血友病患儿唇裂的手术治疗

血友病为一种X染色体伴隐性遗传的出血性疾病.血友病伴唇裂畸形病例更为少见.我院共收治3例血友病唇裂患儿,采用术前、术中、术后48小时内纠正凝血因子缺乏,使凝血因子Ⅷ、Ⅸ在血浆中的浓度术前分别保持在75%、50%以上,48小时后保持在30%、32%以上,手术年龄1岁左右,采用三角瓣法整复,术中注意减少创伤,缝合严密.3例经手术整复成功,无术后出血及复裂.

The Operative Treatment of Cleft Lip with Hemophilia

Hemophilia is a hemorrhagic disease of X-chromosome recessive inheritance. A hemophilia with cleft lip is more rarely. Three such patients were admitted to our hospital. Coagulation factors deficiency was corrected within the period of preoperation, intraoperation and 48h post operation. The plasma level of Factor VIII and Factor IX is beyond 75% and 50% before operation, 30% and 32,% in 48h after operation. The operative age is about one year. The triangular flap was used to perform the operation with little trauma and careful suture. This three cases were successfully repaired without postoperative hemorrhage and fissuration.