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Bone Joint J. 2019 Dec;101-B(12):1563-1569. doi: 10.1302/0301-620X.101B12.BJJ-2019-0735.R1.

Results of growth-friendly management of early-onset scoliosis in children with and without skeletal dysplasias: a matched comparison.

Author information

1
Department of Paediatric Orthopaedic Surgery, University of Turku and Turku University Hospital, Turku, Finland.
2
Department of Orthopaedics and Sports Medicine, Seattle Children's Hospital, Seattle, Washington, USA.
3
Pediatric Spine Study Group, Children's Spine Foundation, Valley Forge, Pennsylvania, USA.
4
Department of Orthopaedics, Faculty of Medicine, Hacettepe University, Sihhiye, Ankara, Turkey.
5
Department of Pediatric Orthopaedics and Spine Surgery, Children's Hospital Colorado, Aurora, Colorado, USA.
6
Division of Pediatric Orthopaedic Surgery, Rainbow Babies & Children's Hospital, Case Western Reserve University, Cleveland, Ohio, USA.
7
Department of Orthopedics, Texas Scottish Rite Hospital, Dallas, Texas, USA.
8
Department of Orthopaedic Surgery, Shiners Hospitals for Children, Philadelphia, Pennsylvania, USA.
9
Columbia University Medical Center, New York, New York, USA.
10
Department of Orthopaedic Surgery, University of California-San Diego, La Jolla, San Diego, California, USA.
11
Department of Orthopaedic Surgery, The Johns Hopkins University, Baltimore, Maryland, USA.

Abstract

AIMS:

The aim of this study was to compare the surgical and quality-of-life outcomes of children with skeletal dysplasia to those in children with idiopathic early-onset scoliosis (EOS) undergoing growth-friendly management.

PATIENTS AND METHODS:

A retrospective review of two prospective multicentre EOS databases identified 33 children with skeletal dysplasia and EOS (major curve ≥ 30°) who were treated with growth-friendly instrumentation at younger than ten years of age, had a minimum two years of postoperative follow-up, and had undergone three or more lengthening procedures. From the same registries, 33 matched controls with idiopathic EOS were identified. A total of 20 children in both groups were treated with growing rods and 13 children were treated with vertical expandable prosthetic titanium rib (VEPTR) instrumentation.

RESULTS:

Mean preoperative major curves were 76° (34° to 115°) in the skeletal dysplasia group and 75° (51° to 113°) in the idiopathic group (p = 0.55), which were corrected at final follow-up to 49° (13° to 113°) and 46° (12° to 112°; p = 0.68), respectively. T1-S1 height increased by a mean of 36 mm (0 to 105) in the skeletal dysplasia group and 38 mm (7 to 104) in the idiopathic group at the index surgery (p = 0.40), and by 21 mm (1 to 68) and 46 mm (7 to 157), respectively, during the distraction period (p = 0.0085). The skeletal dysplasia group had significantly worse scores in the physical function, daily living, financial impact, and parent satisfaction preoperatively, as well as on financial impact and child satisfaction at final follow-up, than the idiopathic group (all p < 0.05). The domains of the 24-Item Early-Onset Scoliosis Questionnaire (EOSQ24) remained at the same level from preoperative to final follow-up in the skeletal dysplasia group (all p > 0.10).

CONCLUSION:

Children with skeletal dysplasia gained significantly less spinal growth during growth-friendly management of their EOS and their health-related quality of life was significantly lower both preoperatively and at final follow-up than in children with idiopathic EOS. Cite this article: Bone Joint J 2019;101-B:1563-1569.

KEYWORDS:

Children; Early-onset scoliosis; Growth-friendly spinal instrumentation; Health-related quality of life; Skeletal dysplasia; Spinal growth

[Indexed for MEDLINE]

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