Prospective Analysis of Bruising in Children with and without an Inherited Bleeding Disorder.
Authors: Hamilton M. , Jenkins B. , Dunstan F. , Kemp A. , Liesner R. , Chalmers E. , Hanley J. , Jungmann A. , Thomas A. , Collins P. 
Published: October 2019

Abstract
All children bruise from everyday play and after accidents. However, children sustain bruises from underlying haemostatic disorders, and bruising is the commonest presentation of non-accidental injury (NAI). The finding of bruises in non-mobile babies is strongly suggestive of NAI, but, in the experience of haemophilia centres, bruising is recognized in non-mobile children with severe inherited bleeding disorders. This may explain why children with undiagnosed inherited bleeding disorders are often investigated for NAI. There are no published longitudinal cohort studies that profile bruising in children over time. After ethical approval and informed consent, three cohorts were recruited 1.Children without an inherited bleeding disorder 2.Children with a severe or moderate inherited bleeding disorder (FVIII/IX <5IU/dL, VWF:Ag <1IU/dL) 3.Children with a mild inherited bleeding disorder. We used body maps and a short proforma to document the frequency, pattern and causes of bruising, weekly for twelve weeks. In each cohort, patients were analyzed in the following groups: non mobile babies, babies who could roll, crawling and walking under age five. Children with prematurity, global developmental delay and coexisting illness were excluded. Children of any race and children with disability were eligible. 110 children were recruited into the trial, and completed data collection. There was a correlation with a child’s developmental stage and their bruise number. Control non-mobile babies did not bruise, but once they started to roll, they developed a small number of bruises. As they crawled, and then walked, the bruise number increased. The difference between the developmental stages was significant, Mann Whitney U test, p < 0.0001.
Bruise numbers - Control cohort Control Number Collections Total bruises Mean bruises / collection (95%CI)[SD] Baby 16 110 0 0 Rolling 8 62 28 0.45 (0.2)[0.8] Crawling 18 134 96 0.72 (0.2)[1.1] Walking 35 359 608 1.69 (0.2)[2.0]
However, children with severe bleeding disorders did develop bruising when non-mobile. Although the number of children in this cohort was small, due to the rarity of newly diagnosed severe bleeding disorders, these children had significantly more bruises than the control group. (Mann Whitney U test p< 0.0001).
Bruise number - Severe cohort Severe Number Collections Total bruises Mean bruises / collection(95%CI)[SD] Baby 3 43 29 0.6(0.3)[1.0] Rolling 4 21 23 1.1(0.8)[1.7] Crawling 4 29 74 2.6(1.0)[2.6] Walking 7 46 296 6.4(1.5)[5.1] Prophylaxis 14 130 562 4.3(0.6)[3.7]
There was no clear trend in bruise size with developmental stage for any group. However, there was a difference in bruise size between the cohorts - controls had smaller bruises than the mild and severe cohorts, when walking p < 0.0001. This study shows that children with severe bleeding disorders develop bruises before they are mobile and bruise more frequently than a control population - for Paediatricians and Haematologists involved in the field of child protection, the consequences of an incorrect diagnosis are significant. This data reinforces the recommendation that bleeding disorders must be excluded in children who present with bruising.