Tamburrini G, Caldarelli M, Massimi L, Gasparini G, Pelo S, Di Rocco C
Pediatric Neurosurgery, Institute of Neurosurgery, Catholic University Medical School, Largo "A. Gemelli", 8, 00168, Rome, Italy.
Childs Nerv Syst. 2012 Sep;28(9):1511-23. doi: 10.1007/s00381-012-1819-4. Epub 2012 Aug 8.
The protocols for clinical evaluation and management of children with complex craniosynostoses are significantly different from those used in single suture forms. The time at which the various anatomical and functional anomalies observed in the affected subjects become clinically relevant varies from patient to patient, consequently requiring a tailored approach. The clinical course is variable and influenced by multiple factors, acting at different steps of the children growth. Intracranial hypertension is a major concern already in the first months of life; active cerebrospinal fluid (CSF) dynamics disorders, venous hypertension, and progressive craniocerebral disproportion are considered the main pathogenetic factors. Cranial vault and skull base sutures synostoses account for the frequently observed increased venous pressure. Skull base abnormalities lead to upper airways obstruction, which, on one side, might create significant upper airways obstructive problems and, on the other, contribute to the increase in the intracranial pressure. Secondary Chiari malformation is common and considered as a progressive disorder, mainly due to progressive craniocerebral disproportion, venous hypertension, and CSF dynamics disorders. Optic nerve and orbit-related eye-globe diseases are also a major concern. Papilledema is mostly related to increased intracranial pressure. The skull base synostotic process is the base of significant abnormalities of the orbital space, ending in the common feature of significant proptosis with the consequent risk of corneal ulcers. Aims of this paper are to analyze the physiopathogenetic mechanisms at the base of the clinical manifestations presented by children with complex craniosynostoses, and the therapeutic options currently available.
复杂颅缝早闭患儿的临床评估和管理方案与单缝型颅缝早闭所使用的方案有显著不同。在受影响的患儿中观察到的各种解剖和功能异常在临床上变得相关的时间因人而异，因此需要采取量身定制的方法。临床病程是可变的，并且受到多种因素的影响，这些因素在儿童生长的不同阶段起作用。颅内高压在生命的最初几个月就是一个主要问题；活跃的脑脊液（CSF）动力学紊乱、静脉高压和进行性的颅脑比例失调被认为是主要的致病因素。颅顶和颅底缝早闭导致经常观察到的静脉压升高。颅底异常会导致上呼吸道阻塞，一方面可能会造成严重的上呼吸道阻塞问题，另一方面会导致颅内压升高。继发性Chiari畸形很常见，被认为是一种进行性疾病,主要是由于进行性的颅脑比例失调、静脉高压和脑脊液动力学紊乱。视神经和眼眶相关的眼球疾病也是一个主要问题。视乳头水肿大多与颅内压升高有关。颅底缝早闭过程是眼眶空间明显异常的基础，最终导致明显眼球突出这一常见特征，从而有发生角膜溃疡的风险。本文的目的是分析复杂颅缝早闭患儿临床表现背后的生理病理机制，以及目前可用的治疗选择。
