Paediatric Neurosurgery Unit, Clinical Department of Neurosurgery, University Medical Centre Ljubljana, 1000 Ljubljana, Slovenia.
Clinical Department of Maxillofacial and Oral Surgery, University Medical Centre Ljubljana, 1000 Ljubljana, Slovenia.
J Integr Neurosci. 2022 Jun 1;21(4):106. doi: 10.31083/j.jin2104106.
Craniosynostosis is a rare congenital disease of the skull. They arise when one or more cranial sutures ossify prematurely. This causes an obstruction to normal brain growth and leads to specific deformations of the skull, which may result in intracranial hypertension and cognitive delay.
We have retrospectively analysed all children treated at the Unit of paediatric neurosurgery of the University Medical Centre Ljubljana between June 2015 and September 2020. The following items have been recorded: affected suture, underlying syndromic condition, hydrocephalus, Chiari malformation, raised intracranial pressure, age at surgery, surgical technique, need for multiple operations and surgical complications.
During the study period, 71 children have been treated for craniosynostosis. The median postoperative follow-up was 31 months. There were: 54.9% sagittal, 25.3% metopic, 14.0% unicoronal, 1.4% bicoronal and 1.4% unilateral lambdoid craniosynostosis. Multiple sutures were affected in 2.8% cases. 7.0% of the cases were syndromic. Overall, 74 surgical procedures have been performed: frontoorbital advancement represented 40.5% of them; biparietal remodelling 32.4%: total cranial vault remodelling 22.9%; posterior distraction 2.7%; posterior expansion 1.3%. Median age at surgery was 12.8 months.
The treatment of craniosynostosis is surgical and requires a multidisciplinary approach, with expertise in plastic and reconstructive surgery, maxillofacial surgery and neurosurgery. The aim of surgical treatment is to release the constrictive and deformative effect that the synostosis has on skull growth. This requires a remodelling of the neurocranium and, if necessary, of the viscerocranium. Beyond aesthetic purposes, the primary aim of surgical treatment is to permit a normal development of the brain.
颅缝早闭是一种罕见的颅骨先天性疾病。当一条或多条颅骨缝过早骨化时就会发生这种情况。这会导致正常脑生长受阻，并导致颅骨特定部位的畸形，这可能导致颅内压升高和认知延迟。
我们回顾性分析了 2015 年 6 月至 2020 年 9 月期间在卢布尔雅那大学医学中心小儿神经外科治疗的所有儿童。记录了以下项目：受累缝、潜在综合征情况、脑积水、Chiari 畸形、颅内压升高、手术年龄、手术技术、需要多次手术和手术并发症。
在研究期间，有 71 名儿童因颅缝早闭接受治疗。术后中位随访时间为 31 个月。其中：矢状缝早闭 54.9%，冠状缝早闭 25.3%，单一冠状缝早闭 14.0%，双冠状缝早闭 1.4%，单侧斜头缝早闭 1.4%。多条缝受累的病例占 2.8%。7.0%的病例为综合征。总共进行了 74 次手术：额眶前移占 40.5%；双额重塑占 32.4%；颅盖全面重塑占 22.9%；后部牵伸占 2.7%；后部扩张占 1.3%。手术年龄中位数为 12.8 个月。
颅缝早闭的治疗是手术性的，需要多学科方法，包括整形和重建外科、颌面外科和神经外科的专业知识。手术治疗的目的是释放早闭对颅骨生长的限制和畸形作用。这需要对神经颅和必要时对内脏颅进行重塑。除了美观目的外，手术治疗的主要目的是允许大脑正常发育。
