Paeds · haematology-oncology-and-transfusion
Venous thromboembolism, pulmonary embolism and thrombophilia in children
Also known as Venous thromboembolism · Deep vein thrombosis · Pulmonary embolism · CVAD-associated thrombosis · Catheter-related thrombosis · Hereditary thrombophilia · Paediatric thrombosis
Fellowship guide to venous thromboembolism, pulmonary embolism and thrombophilia in children: the bimodal epidemiology peaking in neonates and adolescents, the predominance of provoked and central-venous-catheter-associated thrombosis, the Virchow triad pathophysiology modified by developmental haemostasis, the clinical picture of leg swelling and catheter dysfunction, the diagnosis with compression ultrasound and D-dimer, the stepwise anticoagulation with low molecular weight heparin and oral rivaroxaban built on the CHEST and ASH guidelines and the EINSTEIN-Jr trials, the selective approach to hereditary thrombophilia testing, and the care of neonates, critically ill children and the transitioning adolescent.
On this page
Study tools
Your progress
Saved on this device.
Practise this topic
Target exams
Red flags
- A swollen, warm, painful leg in a child with a central venous catheter or critical illness is a deep vein thrombosis until proven otherwise — confirm with compression ultrasound and start therapeutic low molecular weight heparin, because delay risks embolisation and post-thrombotic syndrome
- Sudden dyspnoea, pleuritic chest pain, haemoptysis or unexplained tachycardia and hypoxia in a child with a known or suspected clot is a pulmonary embolism — image urgently and anticoagulate, as paediatric pulmonary embolism is increasingly recognised and frequently missed
- A neonate with a central line and unexplained apnoea, irritability, seizures or a swollen discoloured limb may have catheter-related thrombosis extending into the right atrium or a cerebral sinus venous thrombosis — image and treat, because neonatal thrombosis can be rapidly fatal or cause permanent brain injury
- Warfarin and direct oral anticoagulants must not be used in pregnancy, and a girl on oestrogen-containing contraception with a personal or family history of thrombosis should be screened for thrombophilia before commencing the combined oral contraceptive pill
Life stages
Care settings
Clinical exam formats
Board mappings
- Haematology
- Thrombosis and thrombophilia
- Venous thromboembolism in children
- Paediatric Haematology — venous thromboembolism, pulmonary embolism and thrombophilia
- General Paediatrics learning goal — diagnose and anticoagulate paediatric VTE
- Central venous access device thrombosis and the anticoagulation landscape
- Clinical Applications
- Thromboembolic disease and anticoagulation
- Long Cases
- Short Cases
- Communication scenarios
- Haematology and oncology: recognises and manages venous thromboembolism
- Central venous catheter complications and thrombophilia
- Safe anticoagulation in children
- Foundation of Practice (FOP)
- Applied Knowledge in Practice (AKP)
- Venous thromboembolism and thrombophilia
- Clinical
- History
- Communication
- Cardiorespiratory examination
- General Pediatrics Content Outline — Domain: Haematology
- Subspecialty: Pediatric Hematology — thrombosis and thrombophilia
- Venous thromboembolism and anticoagulation
- Patient Care 1: History and Physical Examination
- Patient Care 4: Clinical Reasoning
- Medical Knowledge 1: Clinical Knowledge of paediatric thrombosis
- Systems-Based Practice 1: multidisciplinary thrombosis and anticoagulation care
- Medical Expert
- Collaborator
- Pediatrics: Core EPA — recognise and manage paediatric thromboembolism
Overview & Definition
Picture the fourteen-year-old girl admitted after spinal fusion surgery for scoliosis, five days postoperative, now breathless and complaining of right-sided chest pain when she takes a deep breath in, her oxygen saturations dipping to 92 percent. That girl has a pulmonary embolism, and the deep vein thrombosis that seeded it has been quietly forming in her immobilised leg. The recent surgery, the immobility and her combined oral contraceptive pill are the three provoking factors stacked together, and the breathlessness and pleuritic pain are the clinical signature of an embolus in a pulmonary artery. [3] [11]
Venous thromboembolism in children is the formation of a pathological clot within the venous system, encompassing deep vein thrombosis (a clot in a deep vein, most often a leg or a central vein), pulmonary embolism (a clot that has embolised to the pulmonary arterial tree), cerebral sinus venous thrombosis, and renal and portal vein thrombosis. The defining feature of paediatric venous thromboembolism, in contrast to the adult disease, is that it is overwhelmingly provoked and secondary. Over 90 percent of paediatric cases have an identifiable trigger, and the single largest category is thrombosis associated with a central venous access device, which accounts for around half of all events. [3] [2]
The disease is also defined by its developmental context. Children are not small adults when it comes to clotting: the neonatal haemostatic system is physiologically immature, with reduced levels of the vitamin K dependent factors and anticoagulant proteins, and this developmental haemostasis shapes both the risk and the response to anticoagulants. Neonates and infants metabolise heparin faster than older children and need higher weight-based doses, which is why dose tables are age-stratified from birth. [7] [1]
References12ShowHide
- [1]Monagle P, Chan AKC, Goldenberg NA, et al. Antithrombotic therapy in neonates and children: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. Chest, 2012.PMID 22315277
- [2]Monagle P, Cuello CA, Augustine C, et al. American Society of Hematology 2018 Guidelines for management of venous thromboembolism: treatment of pediatric venous thromboembolism. Blood Adv, 2018.PMID 30482766
- [3]Raffini L, Huang YS, Witmer C, et al. Dramatic increase in venous thromboembolism in children's hospitals in the United States from 2001 to 2007. Pediatrics, 2009.PMID 19736261
- [4]Male C, Lensing AWA, Palumbo JS, et al. Rivaroxaban compared with standard anticoagulants for the treatment of acute venous thromboembolism in children: a randomised, controlled, phase 3 trial. Lancet Haematol, 2020.PMID 31699660
- [5]Monagle P, Lensing AWA, Thelen K, et al. Bodyweight-adjusted rivaroxaban for children with venous thromboembolism (EINSTEIN-Jr): results from three multicentre, single-arm, phase 2 studies. Lancet Haematol, 2019.PMID 31420317
- [6]Thom K, Lensing AWA, Nurmeev I, et al. Safety and efficacy of anticoagulant therapy in pediatric catheter-related venous thrombosis (EINSTEIN-Jr CVC-VTE). Blood Adv, 2020.PMID 33002131
- [7]Law C, Raffini L A guide to the use of anticoagulant drugs in children. Paediatr Drugs, 2015.PMID 25711916
- [8]van Ommen CH, Nowak-Göttl U Inherited Thrombophilia in Pediatric Venous Thromboembolic Disease: Why and Who to Test. Front Pediatr, 2017.PMID 28352625
- [9]Heleen van Ommen C, Middeldorp S Thrombophilia in childhood: to test or not to test. Semin Thromb Hemost, 2011.PMID 22187402
- [10]Mahajerin A, Obasaju P, Eckert G, et al. Thrombophilia testing in children: a 7 year experience. Pediatr Blood Cancer, 2014.PMID 24249220
- [11]Monagle P Diagnosis and management of deep venous thrombosis and pulmonary embolism in neonates and children. Semin Thromb Hemost, 2012.PMID 23034828
- [12]Pelland-Marcotte MC, Amiri N, Avila ML, et al. Low molecular weight heparin for prevention of central venous catheter-related thrombosis in children. Cochrane Database Syst Rev, 2020.PMID 32557627