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 ...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.展开更多
The results of 31 cases of sutureless esophagogastrostomy by intraluminal elastic circular ligation (IECL) with the biodegradable supporting tube were reported. The fate of the supporting tube could be tracked satisfa...The results of 31 cases of sutureless esophagogastrostomy by intraluminal elastic circular ligation (IECL) with the biodegradable supporting tube were reported. The fate of the supporting tube could be tracked satisfactorily by X-ray, The tube-dislodge time was 15.03 +/- 2.23 days and unaffected by the size of supporting tube or the site of anastomosis, The supporting tube could be safely absorbed or partially discharged through the alimentary tract. IECL, with the merits of saving time, anastomosing tightly and leaving no suture materials in the anastomotic site, can be expected to further reduce the incidence of anastomotic leakage and provide references for other gastrointestinal anastomosis.展开更多
文摘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.
文摘The results of 31 cases of sutureless esophagogastrostomy by intraluminal elastic circular ligation (IECL) with the biodegradable supporting tube were reported. The fate of the supporting tube could be tracked satisfactorily by X-ray, The tube-dislodge time was 15.03 +/- 2.23 days and unaffected by the size of supporting tube or the site of anastomosis, The supporting tube could be safely absorbed or partially discharged through the alimentary tract. IECL, with the merits of saving time, anastomosing tightly and leaving no suture materials in the anastomotic site, can be expected to further reduce the incidence of anastomotic leakage and provide references for other gastrointestinal anastomosis.