title: Surgical management of Ehlers-Danlos syndrome: first report of a pedigree in China.
abstract: 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 lascination and incontrollable hemorrhage.
authors.affiliation: Department of Vascular Surgery, Peking Union Medical College Hospital, CAMS & PUMC, Beijing 100730.
authors.forename: Heng
authors.initials: H
authors.lastname: Guan
authors.forename: Yuehong
authors.initials: Y
authors.lastname: Zheng
authors.forename: Changwei
authors.initials: C
authors.lastname: Liu
authors.forename: Yongjun
authors.initials: Y
authors.lastname: Li
authors.forename: Binglu
authors.initials: B
authors.lastname: Li
authors.forename: Bao
authors.initials: B
authors.lastname: Liu
mesh_terms: D002681:China; D004535:Ehlers-Danlos Syndrome / Q000235:genetics* / Q000601:surgery*; D006801:Humans; D007601:Jugular Veins / Q000601:surgery; D008297:Male; D008875:Middle Aged; D010375:Pedigree