SURGICAL MANAGEMENT OF EHLERS—DANLOS SYNDROME：FIRST REPORT OF A PEDIGREE IN CHINA
管珩;郑曰宏;刘昌伟;李拥军;李秉璐;刘暴摘要 Objective. To describe a case of Ehlers-Danlos syndrome type IV and its pedigree in China. Methods. 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. Results. 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. Conclusions. 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 lacination and incontrollable hemorrhage. 下载全文
Ehlers-Danlos综合征的研究进展郑曰宏;管珩中国现代普通外科进展Ehlers-Danlos综合征(EDS)又名弹力过度性皮肤(cutishyperelastica)、皮肤毛细管破裂(dermatorrhexis)、伴皮肤和关节松弛的皮肤毛细管破裂(dermatorrhexis with derma...