Ehlers-Danlos syndrome: case and pedigree report and review.
OBJECTIVE
To report a case and a firstly described pedigree with Ehlers-Danlos syndrome in China.


METHODS
Clinical materials of a case of Ehlers-Danlos syndrome type IV and a pedigree of 6 members with 4 generations were analyzed. Dilated internal jugular vein in the proband was removed operatively. The diagnosis, surgical treatment, and postoperative complications were retrospectively summarized. Etiology, clinical characteristics and classification of Ehlers-Danlos syndrome were also analysed.


RESULTS
Vessels of the proband in the pedigree were crisp and easily lacinated during the precedure for removal of his internal jugular vein. Repeat postoperative hemotomas were found though complete stanching was made during operation. The patient successfully recovered after prompt debridement in operative theatre and needle sucking. The other 5 members of the pedigrees all had the triads of Ehlers-Danlos syndrome, but no combined vascular diseases were found in them.


CONCLUSIONS
Though the morbidity rate was extremely low, green attention should be paid to the high mortality rate and complication of Ehlers-Danlos syndrome during surgical management of in patients with Ehlers-Danlos syndrome type IV. Surgeons should be aware of the ponderance of its complications and combined diseases to avoid fatal intraoprative vascular lacination and incontrollable hamorrhage.
Zheng
Yuehong
Y
Department of Vascular Surgery, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China.
Guan
Heng
H
Zhang
Jiefeng
J
Liu
Changwei
C
Li
Yongjun
Y
Li
Binglu
B
Huang
Zhongming
Z
D002681:China; D004535:Ehlers-Danlos Syndrome; D006801:Humans; D010375:Pedigree; D012189:Retrospective Studies