<strong>Background:</strong> Klippel Feil syndrome (KFS) is a congenital malformation characterised by the presence of, at least, one fused cervical segment and results from faulty segmentation along the e...<strong>Background:</strong> Klippel Feil syndrome (KFS) is a congenital malformation characterised by the presence of, at least, one fused cervical segment and results from faulty segmentation along the embryo’s developing axis during weeks 3 - 8 of gestation. The KFS increases the risk for spinal cord injury after minor trauma as a result of the disturbance of the biomechanics of the cervical spine. Persons with KFS often have associated congenital anomalies. <strong>Aim:</strong> The purpose of this study was to show the surgical management difficulties of this pathology in a low income country such as Senegal and to make a review of the literature. <strong>Case presentation:</strong> A 32-year-old man developed a tetraparesis secondary to a fall from his height while carrying a bag of rice on his head. Radiological explorations revealed fusedC2-C3 and C4-C5 vertebral bodies with a C3-C4 disc herniation associated to a spine contusion. The patient underwent surgical removal of the herniated disc via an anterior approach followed by interbody fusion and anterior plating. A complete recovery was noted at 4 months follow-up. <strong>Conclusion:</strong> Understanding of the cervical spine biomechanics of Klippel-Feil anomaly may allow an optimal management of patients. Patients with KFS should be warned of the increased risk of spinal cord injury after a low velocity trauma. Timing for surgery should be shortened.展开更多
Klippel-Feil syndrome(KFS) is defined by congenital cervical vertebral spine fusion and is seen with a wide spectrum of dental manifestations and craniofacial profiles. Previous studies on lateral cephalograms have do...Klippel-Feil syndrome(KFS) is defined by congenital cervical vertebral spine fusion and is seen with a wide spectrum of dental manifestations and craniofacial profiles. Previous studies on lateral cephalograms have documented an association between fusion of the cervicalvertebrae and deviations in the craniofacial profile in non-syndromic patients with severe malocclusion. To our knowledge, no previous studies have described the craniofacial profile including the cranial base of KFS patients on lateral cephalograms. Therefore KFS and its craniofacial and dental manifestations were described according to existing literature and additionally the craniofacial profile and cranial base was analysed on lateral cephalograms of two patients with KFS. According to the literature the dental manifestations of KFS-patients included oligodontia, overjet, cross bite, open bite and deep bite. The craniofacial profile was clinically described as reduced lower facial height, midfacial hypoplasia, and mandibular prognathia. The analyses of the two lateral cephalograms showed increased mandibular inclination, increased vertical jaw-relationship, increased jaw angle and maxillary retrognathia. The cranial base was normal in both cases. The sagittal jaw relationship and mandibular prognathia varied between the two cases. The literature review and the analyses of the two lateral cephalograms have shown that deviations in the occipital and cervical spine field as KFS were associated with deviations in the teeth and craniofacial profile.展开更多
文摘<strong>Background:</strong> Klippel Feil syndrome (KFS) is a congenital malformation characterised by the presence of, at least, one fused cervical segment and results from faulty segmentation along the embryo’s developing axis during weeks 3 - 8 of gestation. The KFS increases the risk for spinal cord injury after minor trauma as a result of the disturbance of the biomechanics of the cervical spine. Persons with KFS often have associated congenital anomalies. <strong>Aim:</strong> The purpose of this study was to show the surgical management difficulties of this pathology in a low income country such as Senegal and to make a review of the literature. <strong>Case presentation:</strong> A 32-year-old man developed a tetraparesis secondary to a fall from his height while carrying a bag of rice on his head. Radiological explorations revealed fusedC2-C3 and C4-C5 vertebral bodies with a C3-C4 disc herniation associated to a spine contusion. The patient underwent surgical removal of the herniated disc via an anterior approach followed by interbody fusion and anterior plating. A complete recovery was noted at 4 months follow-up. <strong>Conclusion:</strong> Understanding of the cervical spine biomechanics of Klippel-Feil anomaly may allow an optimal management of patients. Patients with KFS should be warned of the increased risk of spinal cord injury after a low velocity trauma. Timing for surgery should be shortened.
文摘Klippel-Feil syndrome(KFS) is defined by congenital cervical vertebral spine fusion and is seen with a wide spectrum of dental manifestations and craniofacial profiles. Previous studies on lateral cephalograms have documented an association between fusion of the cervicalvertebrae and deviations in the craniofacial profile in non-syndromic patients with severe malocclusion. To our knowledge, no previous studies have described the craniofacial profile including the cranial base of KFS patients on lateral cephalograms. Therefore KFS and its craniofacial and dental manifestations were described according to existing literature and additionally the craniofacial profile and cranial base was analysed on lateral cephalograms of two patients with KFS. According to the literature the dental manifestations of KFS-patients included oligodontia, overjet, cross bite, open bite and deep bite. The craniofacial profile was clinically described as reduced lower facial height, midfacial hypoplasia, and mandibular prognathia. The analyses of the two lateral cephalograms showed increased mandibular inclination, increased vertical jaw-relationship, increased jaw angle and maxillary retrognathia. The cranial base was normal in both cases. The sagittal jaw relationship and mandibular prognathia varied between the two cases. The literature review and the analyses of the two lateral cephalograms have shown that deviations in the occipital and cervical spine field as KFS were associated with deviations in the teeth and craniofacial profile.
