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Σάββατο 30 Σεπτεμβρίου 2017

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

Summary

Background

Genetics discoveries have allowed for better understanding capillary malformations (CMs) with overgrowth syndrome. However, molecular analyses are still not easy to perform or interpret. Other analytical methods are needed.

Objective

We aimed to identify clinical and hemodynamic factors associated with leg length discrepancy (LLD) in children with CM of lower limbs.

Methods

Data were obtained from the multicentre French national cohort CONAPE (COhorte Nationale d'enfants atteints d'Angiome Plan de membrE inférieur), including children from 2 to 12 years old with CM of lower limbs. Clinical characteristics were prospectively collected. Hemodynamic factors were measured by an echographer 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 analyzed 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. Thirty-two patients (33%) had venous anomalies, 13 (14%) lymphatic anomalies, and in 1 child, diagnosis of Parkes Weber syndrome was made. Only increased circumference above the knee was more frequent with than without LLD (50% vs 13%, p=0.02). In all, 10/79 patients (13%) showed a difference in ABF ≥50%: 4 had LLD. The frequency of differences in ABF ≥50% was greater with than without LLD [33.3% (n=4/12) vs 9.0% (n=6/67), p=0.04].

Conclusions

ABF measured by Duplex ultrasonography is a simple, low-cost and non-invasive complementary examination for help in detecting LLD, with a difference ≥ 50% possibly associated.

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