Surgical treatment for scoliosis in patients with Shprintzen-Goldberg syndrome

J Pediatr Orthop. 2011 Mar;31(2):186-93. doi: 10.1097/BPO.0b013e3182093da5.


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.

MeSH terms

  • Arachnodactyly / physiopathology
  • Child
  • Child, Preschool
  • Craniosynostoses / physiopathology
  • Female
  • Humans
  • Intraoperative Complications / epidemiology
  • Kyphosis / etiology
  • Kyphosis / surgery
  • Lumbar Vertebrae / surgery
  • Male
  • Marfan Syndrome / physiopathology
  • Postoperative Complications / epidemiology
  • Scoliosis / etiology
  • Scoliosis / surgery*
  • Surgical Wound Infection / etiology
  • Thoracic Vertebrae / surgery

Supplementary concepts

  • Shprintzen Golberg craniosynostosis