Incidental Finding of Inferior Vena Cava Atresia Presenting with Deep Venous Thrombosis following Physical Exertion

Koppisetty, Shalini; Smith, Alton G.; Dhillon, R.K.

doi:10.1155/2015/146304

Cited by 12 publications

(9 citation statements)

References 18 publications

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“…KILT syndrome is a rare phenomenon and conclusions are based upon the available, limited literature as well as upon pathophysiological considerations. It is suggested that in the so-called idiopathic thrombosis in the young patient < 30 years with no thrombophilia or apparent risk factors (immobilisation, contraceptives, trauma), a vascular malformation of the pelvic/central venous system should be considered as well, especially if increased physical exertion precedes DVT [16, 40]. CT with contrast and MRI are the imaging modalities of choice.…”

Section: Discussionmentioning

confidence: 99%

Thrombophlebitis hiding under a KILT – case report on 40 years long-term follow-up of neonatal renal vein thrombosis

et al. 2019

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Background Neonatal renal vein thrombosis is a recognised cause of renal and inferior caval vein atresia (IVCA). However, the long-term impact of the condition is underrecognized with a high burden of morbidity for the patient, especially in adulthood. IVCA has been shown to be an independent risk factor for deep venous thrombosis (DVT) with a high risk of recurrence. The acronym KILT for kidney and inferior vena cava anomaly with leg thrombosis summarizes the pathological situation. Case presentation We present the case of a 40-year-old patient with pain in the right lower limb resulting from acute thrombophlebitis. No risk factors could be identified. His history was remarkable with two episodes of deep venous thrombosis first of the left, then the right leg 22 years earlier; at that time also, no risk factor was identified. Because of the idiopathic character of that thrombosis, the patient remained on long-term anticoagulation with phenprocoumon. The present thrombophlebitis occurred while the INR was not therapeutic in the preceding weeks. A CT with contrast showed atresia of the inferior vena cava and of the right kidney, and presence of numerous collaterals. A thorough medical history revealed a renal vein thrombosis as a neonate. Anticoagulation was intensified, and stent placement became necessary after a further 2 years. Discussion and conclusions KILT syndrome is a rare but underrecognized condition. Complications may arise in young adulthood only, and it is of prime importance to instruct parents of the pediatric patient of the possible consequences of renal vein thrombosis and to assure guidance from the treating physicians throughout adulthood. Diagnosis of IVCA is by CT with contrast or by MRI, and lifelong anticoagulation may be necessary. Since the KILT syndrome is widely underdiagnosed, we challenge the clinicians to keep it in mind when confronted with thrombophlebitis or thrombosis of the young, male and with no other identifiable risk factors for deep vein thrombosis.

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Section: Discussionmentioning

confidence: 99%

Thrombophlebitis hiding under a KILT – case report on 40 years long-term follow-up of neonatal renal vein thrombosis

et al. 2019

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“…Shaline et al reported a case of IVC atresia presenting with DVT associated in the setting of physical exertion. 8 The DVT commonly involves distal IVC, common, internal and external iliac and femoral veins. Our patient was physically very active and CT scan showed DVT of the right common femoral vein through common iliac vein and the left common iliac vein extending into the left internal and external iliac veins.…”

Section: Discussionmentioning

confidence: 99%

Rare Case of a Kidney and Inferior Vena Cava Abnormalities with Extensive Lower Extremity Deep Vein Thrombosis in a Young Healthy Male

Khalid

Nukavarapu

Shah

et al. 2018

Clinics and Practice

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Kidney and inferior vena cava (IVC) abnormalities with extensive deep vein thrombosis (DVT) is a very rare cause of DVT and has a diverse clinical presentation. Computed tomography (CT) angiography is the gold standard for diagnosis and treatment including thrombectomy, thrombolysis and systemic anticoagulation. We present a rare case of active young healthy male admitted with acute onset of right lower extremity pain and swelling who was found to have extensive DVT on doppler ultrasound. CT abdomen showed extensive clot burden involving right common femoral vein extending into internal and external iliac veins associated with IVC hypoplasia and hypoplastic left kidney. Patient underwent urgent thrombectomy, catheter directed thrombolysis and was discharged home in stable condition on oral anticoagulation.

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“…However, limited data on treatment with direct oral anticoagulants (DOACs) in this setting are so far available. 33,36 The surgical approach (venous thrombectomy and/or replacement of IVC) seems to prevent the deterioration of the chronic venous insufficiency over time and should be considered in patients with acute DVT of the iliofemoral veins, which causes a complete collapse of the collateral system. 42 At present, as patients with AIVC may be at high risk of recurrences, and based on available evidence, lifelong oral anticoagulation should be considered, and ES should be used to prevent the PTS.…”

Section: Discussionmentioning

confidence: 99%

Inferior Vena Cava Agenesis and Deep Vein Thrombosis in the Young: A Review of the Literature and Local Experience

Tufano

Gianno

et al. 2017

Semin Thromb Hemost

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Congenital agenesis of the inferior vena cava (AIVC) is a rare vascular abnormality with a prevalence of 0.0005 to 1% in the general population. This condition is found in almost 5% of young patients (younger than 30 years) with proximal, typically bilateral, deep venous thrombosis (DVT) of the lower limbs, often in the absence of apparent risk factors. However, AIVC in young patients with DVT is probably underestimated because AIVC cannot be detected by the standard DVT diagnostic workup. Inherited thrombophilia has been reported in patients with AIVC-associated DVT, as an additional risk factor, but its role is poorly investigated. The best therapeutic strategy and the optimal duration of anticoagulant treatment of DVT in patients with AIVC are still unclear. Here, we describe 14 young patients (2 females and 12 males, mean age at first DVT: 27.8 ± 10.1 years), with color Doppler ultrasound (US) and abdominal computed tomography scan confirmed AIVC-associated DVT and discuss their characteristics in the framework of a literature review on this topic. Our patients were mainly males (10/12) and experienced proximal DVT events, not complicated by pulmonary embolism. DVT-precipitating factors were detected only in five cases, the two female patients (oral contraceptives and puerperium), and three male patients (one after leg fracture and two after abdominal surgery). Thrombophilic abnormalities were found in eight patients (heterozygous factor V Leiden mutation, n = 3; mild/moderate hyperhomocysteinemia, n = 3; reduced protein C activity, n = 1; antiphospholipid syndrome, n = 1). The majority of patients (13/14) were treated with long-term oral anticoagulant therapy and elastic stockings (ES), with a very low rate of DVT recurrence (1/14, mean follow-up 7 years). These clinical characteristics were largely consistent with those of 161 patients identified by review of the literature. On the whole, although many aspects are still poorly known, our case series and literature review suggest that a search should be undertaken for AIVC in young patients with proximal idiopathic, particularly when bilateral, DVT, which should then be treated with prolonged anticoagulation and ES.

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Incidental Finding of Inferior Vena Cava Atresia Presenting with Deep Venous Thrombosis following Physical Exertion

Cited by 12 publications

References 18 publications

Thrombophlebitis hiding under a KILT – case report on 40 years long-term follow-up of neonatal renal vein thrombosis

Thrombophlebitis hiding under a KILT – case report on 40 years long-term follow-up of neonatal renal vein thrombosis

Rare Case of a Kidney and Inferior Vena Cava Abnormalities with Extensive Lower Extremity Deep Vein Thrombosis in a Young Healthy Male

Inferior Vena Cava Agenesis and Deep Vein Thrombosis in the Young: A Review of the Literature and Local Experience

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