Malformation Of Inferior Vena Cava Research Articles

Agenesis of the inferior vena cava associated with nephropathy, hypertension, and congenital organ anomalies. Case report of a 12-year-old girl

Inferior vena cava agenesis is a rare congenital, often asymptomatic vascular anomaly, usually detected accidentally during medical imaging or interventions. In the literature, an association between inferior vena cava malformations and congenital defects of other organs (anomalies of the heart, spleen, kidneys, bowels) has been reported. This results from the concurrent development of these organs in the embryological process. We describe the agenesis of the lower part of the inferior vena cava coexisting with nephropathy, hypertension, unicornuate uterus, lipoma of the lumbar spine, spina bifida, increased activity of factor VIII and von Willebrand factor in a 12-year-old girl. This combination of defects appears to be unique in the medical literature. Exploring and understanding the nature of the link between these conditions could be crucial for avoiding unnecessary surgical interventions, malpractices, and diagnostic errors.

Special Deep Vein Thrombosis in a Child: A Case Report

Background: Agenesis of the inferior vena cava (IVC) is an extremely rare congenital malformation. Although frequently symptomatic, due to its low prevalence, agenesis of IVC may often be missed in the normal workup. Most of the reports are related to the loss of IVC alone, while the reports of simultaneous loss of IVC and bilateral iliac veins are rare. Acute deep venous thrombosis is also one of the causes of chronic deep venous ulcer, especially in children with acute deep venous thrombosis, which is an important cause of neglect and missed diagnosis. When color Doppler ultrasound cannot be clear, attention must be paid to computerized tomography examination. Case Presentation: We reported a case of IVC below renal vein hypoplasia in an 8-year-old girl who presented bilaterally with persistent pain in both lower limbs. Ultrasonography revealed no clear IVC and iliofemoral venous system under the renal venous plane. Conclusion: This case is a rare congenital malformation of IVC. It is rare in children with venous thrombosis-related symptoms. We intended to demonstrate the etiology of the appearance of venous thrombosis in children through this case.

RARE CASE OF MASSIVE DVT AFTER MRNA COVID-19 VACCINATION IN A PATIENT WITH CONGENITAL IVC ANOMALY

RARE CASE OF MASSIVE DVT AFTER MRNA COVID-19 VACCINATION IN A PATIENT WITH CONGENITAL IVC ANOMALY

Prenatal diagnosis of fetal inferior vena cava malformation using HDlive flow combined with spatiotemporal image correlation.

This study aimed to examine the application value of high-definition live (HDlive) flow combined with spatiotemporal image correlation (STIC) in the diagnosis of fetal inferior vena cava malformation (IVCM). Twenty cases of IVCMs were diagnosed using two-dimensional HDlive flow and HDlive flow combined with STIC and retrospectively analyzed to examine the impact of using HDlive flow combined with STIC in the diagnosis of IVCM. HDlive flow combined with STIC detected one case of duplicated IVC, four cases of left IVC (two cases with complex malformations), and 15 cases of interrupted IVC (two cases of isolated IVC disconnection, five cases with left atrial heterogeneous syndrome, and eight cases with other complex malformations). HDlive flow combined with STIC can help in the diagnosis of IVCM, and this technique has important clinical value.

Inferior Vena Cava Agenesis-Associated Thrombosis in a Neonate

Background: Inferior vena cava (IVC) malformations are rare developmental anomalies and are usually asymptomatic. Agenesis of IVC is an uncommon form of IVC malformation, and symptomatic neonatal presentation is unusual. We describe an unusual case of symptomatic venous thrombosis in the neonatal period after central venous cannulation, which was later found to have agenesis of IVC. Clinical Description: An extreme preterm (28 weeks) baby developed deep vein thrombosis of the femoral and external iliac vein after femoral venous catheterization and required anticoagulation therapy. Later, during the hospital stay, he was noted to have developed dilated abdominal veins over the flanks and paraspinal region, with the direction of flow below upward 2 months after the resolution of the thrombus. Management and Outcome: Ultrasonography (USG) color Doppler ruled out portal hypertension or persistence of IVC obstruction. Computed tomography (CT) venography resolved it to be the case of agenesis of the infrarenal portion of IVC. No consensus guidelines exist for neonatal management and it is usually conservative. Conclusion: IVC developmental anomalies including agenesis of IVC are very rare and are usually an asymptomatic condition, but can cause symptoms due to venous congestion in the lower limb or pressure from enlarged collaterals and rarely associated with deep venous thrombosis usually seen in young adults. Neonatal presentation is extremely rare. Diagnosis is by USG color Doppler and CT venography. Neonatal management guidelines are lacking and the risk of anticoagulation must be cautiously weighed against the risk of hemorrhage.

INFERIOR VENA CAVA AGENESIS: ANATOMICAL VARIANT AS CAUSE OF RECURRENT DVT

SESSION TITLE: Medical Student/Resident Pulmonary Vascular Disease Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Abnormalities of the inferior vena cava (IVC) are rare causes of deep venous thrombosis (DVT). The clinical presentation is often similar to that of an inherited hypercoagulabe condition. CASE PRESENTATION: A 27-year-old man with a past medical history of premature birth presented to the emergency department (ED) with left lower extremity (LLE) tightness and pain for one day. He had no history of recent travel and family history was negative for clotting disorders. Ultrasound (US) of the left leg showed an occlusive thrombus from the left common femoral to the left popliteal vein. The patient was discharged from the ED on rivaroxaban. Two weeks later, he presented to the ED again with progressive LLE swelling despite medication compliance. Physical exam was negative for testicular masses. Interventional Radiology (IR) performed a LLE venogram, which showed thrombus extention to the common iliac vein. US-assisted catheter thrombolysis (EKOS) was initiated. Heparin infusion was started and the patient was transferred to the intensive care unit. Repeat visualization the following day revealed improved clot in the left femoral and external iliac veins and a chronically thrombosed left common iliac vein. LLE pain and swelling improved and EKOS was discontinued twenty-four hours later. Hypercoagulability work-up was negative except for lupus anti-coagulant, the significance of which was difficult to determine, given prior anticoagulant exposure. On discharge, the patient was transitioned back to rivaroxaban. Five days later, the patient presented to the ED again with worsening LLE pain and swelling after initial improvement. Repeat venous US showed occlusive thrombus throughout much of the left femoral vein with only some flow at the distal aspect. An inferior vena cavogram revealed agenesis of the IVC with azygous continuation. CT angiogram of the abdomen and pelvis confirmed an occluded infra-renal IVC secondary to atresia. Patient was discharged on indefinite rivaroxaban therapy. DISCUSSION: This patient presented with an unprovoked DVT involving the common iliac vein that extended despite appropriate anticoagulation. Eventually, a venogram revealed agenesis of the IVC as the predisposing risk factor. In this condition, venous stasis presumably leads to increased risk of clotting. While bilateral DVTs are rare in the general population (approximately 10% of cases), they have been described in up to three quarters of patients with IVC abnormalities. This patient however, had extensive unilateral clot burden, usually seen in more distal anatomical abnormalities, such as May-Thurner syndrome. His premature birth likely predisposed him for an anatomical malformation. CONCLUSIONS: Unprovoked DVT in a young patient, recurrent DVTs despite medication compliance, and occlusion of the iliac veins, should raise suspicion for anatomical abnormalities, such as IVC atresia. Reference #1: 1. Chee YL, Culligan DJ, Watson HG. Inferior vena cava malformation as a risk factor for deep venous thrombosis in the young. Br J Haematol. 2001;114(4):878. Reference #2: 2. Ruggeri M, Tosetto A, Castaman G, Rodeghiero F. Congenital absence of the inferior vena cava: a rare risk factor for idiopathic deep-vein thrombosis. Lancet. 2001;357(9254):441. Reference #3: 3. Hamoud S, Nitecky S, Engel A, Goldsher D, Hayek T. Hypoplasia of the inferior vena cava with azygous continuation presenting as recurrent leg deep vein thrombosis. Am J Med Sci. 2000;319(6):414. DISCLOSURES: No relevant relationships by Amy Cohen, source=Web Response No relevant relationships by David Cohen, source=Web Response No relevant relationships by Aaron Dickinson, source=Web Response No relevant relationships by Mouhanned Eliliwi, source=Web Response No relevant relationships by Jennifer Meyfeldt, source=Web Response

Clinical diagnosis and treatment of 10 cases of congenital inferior vena cava malformation

Objective To analyze the clinical characteristics and significance of congenital inferior vena cava malformation (IVCM). Methods The clinical data of IVCM from 2013 to 2018 were studied. The clinical manifestations, diagnostic methods and characteristics were analyzed. Results There were 2 cases of double inferior vena cava (DIVC), 3 cases of LIVC, 1 case of LIVC + DIVC, 1 case of IVCA, 1 case of LIVC + IVCA near cardiac segment, 1 case of reentrant inferior vena cava (RIVC) and 1 case of linear inferior vena cava (IVC). Among them, 6 cases were complicated with DVT, 3 cases were misdiagnosed as budd-chiari syndrome (BCS), and 1 case was complicated with severe deep venous insufficient (DVI). Conclusion IVCM is rare and often ignored because of nonspecific manifestations, but it is an important risk factor for many diseases. It has important clinical significance in abdominal, pelvic and retroperitoneal surgery and vascular interventional therapy. Neglecting may lead to serious consequences. Key words: Inferior vena cava malformation; Venous thrombosis; Filter; Complications

Diagnosis and prognosis of fetal inferior vena cava malformation by fetal echocardiography

Objective To summarize the characteristics of two-dimensional(2D) ultrasound, spatiotemporal imaging correlation(STIC) and vascular casting of fetal inferior vena cava(IVC) malformations, analyze the clinical characteristics and the prognosis of fetal IVC malformations, and explore the early diagnostic value of fetal echocardiography for fetal IVC malformations. Methods Sixty-one cases of fetal IVC malformation diagnosed by fetal echocardiography in Beijing Anzhen Hospital Maternal Fetal Medical Consultation Center were retrospectively analyzed. The clinical data, echocardiographic features, genetic testing information and prognosis were summarized. Results ①In the 61 cases of IVC malformation, there were 51 cases of absence of IVC, 46 cases of absence of liver segment, 5 cases of absence of left IVC hepatic segment, 9 cases of left IVC (including 5 cases of left IVC and absence of IVC hepatic segment), 5 cases of double IVC, and 1 case of right hepatic vein reflux. ②Ten fetuses of the 61 fetuses were simple IVC deformity without any other malformations. Other venous malformations included 7 cases of absence of right superior vena cava and persistent left superior venacava, 2 cases of absence of venous catheters, and 3 cases of persistent right umbilical vein, 2 cases were merged alone; 49 cases were merged with intracardiac structural malformations and arrhythmia. ③Thirty-eight pregnant women underwent Down′s screening or non-invasive DNA, all of which were at low risk.Four cases underwent amniocentesis, and no obviously abnormality was found. Sixteen cases underwent autopsy and genome sequencing after induced labor, one was single gene abnormalities (ZFPM2 classical mutation). ④STIC images were collected in 20 cases, and 19 cases were clearly visualized. ⑤Vascular cast was made to show the shape of blood vessel more clearly and stereoscopically in 4 cases of absent hepatic segment of IVC. ⑥Eleven fetuses were born and confirmed from the results of postnatal ultrasound, who were healthy in follow-up; 16 fetuses were induced labor; 33 fetuses were losed during follow-up; 1 fetus died after birth (arrhythmia). Conclusions Fetal echocardiography combined with STIC technology can clearly diagnose the anomalies of IVC. Vascular casting can provide stereoscopic and visual manifestations of the anomalies of IVC. The prognosis of simple anomalies of IVC is good. Key words: Echocardiography, Fetal; Inferior vena cava malformation; Spatiotemporal imaging correlation; Vascular cast

Prenatal ultrasonic diagnosis and outcome of fetal inferior vena cava malformation

Objective To evaluate the clinical value of prenatal ultrasound in the diagnosis of inferior vena cava malformation. Methods The ultrasonographic features of 95 cases of fetal inferior vena cava malformation were retrospectively analyzed, and compared with autopsy or postpartum follow-up. Results Among the 95 cases of fetal inferior vena cava malformation, 39 cases were confirmed by autopsy, 56 cases were diagnosed by neonatal ultrasonography, 5 cases were confirmed by angiography or operation. Among them, 37 cases were interrupted inferior vena cava, 37 cases were double inferior vena cava, 21 cases were left inferior vena cava. Forty of the 95 cases were terminate pregnancy (due to intracardiac or extracardiac malformations) or neonatal deaths (42.1%), and 55 cases had good prognoses (57.9%). Conclusions Prenatal ultrasound has important clinical value in diagnosing of inferior vena cava malformation, clearing whether it is associated with other malformations and assessing fetal prognosis. Key words: Ultrasonography, prenatal; Fetal; Inferior vena cava malformation

The Typical Presentation Spectrum of Deep Vein Thrombosis Associated with Inferior Vena Cava Malformations.

Congenital malformations of the inferior vena cava (IVC) are rare and underreported. They can be a risk factor for deep venous thrombosis (DVT) as a result of inadequate venous drainage of the lower extremities through collateral circulation. The significant number of cases reported in the literature highlights their importance, warranting investigating their existence in younger individuals with idiopathic DVT of the lower extremities and pelvic veins. In this systematic review, we depict the typical presentation of IVC malformations, their management, and the management of their associated DVT.

Mayer-rokitansky-kuster-hauser syndrome associated with severe inferior vena cava stenosis.

Precis. The postoperative course of a neovagina creation procedure in a young woman with Meyer-Rokitansky-Kuster-Hauser syndrome was complicated, despite prophylaxis, by extensive pelvic deep venous thrombosis secondary to unsuspected severe inferior vena cava stenosis. Background. Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is characterized by congenital vaginal agenesis and an absent or rudimentary uterus in genotypical females. Malformations of the inferior vena cava (IVC) are not commonly associated with MRKH syndrome. We report a case of a patient with MRKH syndrome with severe IVC stenosis that was diagnosed when the patient presented with extensive pelvic deep venous thrombosis (DVT) during the postoperative course of a neovagina creation. Case. A 19-year-old female underwent a McIndoe procedure. Despite DVT prophylaxis, extensive pelvic DVT of the femoral vein was diagnosed on postoperative day 7. Therapeutic anticoagulation was initiated, and pharmacological and mechanical thrombolysis were performed. During these procedures, a hypoplastic IVC was noted. Conclusion. MRKH syndrome can be associated with IVC malformations, which constitute an anatomical risk factor for postoperative DVT.

Nutcracker syndrome due to left-sided inferior vena cava compression and treated with superior mesenteric artery transposition

Left renal vein hypertension secondary to left renal vein compression has been described as a cause of persistent hematuria in nutcracker syndrome. Malformation of the inferior vena cava (IVC), although rare and frequently asymptomatic, may also result in left renal vein hypertension, with resultant hematuria when it is severely compressed. We report a 20-year-old man with persistent hematuria due to compression of left-sided IVC. The patient was successfully treated by means of superior mesenteric artery (SMA) transposition and division of the fibrous bundle at the origin of the SMA. His postoperative course was uneventful. Compression of the left IVC is a unique form of nutcracker syndrome. SMA transposition, together with division of a fibrous bundle at the origin of the SMA if present, is a safe and effective surgical procedure for this special entity.

Left‐sided and duplicate inferior vena cava: A case series and review

Left-sided and duplicate inferior vena cava (IVC) are two major anatomical variants within the spectrum of IVC malformations, both of which are developmental abnormalities of the supracardinal veins. Four clinical cases are described to highlight the computed tomographic appearances of these vascular malformations and provide novel data on venous dimensions. A systematic review of the recent literature (2000-2011) was conducted focusing on the anatomy, demographics, and associated pathology (congenital and acquired) of isolated left-sided and duplicate IVC. A total of 73 relevant articles were retrieved, consisting of case reports and small case series. The prevalence of left-sided IVC is about 0.1-0.4% and that for duplicate IVC about 0.3-0.4%; both anomalies show a slight male preponderance. In each condition, there are documented variations in the course and tributaries of the IVC. The clinical importance of these anomalies lies in three principal areas: the potential for misdiagnosis on imaging; technical difficulties during retroperitoneal surgery (particularly abdominal aortic aneurysm repair and live donor nephrectomy); and their significance in relation to the etiology and management of venous thromboembolism.

Posterior Nutcracker Syndrome Associated with Interrupted Left Inferior Vena Cava with Azygos Continuation and Retroaortic Right Renal Vein

Various anatomic anomalies have been considered the causes of nutcracker syndrome (NCS). Posterior NCS refers to the condition, in which vascular narrowing was secondary to the compression of the retroaortic left renal vein while it is crossing between the aorta and the vertebral column. Here, we report an unusual case of posterior NCS associated with a complicated malformation of the interrupted left inferior vena cava with azygos continuation and retroaortic right renal vein, diagnosed by both color Doppler ultrasonography and CT angiography.

Diagnosis of horseshoe lung with multi-slice helical CT

Objective To study the multi-slice helical CT (MSCT) features of horseshoe lung and to improve its diagnostic ability. Methods The clinical and imaging data were retrospectively reviewed in 5 patients. All of them underwent plain MSCT scan, and 4 patients also had enhanced MSCT scan and threedimensional reconstruction. Results In horseshoe lung, the basal segments of the right and left lungs were fused together by a isthmus of pulmonary parenchyma, across the midline behind the pericardium and before the esophagus, descending aorta and spine. All patients were companied with bronchopulmonary dysplasia,anomalous origin of the central bronchi, anomalous arterial supply. Anomalous pulmonary venous return to inferior vena cava and congenital cardiovascular malformations were also found in 4 cases. Conclusion MSCT and three-dimensional reconstruction are valuable for identification of horseshoe lung. Key words: Bronchopulmonary dysplasia; Tomography,X-ray computed

Double Inferior Vena Cava in a patient with lymphoma

The inferior vena cava (IVC) develops by a complex process from diverse embryonic sources and drains blood from a large number of structures inferior to the diaphragm. Congenital malformations of the IVC are reported to be rare and most of these malformations are accidental findings through abdominal imaging studies. Although compatible with life, these anomalies are of major clinical importance and can be a contributing factor in thrombus formation. This case report describes a double IVC in an adult male cadaver who died of lymphoma and cardiac events. The common iliac veins were found to continue superiorly and fuse together slightly cranial to the level of the superior mesenteric artery origin from the aorta at the L1 vertebral level. The left IVC received the left gonadal vein. The left suprarenal vein opened into the corresponding renal vein which extended superiorly and fused side to side with the left IVC as well as with the common IVC. Multiple factors may contribute to embryonic IVC malformations including genetic, and environmental including the expression and regulation of growth factors that control epithelial–mesenchymal transformation (e.g. TGF‐â), formation of endothelium, vascular endothelial growth factors (VEGF) and various VEGF receptors.

Deep Venous Thrombosis Caused by Congenital Malformation of the Inferior Vena Cava and Heterozygous Factor V Leiden Presenting as Venous Claudication

A case of an 18-year-old man with deep venous thrombosis of the lower limbs caused by hypoplasia of the inferior vena cava in combination with heterozygous factor V Leiden is presented. Both anomalies were found when the patient complained of venous claudication in both thighs. Inferior vena cava malformation is a rare condition and may predispose to the development of deep venous thrombosis. This patient was at an even higher risk for deep venous thrombosis as the inferior vena cava malformation was combined with a hypercoagulable state.

Inferior Vena Cava Agenesia and a Massive Bilateral Iliofemoral Venous Thrombosis

The absence of the inferior vena cava is a rare congenital anomaly and represents a modest risk for deep-venous thrombosis. When associated with genetic polymorphism or other hypercoagulable states, the risk can increase several times. A case of young man with the inferior vena cava agenesia and deep-venous thrombosis of both legs triggered by the physical effort is reported. The view that the congenital inferior vena cava malformation represents a predisposition to deep-venous thrombosis in spite of well-developed collateral circulation is supported. In these circumstances, various external triggers (physical effort, hormonal contraception etc) may precipitate thrombosis. Because the patient had no other convincing and permanent risk factors for thrombosis, the lifelong anticoagulant therapy was not recommended.

724 Evaluation of mechanical arterial properties in patients with coronary vasoconstriction

Eur J Echocardiography Abstracts Supplement, December 2006 Conclusions: In children, deep venous thrombosis was the result of the association between a thrombophilic factor (found in all cases), a vascular or cardiac anatomic anomaly and a precipitating factor. Doppler ultrasonography was the main diagnosis method, concordant with angio CT in the identification of all iliac veins anomalies, but not always concordant for inferior vena cava malformations.

Right Varicocele Associated with Inferior Vena Cava Malformation in Situs Inversus: Percutaneous Treatment with Retrograde Sclerotherapy

Isolated right varicocele is a rare condition. It could be secondary to a retroperitoneal neoplastic mass involving the right internal spermatic vein, but sometimes, an anatomic variant must be considered. We present a case of a young man with situs inversus and right varicocele, a mirror image of the normal condition, associated with inferior vena cava malformation, who was treated successfully with retrograde selective percutaneous sclerotherapy of the right internal spermatic vein. Selective sclerotherapy of the internal spermatic vein at the time of venography has proved to be a valuable therapeutic option in right-sided varicocele associated with anatomic abnormalities.