Naran Sanjay, Miller Matthew, Shakir Sameer, Ware Benjamin, Camison Liliana, Ford Matthew, Goldstein Jesse, Losee Joseph E
Pittsburgh and Philadelphia, Pa.
From the Department of Plastic Surgery and the School of Medicine, University of Pittsburgh; and the Division of Plastic Surgery, University of Pennsylvania.
Plast Reconstr Surg. 2017 Jul;140(1):62e-69e. doi: 10.1097/PRS.0000000000003423.
Although many metrics for neurodevelopment in children with nonsyndromic craniosynostosis have been analyzed, few have directly examined early language acquisition and speech development. The authors characterized language acquisition and speech development in children with nonsyndromic craniosynostosis.
The authors' institutional database was queried for nonsyndromic craniosynostosis from 2000 to 2014. Patients with an identified syndrome were excluded. Specific data elements included age, gender, velopharyngeal adequacy by means of the Pittsburgh Weighted Speech Scale, evaluation for anatomical motor delay, language acquisition delay/disorder, articulation or speech sound production delays/disorders, and whether speech therapy was recommended. Diagnosis of a submucous cleft palate was noted.
One hundred one patients met inclusion criteria, of which 57.4 percent were male. Average age at the time of the most recent speech evaluation was 6.1 years (range, 2.31 to 17.95 years); 43.6 percent had normal speech/language metrics and 56.4 percent had one or more abnormalities, including anatomical motor delay/disorder (29.7 percent), language acquisition delay/disorder (21.8 percent), articulation or speech production delay/disorder (4.0 percent), hypernasality (15.8 percent), and velopharyngeal insufficiency or borderline competency (23.8 percent). Average Pittsburgh Weighted Speech Scale score was 1.3 (range, 0 to 5), and 29.7 percent (n = 30) of patients were recommended to have speech therapy. In addition, 25.8 percent of patients were diagnosed with a submucous cleft palate.
One in four patients with nonsyndromic craniosynostosis carried a diagnosis of submucous cleft palate. The authors found that abnormal speech and language development occurs in one in 1.7 patients with nonsyndromic craniosynostosis, and that speech therapy for such abnormal development is warranted in one in 3.4 of them-a prevalence two to five times higher compared with the general pediatric population.
尽管已经分析了许多用于评估非综合征性颅缝早闭患儿神经发育的指标，但很少有研究直接考察早期语言习得和言语发育情况。作者对非综合征性颅缝早闭患儿的语言习得和言语发育进行了特征描述。
查询作者所在机构2000年至2014年期间非综合征性颅缝早闭的数据库。排除患有明确综合征的患者。具体数据元素包括年龄、性别、通过匹兹堡加权言语量表评估的腭咽功能、解剖性运动延迟评估、语言习得延迟/障碍、发音或语音产生延迟/障碍，以及是否建议进行言语治疗。记录黏膜下腭裂的诊断情况。
101例患者符合纳入标准，其中57.4%为男性。最近一次言语评估时的平均年龄为6.1岁（范围为2.31至17.95岁）；43.6%的患者言语/语言指标正常，56.4%的患者有一项或多项异常，包括解剖性运动延迟/障碍（29.7%）、语言习得延迟/障碍（21.8%）、发音或语音产生延迟/障碍（4.0%）、鼻音过重（15.8%）以及腭咽功能不全或临界功能（23.8%）。匹兹堡加权言语量表的平均得分为1.3（范围为0至5），29.7%（n = 30）的患者被建议进行言语治疗。此外，25.8%的患者被诊断为黏膜下腭裂。
四分之一的非综合征性颅缝早闭患者被诊断为黏膜下腭裂。作者发现，1.7例非综合征性颅缝早闭患者中就有1例存在言语和语言发育异常，其中3.4例中有1例因这种异常发育需要进行言语治疗——这一患病率比普通儿科人群高两到五倍。
