No abstract available.
Ehlers-Danlos Syndrome*

No abstract available.
Corneal Opacity ; Ehlers-Danlos Syndrome*

Ehlers-Danlos syndrome type IV(the arterial-ecchymotic type) which has skin fragility, easy bruisability, and j oint hyperextensibility is occasionally combined with large vessel involve-ment and spontaneous catastrophic bleeding. As even a small inj ury can cause profound vascular tearing and damage, surgical management is hazardous and often unrewarding. We report a successful surgical treatment of an acute type A aortic dissection associated with Ehlers-Danlos syndrome.
Ehlers-Danlos Syndrome* ; Hemorrhage ; Skin

Ehlers-Danlos Syndrome ; Humans ; Infant, Newborn ; Male

Ehlers-Danlos syndrome is an autosomal dominant hereditary disorder of connective tissue, while familial gigantiform cementoma is a condition that usually manifests as multiple radiopaque cementum-like masses throughout the jaws. This case report discusses the oral management and prosthetic rehabilitation of two patients presenting familial gigantiform cementoma with Ehlers-Danlos Syndrome.
Cementoma* ; Connective Tissue ; Ehlers-Danlos Syndrome ; Humans ; Jaw ; Rehabilitation

Ehlers-Danlos syndrome is an autosomal dominant hereditary disorder of connective tissue, while familial gigantiform cementoma is a condition that usually manifests as multiple radiopaque cementum-like masses throughout the jaws. This case report discusses the oral management and prosthetic rehabilitation of two patients presenting familial gigantiform cementoma with Ehlers-Danlos Syndrome.
Cementoma* ; Connective Tissue ; Ehlers-Danlos Syndrome ; Humans ; Jaw ; Rehabilitation

OBJECTIVETo report a case and a firstly described pedigree with Ehlers-Danlos syndrome in China.

METHODSClinical 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.

RESULTSVessels 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.

CONCLUSIONSThough 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.

China ; Ehlers-Danlos Syndrome ; Humans ; Pedigree ; Retrospective Studies

The Ehlers-Danlos syndrome is a familial disorder of connective tissue, and seven distinct clinical forms are reported. Author experienced two cases of the type 3 Ehlers-Danlos syndrome which present hypermobility of the joints, asymmetry of the thorax, anomalies of the thoracic spine, and flatfoot. So, we report these patients with references.
Connective Tissue ; Ehlers-Danlos Syndrome ; Flatfoot ; Humans ; Joints ; Spine ; Thorax

OBJECTIVETo describe a case of Ehlers-Danlos syndrome type IV and its pedigree in China.

METHODSClinical 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.

RESULTSVessels 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.

CONCLUSIONSThough 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.

China ; Ehlers-Danlos Syndrome ; genetics ; surgery ; Humans ; Jugular Veins ; surgery ; Male ; Middle Aged ; Pedigree

Ehlers-Danlos Syndrome ; pathology ; Humans ; Lung ; abnormalities ; pathology ; Male ; Patella ; abnormalities