Becker Devra B, Petersen Jason D, Kane Alex A, Cradock Mary Michaeleen, Pilgram Thomas K, Marsh Jeffrey L
The Cleft Palate and Craniofacial Deformities Institute, Department of Pediatric Psychology, St. Louis Children's Hospital, St. Louis, MO, USA.
Plast Reconstr Surg. 2005 Aug;116(2):400-7. doi: 10.1097/01.prs.0000172763.71043.b8.
The neuropsychological morbidity of nonsyndromic craniosynostosis is incompletely understood. The purpose of this study was to establish the prevalence of speech-language, cognitive, and behavioral abnormalities in this population and to stratify the findings on the basis of the affected suture and age of diagnosis with speech-language or psychological abnormalities.
Charts of all patients with nonsyndromic craniosynostosis evaluated between 1978 and 2000 were reviewed, noting diagnoses of speech-language, cognitive, or behavioral abnormalities. Findings were statistically analyzed for variance with regard to affected suture and diagnosis of abnormalities.
Two hundred fourteen patients with nonsyndromic craniosynostosis had documented follow-up evaluations with an average age of 6 years 4 months at last visit. Speech, cognitive, and/or behavioral abnormalities were manifest in 49 percent of the patients with specific rates for each suture as follows: right unilateral coronal, 61 percent; bilateral coronal, 55 percent; multiple, 47 percent; metopic, 57 percent; left unilateral coronal, 52 percent; lambdoid, 44 percent; and sagittal, 39 percent. This prevalence of abnormalities was a statistically significant increase from the general population. Logistic regression demonstrated that as patient age increased, the percentage of abnormal diagnoses also increased.
Nonsyndromic craniosynostosis is often associated with cognitive, speech, and/or behavioral abnormalities. The etiopathology of this association is unknown. Furthermore, the proportion of children diagnosed with cognitive and behavioral dysfunction increases with age. Therefore, longitudinal cognitive, behavioral, and speech assessment and treatment are integral to the care of these patients.
非综合征性颅缝早闭的神经心理学发病情况尚未完全明了。本研究的目的是确定该人群中言语、认知和行为异常的患病率,并根据受影响的颅缝以及言语或心理异常的诊断年龄对研究结果进行分层。
回顾了1978年至2000年间评估的所有非综合征性颅缝早闭患者的病历,记录言语、认知或行为异常的诊断情况。对受影响的颅缝和异常诊断结果进行方差统计分析。
214例非综合征性颅缝早闭患者有随访评估记录,最后一次就诊时的平均年龄为6岁4个月。49%的患者存在言语、认知和/或行为异常,各颅缝的具体发生率如下:右侧单侧冠状缝,61%;双侧冠状缝,55%;多发,47%;额缝,57%;左侧单侧冠状缝,52%;人字缝,44%;矢状缝,39%。这种异常患病率与普通人群相比有统计学意义的增加。逻辑回归分析表明,随着患者年龄的增加,异常诊断的百分比也增加。
非综合征性颅缝早闭常与认知、言语和/或行为异常相关。这种关联的病因尚不清楚。此外,被诊断为认知和行为功能障碍的儿童比例随年龄增长而增加。因此,纵向的认知、行为和言语评估及治疗是这些患者护理中不可或缺的一部分。