额眶内牵张成骨术治疗突眼严重的学龄期Crouzon综合征

Frontal-orbital internal distraction for severe proptosis in school-aged Crouzon syndrome

目的探讨额眶内牵张成骨术治疗突眼严重的学龄期Crouzon综合征患儿的效果。方法回顾性分析2016年1月至2021年2月南京医科大学附属儿童医院应用额眶内牵张成骨术治疗的学龄期Crouzon综合征患儿临床资料,统计并分析临床效果、并发症(脑脊液漏、感染等)及手术安全性情况。结果共纳入12例患儿,男9例,女3例,年龄6岁~12岁1个月,平均7.1岁;颅型4例,颅面型6例,其他2例;均伴有明显的突眼畸形。手术过程顺利。随访3~20个月,平均14个月。术后头颅前后径延长距离为(19.1±3.3)mm,范围15.2~25.6 mm,头颅指数从术前的97.5%降低至术后的80.0%,颅面外形良好,突眼和后颅平坦外观均得到较好改善;除1例患儿出现牵张成骨器延长杆外置部位感染外,无额眶回缩、脑脊液漏、颅内感染等其他并发症发生。结论额眶内牵张成骨术治疗学龄期伴严重突眼的Crouzon综合征效果肯定,突眼改善明显,术后脑脊液漏及感染发生率低,效果稳定。该术式是伴严重突眼的大龄Crouzon综合征患儿的一种较好选择。

Objective To investigate the effect of intrafrontal-orbital distraction osteogenesis in the treatment for school-aged children with severe proptosis in Crouzon syndrome.Methods We retrospectively analyzed the clinical data of school-aged children with Crouzon syndrome treated with intrafrontal orbital distraction osteotomy at the Children’s Hospital of Nanjing Medical University from January 2016 to February 2021,and counted and analyzed the clinical results,complications(cerebrospinal fluid leakage,infection,etc.),and surgical safety.Results A total of 12 children,9 males and 3 females,aged 6-12.1 years,with an average age of 7.1 years,were included;4 were cranial,6 were craniofacial,and 2 were other types.All were shown with obvious proptosis.The surgical procedure was successful.Follow-up ranged from 3 to 20 months,with a mean of 14 months.The postoperative distraction distance was(19.1±3.3)mm,ranging from 15.2 to 25.6 mm.And the mean cranial index(CI)before and after surgery were 97.5%and 80.0%,respectively,with good craniofacial appearance and better improvement of proptosis and posterior cranial flattening.There were no other complications but for one child with infection at the external site of the distraction rod.Conclusions The effect of frontal-orbital distraction osteotomy in the treatment of Crouzon syndrome with severe proptosis in school-aged children was positive,with significant improvement in proptosis and low incidence of postoperative cerebrospinal fluid leakage and infection.This procedure is a good choice for older children with severe proptosis.