Guan Heng, Zheng Yuehong, Liu Changwei, Li Yongjun, Li Binglu, Liu Bao
Department of Vascular Surgery, Peking Union Medical College Hospital, CAMS & PUMC, Beijing 100730.
Chin Med Sci J. 2002 Sep;17(3):178-82.
To describe a case of Ehlers-Danlos syndrome type IV and its pedigree in China.
Clinical materials of a case of Ehlers-Danlos syndrome type IV and a pedigree of 6 members within 4 generations were analyzed. Dilated internal jugular vein in the proband was removed by operation. The diagnosis, surgical treatment, and postoperative complications were retrospectively reviewed.
Vessels of the proband in the pedigree were crisp and easily lacinated during the procedure of removing his internal jugular vein. Repeating postoperative hematomas were found though complete stanching was achieved during the operation. The patient was successfully recovered by promptly debridgement and needle sucking. The other 5 members of the pedigree all had the triads of Ehlers-Danlos syndrome.
Though it was of extremely low morbidity rate, the high mortality rate and complication of Ehlers-Danlos syndrome deserve great attention during surgical management, especially in patients with Ehlers-Danlos syndrome type IV. Surgeons should be aware of the ponderance of its complications and combined diseases to avoid fatal intraoperative vascular lascination and incontrollable hemorrhage.
描述中国1例Ⅳ型埃勒斯-当洛综合征及其家系情况。
分析1例Ⅳ型埃勒斯-当洛综合征患者的临床资料及一个4代6人的家系。对先证者扩张的颈内静脉进行手术切除。回顾性分析诊断、手术治疗及术后并发症情况。
家系中先证者在颈内静脉切除过程中血管脆且易撕裂。术中虽实现完全止血，但术后仍反复出现血肿。通过及时清创和穿刺抽吸，患者成功康复。家系中的其他5名成员均有埃勒斯-当洛综合征三联征。
尽管Ⅳ型埃勒斯-当洛综合征发病率极低，但在手术治疗过程中，其高死亡率和并发症值得高度关注，尤其是对于Ⅳ型埃勒斯-当洛综合征患者。外科医生应意识到其并发症和合并疾病的严重性，以避免术中致命的血管撕裂和难以控制的出血。
