Clinical and haemodynamic risk factors associated with discrepancies in lower limb length with capillary malformations: data from the national paediatric French cohort CONAPE

Abstract : Background Genetics discoveries have allowed for a better understanding of capillary malformations (CMs) associated with overgrowth syndrome. However, molecular analyses are still not easy to perform or interpret. Other analytical methods are needed. Objectives To identify clinical and haemodynamic factors associated with leg length discrepancy (LLD) in children with CMs of the lower limbs. Methods Data were obtained from the multicentre French national cohort CONAPE (COhorte Nationale d'enfants atteints d'Angiome Plan de membrE inférieur), from children aged 2–12 years old with CMs of the lower limbs. Clinical characteristics were prospectively collected. Haemodynamic factors were measured by an sonographer who calculated the arterial blood flow (ABF) in both lower limbs. An ABF difference ≥ 50% between the two lower limbs was considered relevant. LLD ≥ 2% was determined by the same radiologist on centralized radiographs. Results We analysed data at baseline for 96 children. The mean ± SD age was 5·6 ± 3·1 years; 49 (51%) were male; and 14 (15%) showed LLD. In total, 32 patients (33%) had venous anomalies, 13 (14%) lymphatic anomalies and in one child a diagnosis of Parkes Weber syndrome was made. Only an increased circumference above the knee was more frequent with than without LLD (43% vs. 13%, P = 0·02). In all, 10/79 patients (13%) showed a difference in ABF ≥ 50%: four had LLD. The frequency of differences in ABF ≥ 50% was greater with than without LLD [33% (n = 4/12) vs. 9% (n = 6/67), P = 0·04]. Conclusions ABF measured by Duplex ultrasonography is a simple, low‐cost and noninvasive complementary examination for help in detecting LLD, with a difference of ≥ 50% possibly associated.
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https://hal-univ-bourgogne.archives-ouvertes.fr/hal-01737962
Contributeur : Lnc - Université de Bourgogne <>
Soumis le : mardi 20 mars 2018 - 09:40:49
Dernière modification le : vendredi 26 juillet 2019 - 01:19:15

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M. Samimi, M. Durieux-Verde, A. Caille, J. Mazereeuw-Hautier, O. Boccara, et al.. Clinical and haemodynamic risk factors associated with discrepancies in lower limb length with capillary malformations: data from the national paediatric French cohort CONAPE. British Journal of Dermatology, Wiley, 2018, 178 (2), pp.520 - 526. ⟨https://onlinelibrary.wiley.com/doi/pdf/10.1111/bjd.16017⟩. ⟨10.1111/bjd.16017⟩. ⟨hal-01737962⟩

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