ESPE2023 Poster Category 1 Growth and Syndromes (75 abstracts)
Does cervical medullary decompression have an impact on growth in children with achondroplasia?
Daniela Fava 1,2 , Caterina Tedesco 2 , Alessia Angelelli 1,2 , Flavia Napoli 2 , Carlotta Pepino 1 , Alessia Pepe 1 , Daniela Teruzzi 1 , Angelica Pisati 1 , Maria Fay Cortella 1 , Maria Binelli 1 , Mohamad Maghnie 1,2 , Natascia Di Iorgi 1,2 & Anna Elsa Maria Allegri 2
1University of Genoa, Genoa, Italy. 2Department of Pediatric, IRCCS Istituto Giannina Gaslini, Genoa, Italy
Background: Foramen magnum stenosis (FMS) is a life-threatening complication in children with achondroplasia (ACH) which may require cervicomedullary decompression (CMD). There is no evidence if FMS and CMD affects growth in children with ACH.
Aim: To evaluate the impact of FSM and CMD surgery on anthropometric measurements in children with ACH.
Methods: Sixty-five patients with ACH (32 females -F-, 33 males-M-) were evaluated between 4-6 yrs of age. Height SDS (H-SDS), weight SDS (W-SDS) and body mass index (BMI) SDS (BMI-SDS) were calculated according to Merker et al.
Results: Thirty-nine children (60%; 21 F, 18 M) underwent CMD, n= 21 (32%; 13 F, 8 M) within the first yr (mean 0.62±0.20 yrs) and n= 18 after the age of 1 yr (mean 1.84±0.84 yrs). Mean age at anthropometric evaluation was 5.2± 0.4 yrs. H-SDS of patients with CMD was significantly lower (-0.55±0.99 vs 0.85±1.24, P=0.01) and even lowest when CMD occurred after 1 yr of age (-0.81±0.95 vs 0.07±1.04, P=0.006). Furthermore, H-SDS was more affected in M (-0.90±1.06 vs-0.10±1.22, P=0.01) than F. There were no significant differences in BMI-SDS, but in patients without FMS W-SDS was significantly higher than in patients underwent CMD after 1 yr of age (-0.07±1.22 vs -0.86±0.97, P=0.03).
Conclusions: H-SDS evaluated at the age of 5 years is more affected in children with ACH who underwent CMD. Furthermore, height and weight appear to be influenced by age at the time of surgery. Our data suggest that FMS and/or CMD may have a negative impact on growth in children with ACH, and that early initiation of medical therapy is desirable in these children.
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