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SUMMARY: Situs ambiguous is the placement of vessels and organs in the thoracoabdominal space that are anatomically located outside its normal position in a certain order. This condition is a broad definition that includes many variations. In this case report, we reported a patient with Situs ambiguus with an abnormal hepatic vein who was diagnosed incidentally during medical imaging (computed tomography, sonography and MRI study).

RESUMEN: El Situs ambiguous es la colocación de vasos y órganos en el espacio toracoabdominal que anatómicamente se encuentran fuera de su posición normal en un cierto orden. Esta condición es una definición amplia que incluye muchas variaciones. En este reporte de caso, reportamos un paciente con Situs ambiguous con una vena hepática anormal que fue diagnosticado incidentalmente durante un estudio de imagen médica (tomografía computarizada, ecografía y resonancia magnética).

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The incidence of detection of accessory hepatic vein (AHV) using MRI or CT has been reported. However, previous studies had a small sample size or only reported on the incidence of hepatic vein variants. To the best of our knowledge, there has been no previous report evaluating the factors predictive of the presence of an AHV. To evaluate the incidence and morphology of the accessory hepatic vein (AHV) using multidetector row computed tomography (MDCT) and to investigate the factors which may be helpful in predicting the presence of an AHV. We enrolled 360 patients who underwent abdominal MDCT. We investigated whether the AHV was present and evaluated the frequency of AHVs greater than 5 mm in diameter. We classified the morphology of the AHV entering the inferior vena cava (IVC). We also examined the factors that predicted the presence of an AHV by comparing the diameter of the middle hepatic vein (MHV) and the right hepatic vein (RHV). We identified an AHV in 164 of the 360 patients (45.6 %). Among the 164 AHVs, 56.7 % were larger than 5 mm in diameter. The most common morphologies of the inferior RHV were a single main trunk (58.5 %), followed by two main trunks with a V-shape (19.5 %) and two trunks entering the IVC separately (17.0 %). The possibility that an AHV will be present was significantly higher when the diameter of the RHV was smaller than that of the MHV. MDCT can provide important information regarding AHV incidence and morphology. The possibility of an AHV being present was significantly higher when the diameter of the RHV was smaller than that of the MHV.

Se ha informado de la incidencia de la detección de la vena hepática accesoria (VHA) mediante RM o TC. Sin embargo, estudios previos tenían un tamaño muestral pequeño o solo informaban sobre la incidencia de variantes de las venas hepáticas. Hasta donde sabemos, no ha habido ningún informe previo que evalúe los factores predictivos de la presencia de una VHA. El objetivo del estudio fue evaluar la incidencia y morfología de la vena hepática accesoria (VHA) mediante tomografía computarizada multidetector (TCMD) e investigar los factores que pueden ser útiles para predecir la presencia de un VHA. Se evaluaron 360 pacientes que se sometieron a TCMD abdominal. Se investigó si la VHA estaba presente y se evaluó la frecuencia de VHA mayores de 5 mm de diámetro. Se clasificó la morfología del VHA que drenaba en la vena cava inferior (VCI). Además, se examinaron los factores que predijeron la presencia de una VHA mediante la comparación del diámetro de la vena hepática media (VHM) y la vena hepática derecha (VHD). Se identificó un VHA en 164 de los 360 pacientes (45,6%). Entre las 164 VHA, el 56,7% tenía más de 5 mm de diámetro. Las morfologías más frecuentes del VHD inferior fueron un tronco principal único (58,5%), seguido por dos troncos principales con forma de V (19,5%) y dos troncos que drenaban en la VCI por separado (17,0%). La posibilidad de que una VHA esté presente fue significativamente mayor cuando el diámetro de la VHD era menor que la de la VHM. La MDCT puede proporcionar información importante sobre la incidencia de la VHA y su morfología. La posibilidad de que un VHA estuviera presente era significativamente mayor cuando el diámetro del VHD era menor que la VHM.

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BACKGROUND: Bisegmentectomy 7-8 is feasible even in the absence of a large inferior right hepatic vein. To our knowledge, this operation has never been performed by laparoscopy. This study was designed to present video of pure laparoscopic bisegmentectomy 7-8 and bisegmentectomy 2-3 in one-stage operation for bilateral liver metastasis. METHODS: A 67-year-old man with metachronous bilobar colorectal liver metastasis was referred for surgical treatment after neoadjuvant chemotherapy. CT scan disclosed two liver metastases: one located between segments 7 and 8 and another one in segment 2. At liver examination, another metastasis was found on segment 3. We decided to perform a bisegmentectomy 7-8 along with bisegmentectomy 2-3 in a single procedure. The operation began with mobilization of the right liver with complete dissection of retrohepatic vena cava. Inferior right hepatic vein was absent. Right hepatic vein was dissected and encircled. Upper part of right liver, containing segment 7 and 8, was marked with cautery. Selective hemi-Pringle maneuver was performed and right hepatic vein was divided with stapler. At this point, liver rotation to the left allowed direct view and access to the superior aspect of the right liver. Liver transection was accomplished with harmonic scalpel and endoscopic stapling device. Bisegmentectomy 2-3 was performed using the intrahepatic Glissonian approach. The specimens were extracted through a suprapubic incision. Liver raw surfaces were reviewed for bleeding and bile leaks. RESULTS: Operative time was 240 minutes with no need for transfusion. Recovery was uneventful. Patient was discharged on the fifth postoperative day. Patient is well with no evidence of disease 14 months after liver resection. Tumor markers are within normal range. CONCLUSIONS: Bisegmentectomy 7-8 may increase resectability rate in patients with bilateral lesions. This operation can be performed safely by laparoscopy. Preservation of segments 5 and 6 permitted simultaneous resection of segments 2 and 3 with adequate liver remnant.

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Two patients with symptomatic congenital portal vein (PV) fistulas (one case of PV-right hepatic vein fistula and one case of PV-inferior vena cava fistula) causing hepatopulmonary syndrome (hepatic dysfunction, lung vascular alteration in the form of arteriovenous shunts, and hypoxemia) were successfully treated with the Amplatzer patent ductus arteriosus (PDA) occlusion device. This device was chosen in light of the anatomic characteristics of the vascular malformations and the specific properties of the prosthesis, especially those related to relocation and retrievability when not properly positioned. Embolization with an Amplatzer PDA occlusion device should be considered as a useful therapeutic alternative in the treatment of congenital portal fistulas that can obviate complex surgeries.

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OBJECTIVE: Infantile hemangioma and arteriovenous malformation (AVM) of the liver have a similar presentation but a different natural history, and therefore require different treatment. This study was undertaken to clarify differential diagnosis and management of these two biologically distinct vascular disorders. STUDY DESIGN: We retrospectively analyzed the records of 43 children with hepatic vascular anomalies treated during the past 27 years. RESULTS: Ninety percent were hemangiomas (n = 39); 10% were AVM (n = 4). Infants with AVM or large solitary hemangioma had hepatomegaly, congestive heart failure, and anemia as presenting symptoms at birth. Multiple hepatic hemangiomas manifested at 1 to 16 weeks of age with the same clinical triad, plus multiple cutaneous lesions (19/23). The mortality rate after treatment of hepatic AVM was 50% (2/4). The mortality rates after treatment of liver hemangiomas were as follows: resection of solitary lesions, 20% (2/10); embolization, 43% (3/7); corticosteroids, 30% (3/10); and interferon alfa-2a, 15% (2/13). CONCLUSION: Solitary hepatic hemangioma cannot always be distinguished from hepatic AVM without radiologic studies. Multiple hepatic hemangiomas are differentiated from hepatic AVM by coexistence of multiple cutaneous hemangioma and by radiologic imaging. We recommend combined embolization and surgical resection for hepatic AVM and for solitary symptomatic hemangioma, if drug therapy fails. Pharmacologic treatment is used for symptomatic multiple liver hemangiomas. Embolization allows interim control of heart failure. A decreased mortality rate after interferon alfa-2a therapy is encouraging.

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Los autores revisaron 92 expedientes de 92 niños con conexión anómala total de venas pulmonares (CATVP). Los pacientes se diagnosticaron invariablemente con estudio hemodinámico. Se dividieron en cinco grupos: a)CATVP supracardiaca, a la vena cava superior, 43 casos. 2) Intracardiaca, 37 de los cuales 28 al seno coronario y nueve a la aurícula derecha. 3) Infracardiaca, a las venas suprahepáticas, cuatro casos. 4) Mixta, a la vena cava superior y al seno coronario, cuatro casos. 5) Malformaciones cardiacas complejas con CATVP acompañante: tres a la vena capa superior y una al seno coronario. Se discuten los datos clínicos, radiológicos y hemodinámicos y se señala que en un alto porcentaje de los casos al cuadro clínico y la silueta cardiaca permiten hacer el diagnóstico presuntivo sin estudios cruentos. La mortalidad quirúrgica general se relaciona con la gravedad del enfermo, lo que a su vez se expresa por insuficiencia cardiaca resistente a tratamiento, a edades tempranas, en pacientes que en esta serie tenían menos de cuatro meses de edad. La gravedad depende en gran medida, de la pequeñez de la comunicación interauricular invariablemente presente; si es pequeña o "restritiva", la situación es grave. Los pacientes que en esta serie alcanzaron nueve a 13 años de edad tuvieron grandes defectos auriculares y esto explica la supervivencia. Si bién es deseable la cirugía temprana para corrección completa, tiene alto riesgo pero no tanto como el tratamiento conservador, el cual puede causar muertes repentinas. Siempre debe intentarse septostomía con globo en el momento de lograr el diagnóstico con cateterismo. Esta septostomía sin duda es benéfica y muchas veces permite que se realice la cirugía correctora tiempo después, sin embargo, el beneficio de la septostomía a menudo es temporal.El tratamiento médico de la insuficiencia cardiaca siempre se emplea pero también es una medida paliativa temporal que debe ir seguida de cirugía correctora

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