Inferior vena cava atresia predisposing to acute lower extremity deep vein thrombosis in children: A descriptive dual‐center study

Tarango, Cristina; Kumar, Riten; Patel, Manish; Blackmore, Anne; Warren, Patrick; Palumbo, Joseph S.

doi:10.1002/pbc.26785

Cited by 14 publications

(14 citation statements)

References 32 publications

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“…Of note, in DVT with IVCA, pulmonary embolism due to deep vein thrombosis in the legs is unlikely due to the anatomic situation [14, 16]; exceptions are rare [12, 37–39]. In our case, no pulmonary embolism was found, and dyspnoea resolved spontaneously.…”

Section: Discussionmentioning

confidence: 59%

“…For detection of IVCA, CT with contrast or MRI are considered the most accurate modalities, as ultrasound is limited by habitus, bowel gas and because the abdominal vessels cannot be compressed [14, 16, 30]. Also, indefinite anticoagulant therapy as well as wearing of medical compression stockings is advocated [12, 16, 20]. In selected cases of acute DVT in the presence of IVCA, endovascular approaches such as catheter-directed thrombolysis and stenting have been successfully applied [16, 30–32].…”

Section: Discussionmentioning

confidence: 99%

“…Moreover, atresia of the IVC has been identified as an underrecognized risk factor for deep-vein thrombosis of the legs [9, 10], and IVC anomalies were found in 16.2% of iliac vein thrombosis [11]. In a survey of an institutional cohort, ten out of 18 patients (56%), aged 12–18 years, suffered from subsequent post-thrombotic syndrome [12]. Finally, the acronym KILT for kidney and IVC anomalies with leg thrombosis has been proposed for the association of kidney atrophy, IVC atresia and thrombosis in the legs [13].…”

Section: Introductionmentioning

confidence: 99%

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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: 59%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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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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“…A recent two centre study of 18 paediatric patients with IVC atresia and DVT managed with long term therapeutic anticoagulation, with or without thrombolysis, reported a re-thrombosis rate of 17%. 9 …”

Section: Discussionmentioning

confidence: 99%

Endovascular IVC Reconstruction in an 18 Year Old Patient with Subtotal IVC Atresia

Hung

Kwon

Sudheendra

2021

EJVES Vascular Forum

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Introduction Inferior vena cava (IVC) atresia is an uncommon venous anomaly that is an under recognised cause of unprovoked acute deep venous thrombosis (DVT) in young adults. The purpose of this case report is to highlight endovascular IVC reconstruction as a feasible treatment option, particularly in challenging cases when other therapeutic modalities have failed. Report This is the report of an 18 year old patient with near complete IVC atresia and a longstanding history of exertional nausea of unknown aetiology, who presented with extensive acute DVT. He was treated successfully by endovascular IVC reconstruction after failing initial anticoagulation and thrombolysis. Symptom resolution and venous patency were maintained at 2.5 year follow up. Discussion IVC atresia is an important aetiology to consider in a young patient presenting with unprovoked DVT. Endovascular stenting can restore venous patency and is feasible even when there is near complete IVC atresia. This case was uniquely challenging in the length of atretic IVC that was reconstructed and also highlights an atypical clinical presentation of IVC atresia.

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“…1 Otras manifestaciones son dolor lumbar, tromboembolismo pulmonar, edema, dolor o várices de miembros inferiores, síndrome de compresión de la cola de caballo y varicocele bilateral. [10][11][12][13][14] Aunque hay muchas causas posibles de ascitis fetal, la AVCI debe ser incluida entre los diagnósticos diferenciales, debido a sus repercusiones a futuro.…”

Section: Venas Supracardinalesunclassified

Ascitis fetal como forma de presentación de agenesia de la vena cava inferior

Mercado

Liberto²,

Udaquiola

et al. 2018

Arch Argent Pediatr

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Inferior Vena Cava (IVC) is composed of three segments from different embryological origin. Its lack of fusion originates a wide spectrum of anomalies of the IVC. These malformations are present in 0.07-8.7% of the population. It is generally diagnosed as an incidental finding in adult life. It represents between 5 and 9.5% of idiopathic deep vein thrombosis in patients younger than 30 years old without associated risk factors. We present a case of a term newborn with prenatal diagnosis of ascites during the 20th week of gestation. IVC Agenesis was diagnosed with the use of abdominal angiotomography. The treatment of patients with IVC Agenesis is based on the management of its complications. Due to the increased thrombotic risk of these patients, we should consider lifelong anticoagulation. We suggest initiating it during puberty.

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Inferior vena cava atresia predisposing to acute lower extremity deep vein thrombosis in children: A descriptive dual‐center study

Cited by 14 publications

References 32 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

Endovascular IVC Reconstruction in an 18 Year Old Patient with Subtotal IVC Atresia

Ascitis fetal como forma de presentación de agenesia de la vena cava inferior

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