described in the literature until 2015 and most affected females. In this anomaly, also known as congenital extrahepatic porto-systemic shunt (CEPS), intestinal venous drainage is derived towards the territory of the systemic veins and could be associated with other birth defects, including heart and biliary tract anomalies, metabolic diseases, mental retardation. We present the case of a 2-year-old boy who died because of multifocal pneumonia and necropsy showed anatomical findings consistent with this malformation as an incidental finding. The portal vein followed an anomalous course and joined the right renal vein, forming the "mesenteric-splenic-renal" venous collector, which then received the right gonadal vein and ended at the confluence of the common iliac veins. In our case this anomaly was associated to intestinal malrotation, aberrant hepatic artery and persistent left inferior vena cava, situation described once in the literature. The finding of these anatomical abnormalities in cadavers has a great teaching value for our undergraduate and graduate students who are learning anatomy and they can also assess the associated clinical.

Síndrome da cimitarra: A propósito de três casos singulares / Scimitar syndrome: the purpose of three individual cases

Os autores relatam três casos de síndrome da cimitarra (SC), que em sua forma habitual cursa com drenagem venosa anômala no pulmão direito, de aspecto radiográfico característico, hipoplasia no referido pulmão e dextroposição cardíaca. Enfatizam sobre a radiografia simples do tórax, como principal exame na investigação diagnóstica, e o valor do estudo angiográfico, na elucidação de anomalias vasculares quando houver indicação cirúrgica. Outros exames complementares, como a tomografia computadorizada, são avaliados para a perfeita caracterização dessa síndrome e de suas variantes...

The authors write about three cases of scimitar syndrome which, commonly, releases as an atypical venous drainage of the right lung, with characteristic image, as well as hypoplasia of the same lung and dextropositioned heart. They emphasize the thoracic radiographic image as the most important complementary method of diagnosis and the importance of the angiographic study for the diagnose of vascular abnormalities with surgical indication. Other exams such as computerized tomography are of value in characterization of syndrome and its variants...

Trombosis parcial de la vena cava inferior / Partial thrombosis of the inferior vena cava

La patología que suele afectar más frecuentemente a la vena cava inferior es la trombosis. Numerosas neoplasias han sido asociadas con trombosis de la vena cava inferior. Algunos tumores liberan sustancias protrombóticas, aumentando el riesgo de tromboembolia.

Ressonância magnética cardiovascular em veia cava inferior interrompida não prevista / Cardiovascular magnetic resonance in unsuspected interrupted inferior vena cava / Cardiovascular magnetic resonance in unsuspected interrupted inferior vena cava

A Veia Cava Inferior (VCI) interrompida é uma anomalia rara. As anomalias da VCI são clinicamente importantes para os cardiologistas e radiologistas que pretendem intervir na cavidade cardíaca direita. Descrevemos três casos de interrupção da VCI diagnosticados por meio de estudo imaginológico de ressonância magnética cardíaca.

Interrupted inferior vena cava (IVC) is a rare anomaly. Anomalies of IVC are clinically important for cardiologists and radiologists who plan to intervene in the right heart. We describe three cases of IVC interruption diagnosed by cardiac magnetic resonance imaging study.

La Vena Cava Inferior (VCI), interrumpida es una anomalía rara. Las anomalías de la VCI son clínicamente importantes para los cardiólogos y radiólogos que pretenden intervenir en la cavidad cardíaca derecha. Aquí describimos tres casos de interrupción de la VCI diagnosticados por medio de un estudio imaginológico de resonancia magnética cardíaca.

Valva de Eustáquio proeminente ao ecocardiograma transesofágico: implicações clínicas e cirúrgicas do diagnóstico morfológico / Transesophageal view of a prominent Eustachian valve: clinical and surgical implications of the morphological diagnosis

The Eustachian valve (EV) is an embryonic remnant of the valve of the inferior vena cava (IVC). In some cases, the EV can be prominent and presenting as a thin, elongated and ecodense membrane, superimposed to the septal aspect of the RA, below the fossaovalis, partially dividing the RA in two chambers. Echocardiography is the method of choice for the diagnosis of this finding. The correct identification of this anatomic variant is very important for the differential diagnosis of the masses and tumors in the RA. Inpatients referred for cardiac surgery, as in the closure of atrial septal defects (ASD), it is fundamental the identification of this structureto avoid complications as the inadvertent closure of an EV considered as part of the atrial septum. The finding of a redundant EV can also add technical difficulties for the percutaneous treatment of ASD. The correct recognition and detailed description of this finding are important to avoid equivocal diagnosis and complications in patients referred for cardiac surgery. Thus, attention to this anatomicvariant, and careful TEE examination are fundamental for the correct diagnosis and therapeutic approach.