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