Postoperative mental and morphological outcome for nonsyndromic brachycephaly
Postoperative mental and morphological outcome for nonsyndromic brachycephaly
复制标题
DOI：
10.1097/00006534-200207000-00002
复制
发表时间：
2002-07-01
影响因子：
3.6
通讯作者：
Renier, D
中科院分区：
文献类型：
--
作者：
Arnaud, E;Meneses, P;Renier, D
双侧冠状骨连接会导致功能和形态问题，需要在婴儿期进行额眶前移以纠正短头畸形并防止颅内高压引起的精神障碍。在这项研究中，对 99 名患有短头畸形的孤立病例进行了前瞻性随访，以研究他们的术前和术后心理结果，并使用发育商或智商进行评估。分析了几个因素：治疗前的年龄、手术时的年龄以及手术前后精神评估之间的相关性。在接受 FGFR3 P250R 突变测试的亚组或患者中 (n = 48)，对精神和形态评估进行了分析。手术前，1 岁前接受测试的患者精神状态较好 (p < 0.001)。术前心理评估始终与术后评估相关（P < 0.0001）。在患者 1 岁之前进行手术时，术后精神结果更好 (p < 0.02)。尽管非突变携带者亚组的形态和功能结果均较好，但差异无统计学意义。颞窝的突出凸出常常是突变携带者形态学结果不佳的原因。这项研究证实短头畸形患者需要在 1 岁之前进行早期矫正手术，以防止其智力发育受损。携带 FGFR3 P250R 突变的非综合征性短头畸形患者的形态和心理结果可能会不佳。颞部膨出的初步矫正应与额眶前移结合进行，以改善突变患者的形态学结果。
Bilateral coronal synostosis causes functional and morphological problems that require fronto-orbital advancement in infancy to correct the brachycephalic deformity and to prevent mental impairment caused by the intracranial hypertension. In this study, 99 children with isolated cases of brachycephaly were prospectively followed to Study their preoperative and postoperative mental outcome, which was evaluated using developmental or intelligence quotients. Several factors were analyzed: age before treatment, age at the time of surgery, and the correlation between mental assessments before and after surgery. In a subgroup or patients tested for the FGFR3 P250R mutation (n = 48), mental and morphological assessments were analyzed. Before surgery, mental status was better in the patients tested before 1 year of age (p < 0.001). The preoperative mental assessment always correlated with the postoperative assessment (P < 0.0001). The postoperative mental outcome was better when surgery was per-formed before the patient reached 1 year of age (p < 0.02). Although both the morphological and functional outcomes were better in the subgroup of non-carriers of the mutation, the differences were not statistically significant. Prominent bulging of the temporal fossae was frequently responsible for poor morphological outcome in carriers of the mutation. This study confirms the need for early corrective surgery before I year of age in brachycephalic patients to prevent impairment of their mental development. Suboptimal morphological and mental outcomes can be expected in patients with non-syndromic brachycephaly who carry the FGFR3 P250R mutation. Primary correction of the temporal bulging should be performed in conjunction with fronto-orbital advancement to improve the morphological outcome in patients with the mutation.