Mouradian W E
Craniofacial Program, Children's Hospital Medical Center, University of Washington, Seattle 98105-0371, USA.
Cleft Palate Craniofac J. 1998 May;35(3):190-3. doi: 10.1597/1545-1569_1998_035_0190_citdam_2.3.co_2.
This introductory article summarizes and comments on a group of four papers based in part on a panel discussion of craniosynostosis (CS) held at the 53rd annual meeting of the American Cleft Palate-Craniofacial Association. The purpose of this panel was to review the differential diagnosis of CS and the evidence for increased intracranial pressure (ICP) and developmental problems in CS patients. First, a correct diagnosis must be made, with true synostosis being differentiated from positional deformities and other normal variants. Second, medical indications must be balanced against the risks of operating on CS patients. Release of fused sutures is commonly undertaken to avoid increased ICP, although studies of ICP in infants and children with CS are hampered by a lack of normative data and by difficulties with measurement techniques. A subgroup of children with isolated craniosynostosis may have increased ICP, which can be deleterious to brain function. Longitudinal studies of children with isolated CS suggest an increased risk of mental retardation and learning disorders; surgical release of the suture may not diminish this risk. Patients with metopic suture fusion appear to be particularly at risk. These findings must be confirmed with a larger sample size. These uncertainties raise ethical issues and complicate medical decision-making for the infant with CS. A trusting and truthful relationship between the parents and the professional is necessary for a balanced discussion of the best interests of the child. All patients with confirmed synostosis should be followed for evidence of progressive deformity, intracranial hypertension, and neurodevelopmental problems.
这篇介绍性文章总结并评论了一组四篇论文，这些论文部分基于在美国腭裂-颅面协会第53届年会上举行的关于颅缝早闭（CS）的小组讨论。该小组的目的是回顾CS的鉴别诊断以及CS患者颅内压（ICP）升高和发育问题的证据。首先，必须做出正确的诊断，将真正的骨缝早闭与位置性畸形和其他正常变异区分开来。其次，必须在医学指征与对CS患者进行手术的风险之间取得平衡。虽然由于缺乏规范数据和测量技术的困难，对CS婴儿和儿童的ICP研究受到阻碍，但通常会进行融合缝线的松解以避免ICP升高。患有孤立性颅缝早闭的儿童亚组可能有ICP升高，这可能对脑功能有害。对患有孤立性CS的儿童进行的纵向研究表明，智力迟钝和学习障碍的风险增加；缝线的手术松解可能无法降低这种风险。额缝融合的患者似乎特别危险。这些发现必须用更大的样本量来证实。这些不确定性引发了伦理问题，并使CS婴儿的医疗决策复杂化。父母与专业人员之间信任和真实的关系对于平衡讨论儿童的最大利益是必要的。所有确诊为骨缝早闭的患者都应进行随访，以观察是否有进行性畸形、颅内高压和神经发育问题的迹象。
