罕见先天性右面部不典型面横裂伴颊瘘及下颌骨角化囊肿1例报道

Congenital atypical transverse facial cleft of the right face with buccal fistula and odontogenic keratocyst of mandible:A rare case report

面横裂属于颅面裂畸形中的一种临床类型。面横裂是一种较唇腭裂更为少见的先天性面裂畸形,其发生是由于胚胎时上颌突与下颌突未能完全融合所致,表现为口角至颊部呈水平裂开,除口颊畸形外,还可伴有第一鳃弓的发育畸形,如颜面一侧发育不良,耳前瘘管及附耳等畸形。面横裂的发生机制目前尚不清楚,可能与遗传、营养或环境因素有关。本文就临床中发现的1例先天性右面部不典型面横裂伴颊瘘合并同侧下颌骨角化囊肿的病例进行报道,具有一定的临床参考价值。

Transverse facial cleft is a clinical type of craniofacial cleft malformation,which constitutes a rare congenital facial cleft deformity compared to cleft lip and palate.The pathogenesis of transverse facial cleft arises from incomplete fusion of maxillary process and mandibular process during embryo,resulting in a transverse facial cleft extending from the oral commissure to the buccal region.In addition to the oral and buccal malformations,developmental malformations of the first branchial arch may also co-occur,such as unilateral facial hypoplasia,preauricular fistula,appendage and other deformity.The precise pathogenesis remains unclear,potentially stemming from genetic,nutritional,or environmental factors.We present a clinical case of congenital atypical transverse facial cleft of the right face with buccal fistula and odontogenic keratocyst of mandible,thereby contributing to the clinical understanding of this condition.