Pollack I F, Losken H W, Biglan A W
Department of Neurosurgery, Children's Hospital of Pittsburgh, Pa. 15213, USA.
Pediatr Neurosurg. 1996;24(4):202-9. doi: 10.1159/000121038.
The late development of intracranial hypertension after an aesthetically successful cranial vault expansion has been noted anecdotally in children with syndromic synostosis. If untreated, this process can lead to neurological and visual deterioration; however, the frequency of this problem is uncertain. In an attempt to detect this process before the onset of irreversible impairment, the authors have since 1991 incorporated routine ophthalmological evaluations into the multidisciplinary follow-up protocol for all patients with complex craniosynostosis. These examinations were performed at initial evaluation and at 6-month intervals thereafter. The present report focuses on the results in 22 consecutive infants with syndromic synostosis who underwent initial surgery between 1991 and 1994. All but 4 children underwent initial cranial reconstruction at 6-18 months of age. Four patients had papilledema preoperatively which in each instance resolved postoperatively. During the follow-up period, 8 children manifested evidence of late intracranial hypertension at a median of 16.5 months after initial operation(s): 4 developed asymptomatic papilledema, 1 had progressive proptosis, and 3 had other subtle clinical signs of increased intracranial pressure (ICP). Further evaluation disclosed a Chiari 1 malformation in 2 children; 4 had a 'beaten copper' appearance on skull radiographs, and 4 underwent lumbar puncture, which in each case demonstrated an opening pressure above 20 cm H2O. Only 3 children exhibited symptoms from the ICP elevation. Six children with a head size < or = the 25th percentile underwent a repeat cranial expansion; after the cranial vault expansion, one child who initially had slit-like ventricles developed ventriculomegaly and, because of persistent papilledema, underwent insertion of ventriculoperitoneal (VP) shunt. Two other patients with a head circumference above the 50th percentile and the development of moderate ventriculomegaly underwent VP shunt insertion alone. All children have had complete resolution of papilledema as well as other symptoms and signs of increased ICP without evidence of optic atrophy or neurological sequelae. This study indicates that the incidence of delayed, asymptomatic increases in ICP among children with complex craniosynostosis is higher than previously estimated. The pathophysiologic bases for this process are discussed. Because the detection of this problem at a presymptomatic stage should optimize the likelihood of a good functional outcome, we strongly recommend close ophthalmological and clinical follow-up as a part of the comprehensive care of all young children with complex craniosynostosis, even after a cosmetically successful cranial expansion.
在患有综合征性颅缝早闭的儿童中，曾有轶事报道称，在颅骨穹窿扩张手术在美学上取得成功后，颅内高压会延迟出现。如果不进行治疗，这一过程可能导致神经功能和视力恶化；然而，这个问题的发生频率尚不确定。为了在不可逆损伤发生之前检测到这一过程，自1991年以来，作者将常规眼科评估纳入了所有复杂颅缝早闭患者的多学科随访方案中。这些检查在初次评估时进行，此后每隔6个月进行一次。本报告重点关注1991年至1994年间接受初次手术的22例连续患有综合征性颅缝早闭的婴儿的结果。除4名儿童外，所有儿童均在6至18个月大时接受了初次颅骨重建。4例患者术前有视乳头水肿，术后均消退。在随访期间，8名儿童在初次手术后中位16.5个月出现晚期颅内高压迹象：4例出现无症状性视乳头水肿，1例出现进行性眼球突出，3例出现其他颅内压升高（ICP）的细微临床体征。进一步评估发现2名儿童有Chiari 1畸形；4例颅骨X线片显示有“铜击样”外观，4例接受了腰椎穿刺，每例腰椎穿刺的初压均高于20 cm H2O。只有3名儿童出现了ICP升高的症状。6名头围小于或等于第25百分位数的儿童接受了再次颅骨扩张；在颅骨穹窿扩张后，1名最初有裂隙样脑室的儿童出现脑室扩大，由于持续存在视乳头水肿，接受了脑室腹腔（VP）分流术。另外2名头围大于第50百分位数且出现中度脑室扩大的患者仅接受了VP分流术。所有儿童的视乳头水肿以及其他ICP升高的症状和体征均已完全消退，且无视神经萎缩或神经后遗症的证据。这项研究表明，复杂颅缝早闭儿童中延迟出现的无症状ICP升高的发生率高于先前的估计。本文讨论了这一过程的病理生理基础。由于在症状前期阶段检测到这个问题应该会优化获得良好功能结果的可能性，我们强烈建议对所有患有复杂颅缝早闭的幼儿进行密切的眼科和临床随访，即使在颅骨扩张在美容上取得成功之后，这也是综合护理的一部分。
