Hypertensive crisis in an 11-year-old girl with kidney and inferior vena cava abnormalities and leg thrombosis: Answers
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Hypertensive crisis in an 11-year-old girl with kidney and inferior vena cava abnormalities and leg thrombosis: Answers Ľubica Kováčiková 1 & Mária Chocholová 1 & Milan Ilčík 2 & Žofia Varényiová 1,3 & Tomáš Seeman 3 & Ľudmila Podracká 1 Received: 11 August 2020 / Revised: 22 September 2020 / Accepted: 13 October 2020 # IPNA 2020
Keywords Child . Hypertensive crisis . Kidney hypoplasia . Venous thrombosis . Inferior vena cava abnormalities . KILT syndrome . Kidney and inferior vena cava abnormalities with leg thrombosis
Answers What is the final diagnosis of a condition in which leg thrombosis is associated with renal and vena cava inferior anomalies? As a part of diagnostic workup in a girl with hypertensive crisis and leg pain and swelling, imaging studies revealed deep venous thrombosis of the lower extremities and anomalies of the inferior vena cava (IVC) and hypoplastic left kidney. In 2002, Van Veen et al. [1] reported a 16-year-old girl with an absent IVC, atrophic left kidney and deep venous thrombosis of the legs, and the authors termed this association KILT (Kidney and Inferior vena cava abnormalities with Leg Thrombosis) syndrome. Thirteen years later, Bami et al. [2] published another case of KILT syndrome in a 14-year-old boy with instable painful gait and interruption of the IVC accompanied by a lower leg thrombosis. As the condition became better studied and described, further patients were This refers to the article that can be found at https://doi.org/10.1007/ s00467-020-04825-8. * Ľudmila Podracká [email protected] 1
Department of Pediatrics, Medical Faculty, Comenius University and National Institute of Children′s Diseases, Limbova 1, 833 40 Bratislava, Slovakia
2
Department of Pediatric radiology, Medical Faculty, Comenius University and National Institute of Children′s Diseases, Bratislava, Slovakia
3
Department of Pediatrics, 2nd Faculty of Medicine and Faculty of Medicine, Charles University in Prague and Motol University Hospital, Prague, Czech Republic
recognized as having KILT syndrome manifesting mostly with structural IVC malformations or its complete absence together with thrombosis and kidney atrophy or dysplasia [3, 4]. The patients mostly suffered from abdominal pain and leg edema and pain. Interestingly, quite a few of the reported patients were siblings, suggesting a possible pattern of inheritance of this pathology [5]. In 2018, Pomeranz et al. [6] also published a case of an 11year-old girl who complained of difficulties with gait and persistent leg pain. Imaging studies confirmed an absence of the portions of the IVC critical for blood drainage from kidneys, and multiple thromboses of deep pelvic and femoral veins. As a result of the flawed vasculature, the development of the right kidney was deteriorated, leading to its complete atrophy. Malformation or complete absence of the IVC is a rare entity affecting only about 0.5% of the population. It is presumably a result of an intrauterine or perinatal thrombosis which leads to an obstruction and subsequen
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