Background: Klippel-Feil syndrome (KFS) is a rare congenital disorder characterized by the fusion of two or more cervical vertebrae during early prenatal development. This fusion results from a failure of segmentation during the first trimester. Although six genes have previously been associated with KFS, they account for only a small proportion of cases.
View Article and Find Full Text PDFBackground: Patients with "sandwich" fusion (concomitant C1 occipitalization and C2-C3 nonsegmentation), a subtype of Klippel-Feil syndrome, are at particular risk for developing atlantoaxial dislocation (AAD). However, the clinical and surgical characteristics of AAD in patients with sandwich fusion have not been clearly defined.
Methods: A retrospective case-control study with a large sample size and a minimum 2-year follow-up was performed.
Purpose: To elucidate the anatomic relationship between the internal carotid artery (ICA) and the bony structures of the craniovertebral junction among "sandwich" atlantoaxial dislocation (AAD) patients, and to analyze the risks of injury during surgical procedures.
Methods: The distance from the medial wall of ICA to the midsagittal plane (D1), the shortest distance between the ICA wall and the anterior cortex of the lateral mass of atlas (LMA) (D2) on the most caudal and cranial levels of LMA and the angle (A) between the sagittal plane passing through the screw entry point of C1 lateral mass(C1LM) screw and the medial tangent line of the vessel passing through the entry point were measured. Besides, the location of ICA in front of the atlantoaxial vertebra was divided into 4 categories (Z1-Z4).
Objective: To summarize the vertebral artery (VA) pattern of 96 "sandwich" atlantoaxial dislocation (AAD) patients and to describe the strategies of reducing the injury of VA during surgery.
Methods: From 2009 to 2020, we retrospectively reviewed the 3-dimensional computed tomography angiography data of 96 AAD patients combined with atlas occipitalization and C2-3 fusion, which were diagnosed as "sandwich" AAD and 96 patients as control group patients who were without atlas occipitalization, C2-3 fusion and any other cervical bone deformity at our institution. The variations of each side of VA were described in 3 different parts (C0-1, C1-2, and C2-3) according to the characteristics of the 3-part pathological structures in "sandwich" subgroup.