Ventral Portal Vein Over Gallbladder and Pancreas---An Exceptional Anatomical Variation Found in a Liver Transplant Patient: A Case Report.

BACKGROUND: Anatomic variations are well known in the liver hilum. A rare precholecystic, preduodenal, prepancreatic portal vein is described as found in a liver transplant candidate. Precholecystic location of portal vein is an exceptional finding and does not seem to have been previously described. It is associated with a preduodenal portal vein. Its position is challenging, as its surface can be mistaken with the gallbladder wall. We present the case of a patient candidate to liver transplantation. In the preoperative studies, a portal thrombosis was suspected, with recanalization by collaterals, but also a malformation was suggested. The patient had a primary biliary cirrhosis. Other findings included agenesis of inferior vena cava on the right side. During operation, the portal vein was found over the gallbladder and fixed to it, making it at first difficult to distinguish one from the other. CONCLUSIONS: A precholecystic portal vein is a rare finding that poses a challenge for the surgeon. It must be ruled out in the preoperative workout.

Neonatal cerebral sinovenous thrombosis.

Cerebral sinovenous thrombosis (CSVT) is a focal or diffuse disruption of cerebral blood flow secondary to occlusion of cerebral veins and/or sinuses. The challenge of CSVT during the neonatal age has led to a great interest in this condition among neonatologists, child neurologists, and pediatric neuroradiologists. The highly variable clinical spectra, etiologies, and prognosis require fine medical skills and a high level of suspicion. Nevertheless, the diagnosis is often delayed or missed altogether. Differences in brain vulnerability at different stages of maturation may explain the spectrum of associated brain lesions, which varies with gestational age. Treatment is controversial and reported clinical outcomes vary widely. The controversial treatment of CSVT with anticoagulant therapy is based only on case series and expert consensus, there is lack of safety data.

Absent Inferior Vena Cava Leading to Recurrent Lower Extremity Deep Vein Thrombosis in a United States Marine.

Introduction: Anomalies of the inferior vena cava (AIVC) are rare but well-recognized anatomic abnormalities that can lead to clinically significant deep vein thrombosis (DVT) in a subset of otherwise healthy patients. This report illustrates an uncommon congenital anomaly that military clinicians should consider when evaluating unprovoked DVT in young patients. Materials and Methods: Single case report and literature review. Results: We describe a case of a 24-yr-old United States Marine who presented with abdominal pain for 2 wk. After conservative therapy failed, a contrast-enhanced abdominal computed tomography (CT) scan was performed. The CT scan revealed an absent inferior vena cava with evidence of right venous thrombophlebitis. We include four contrast-enhanced helical CT scans that illustrate this phenomenon. Conclusion: Due to the lack of available studies and data, we do not know the relative risk of DVT in patients with AIVC. However, the literature review suggests that there is a pro-thrombogenic effect of this congenital anomaly. Clinicians should include AIVC in their differential when treating young, otherwise healthy patients with unprovoked DVT. This population is much more likely to have an AIVC than the general population. In addition to thrombophilia markers, a contrast-enhanced CT scan should be considered as part of the initial workup.

Aneurysmal dilatation of persistent vitelline vein with thrombus in a neonate.

The paired vitelline veins selectively involute and form a part of the portal vein during embryonic development. The presence of a persistent vitelline vein segment after birth is very rare and can be confused with anomalies of the portal and umbilical veins. We present sonographic, CT and MRI findings of aneurysmal dilatation of a persistent vitelline vein with thrombus in a neonate; this case was first misdiagnosed as an umbilical vein varix by prenatal US. MRI was used to identify the persistent vitelline vein segment and the remnant umbilical vein segment.

Congenital extensive central venous thrombosis with chylous ascites and chylothoraces.

We describe a case of congenital extensive central venous thrombosis presenting as polyhydramnios and massive ascites, requiring amnioreduction prenatally and refractory chylous ascites and chylothoraces postnatally. Echocardiography, computed tomography angiogram (CTA), and magnetic resonance venogram (MRV) were helpful in defining the nature and extent of the lesion. The patient underwent staged procedures of repeated abdominal paracentesis, chest drain insertion, and right internal jugular vein exploration initially. Subsequently, open thromboembolectomy from the upper venous system veins and pericardial patch angioplasty of the right internal jugular and right innominate veins were required and managed by catheter-directed thrombolysis for the residual thrombosis with successful recovery.

Evolution of congenital malformations of the umbilical-portal-hepatic venous system.

OBJECTIVE: The objective of this study is to describe the evolution of 8 cases of congenital malformations of the umbilical-portal-hepatic venous system diagnosed before the first month of life. MATERIALS AND METHODS: All cases of congenital malformation of the portal and hepatic venous system diagnosed prenatally or during the first month of life in our institution were systematically reviewed since November 2000. Clinical features, imaging, and anatomical findings were reviewed, focusing primarily on clinical and radiologic evolution. RESULTS: Eight cases of congenital malformation of the umbilical-portal-hepatic venous system were studied. Fifty percent of these malformations were diagnosed prenatally. We report 4 portosystemic shunts. Three involuted spontaneously, and the fourth one required surgical treatment. We report a variation of the usual anatomy of portal and hepatic veins that remained asymptomatic, an aneurysmal dilatation of a vitelline vein causing portal vein thrombosis that needed prompt surgical treatment with good result, a complex portal and hepatic venous malformation treated operatively, and a persistent right umbilical vein that remained asymptomatic. CONCLUSION: Prenatal diagnosis of malformations of the umbilical-portal-hepatic venous network is uncommon. Little is known about the postnatal prognosis. Clinical, biologic, and radiologic follow-up by ultrasonography is essential to distinguish pathologic situations from normal anatomical variants.

Ductus arteriosus aneurysm with massive thrombosis of pulmonary artery and fetal hydrops.

Ductus arteriosus aneurysm (DAA) is a rare cardiovascular lesion usually diagnosed within the first 2 months of life, or less frequently in the 3rd trimester, by antenatal sonography. The true in utero incidence of DAA is unknown, as most affected fetuses are asymptomatic at birth. Potential complications include thromboembolism, rupture, and death. We report a unique lethal case of a large DAA detected by mid-2nd trimester fetal echocardiography, complicated by stricture and massive occlusive thrombosis extending into the pulmonary artery branches. Stricture and thrombosis of the DAA led to interruption of fetal circulation, cardiac failure, and fetal hydrops, ultimately resulting in fetal demise.

Tratamiento de la trombosis venosa profunda asociada a agenesia de vena cava inferior. Revisión de las anomalías de vena cava inferior / Treatment of deep venous thrombosis associated with inferior vena cava agenesis. A review of inferior vena cava variants

La complejidad de la embriología de la vena cava inferior, con numerosas anastomosis entre pares de venas, puede conducir a multitud de variantes anatómicas. En la población general la agenesia de la vena cava inferior constituye una malformación poco frecuente (0,07%), pero su presencia representa el 5-9,5% de las trombosis venosas profundas idiopáticas en pacientes menores de 30 años y su tratamiento continúa siendo un tema controvertido(AU)

The development of the inferior vena cava is a complex embryological process, with numerous anastomoses among pairs of veins, which may result in many anatomic variants. In the general population, the absence of inferior vena cava is a rare anomaly (0.07%), but this represents 5-9.5% of idiopathic deep venous thrombosis in patients under 30 years-old and its treatment options remain (AU)

Bilateral renal venous thrombosis and adrenal hemorrhage: sequential prenatal US with postnatal recovery.

A neonate presented with bilateral renal venous thrombosis (RVT) and adrenal hemorrhage. Serial fetal ultrasonography showed progressive development of severe atrophy of one kidney and mild atrophy of the other. At sequential postnatal imaging, both kidneys showed progressive growth. Bilateral fetal RVT is quite uncommon, and postnatal recovery of a kidney severely affected by RVT is very rare.

Prenatal demonstration of afferent vessels and progressive thrombosis in a torcular malformation.

OBJECTIVES: To elucidate a part of the prenatal natural history of dural sinus malformation of the posterior fossa. METHODS: Ultrasound and magnetic resonance imaging were performed from 31 to 32 weeks' gestation. RESULTS: We observed the progressive development of a thrombus that was visible as an expanding hyperechoic round area within a cystic mass of the posterior fossa. It was characterized, as expected for a vascular malformation, by the presence of blood flow into the aneurismal cavity. Color doppler identified superior sagittal and straight sinuses, and distinguished that their flow continued into the dilated torcular. Prenatal magnetic resonance imaging confirmed an arteriovenous malformation involving the dural sinus. CONCLUSION: The vascular malformation had a fixed volume and preceded the thrombosis, which formed within several days. The present case is the first report with all the prenatal sonographic features of this condition.

Congenital prothrombotic disorders in children with peripheral venous and arterial thromboses.

AIMS: To evaluate the prevalence of congenital prothrombotic disorders in children with peripheral venous and arterial thromboses. METHODS: Deficiencies in antithrombin (AT), proteins C (PC) and S (PS), and increased lipoprotein (a), and the presence of factor V (FV) G1691A, prothrombin G20210A and methylenetetrahydrofolate reductase (MTHFR) mutations were investigated. RESULTS: Forty-eight patients (mean age, 3.4 years) were investigated. Of these patients, 23 had venous thrombosis, 22 had arterial thrombosis, and 3 had both. No patients had AT, PC or PS deficiency. FV G1691A mutation was present in 2 (7.6%) and 3 (12%) patients with venous and arterial thromboses, respectively. The prothrombin G20210A mutation was present in 1 (4%) patient with arterial thrombosis. Homozygous MTHFR C677T mutation was detected in 4 (18%) and 2 (9%) patients with venous and arterial thromboses, respectively. Increased lipoprotein (a) was present in 2 (10%) and 1 (4.5%) patients with venous and arterial thromboses, respectively. Regarding acquired risk factors, 79% of all thrombotic events were related to catheter usage. An underlying disease was present in 96% of the patients. CONCLUSIONS: Compared to acquired risk factors, congenital prothrombotic disorders are rarely present in children with peripheral venous and arterial thromboses. These results do not support general screening of children with venous and arterial thromboses for congenital prothrombotic disorders.

[A case report of posttraumatic deep vein thrombosis of the lower limbs in a patient with factor V Leiden mutation and the congenital absence of the inferior vena cava]. / Opis przypadku pourazowej zakrzepicy zyl glebokich konczyn dolnych u chorego obciazonego mutacja czynnika V typu Leiden oraz wrodzonym brakiem zyly glównej dolnej.

The congenital absence of the inferior vena cava (AIVC) is a rare vessels' malformation which may predispose to the development of thrombosis. Although AIVC is very rare, its occurrence should be considered in young patients, under 40 years old, with deep vein thrombosis (DVT). We are describing a case of a young male with bilateral deep vein thrombosis, in whom we defined three risk factors for DVT--trauma, factor V Leiden and the absence of the inferior vena cava. It is worth to consider the occurrence of malformation of the inferior vena cava in the young patients with deep vein thrombosis even when the other obvious risk factors are present (trauma, factor V Leiden mutation). The clinical state and the diagnostic process are discussed.

Infantile spasms as an adverse outcome of neonatal cortical sinovenous thrombosis.

Cerebral sinovenous thrombosis is a rare but potentially serious condition often occurring in children with nonspecific presenting features. Much remains to be learned about the long-term outcome of infants with cerebral sinovenous thrombosis. We report a series of four patients taken from a prospective database of neonates with sinovenous thrombosis who subsequently developed infantile spasms, three with hypsarrythmia on electroencephalography and one with multiple independent spike foci. The first patient presented at 2 weeks of age with hypernatremia, dehydration, and seizures. He was found to have extensive thrombosis and hemorrhagic infarction of the right basal ganglia. The second patient presented at 5 weeks of life and was found to have sagittal sinus thrombosis with bilateral intracranial hemorrhage. The third patient presented with seizures on day 1 of life and was found to have venous thrombosis involving the torcular, extending into the sagittal sinus. The fourth patient presented at 3 weeks with lethargy and seizures. He was diagnosed with bacterial meningitis and also had extensive sinus thrombosis. All patients developed infantile spasms at ages 9, 7, 11, and 10 months, respectively. This is the first report in the English literature describing infantile spasms as a possible outcome of sinovenous thrombosis in early infancy.

Percutaneous hydrodynamic thrombectomy for congenital deep vein thrombosis in a neonate.

A 1-day-old boy with a complete occlusive inferior vena cava and bilateral renal vein thrombus removed successfully using a hydrodynamic thrombectomy catheter is reported. Although blood flow to the inferior vena cava and bilateral renal veins was restored with no distal embolism or vascular injury, he died of bleeding complications due to fibrinolytic therapy after hydrodynamic thrombectomy. To the best of our knowledge, this is the first report of hydrodynamic thrombectomy of a neonate.

[Venous thrombosis and thrombophilic management of prevention and therapy]. / Venöse thrombose und thrombophilie management von prophylaxe und therapie.

Cardiovascular disease, to which venous thrombosis and pulmonary embolism contribute a major part, are the leading cause of death in industrialized countries. Thrombosis is caused by temporary risk factors or by thrombophilia. Increasing knowledge concerning the pathophysiology of thrombosis and new diagnostic tools allow a thrombotic risk assessment. In this paper thrombotic risk factors will be discussed with respect to their significance and diagnosis as well as methods of prophylaxis and treatment for patients at risk for or with diagnosed thrombosis. Furthermore, currently available as well as new anticoagulants (pentasaccharide, melagatran) will be discussed and their potential for patient-oriented and cost-efficient prophylaxis and treatment of thromboembolic complications outlined.