Post-thrombotic syndrome in children: a systematic review of frequency of occurrence, validity of outcome measures, and prognostic factors

Abstract

BackgroundPost-thrombotic syndrome is a manifestation of chronic venous insufficiency following deep venous thrombosis. This systematic review was conducted to critically evaluate pediatric evidence on frequency of occurrence, validity of outcome measures, and prognostic indicators of post-thrombotic syndrome. Design and MethodsA comprehensive literature search of original reports revealed 19 eligible studies, totaling 977 patients with upper/lower extremity deep venous thrombosis. Calculated weighted mean frequency of post-thrombotic syndrome was 26% (95% confidence interval: 23-28%) overall, and differed significantly by prospective/non-prospective analysis and use/non-use of a standardized outcome measure. ResultsStandardized post-thrombotic syndrome outcome measures included an adaptation of the Villalta scale, the Clinical-Etiologic-Anatomic-Pathologic classification, and the Manco-Johnson instrument. Data on validity were reported only for the Manco-Johnson instrument. No publications on post-thrombotic syndrome-related quality of life outcomes were identified. Candidate prognostic factors for post-thrombotic syndrome in prospective studies included use/non-use of thrombolysis and plasma levels of factor VIII activity and D-dimer. ConclusionsGiven that affected children must endure chronic sequelae for many decades, it is imperative that future collaborative pediatric prospective cohort studies and trials assess as key objectives and outcomes the incidence, severity, prognostic indicators, and health impact of post-thrombotic syndrome, using validated measures.

Phase 3, single‐arm, multicenter study of dabigatran etexilate for secondary prevention of venous thromboembolism in children: Rationale and design

“…Use of recognized pediatric‐specific VTE and bleeding‐event classification criteria13 and of a pediatric PTS scoring system10, 37, 38 will also aid such comparisons.…”

“…Further, although estimates of rates of PTS in children vary considerably,7, 9, 10, 11 it has been found to be associated with increased morbidity and cost 39. There have been far fewer studies on the natural history, treatment, and secondary prevention of VTE in children than in adults 1, 9, 40.…”

“…Estimates of the rate of pediatric VTE recurrence vary widely,6, 7 ranging between 2% and 21% 8. Post‐thrombotic syndrome (PTS), another long‐term consequence of VTE, has been reported in 12‐65% of children following an initial VTE 1, 7, 9, 10, 11, 12. Risk factors associated with VTE recurrence include underlying chronic conditions associated with a prothrombotic tendency, such as malignancies, cardiac/vascular abnormalities, short bowel syndrome with total parenteral nutrition dependency, or congenital disorders (eg, severe inherited thrombophilias).…”

Pediatric post-thrombotic syndrome in children: Toward the development of a new diagnostic and evaluative measurement tool

“…A systematic review of PTS in the pediatric population reported an overall frequency of 26% [95% Confidence Interval (CI): [23][24][25][26][27][28] and a frequency of 17% [95% CI: [14][15][16][17][18][19][20] among prospective studies (9), including both UE and LE venous territories.…”

“…A recent systematic review identified two outcome-assessment tools that are commonly used in pediatric patients: the Modified Villalta Scale and the Manco-Johnson Instrument (9).…”