额眶面骨发育不全症的治疗结果：颅后窝撑开延长术与额眶部推进术的比较。

Apert Syndrome Outcomes: Comparison of Posterior Vault Distraction Osteogenesis Versus Fronto Orbital Advancement.

机构信息

Institute of Plastic and Craniofacial Surgery, SOBRAPAR Hospital.

Department of Neurology, University of Campinas (UNICAMP), Campinas, Brazil.

出版信息

J Craniofac Surg. 2022;33(1):66-69. doi: 10.1097/SCS.0000000000007959.

DOI:10.1097/SCS.0000000000007959

PMID:34261966

Abstract

BACKGROUND

Presenting a wide clinical spectrum and large variety of clinical features, successful treatment of Apert syndrome necessitates performance of sequential multiple surgeries before a patient's facial skeleton growth is complete.The objective of this study is to compare forehead contour asymmetry and clinical outcomes between Apert patients who underwent either fronto-orbital advancement (FOA) or posterior vault distraction osteogenesis (PVDO).

METHODS

A retrospective study was performed on consecutive patients with Apert syndrome who underwent either FOA or PVDO between 2007 and 2019, and participated in at least 6 months of follow-up care. Forehead contour asymmetry and surgical outcomes for each of the included patients were verified through medical records, clinical photographs, and interviews with the parents of the patients. The need for additional craniofacial procedures based on the surgical outcomes of each patient was graded from I to IV utilizing the Whitaker outcome classification system.

RESULTS

Forehead contour asymmetry for all included patients was rated under the Whitaker grading scale as type II (n = 4) 44.4%, type III (n = 2) 22.2%, and type IV (n = 3) 33.3% for FOA, and type I (n = 5) 35.7%, type II (n = 7) 50%, and type III (n = 2) 14.3%, for PVDO (P < 0.05). The average transfused blood volume was 47.77 ± 9.42 mL/kg for patients who underwent FOA, and 22.75 ± 10.31 mL/kg for patients who underwent PVDO (P < 0.05).

CONCLUSIONS

Patients who underwent PVDO had lower forehead contour asymmetry as per the Whitaker outcome grading scale than patients who underwent FOA.

摘要

背景

Apert 综合征表现出广泛的临床谱和多种临床特征，在患者面颅骨生长完全之前，需要进行多次序贯手术才能获得成功的治疗。本研究的目的是比较接受额眶前移术（FOA）或颅后窝容积扩大术（PVDO）的 Apert 综合征患者的额部轮廓不对称和临床结果。

方法

对 2007 年至 2019 年间接受 FOA 或 PVDO 的连续 Apert 综合征患者进行回顾性研究，且至少接受了 6 个月的随访。通过病历、临床照片和对患者家长的访谈，验证了每位患者的额部轮廓不对称和手术结果。根据每位患者的手术结果，使用 Whitaker 结果分类系统对需要进行额外的颅面手术的情况进行分级，从 I 级到 IV 级。

结果

所有纳入患者的额部轮廓不对称根据 Whitaker 分级系统评定为 FOA 型 II（n = 4）44.4%、型 III（n = 2）22.2%和型 IV（n = 3）33.3%，以及 PVDO 型 I（n = 5）35.7%、型 II（n = 7）50%和型 III（n = 2）14.3%（P < 0.05）。行 FOA 的患者平均输血量为 47.77±9.42ml/kg，而行 PVDO 的患者平均输血量为 22.75±10.31ml/kg（P < 0.05）。