Division of Plastic and Reconstructive Surgery, Children's Hospital of Philadelphia.
Division of Plastic and Reconstructive Surgery, Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center.
J Craniofac Surg. 2022;33(8):2372-2378. doi: 10.1097/SCS.0000000000008748. Epub 2022 Jul 22.
A subset of patients with metopic craniosynostosis are noted to have elevated intracranial pressure (ICP). However, it is not known if the propensity for elevated ICP is influenced by the severity of metopic cranial dysmorphology.
Children with nonsyndromic single-suture metopic synostosis were prospectively enrolled and underwent optical coherence tomography to measure optic nerve head morphology. Preoperative head computed tomography scans were assessed for endocranial bifrontal angle as well as scaled metopic synostosis severity score (MSS) and cranial morphology deviation score determined by CranioRate, an automated severity classifier.
Forty-seven subjects were enrolled between 2014 and 2019, at an average age of 8.5 months at preoperative computed tomography and 11.8 months at index procedure. Fourteen patients (29.7%) had elevated optical coherence tomography parameters suggestive of elevated ICP at the time of surgery. Ten patients (21.3%) had been diagnosed with developmental delay, eight of whom demonstrated elevated ICP. There were no significant associations between measures of metopic severity and ICP. Metopic synostosis severity score and endocranial bifrontal angle were inversely correlated, as expected ( r =-0.545, P <0.001). A negative correlation was noted between MSS and formally diagnosed developmental delay ( r =-0.387, P =0.008). Likewise, negative correlations between age at procedure and both MSS and cranial morphology deviation was observed ( r =-0.573, P <0.001 and r =-0.312, P =0.025, respectively).
Increased metopic severity was not associated with elevated ICP at the time of surgery. Patients who underwent later surgical correction showed milder phenotypic dysmorphology with an increased incidence of developmental delay.
有一部分额缝早闭患者存在颅内压升高（ICP）。但是，目前尚不清楚 ICP 升高的倾向是否受到额部颅面畸形严重程度的影响。
前瞻性纳入患有非综合征性单一额缝早闭的儿童，并进行光学相干断层扫描以测量视神经乳头形态。对术前头部 CT 扫描进行评估，包括颅内腔额角以及经 CranioRate 确定的额缝早闭严重程度评分（MSS）和颅形偏离评分，CranioRate 是一种自动严重程度分类器。
2014 年至 2019 年间共纳入 47 例患者，术前 CT 平均年龄为 8.5 个月，手术时平均年龄为 11.8 个月。14 例患者（29.7%）在手术时存在提示 ICP 升高的光学相干断层扫描参数。10 例患者（21.3%）被诊断为发育迟缓，其中 8 例存在 ICP 升高。额部严重程度测量值与 ICP 之间无显著相关性。额缝早闭严重程度评分与颅内腔额角呈负相关（r=-0.545，P<0.001）。MSS 与确诊的发育迟缓呈负相关（r=-0.387，P=0.008）。同样，手术时年龄与 MSS 和颅形偏离之间也呈负相关（r=-0.573，P<0.001 和 r=-0.312，P=0.025）。
手术时，额部严重程度增加与 ICP 升高无关。接受较晚手术矫正的患者具有较轻的表型畸形，发育迟缓的发生率增加。
