PDF(Pubmed)
Abstract：
UNASSIGNED: The Klippel-Feil syndrome (KFS) is a rare congenital anomaly characterized by the fusion of cervical vertebrae, which may be associated with other malformations, such as dermoid tumors and teratoma. Some theories explain the embryology of these associations. Another condition that may be present is the dermal sinus (DS), communication between intracranial tumors and the subcutaneous tissue, and predisposing infections. This case report aims to describe an association between these three pathologies as well as correlate them from the literature. This report was based on medical records retrospectively reviewed associated with the systematic bibliographical consultation using indexed databases based on inclusion and exclusion methods.
UNASSIGNED: An adult male patient, 24 years old, was admitted to our service, presenting fever and meningeal irritation as initial symptoms. In the patient\'s clinical history, he was diagnosed with an occipital DS in his childhood, which was previously instructed to be operated on by another neurosurgical team, but the patient chose not to perform the procedure. The magnetic resonance imaging investigation showed a DS associated with a cerebellar infected mass with 2 cm on its main diameter. The patient was treated with preoperative antibiotic therapy and underwent gross total surgical resection of the tumor as well as DS correction, confirmed in the histopathological examination as a teratoma. After surgery, further computed tomography scan analysis showed the presence of cervical vertebrae fusion, compatible with KFS diagnosis.
UNASSIGNED: The association between KFS, cerebellar teratoma, and DS has not yet been described in the literature, with only the association of the first two being extremely rare.
UNASSIGNED: An adult male patient, 24 years old, was admitted to our service, presenting fever and meningeal irritation as initial symptoms. In the patient\'s clinical history, he was diagnosed with an occipital DS in his childhood, which was previously instructed to be operated on by another neurosurgical team, but the patient chose not to perform the procedure. The magnetic resonance imaging investigation showed a DS associated with a cerebellar infected mass with 2 cm on its main diameter. The patient was treated with preoperative antibiotic therapy and underwent gross total surgical resection of the tumor as well as DS correction, confirmed in the histopathological examination as a teratoma. After surgery, further computed tomography scan analysis showed the presence of cervical vertebrae fusion, compatible with KFS diagnosis.
UNASSIGNED: The association between KFS, cerebellar teratoma, and DS has not yet been described in the literature, with only the association of the first two being extremely rare.
摘要：
■Klippel-Feil综合征（KFS）是一种罕见的先天性异常，其特征是颈椎融合，可能与其他畸形有关，例如皮样肿瘤和畸胎瘤。一些理论解释了这些关联的胚胎学。可能存在的另一种情况是真皮窦（DS），颅内肿瘤和皮下组织之间的沟通，和易感感染。本病例报告旨在描述这三种病理之间的关联，并将它们与文献相关联。本报告基于与系统书目咨询相关的病历回顾性审查，使用基于纳入和排除方法的索引数据库。
■一名成年男性患者，24岁,被接纳为我们的服务，以发热和脑膜刺激为初始症状。在患者的临床病史中，他在童年时期被诊断出患有枕骨DS,先前被指示由另一个神经外科小组进行手术，但患者选择不进行手术.磁共振成像研究显示，DS与小脑感染的肿块相关，其主要直径为2厘米。患者接受了术前抗生素治疗，并接受了肿瘤的大体全手术切除以及DS矫正，在组织病理学检查中证实为畸胎瘤。手术后,进一步的计算机断层扫描分析显示颈椎融合的存在，与KFS诊断兼容。
■KFS，小脑畸胎瘤,DS尚未在文献中描述，只有前两者的关联极为罕见。
■一名成年男性患者，24岁,被接纳为我们的服务，以发热和脑膜刺激为初始症状。在患者的临床病史中，他在童年时期被诊断出患有枕骨DS,先前被指示由另一个神经外科小组进行手术，但患者选择不进行手术.磁共振成像研究显示，DS与小脑感染的肿块相关，其主要直径为2厘米。患者接受了术前抗生素治疗，并接受了肿瘤的大体全手术切除以及DS矫正，在组织病理学检查中证实为畸胎瘤。手术后,进一步的计算机断层扫描分析显示颈椎融合的存在，与KFS诊断兼容。
■KFS，小脑畸胎瘤,DS尚未在文献中描述，只有前两者的关联极为罕见。
