Sulong Shukriyah, Alias Azmi, Johanabas Fadzlishah, Yap Abdullah Johari, Idris Badrisyah
Department of Neurosurgery, Hospital Kuala Lumpur, Kuala Lumpur.
Department of Neurosciences, School of Medical Sciences, Universiti Sains Malaysia.
J Craniofac Surg. 2020 Jan/Feb;31(1):46-50. doi: 10.1097/SCS.0000000000005810.
Craniosynostosis is a congenital defect that causes ≥1 suture to fuse prematurely. Cranial expansion surgery which consists of cranial vault reshaping with or without fronto-orbital advancement (FOA) is done to correct the skull to a more normal shape of the head as well as to increase the intracranial volume (ICV). Therefore, it is important to evaluate the changes of ICV after the surgery and the effect of surgery both clinically and radiologically.
The aim of this study is to evaluate the ICV in primary craniosynostosis patients after the cranial vault reshaping with or without FOA and to compare between syndromic and nonsyndromic synostosis group, to determine factors that associated with significant changes in the ICV postoperative, and to evaluate the resolution of copper beaten sign and improvement in neurodevelopmental delay after the surgery.
This is a prospective observational study of all primary craniosynostosis patients who underwent operation cranial vault reshaping with or without FOA in Hospital Kuala Lumpur from January 2017 until Jun 2018. The ICV preoperative and postoperative was measured using the 3D computed tomography (CT) imaging and analyzed. The demographic data, clinical and radiological findings were identified and analyzed.
A total of 14 cases (6 males and 8 females) with 28 3D CT scans were identified. The mean age of patients was 23 months. Seven patients were having syndromic synostosis (4 Crouzon syndromes and 3 Apert syndromes) and 7 nonsyndromic synostosis. The mean preoperative ICV was 880 mL (range, 641-1234 mL), whereas the mean postoperative ICV was 1081 mL (range, 811-1385 mL). The difference was 201 mL which was statistically significant (P < 0.001). In comparison, the mean volume increment for syndromic synostosis and nonsyndromic synostosis was 282 mL and 120 mL, respectively. The difference was statistically significant (P < 0.004). Three months post-operation, the copper beaten sign was still present in the CT scan which was statistically not significant in this study (P > 1.0). However, there was 100% (n = 13) improvement of this copper beaten sign. However, the neurodevelopmental delay showed no improvement which was statistically not significant (P > 1.0).
Surgery in craniosynostosis patient increases the ICV besides it improves the shape of the head. From this study, the syndromic synostosis had better increment of ICV compared to nonsyndromic synostosis.
颅缝早闭是一种先天性缺陷，导致≥1条颅缝过早融合。颅骨扩张手术包括颅骨重塑，可伴有或不伴有额眶前移（FOA），目的是将颅骨矫正为更正常的头部形状，并增加颅内体积（ICV）。因此，从临床和放射学角度评估手术后ICV的变化以及手术效果非常重要。
本研究旨在评估原发性颅缝早闭患者在进行伴有或不伴有FOA的颅骨重塑术后的ICV，并比较综合征性和非综合征性颅缝早闭组，确定与术后ICV显著变化相关的因素，以及评估手术后“铜击征”的消退情况和神经发育迟缓的改善情况。
这是一项前瞻性观察研究，对象为2017年1月至2018年6月在吉隆坡医院接受伴有或不伴有FOA的颅骨重塑手术的所有原发性颅缝早闭患者。术前和术后的ICV通过三维计算机断层扫描（CT）成像进行测量和分析。收集并分析人口统计学数据、临床和放射学检查结果。
共纳入14例患者（6例男性，8例女性），有28次三维CT扫描结果。患者的平均年龄为23个月。7例患者为综合征性颅缝早闭（4例克鲁宗综合征和3例阿佩尔综合征），7例为非综合征性颅缝早闭。术前ICV的平均值为880mL（范围为641 - 1234mL），而术后ICV的平均值为1081mL（范围为811 - 1385mL）。差值为201mL，具有统计学意义（P < 0.001）。相比之下，综合征性颅缝早闭和非综合征性颅缝早闭的平均体积增加量分别为282mL和120mL。差异具有统计学意义（P < 0.004）。术后3个月，CT扫描中仍存在“铜击征”，在本研究中无统计学意义（P > 1.0）。然而，该“铜击征”有100%（n = 13）得到改善。但是，神经发育迟缓没有改善，无统计学意义（P > 1.0）。
颅缝早闭患者的手术除了改善头部形状外，还增加了ICV。从本研究来看，综合征性颅缝早闭的ICV增加量比非综合征性颅缝早闭更好。
