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.

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

Ehlers-Danlos syndrome (EDS) is a rare inherited connective disease. Among several subgroups, type IV EDS is frequently associated with spontaneous catastrophic bleeding from a vascular fragility. We report on a case of carotid-cavernous fistula (CCF) in a patient with type IV EDS. A 46-year-old female presented with an ophthalmoplegia and chemosis in the right eye. Subsequently, seizure and cerebral infarction with micro-bleeds occurred. CCF was completely occluded with transvenous coil embolization without complications. Thereafter, the patient was completely recovered. Transvenous coil embolization can be a good treatment of choice for spontaneous CCF with type IV EDS. However, every caution should be kept during invasive procedure.
Cerebral Infarction ; Ehlers-Danlos Syndrome* ; Embolization, Therapeutic ; Female ; Fistula* ; Hemorrhage ; Humans ; Middle Aged ; Ophthalmoplegia ; Seizures

The Ehlers-Danlos syndrome is a congenital anomaly having collagen metabolism with clinical and genetic heterogeneity. It has been classified into ten distinct clinical forms. Urinary bladder diverticula associated with Ehlers-Danlos syndrome appear to be relatively rare and all of the reported cases were male. A 7 years old female patient with Ehlers-Danlos syndrome visited to our hospital because of gross hematuria after blunt trauma to lower abdomen. Markedly distended urinary bladder with diverticulum was found on abdominopelvic computed tomography. Upper tract was unremarkable. We report a 7 years old female patient with Ehlers-Danlos syndrome associated with megabladder and bladder diverticulum.
Abdomen ; Child ; Collagen ; Diverticulum* ; Ehlers-Danlos Syndrome* ; Female* ; Genetic Heterogeneity ; Hematuria ; Humans ; Male ; Metabolism ; Urinary Bladder*