Abulezz Tarek Abdelhameed, Allam Karam A, Wan Derrick C, Lee Justine C, Kawamoto Henry K
From the Plastic Surgery Department, Sohag University, Sohag, Egypt.
Hagey Laboratory for Pediatric Regenerative Medicine, Division of Plastic and Reconstructive Surgery, Stanford University School of Medicine, Stanford, CA.
Ann Plast Surg. 2020 Sep;85(3):251-255. doi: 10.1097/SAP.0000000000002391.
Saethre-Chotzen syndrome is a genetic condition characterized by craniofacial and limb anomalies, with craniosynostosis (mainly coronal) being the most frequent craniofacial finding. Cranial and facial deformities can be extremely variable requiring individualization of treatment strategies. We present our case series to highlight clinical findings, treatment philosophy, and challenges facing Saethre-Chotzen patients.
A retrospective review was performed on records of patients given a diagnosis of Saethre-Chotzen syndrome at the University of California Los Angeles (UCLA) Craniofacial Clinic (n = 7) between 1980 and 2010. Patients with complete records were included in this study, and review of demographic data, clinical findings, surgical interventions and postoperative follow-up, and stability were performed.
Seven patients (1 male and 6 female) were included in this study. The average age at which the patients were first seen was 6.5 years. Suture involvement was bicoronal (n = 6) and unicoronal (n = 1). There was 1 patient having superimposed metopic synostosis, and there was another patient having Kleeblattschädel deformity. Previous procedures performed for patients before establishing care at UCLA were strip craniectomy (n = 2) and fronto-orbital advancement (n = 2). All patients (n = 7) had fronto-orbital advancements at UCLA. Other skeletal operations included the following: redo forehead advancement and contouring (n = 3), monobloc advancement (n = 1), and LeFort III distraction (n = 1). Five patients reached skeletal maturity, and 2 patients received LeFort I advancement for class III malocclusion, one of which also required a bilateral sagittal split osteotomy of the mandible.
Clinical presentation and severity of deformity in Saethre-Chotzen syndrome are variable. Our current report reviews our treatment strategies and illustrates the predominance of cranial and upper face deformities and frequent need for redo surgeries to address forehead asymmetry in this group of syndromic craniosynostosis patients.
赛-乔综合征是一种遗传性疾病，其特征为颅面和肢体异常，颅缝早闭（主要为冠状缝）是最常见的颅面表现。颅面畸形差异极大，需要针对个体制定治疗策略。我们展示我们的病例系列，以突出赛-乔综合征患者的临床发现、治疗理念及面临的挑战。
对1980年至2010年间在加利福尼亚大学洛杉矶分校（UCLA）颅面诊所被诊断为赛-乔综合征的患者记录进行回顾性研究（n = 7）。本研究纳入记录完整的患者，并对人口统计学数据、临床发现、手术干预、术后随访及稳定性进行了回顾。
本研究纳入7例患者（1例男性，6例女性）。患者首次就诊的平均年龄为6.5岁。缝线受累情况为双侧冠状缝（n = 6）和单侧冠状缝（n = 1）。有1例患者合并额缝早闭，另有1例患者患有三角头畸形。在患者于UCLA接受治疗前，曾接受的手术包括颅骨条带切除术（n = 2）和额眶前移术（n = 2）。所有患者（n = 7）在UCLA均接受了额眶前移术。其他骨骼手术包括：再次前额前移及轮廓整形（n = 3）、整块前移术（n = 1）和勒福III型牵张成骨术（n = 1）。5例患者达到骨骼成熟，2例患者因III类错牙合接受勒福I型前移术，其中1例还需要进行双侧下颌矢状劈开截骨术。
赛-乔综合征的临床表现和畸形严重程度各不相同。我们目前的报告回顾了我们的治疗策略，并说明了在这组综合征性颅缝早闭患者中，颅骨和上半面部畸形的主导地位以及频繁需要再次手术以解决前额不对称问题。
