Surgical treatment for scoliosis in patients with Shprintzen-Goldberg syndrome

Kota Watanabe, Eijiro Okada, Kenjiro Kosaki, Takashi Tsuji, Ken Ishii, Masaya Nakamura, Kazuhiro Chiba, Yoshiaki Toyama, Morio Matsumoto

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)186-193
Number of pages8
JournalJournal of Pediatric Orthopaedics
Volume31
Issue number2
DOIs
Publication statusPublished - 01-03-2011
Externally publishedYes

Fingerprint

Scoliosis
Therapeutics
Kyphosis
Wound Infection
Shprintzen Golberg craniosynostosis
Craniosynostoses
Pseudarthrosis
Postoperative Care
Connective Tissue
Bone and Bones

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Orthopedics and Sports Medicine

Cite this

Watanabe, Kota ; Okada, Eijiro ; Kosaki, Kenjiro ; Tsuji, Takashi ; Ishii, Ken ; Nakamura, Masaya ; Chiba, Kazuhiro ; Toyama, Yoshiaki ; Matsumoto, Morio. / Surgical treatment for scoliosis in patients with Shprintzen-Goldberg syndrome. In: Journal of Pediatric Orthopaedics. 2011 ; Vol. 31, No. 2. pp. 186-193.
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abstract = "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.",
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Watanabe, K, Okada, E, Kosaki, K, Tsuji, T, Ishii, K, Nakamura, M, Chiba, K, Toyama, Y & Matsumoto, M 2011, 'Surgical treatment for scoliosis in patients with Shprintzen-Goldberg syndrome', Journal of Pediatric Orthopaedics, vol. 31, no. 2, pp. 186-193. https://doi.org/10.1097/BPO.0b013e3182093da5

Surgical treatment for scoliosis in patients with Shprintzen-Goldberg syndrome. / Watanabe, Kota; Okada, Eijiro; Kosaki, Kenjiro; Tsuji, Takashi; Ishii, Ken; Nakamura, Masaya; Chiba, Kazuhiro; Toyama, Yoshiaki; Matsumoto, Morio.

In: Journal of Pediatric Orthopaedics, Vol. 31, No. 2, 01.03.2011, p. 186-193.

Research output: Contribution to journalArticle

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T1 - Surgical treatment for scoliosis in patients with Shprintzen-Goldberg syndrome

AU - Watanabe, Kota

AU - Okada, Eijiro

AU - Kosaki, Kenjiro

AU - Tsuji, Takashi

AU - Ishii, Ken

AU - Nakamura, Masaya

AU - Chiba, Kazuhiro

AU - Toyama, Yoshiaki

AU - Matsumoto, Morio

PY - 2011/3/1

Y1 - 2011/3/1

N2 - 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.

AB - 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.

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