Benjamin J. Shore, Syeda Mehwish, Muhammad Amin Chinoy.
Response/Recommendation: The overall risk of venous thromboembolism (VTE) in children with chronic neuromuscular conditions is very low. Routine VTE prophylaxis in children with chronic neuromuscular conditions is not necessary unless additional VTE risk factors are identified.
Strength of Recommendation: Moderate.
Rationale: In comparison to adults, the incidence of VTE in children is significantly lower1-3. Large multicenter investigations of pediatric VTE have reported an incidence of 5.3 per 10,000 hospital admission and 0.7 per 100,000 children3,4. More than 80% of pediatric VTE events occur in children with 1 or more risk factors5. Furthermore, the incidence of ‘idiopathic’ VTE is only 5% in children, compared to 40% in adults6-11. Pediatric incidence of VTE has a bimodal distribution, with the highest proportions reported in infants aged 1 to 23 months and adolescent females7,12. The incidence peaks in adolescents due to contraception use, smoking, and obesity. Additionally, the composition of the hemostatic system in adolescents is simultaneously transitioning to the comparable adult system13,14. Despite the significant differences in the epidemiology and pathophysiology between pediatric and adult VTE, most clinical management guidelines for pediatric patients are extrapolated from adult literature without targeted evidence14,15.
In 2000, Feudtner et al.16, developed the definition for children with complex chronic conditions (CCC), of which there are 12 categories. Neuromuscular complex chronic conditions (NCCC) was defined to encompass a host of conditions including cerebral palsy, spina bifida, brain malformations, muscular dystrophy, and seizure disorder17. VTE events occurs much less frequently in disabled children than in their adult counterparts18. Children with NCCC frequently require hip and spine surgery that is resource expensive19. Additionally, spine and lower-extremity orthopaedic surgery can be associated with a substantial increase in VTE risk, attributed to immobility, and inconsistent use of thromboprophylaxis20. However, in children with NCCC, due to their chronic immobility, the general consensus is that the risk of VTE is rare, yet little research exists to support this assertion.
Adults with neuromuscular disease undergoing orthopaedic total joint arthroplasty (TJA) have been shown to experience an increased risk for perioperative complications21-24. Specifically, abnormalities in muscle tone associated with cerebral palsy, among others, are associated with a high rate of complications of the vascular system, especially deep venous thrombosis (DVT)25. In an adult study of 28 patients with severe motor and intellectual disabilities, the authors found that the rate of asymptomatic DVT was high. Additionally, thrombosis was absent in the soleal veins but present in the femoral and common femoral veins25. Conversely, similar findings in children have not been noted. Over a 4-year period at one rehabilitation institution, 532 children’s (< 18 years old) charts were reviewed, 9 of whom were diagnosed with a DVT. The authors found that the majority of these children had suffered a spinal cord injury leading to an acute change in their baseline mobility18. In this study, the majority of patients diagnosed with DVT were over 13 years of age, leading the authors to conclude that VTE chemoprophylaxis in the disabled pediatric population, especially prepubertal patients, should be questioned.
Previous research has demonstrated that overall complication rates are considerably higher amongst patients undergoing surgical correction for neuromuscular scoliosis, vs. those undergoing surgical correction for idiopathic scoliosis26-28. VTE is a known complication after pediatric spinal fusion29. However, a detailed review of the neuromuscular complications from the Scoliosis Research Society Morbidity and Mortality (SRSMM) database showed that the reported annual rate of VTE was low, varying between 0 to 0.31%.
Shore et al.17, utilized the Pediatric Health Information System Plus (PHIS+) database to assess the incidence of VTE after elective hip and spine surgery in children with NCCC. They found that of 4,583 pediatric patients undergoing orthopaedic surgery, only four experienced a VTE event, all of whom had a prior diagnosis of cerebral palsy. However, 2 of the 4 children experienced their VTE episode prior to surgery, resulting in a corrected VTE rate of 0.04%. It is also important to note that in both cases of VTE, patients had a preoperative diagnosis of a coagulation disorder, leading the authors to conclude that based on their findings, no prophylaxis is required in children with NCCC undergoing elective hip and spine surgery, unless other known risk factors are also present.
VTE in pediatric orthopaedic patients is a rare occurrence. Furthermore, limited data is currently available to guide decision-making and practice management. The presence of a neuromuscular disorder has been shown to increase the risk of VTE in adults undergoing TJA by 30%24. Despite this, a similar association has not been demonstrated in the limited literature on orthopaedic procedures in pediatric patients. In summary, based on the limited evidence available to date, VTE prophylaxis for children with NCCC undergoing orthopaedic surgery is not recommended, unless additional identified risk factors for thrombosis exist.
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