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Abstract
BackgroundNeonatal 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 presentationWe 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 conclusionsKILT 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.
Highlights
Neonatal renal vein thrombosis is a recognised cause of renal and inferior caval vein atresia (IVCA)
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 Inferior vena cava atresia (IVCA) is by Computed tomography (CT) with contrast or by Magnetic resonance imaging (MRI), and lifelong anticoagulation may be necessary
Summary
This case is remarkable in many aspects. First, the discovery of the IVC anomaly was fortuitous and the computed tomographies were prompted by dyspnoea of the patient and suspicion of pulmonary embolism; secondly, it highlights a more general unawareness of this condition, since haematologists diagnosed a normal laboratory thrombophilia work-up and angiologists a postthrombotic syndrome in both legs, and no abdominal imaging study was performed; thirdly, the case demonstrates the outcome after renal vein thrombosis in the neonate and no longterm anticoagulation at that time; and, lastly, the longterm follow-up of over 40 years in this case is unique, and we did not find a similar case in the literature. Most importantly and as shown by the present case is a thorough information of the patient and the parents at the time of renal vein thrombosis to ensure a lifelong correct management of possible complications, and, when DVT occurs in early adulthood, a thorough workup of the previous medical history targeting at early childhood. The only cost it takes is time, and the benefit may be prevention of further complications by lifelong anticoagulation.
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