Department of Neurosurgery, Miyagi Children's Hospital, 4-3-17 Ochiai, Aoba-ku, Sendai, Miyagi, 989-3126, Japan.
Department of Neurosurgery, Kitasato University School of Medicine, Sagamihara, Japan.
Childs Nerv Syst. 2024 Feb;40(2):575-580. doi: 10.1007/s00381-023-06141-6. Epub 2023 Sep 6.
Sagittal suture synostosis is one of the most common craniosynostoses and is often diagnosed by characteristic narrow and long skull shape, scaphocephaly. However, some patients with sagittal suture synostosis do not present with typical scaphocephaly, making early diagnosis difficult. In this study, five cases of characteristic skull deformity showing a narrowing of the cranium posterior to the coronal suture on computed tomography (CT) are presented. The three older children presented with papilledema and intellectual disability and a closed sagittal suture on CT. The two infant cases were diagnosed with the characteristic cranial deformities with aggravation of the deformity over time, but sagittal suture closure was not evident on CT. All patients underwent cranial remodeling surgery. In the two infant cases, the histopathological findings showed that the anterior part of the sagittal suture was firmly fused with fibrous tissue without bony fusion. These findings suggested that narrowing of the cranium posterior to the coronal suture might be due to functional fusion of the anterior portion of the sagittal suture prior to bony fusion. In an infant presenting with such a deformity that shows aggravation of the deformity over time, surgical treatment should be considered.
矢状缝早闭是最常见的颅缝早闭之一，其特征性表现为颅骨狭窄且狭长，舟状头畸形。然而，一些矢状缝早闭患者并不表现出典型的舟状头畸形，这使得早期诊断变得困难。本研究中，我们报告了 5 例具有特征性颅骨畸形的病例，这些病例在 CT 上表现为冠状缝后颅腔狭窄。3 例年长儿童表现为视乳头水肿和智力障碍，CT 显示矢状缝闭合。2 例婴儿病例被诊断为具有特征性的头颅畸形，随着时间的推移畸形加重，但 CT 上未见矢状缝闭合。所有患者均接受了颅骨重塑手术。在 2 例婴儿病例中，组织病理学检查发现矢状缝前部与纤维组织牢固融合，无骨性融合。这些发现提示冠状缝后颅腔狭窄可能是由于矢状缝前部在骨性融合之前发生了功能性融合。对于出现这种进行性加重的畸形的婴儿，应考虑手术治疗。
