背景:脊髓支气管囊肿是由胚胎发育错误引起的罕见实体,由呼吸道上皮细胞组成。迄今为止,还有其他三个关于髓内囊肿的报道,部分切除,因此需要密切随访和监测。作者介绍了一例患者出现Klippel-Feil异常和上颈椎大的髓内支气管囊肿的情况。
方法:作者在进行C2-6椎板切除术时注意到C5-6椎板的融合。硬脑膜开放后,髓内病变有光滑,立即观察到从脊髓背侧出现的纤维成分。脊髓背侧未受累于该囊肿壁或其他较小的囊肿,都含有灰色液体。囊肿壁部分切除,送病理检查。
结论:脊柱发育性囊肿与其他解剖异常有关,比如Klippel-Feil异常,胚胎发育中的错误引起的。对于髓内病变,例如该患者的支气管囊肿,部分切除和减压是手术的目标,因为积极的减积可能导致神经系统受损。密切成像随访是必要的。
BACKGROUND: Spinal bronchogenic cysts are rare entities arising from errors in embryogenesis and consisting of respiratory epithelial cells. To date, there are three other published accounts of intramedullary cysts, which were partially resected and thereby warrant close follow-up and monitoring. The authors present an illustrative
case of a patient presenting with Klippel-Feil anomaly and a large intramedullary bronchogenic cyst in the upper cervical spine.
METHODS: The authors noted fusion of the C5-6 laminae as they performed the C2-6 laminectomy. After dural opening, an intramedullary lesion with a smooth, fibrous component emerging from the dorsal spinal cord was immediately observed. The dorsal spinal columns were not involved with this cyst wall or the other smaller cysts, which all contained gray fluid. The cyst walls were partially resected and sent for pathological examination.
CONCLUSIONS: Spinal developmental cysts are associated with other anatomical anomalies, such as Klippel-Feil anomaly, arising from errors in embryogenesis. For intramedullary lesions such as this patient\'s bronchogenic cyst, partial resection and decompression are the goals of surgery because aggressive debulking may lead to neurological compromise. Close imaging follow-up is warranted.