Hoang Thom Dang, Son Tran Thiet, Trung Dong Ha, Anh Hoang Tuan
Department of Plastic and Craniofacial Surgery, Vietnam National Children's Hospital.
Department of Plastic Surgery, Hanoi Medical University.
J Craniofac Surg. 2025;36(5):1556-1559. doi: 10.1097/SCS.0000000000011194. Epub 2025 Mar 11.
This study aimed to investigate the clinical characteristics according to Tessier classification and evaluate the surgical outcomes in patients with rare craniofacial cleft (RCC) primary repair. A retrospective study on 30 patients with RCC was conducted at the Department of Craniofacial and Plastic Surgery of the Vietnam National Hospital of Pediatrics. Rare craniofacial cleft was recorded according to Tessier's classification and was analyzed for gender, affected side, clinical characteristics, and associated abnormalities. Primary surgical correction was based on the concept of aesthetic units with multiple Z-plasty. Preoperative deformities and postoperative outcomes were evaluated with anthropometric measurements using the Versnel scoring system. The most common type was Tessier 7 cleft (T7), followed by T0. There was no difference between the frequency of males and females. Patients with unilateral cleft accounted for a larger proportion than bilateral cleft (76.5% versus 23.5%; P = 0.029 <0.05). The median and paramedian cleft groups (T0, T1, T30) affected orbit 22.2%-nose 77.8%-mouth 44.4%. The oblique clefts (T3, T4, T5, T11) affected orbit 100%-nose 50%-mouth 50%. The transverse cleft group (T6, T7, T8) affected mouth 94.1%-ear 29.4%. Rare craniofacial cleft may present alone or in a syndrome (Treacher Collin, Goldenhar, Hemifacial atrophy), or in combination with other abnormalities. Repairing RCC with aesthetic units with multiple Z-plasty has improved facial balance and restored key landmarks with acceptable scar position. The Versnel scoring system can serve as an objective instrument to measure the surgical outcomes of RCC repair and can be used to evaluate the influence of growth.
本研究旨在根据泰西埃分类法调查罕见颅面裂（RCC）一期修复患者的临床特征，并评估手术效果。越南国家儿童医院颅面整形外科对30例RCC患者进行了一项回顾性研究。根据泰西埃分类法记录罕见颅面裂情况，并分析性别、患侧、临床特征及相关异常情况。一期手术矫正基于美学单位概念并采用多个Z成形术。术前畸形和术后效果采用韦斯内尔评分系统通过人体测量进行评估。最常见的类型是泰西埃7型裂（T7），其次是T0型。男性和女性的发病率无差异。单侧裂患者占比高于双侧裂患者（76.5%对23.5%；P = 0.029<0.05）。正中裂和旁正中裂组（T0、T1、T30）累及眼眶22.2%、鼻77.8%、口44.4%。斜裂组（T3、T4、T5、T11）累及眼眶100%、鼻50%、口50%。横裂组（T6、T7、T8）累及口94.1%、耳29.4%。罕见颅面裂可能单独出现，或与综合征（如特雷彻·柯林斯综合征、戈尔登哈综合征、半侧颜面萎缩）相关，或与其他异常合并出现。采用多个Z成形术的美学单位修复RCC可改善面部平衡，并在瘢痕位置可接受的情况下恢复关键标志。韦斯内尔评分系统可作为衡量RCC修复手术效果的客观工具，并可用于评估生长的影响。
