颅颈交界区-后颅窝区域的先天性异常：两例报告。
Congenital anomalies at the craniovertebral junction-posterior fossa region: report of two cases.
作者信息
Madhugiri Venkatesh S, Bhagavatula Indira Devi
机构信息
Department of Neurosurgery, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry, India.
Department of Neurosurgery, National Institute of Mental Health and Neuro Sciences (NIMHANS), Bangalore, Karnataka, India.
出版信息
J Neurol Surg A Cent Eur Neurosurg. 2013 Dec;74 Suppl 1:e279-83. doi: 10.1055/s-0033-1349335. Epub 2013 Aug 19.
BACKGROUND
Various combinations of bony and soft tissue anomalies have been described at the craniovertebral junction (CVJ). These include posterior fossa dermoids (with or without dermal sinuses) associated with Klippel-Feil anomalies and dermoids (no previous reports of overlying dermal sinuses) associated with bony anomalies.
PATIENTS
Two patients with a combination of CVJ anomalies are described.
RESULTS
Both patients had a posterior fossa dermoid, dermal sinus, and occipitalized C1 arch. Furthermore, one patient had a complex bony CVJ anomaly, and the other had a Klippel-Feil anomaly in addition. The clinical presentation and management are elucidated.
CONCLUSIONS
Patients who present with any one of these lesions need to be evaluated for the existence of other congenital anomalies. These are complex malformations to treat.
背景
颅颈交界区（CVJ）存在多种骨与软组织异常的组合情况。这些情况包括与Klippel-Feil异常相关的后颅窝皮样囊肿（伴或不伴有皮样窦道）以及与骨异常相关的皮样囊肿（此前无覆盖皮样窦道的报道）。
患者
描述了两名患有CVJ异常组合的患者。
结果
两名患者均有后颅窝皮样囊肿、皮样窦道和枕化的C1椎弓。此外，一名患者有复杂的CVJ骨异常，另一名患者还伴有Klippel-Feil异常。阐述了临床表现及治疗情况。
结论
出现这些病变中任何一种的患者都需要评估是否存在其他先天性异常。这些是复杂的畸形，治疗难度较大。
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