Tessier 7 cleft

  • 文章类型: Journal Article
    Tessier号7个裂口,被称为面部外侧裂,是一个罕见且研究不足的实体,发病率为1/80,000-1/300,000活产。除了口周组织异常表现为巨大口炎,Tessier7裂口还涉及底层骨结构的异常。它可以作为综合症的一部分出现,如Treacher-Collins综合征或Goldenhar/Orbito-Auriculo-Vestarospective,或作为具有可变表达式的孤立形式(单边或双边)。Tessier7裂的双侧上颌重复被认为极为罕见,只考虑了以前提出的两个案例。鉴于文献中介绍的病例主要集中在临床表现和手术治疗上,没有提供足够的成像,我们的目标是在评估涉及的结构方面提出Tessier7裂隙的关键放射学特征,这对于治疗方法和最终结果至关重要。一名17岁的男性,嘴唇不称职且正畸异常,被转诊到我们的放射科进行正畸造影(OPG)和CT检查。异质记忆数据显示,在出生后2个月进行了手术治疗,以喂养连合c裂。口内检查显示上颌裂和多余牙齿。由于给定的临床表现尚无定论,放射诊断在阐明这个复杂实体方面占据了优先地位。
    Tessier No. 7 cleft, known as lateral facial cleft, is a rare and understudied entity with an incidence of 1/80,000-1/300,000 live births. Besides perioral tissue abnormalities manifesting as macrostomia, Tessier 7 cleft also involves anomalies of the underlying bony structures. It can appear as part of a syndrome, such as Treacher-Collins syndrome or Goldenhar/Orbito-Auriculo-Vestibular Spectrum, or as an isolated form (unilateral or bilateral) with variable expressions. Bilateral maxillary duplication in Tessier 7 cleft is considered extremely rare, accounting for only two previously presented cases. Given that the cases presented in the literature mainly focus on clinical appearance and surgical treatment, without providing sufficient imaging, we aim to present key radiological features of Tessier 7 cleft in terms of evaluating the involved structures, which is essential for the therapeutic approach and final outcome. A 17-year-old male with incompetent lips and orthodontic abnormalities was referred to our Radiology Department for orthopantomography (OPG) and CT examinations. Hetero-anamnestic data revealed a history of surgical treatment of the commissural cleft conducted 2 months after the birth to enable feeding. Intraoral examination showed a maxillary cleft and supernumerary teeth. Since the given clinical presentation was inconclusive, radiological diagnostics took precedence in elucidating this complex entity.
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  • 文章类型: Case Reports
    先天性大口,或者Tessier7号裂缝,是一种罕见的颅面异常.我们介绍了一名独特的双侧大口患者,包括左侧的“双”横向裂口和右侧的单个横向裂口。分阶段重建方法用于修复“双”左侧裂隙。这种分阶段的技术产生了令人满意的美学和功能结果。
    Congenital macrostomia, or Tessier number 7 cleft, is a rare craniofacial anomaly. We present a unique patient with bilateral macrostomia that consisted of a \"double\" transverse cleft on the left side and a single transverse cleft on the right side. A staged reconstructive approach was used to repair the \"double\" left-sided clefts. This staged technique produced a satisfactory aesthetic and functional outcome.
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  • 文章类型: Case Reports
    Goldenhar syndrome, a variant of hemifacial microsomia, is a well-known developmental anomaly of maxillofacial skeleton that is apparent at birth. The first and second branchial arch involvement during early embryonic development results in a wide spectrum of anomalies that may include macrostomia and lateral facial clefts. Though clefts of the orofacial region are among the most common congenital facial defects, the occurrence of lateral facial clefts (Tessier 7 cleft) in conditions such as the Goldenhar syndrome, is very rare (<5%). The lateral facial cleft, which results because of improper development of the perioral muscles of the face, gives an appearance of macrostomia giving rise to potential psychological, aesthetic and feeding problems. This clinical report describes the closure of a Tessier 7 cleft and the use of distraction osteogenesis to treat mandibular asymmetry in an 11-year-old female patient with Goldenhar\'s syndrome.
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