BACKGROUND: Shprintzen-Goldberg syndrome (SGS) is characterized by 
craniosynostosis and marfanoid habitus. The clinical findings of SGS include 
neurological, cardiovascular, connective tissue, and skeletal abnormalities. 
Among these skeletal findings, developmental scoliosis is recognized in half of 
all patients with SGS. However, no earlier reports have described the surgical 
treatment of scoliosis associated with SGS.
METHODS: Four patients (2 boys and 2 girls; mean age at the time of surgery, 
7.3±4.4 y) with SGS who underwent surgical treatment for progressive scoliosis 
were reviewed. The radiologic findings, operative findings, and perioperative 
complications were evaluated.
RESULTS: The mean preoperative Cobb angle was 102.8±16.9 degrees. The curve 
patterns were a double curve in 2 cases and a triple curve in 2 cases. Local 
kyphosis at the thoracolumbar area was recognized in all the cases with a mean 
kyphosis angle of 49±16 degrees. Growing rod procedures were performed in 2 
patients, and posterior correction and fusion were performed in 2 patients. The 
mean correction rate was 45% in the patients who underwent the growing rod 
procedures at the time of growing rod placement and 51% in the patients who 
underwent posterior correction and fusion. Dislodgement of the proximal anchors 
occurred in 3 of the 4 patients. One patient developed pseudoarthrosis. Two 
patients developed deep wound infections, and implant removal was necessary in 1 
patient.
CONCLUSIONS: Surgical treatment for scoliosis in patients with SGS was 
associated with a high incidence of perioperative and postoperative 
complications including implant dislodgements and deep wound infections 
attributable to poor bone quality and a thin body habitus, which are 
characteristic clinical features of this syndrome. Careful preoperative surgical 
planning and postoperative care are critical for the surgical treatment of 
scoliosis associated with SGS, especially in infants requiring multiple 
surgeries.
LEVEL OF EVIDENCE: Level IV.