Santangelo Gabrielle, Megas Andrew, Mandalapu Aniruddh, Haddas Ram, Mesfin Addisu
Department of Neurosurgery, University of Rochester Strong Memorial Hospital, Rochester, NY, USA.
Department of Orthopedic Surgery, University of Rochester Strong Memorial Hospital, Rochester, NY, USA.
Spine Deform. 2025 Jul;13(4):1189-1195. doi: 10.1007/s43390-025-01072-5. Epub 2025 Apr 14.
To characterize the patient demographics, medical comorbidities, spinal anatomic characteristics, and surgical management of patients with Klippel-Feil Syndrome (KFS).
An institutional billing database was used to identify patients with KFS, which was confirmed by the authors' review of imaging. The patient data were categorized by patient demographics, medical comorbidities, spinal anatomic characteristics, and surgical management.
In our cohort of 93 patients with KFS, more females than males (53% vs 47%) were affected. The mean age was 46 years old. The most common levels of fusion were C2-C3 (15%) and C5-C6 (13%). Following the Samartzis classification, 67% were type I, 14% were type II, and 22% were type III. There was a 29% occurence of scoliosis. Among those with scoliosis, 27% were diagnosed in adolescence, 40% had scoliosis in the cervical region, 20% thoracic, and 20% lumbar. A total of 26% of patients underwent spine surgery primarily for stenosis (6/24) and myelopathy (5/24). Of the spine surgeries, 58.3% were posterior-based procedures, 16.7% anterior and posterior-based procedures, 12.5% anterior-based procedures, 8.3% of patients had a spinal discectomy, and 1 patient underwent a torticollis release. A total of 18 out of 24 of the surgical procedures (75%) were at or adjacent to the level of congenital fusion.
KFS is commonly associated with degenerative changes, neural compression, and traumatic instability in intervertebral discs adjacent to fused vertebrae. Surgical intervention in patient with KFS largely involved posterior spinal fusions involving or adjacent to levels of congenital vertebral fusions.
描述Klippel-Feil综合征（KFS）患者的人口统计学特征、合并症、脊柱解剖特征及手术治疗情况。
利用机构计费数据库识别KFS患者，并经作者影像学审查确认。患者数据按人口统计学特征、合并症、脊柱解剖特征及手术治疗进行分类。
在我们93例KFS患者队列中，女性患者多于男性（53%对47%）。平均年龄为46岁。最常见的融合节段是C2-C3（15%）和C5-C6（13%）。根据Samartzis分类，67%为I型，14%为II型，22%为III型。脊柱侧弯发生率为29%。在脊柱侧弯患者中，27%在青春期被诊断出，40%的脊柱侧弯位于颈椎区域，20%位于胸椎，20%位于腰椎。共有26%的患者主要因狭窄（6/24）和脊髓病（5/24）接受脊柱手术。在脊柱手术中，58.3%为后路手术，16.7%为前后联合手术，12.5%为前路手术，8.3%的患者接受了椎间盘切除术，1例患者接受了斜颈松解术。24例手术中有18例（75%）在先天性融合节段或其附近进行。
KFS通常与融合椎体相邻椎间盘的退变、神经受压及创伤性不稳定有关。KFS患者的手术干预主要涉及在先天性椎体融合节段或其附近进行后路脊柱融合术。
