Assessment of long-term quality of life in patients with syndromic craniosynostosis
Assessment of long-term quality of life in patients with syndromic craniosynostosis
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DOI：
10.1016/j.bjps.2020.08.102
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发表时间：
2021-01-19
影响因子：
2.7
通讯作者：
Kishi, Kazuo
中科院分区：
文献类型：
--
作者：
Sakamoto, Yoshiaki;Takenouchi, Toshiki;Kishi, Kazuo
一些研究分析了颅骨成形术和面中部牵引术在颅缝早闭患者中的长期稳定性;然而，很少有研究调查了综合征性颅缝早闭成人的长期生活质量（QOL）和并发症。本研究旨在探讨成人综合征性颅缝早闭患者的生活质量（社会、身体和心理社会）。年龄≥ 20岁的综合征性颅缝早闭患者，在一家颅面机构接受手术治疗，被纳入本研究。我们调查了日常不便（使用世界卫生组织残疾评估表问卷），任何正在进行的治疗，婚姻状况和孩子的数量。共有18例患者（平均年龄：31.4 ± 9.2岁）回答了问卷（Crouzon综合征，9例; Apert综合征，5例; Pfeiffer综合征，4例）。其中，只有一名Crouzon综合征患者已婚，她也是唯一一个有孩子的人。Apert综合征患者被发现有困难的理解，沟通，和自我照顾，因为他们的精神发育迟滞和手和脚的残疾，但是，他们的参与社会是最积极的。相比之下，Crouzon综合征患者的社会参与度特别低。在所有患者中，任何正在进行的医院治疗都是由于眼科疾病。Crouzon综合征患者的生活质量极差;没有精神发育迟滞和手脚残疾迫使他们生活在主流社会中，他们在情感上装备不良。因此，有必要对这些患者进行无任何残余畸形的治疗，以提供心理支持并创造一个接受的社会。(C)2020年英国整形，重建和美容外科医生协会。由爱思唯尔有限公司出版。保留所有权利。
Several studies have analyzed the long-term stability of cranioplasty and mid-face distraction in patients with craniosynostosis; however, few studies have investigated long-term quality of life (QOL) and complications in adults with syndromic craniosynostosis. This study aimed to investigate the QOL (social, physical, and psychosocial) of patients with adult syndromic craniosynostosis. Patients aged >= 20 years with syndromic craniosynostosis, who were surgically treated at a single craniofacial institution, were included in this study. We investigated everyday inconvenience (using the World Health Organization Disability Assessment Schedule questionnaire), any ongoing treatment, marital status, and number of children. Totally, 18 patients aged 22-48 years (mean: 31.4 +/- 9.2 years) answered the questionnaire (Crouzon syndrome, 9; Apert syndrome, 5; Pfeiffer syndrome, 4). Of these, only one Crouzon syndrome patient was married; she was also the only one with a child. Apert syndrome patients were found to have difficulty in understanding, communication, and self-care because of their mental retardation and hand and foot handicaps; however, their participation in society was the most aggressive. In contrast, Crouzon syndrome patients had especially poor participation in society. In all patients, any ongoing hospital treatment was due to ophthalmological conditions. Crouzon syndrome patients have extremely poor QOL; the absence of mental retardation and hand and foot handicaps forces them to live in mainstream society, for which they are emotionally ill-equipped. It is necessary to treat these patients without any residual deformity to provide psychological support and to create an accepting society. (C) 2020 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.