Early outcomes of growth friendly instrumentation in children with Williams syndrome

Although scoliosis and kyphosis have been associated with Williams Syndrome (WS), no previous literature has reported on surgical treatment for early onset scoliosis (EOS) in WS. The aim of this case series is to report on the outcomes of spine deformity surgery in patients with EOS and WS and any perioperative anesthetic or cardiovascular complications.

One multicenter database was queried for all patients with WS who underwent growth-friendly (GF) treatment before age 12 between 2000 and 2017. Demographics, surgical, and growth-friendly data were queried. Radiographs were measured for curve magnitude, T1-T12 length, and T1-S1 length.

Seven patients were analyzed (3 males, 4 females). Patients were at a median age of 2.8 years at initial surgery with median follow-up 3.6 years (range 2.0-12 years) after index surgery. The initial surgical treatments were as follows: 2 traditional growing rods (TGR), 2 magnetically controlled growing rods (MCGR), and 3 vertical expandable prosthetic titanium ribs (VEPTR). The median duration of growth-friendly treatment was 5.0 years (range, 2.6-10.4 years) with a median number of 9 device lengthenings. The median improvement in coronal curve magnitude from preoperative to most recent follow-up was 19° (range, 54°-9°). Three patients have completed GF treatment: one underwent definitive fusion, and two are under observation with apparent spontaneous fusion and retain the original GF implants. No peri-operative anesthetic or cardiovascular complications occurred.

Few studies have reported on surgical outcomes in WS patients with EOS. In this case series, 6/7 patients experienced curve improvement with growth-friendly spine instrumentation. This study suggests that growth-friendly instrumentation for severe EOS in WS can be used for control of spinal deformity while allowing for further growth. Associated complications were typical of distraction-based EOS surgical treatment. There were 62 total procedures with general anesthesia, but no perioperative cardiac complications occurred.