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Objective <p>Klippel‒Feil syndrome (KFS) frequently coexists with congenital cervicothoracic scoliosis (CTS), but its impact on coronal alignment and surgical prognosis remains unclear. This study aimed to assess the differences in coronal morphology and surgical outcomes between CTS patients with and without KFS.</p> Methods <p>Seventy-three CTS patients who underwent hemivertebra (HV) resection and fusion were included retrospectively and divided into KFS (<i>n</i> = 43) and non-KFS (<i>n</i> = 30) groups. Primary coronal parameters (cervicothoracic curve, head tilt/shift) and secondary coronal compensatory parameters (trunk tilt, cranial/distal compensatory curves [CCC/DCC]) were measured preoperatively, postoperatively, and at final follow-up. Radiographic parameters, coronal subtypes, fixation length, blood loss, surgical duration, and complications were compared between the two groups.</p> Results <p>In the KFS group, 53.5% of patients had cervical fusion involving three or more segments, and 74.4% of patients were classified as Samartzis type III (multiple contiguous congenital fusions). Before surgery, the KFS group exhibited a smaller CCC but greater head tilt, head shift, trunk tilt, and DCC compared to the non-KFS group (all <i>P</i> &lt; 0.05). In the KFS group (60.5%), the proportion of patients with significant trunk tilt or compensatory thoracic curves (Type B or Type C) was notably higher than in the non-KFS group (10.0%, <i>P</i> &lt; 0.001). Although there were significant improvements in all deformity parameters after surgery (all <i>P</i> &lt; 0.05), at the last follow-up, the head shift, trunk tilt, and DCC in the KFS group showed a notable worsening compared to postoperative values and were significantly greater than those in the non-KFS group (all <i>P</i> &lt; 0.05). Despite having longer initial fixation segments (<i>P</i> = 0.002), five patients in the KFS group required revision surgery due to progressive trunk tilt and DCC, compared to only one patient in the non-KFS group. No significant differences were observed between the groups regarding blood loss, surgical duration, or neurological complications.</p> Conclusions <p>KFS-associated CTS is prone to develop secondary trunk tilt or DCC in the coronal plane. Such deformities may not be effectively corrected by initial short-segment osteotomy surgery and may largely continue to progress with growth and potentially require revision surgery thereafter. Long-term surveillance should thus be considered.</p>

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The influence of Klippel-Feil syndrome on coronal plane deformity characteristics and surgical outcomes in congenital cervicothoracic scoliosis: a comparative analysis

  • 期刊论文

  • 发表日期：

    2025年9月26日

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  • Kai Sun,
  • Song Li,
  • Benlong Shi,
  • Xu Sun,
  • Zhen Liu,
  • Zezhang Zhu,
  • Yong Qiu,
  • Saihu Mao

摘要

Objective

Klippel‒Feil syndrome (KFS) frequently coexists with congenital cervicothoracic scoliosis (CTS), but its impact on coronal alignment and surgical prognosis remains unclear. This study aimed to assess the differences in coronal morphology and surgical outcomes between CTS patients with and without KFS.

Methods

Seventy-three CTS patients who underwent hemivertebra (HV) resection and fusion were included retrospectively and divided into KFS (n = 43) and non-KFS (n = 30) groups. Primary coronal parameters (cervicothoracic curve, head tilt/shift) and secondary coronal compensatory parameters (trunk tilt, cranial/distal compensatory curves [CCC/DCC]) were measured preoperatively, postoperatively, and at final follow-up. Radiographic parameters, coronal subtypes, fixation length, blood loss, surgical duration, and complications were compared between the two groups.

Results

In the KFS group, 53.5% of patients had cervical fusion involving three or more segments, and 74.4% of patients were classified as Samartzis type III (multiple contiguous congenital fusions). Before surgery, the KFS group exhibited a smaller CCC but greater head tilt, head shift, trunk tilt, and DCC compared to the non-KFS group (all P < 0.05). In the KFS group (60.5%), the proportion of patients with significant trunk tilt or compensatory thoracic curves (Type B or Type C) was notably higher than in the non-KFS group (10.0%, P < 0.001). Although there were significant improvements in all deformity parameters after surgery (all P < 0.05), at the last follow-up, the head shift, trunk tilt, and DCC in the KFS group showed a notable worsening compared to postoperative values and were significantly greater than those in the non-KFS group (all P < 0.05). Despite having longer initial fixation segments (P = 0.002), five patients in the KFS group required revision surgery due to progressive trunk tilt and DCC, compared to only one patient in the non-KFS group. No significant differences were observed between the groups regarding blood loss, surgical duration, or neurological complications.

Conclusions

KFS-associated CTS is prone to develop secondary trunk tilt or DCC in the coronal plane. Such deformities may not be effectively corrected by initial short-segment osteotomy surgery and may largely continue to progress with growth and potentially require revision surgery thereafter. Long-term surveillance should thus be considered.