Long term complications of inferior vena cava thrombosis

AIM—To evaluate the long term outcome after paediatric inferior vena cava (IVC) thrombosis.
METHODS—A combined retrospective and prospective study on infants and children with IVC thrombosis treated at Aachen and Maastricht University Hospitals between 1980 and 1999.
RESULTS—Forty patients were enrolled, including four with preceding cardiac catheterisation, 18 with central venous saphenous lines, and an additional eight with umbilical venous catheters. Six patients died within three months of diagnosis; one patient was lost to follow up. Twelve of the remaining 33 patients had suffered from limited IVC thrombosis; during follow up (for up to nine years) none showed persisting caval obstruction (successful thrombolysis, n = 2; spontaneous recanalisation, n = 10). The remaining 21 patients presented with extensive IVC thrombosis. During follow up (for up to 18 years) complete restitution was found in only four cases (one thrombolysis, two surgery, one spontaneous recanalisation). Persisting iliac and/or caval venous obstruction occurred in 17patients, including six with thrombolysis. Varicose veins were found in 12, and post-thrombotic syndrome in seven of these cases. According to Kaplan-Meier analysis, 30% of patients with persisting venous disease will develop post-thrombotic syndrome within 10years of the thrombotic event.
CONCLUSIONS—Infants and children with extensive IVC thrombosis are at high risk for persisting venous disease and serious long term complications. Prospective trials are urgently needed to establish effective treatment strategies and to improve long term prognosis. Central venous catheters, contributing to IVC thrombosis in the majority of cases reported here, should be inserted only if essential.

     

Duplex Doppler ultrasound evaluation of calcified inferior vena cava thrombosis

We describe the use of duplex Doppler ultrasound in the noninvasive assessment of inferior vena cava patency and abdominal venous flow dynamics in a four month old infant with calcified inferior vena cava thrombosis.     

Renal transplant in children with previous inferior vena cava thrombosis

Our experience with renal transplantation in children with inferior vena cava thrombosis is presented in this study. Of the 238 children who have received renal transplants at our institution, four had IVC thrombosis (discovered during pretransplant evaluation: three patients; found at surgery: one patient). The pretransplant US evaluation diagnosis of IVC thrombosis in three patients was confirmed by transjugular retrograde cavography. There were no signs of hypercoagulability or IVC thrombosis symptoms prior to diagnosis in any patient. The graft was implanted in a left orthotopic position in three patients. Venous drainage was attained to the infrahepatic vena cava or native renal vein after ipsilateral nephrectomy. The renal artery of the graft was anastomosed to the aorta. In one patient, the graft was placed in the left iliac fossa. Patient and graft survival are 100%. Three grafts are functioning normally after a mean follow-up of 3.7 yr. The graft placed in the iliac fossa has moderate dysfunction due to high pressure venous outflow. Children with IVC thrombosis can be successfully transplanted orthotopically. Candidates with any suspicious-looking occlusion on ultrasound should be studied by retrograde venography to confirm diagnosis prior to transplantation.     

Pediatric child abuse victim with posttraumatic inferior vena cava thrombosis

Posttraumatic inferior vena cava thrombosis (IVCT) is very rare, with only a few cases reported in the literature. While thromboembolism as a result of trauma is common in adult patients, it is very rare in young children and seldom involves the inferior vena cava. We report the youngest patient to date with IVCT and the only child reported whose findings are the result of child physical abuse. The diagnosis can be challenging both clinically and radiographically. Additionally, the risks of morbidity and even mortality associated with an IVCT if untreated are significant.     

The clinical outcome after inferior vena cava thrombosis in early infancy

The clinical outcome after inferior vena cava thrombosis in early infancy is unknown. We report the clinical long-term follow-up of 12 patients presenting inferior vena cava thrombosis within their first months of life (gestational age: 24–41 weeks; follow-up: 7±3 years). Accompanying renal venous thrombosis occurred in 9, and adrenal bleeding in 4 patients. A central venous catheter was related to the thrombosis in only four patients. Heterozygous factor V Leiden mutation was found in two of the eight infants without central venous catheter. Thrombolysis was performed in seven and effective in three infants; one infant required surgical thrombectomy. In three of eight infants with ineffective or with no therapy, spontaneous recanalization occurred during follow-up. No patient died of the thrombosis. Although no long-term anticoagulatory prophylaxis was performed, none of the children with persisting occlusion (n=5) or stenosis (n=1) of the inferior vena cava developed symptomatic thrombo-embolic complications. However, extensive internal collaterals (n=6), visible varicosis (n=5), pain in the legs (n=3) and persisting renal disease (n=3) with arterial hypertension (n=2) were observed during follow-up. Conclusion Inferior vena cava thrombosis of early infancy frequently persists and may cause considerable long-term morbidity. New strategies for early and long-term therapy are necessary. Received: 6 April 1998 / Accepted in revised form: 24 July 1998     

Neonatal inferior vena cava and renal venous thrombosis treated by thrombectomy and nephrectomy.

Neonatal inferior vena cava and renal venous thrombosis with obstruction was diagnosed clinically and confirmed by ultrasonography. Successful thrombectomy and nephrectomy were performed at 40 hours of age. Thrombus with obstruction occluding the inferior vena cava favours immediate surgery.     

Deep venous thrombosis in a child associated with an abnormal inferior vena cava

Congenital anomalies of the inferior vena cava (IVC), such as absence or atresia, although well documented, are uncommon and result from aberrant development during embryogenesis. Absence or atresia of the IVC is usually discovered accidentally. Patients are typically asymptomatic of the condition itself. Many concurrent cardiovascular-associated abnormalities have been described. We report a 10-y-old boy admitted to the emergency room with painful swelling of his right lower limb without previous trauma or surgery. After 3 d, swelling also involved the left lower limb. A Doppler ultrasound of the lower limbs revealed bilateral thrombosis of the vena iliaca communis, vena iliaca externa, femoral vein communis and superficial extending to the IVC. Magnetic resonance imaging (MRI) of the abdomen was performed. On MRI, we demonstrated a hypoplastic IVC. The results of blood coagulation studies, including levels of antiphospholipid antibodies, proteins C and S, and antithrombin III, were normal. The patient was treated with intravenous heparin for 8 d and discharged with oral warfarin therapy, which has been recommended for life.
Conclusion: Therapy against deep venous thrombosis must be focused on its prevention in the future. An abnormal inferior vena cava should be considered in young patients with deep venous thrombosis without apparent cause.     

Congenital calcified thrombosis of inferior vena cava,bilateral renal veins and left spermatic vein

An infant with severe congenital calcified thrombosis in the inferior vena cava, bilateral renal veins and left spermatic vein is presented. Five previously published cases and pertinent points concerning this rare occurence are discussed. The etiology of this calcified thrombosis remained unknown.The institution from which the work originated     

Sturge-Weber and Klippel-Trenaunay syndromes with absence of inferior vena cava.

A baby girl born at 33 weeks'' gestation weighing 2250 g presented with Sturge-Weber syndrome, features of the Klippel-Trenaunay syndrome, and absence of the inferior vena cava. We suggest that aplasia of the vena cava may be a feature of Klippel-Trenaunay syndrome when the capillary malformation affects the trunk.     

Thoracoabdominal intestinal duplication with absent inferior vena cava

We report a rare case of thoracoabdominal intestinal duplication with absent inferior vena cava (IVC). The patient was initially explored with a mistaken diagnosis of diaphragmatic hernia on the basis of a chest radiograph and barium meal. However, a subsequent computed tomography scan revealed a mediastinal mass with an air–fluid level, a hugely dilated azygos vein, and an absent IVC. Thoracoabdominal exploration was required to excise the duplication cyst arising from the jejunum. We believe that this is the first report of this association. Accepted: 27 October 2000     

Noninvasive evaluation of the inferior vena cava. The value of sonography

Children who have abnormalities of the inferior vena cava (IVC) appear with diverse signs and symptoms. Those with congenital IVC malformations have symptoms related to associated cardiac disease. Those with IVC compression or obstruction may have ascites, hepatomegaly, edema of the lower extremities, and/or signs of portal hypertension. Sonography of the IVC has become a routine part of the evaluation of congenital heart disease and also part of the examination of patients with abdominal masses. Sonography should be the initial imaging modality in children at high risk for caval disorders. Previously used diagnostic procedures such as the inferior venacavogram should now be used only as a secondary investigative tool.