Kalantar-Hormozi Abdoljalil, Abbaszadeh-Kasbi Ali, Goravanchi Farhood, Davai Nazanin Rita
*Department of Plastic and Craniofacial Surgery, Medical College of Shahid Beheshti University of Medical Science (SBMU), 15 Khordad Hospital †Tehran University of Medical Science ‡Medical College of Shahid Beheshti University of Medical Science (SBMU), 15 Khordad Hospital §Private Practice, Tehran, Iran.
J Craniofac Surg. 2017 Jul;28(5):e467-e470. doi: 10.1097/SCS.0000000000003771.
Craniofacial clefts are extremely rare congenital malformations that have adverse functional, psychosocial, and aesthetic effects on patients' life. Although the exact incidence is unclear, it is estimated between 1.4 and 4.9 per 100,000 live births. Prevalence of the rare craniofacial clefts is imprecise due to the paucity of literature as well as their etiologies.
All the patients with rare craniofacial clefts during 10 years in a plastic surgery tertiary referral hospital were included, and Tessier craniofacial clefting classification was used for classifying the clefts.
Of 964 patients with craniofacial clefts, 80 (8.29%) patients were identified with rare craniofacial clefts. There were 39 (48.7%) males and 41 (51.3%) females. Family history was determined positive in 30 (37.5%) patients. Tessier number 0 (58.7%) was the most common cleft in the authors' study. Tessier numbers 8, 13, and 30 were the rarest clefts. There was no patient with Clefts numbers 5, 6, or 9. Maternal smoking during pregnancy was observed in 1 (1.3%) of the women and 3 of the women had used drugs, 1 of them used the dexamethasone tablets and 2 of them could not remember name of the used drug.
Tessier number 0 was the most common cleft and Tessier numbers 8, 13, and 30 were the rarest types. The precise etiology of rare craniofacial clefts remained undetermined in this study. Women should be educated about the risk factors and subsequent ways of preventing from these risk factors.
颅面裂是极其罕见的先天性畸形，对患者的生活有不良的功能、心理社会和美学影响。虽然确切发病率尚不清楚，但估计每10万例活产中为1.4至4.9例。由于文献匮乏及其病因，罕见颅面裂的患病率尚不准确。
纳入一家整形外科三级转诊医院10年间所有患有罕见颅面裂的患者，并采用泰西埃颅面裂分类法对裂进行分类。
在964例颅面裂患者中，80例（8.29%）被确定为患有罕见颅面裂。其中男性39例（48.7%），女性41例（51.3%）。30例（37.5%）患者家族史呈阳性。泰西埃0号裂（58.7%）是作者研究中最常见的裂。泰西埃8号、13号和30号裂是最罕见 的裂。没有患者患有5号、6号或9号裂。1名（1.3%）女性在孕期有吸烟史，3名女性曾使用药物，其中1名使用地塞米松片，2名记不起所使用药物的名称。
泰西埃0号裂是最常见的裂，泰西埃8号、13号和30号裂是最罕见的类型。本研究中罕见颅面裂的确切病因仍未确定。应教育女性了解危险因素以及预防这些危险因素的后续方法。
