Anderson P J, Hall C M, Evans R D, Hayward R D, Jones B M
Craniofacial Centre, Great Ormond Street Hospital for Children, London, UK.
J Craniofac Surg. 1998 May;9(3):201-6. doi: 10.1097/00001665-199805000-00002.
Craniosynostosis may occur in conjunction with limb and visceral anomalies in more than 100 syndromes and may include anomalies of the elbow. Apert's, Pfeiffer's, Crouzon's, and Saethre-Chotzen syndrome have been linked with anomalies of the elbow, but the incidence and severity of such anomalies is unknown. A prospective radiographic study was undertaken to establish the types, incidence, and severity of elbow anomalies in patients with either Apert's, Pfeiffer's, Crouzon's, or Saethre-Chotzen syndrome attending the Craniofacial Centre at Great Ormond Street Hospital during a 12-month period. This study showed that elbow anomalies were very common in Apert's and Pfeiffer's syndrome, but less so in Crouzon's syndrome. The elbows in all patients with Saethre-Chotzen syndrome were normal. A range of anomalies was seen, with overlap between the syndromes. The severest anomaly seen in children with Crouzon's, Pfeiffer's, and Apert's syndrome was complete synostosis, which may require surgical intervention in due course. The results of this study suggest that the incidence of elbow anomalies in Apert's, Pfeiffer's, and Crouzon's syndrome is higher than the current literature suggests. Synostosis can be so severe that orthopedic review as part of the management of these children may be beneficial and may become increasingly important as more of these children survive into adulthood.
颅缝早闭可能与100多种综合征中的肢体和内脏异常同时出现，可能包括肘部异常。Apert综合征、Pfeiffer综合征、Crouzon综合征和Saethre-Chotzen综合征都与肘部异常有关，但此类异常的发生率和严重程度尚不清楚。一项前瞻性影像学研究旨在确定在大奥蒙德街医院颅面中心就诊的Apert综合征、Pfeiffer综合征、Crouzon综合征或Saethre-Chotzen综合征患者在12个月期间肘部异常的类型、发生率和严重程度。这项研究表明，肘部异常在Apert综合征和Pfeiffer综合征中非常常见，但在Crouzon综合征中较少见。所有Saethre-Chotzen综合征患者的肘部均正常。观察到一系列异常情况，各综合征之间存在重叠。在Crouzon综合征、Pfeiffer综合征和Apert综合征患儿中观察到的最严重异常是完全性骨融合，这可能需要在适当的时候进行手术干预。这项研究的结果表明，Apert综合征、Pfeiffer综合征和Crouzon综合征中肘部异常的发生率高于当前文献所显示的情况。骨融合可能非常严重，以至于作为这些儿童管理一部分的骨科评估可能是有益的，并且随着越来越多的此类儿童存活至成年，其重要性可能会日益增加。
