Gischler Saskia J, Mazer Petra, Duivenvoorden Hugo J, van Dijk Monique, Bax Nikolaas M A, Hazebroek Frans W J, Tibboel Dick
Surgical Intensive Care Unit, Department of Pediatric Surgery, Erasmus MC, Sophia Children's Hospital, Erasmus University Medical Center Rotterdam, 3000 CA Rotterdam, The Netherlands.
J Pediatr Surg. 2009 Jul;44(7):1382-9. doi: 10.1016/j.jpedsurg.2008.12.034.
Information on physical and developmental outcomes of children with anatomical congenital anomalies (CAs) may indicate the need for early intervention and reduce impact on the child's life and parental burden.
From 1999 to 2003, 101 children with CA (76.5% of initial survivors) were seen 6-monthly in a tertiary children's hospital. Growth, neurologic outcome, mental and psychomotor development as determined with the Bayley Scales of Infant Development, and categorization of predictive sociodemographic and medical variables of the children were evaluated prospectively and longitudinally.
Congenital diaphragmatic hernia (CDH) and esophageal atresia patients showed impaired growth, that is, both height for age (-1.5 standard deviation score [SDS]) and weight for height (-1.0 SDS). Overall neurologic outcome was normal, however, suspect or abnormal for 40% of CDH patients. Overall mental development was normal, but psychomotor scores were significantly lower than the norm (95% confidence interval, 83.8-92.2 at 6 months and 87.9-98.5 at 24 months). Sex, maternal age, socioeconomic status, CA, severity-of-disease covariables, and need of medical appliances at home could predict negative outcome significantly (P < .05).
The CA survivors show impaired growth and psychomotor developmental delay up to age 2 years. This warrants specific follow-up programs and infrastructure for these patients.
有关先天性解剖结构异常(CA)患儿身体和发育结局的信息可能提示早期干预的必要性,并减轻对患儿生活及家长负担的影响。
1999年至2003年期间,一家三级儿童医院每6个月对101例患有CA的患儿(占初始存活者的76.5%)进行一次检查。对患儿的生长情况、神经学结局、使用贝利婴儿发育量表测定的智力和精神运动发育情况,以及对患儿社会人口统计学和医学预测变量进行分类,进行前瞻性和纵向评估。
先天性膈疝(CDH)和食管闭锁患儿生长发育受损,即年龄别身高(-1.5标准差评分[SDS])和身高别体重(-1.0 SDS)均较低。总体神经学结局正常,然而,40%的CDH患儿存在可疑或异常情况。总体智力发育正常,但精神运动评分显著低于正常水平(6个月时95%置信区间为83.8 - 92.2,24个月时为87.9 - 98.5)。性别、母亲年龄、社会经济地位、CA、疾病严重程度协变量以及家庭是否需要医疗设备可显著预测不良结局(P < .05)。
CA存活者在2岁前生长发育受损且存在精神运动发育迟缓。这就需要为这些患者制定特定的随访计划和基础设施。