Department of Pediatric Surgery, Erasmus MC, Sophia Children's Hospital, 3000 CB, Rotterdam, the Netherlands.
J Pediatr Surg. 2013 Apr;48(4):818-25. doi: 10.1016/j.jpedsurg.2012.09.068.
BACKGROUND/PURPOSE: To evaluate growth and development in children with anorectal malformations and to analyze effects of type of malformation and comorbidities.
Non-syndromal children with anorectal malformations were prospectively evaluated at 0.5, 1, 2, and 5 years. Biometrics were obtained at all visits. Mental and psychomotor function development was determined.
108 children (59% male) were included. 49% had a high malformation, and 46% had ≥ 1 additional major comorbidity. All growth parameters were below the norm at all ages (p<0.01), irrespective of type of malformation. Children with ≥ 1 additional major anomaly had lower height at all ages; at 5 years, mean (95% CI) height was -1.83 (-2.7 to -1.1) and -0.70 (-1.3 to -0.1) in children with and without comorbidities, respectively (p=0.019). Mental development was normal, irrespective of the type of malformation or comorbidities. Motor development was delayed at all ages. At 5 years, motor development (n=30) was normal in 70%, borderline in 23%, and 7% had definitive motor problems (p=0.043).
Non-syndromal children with anorectal malformations are at risk for growth impairment, especially those with additional major comorbidity. Mental development is normal. Motor development is slightly impaired. Supportive care should focus on growth, dietary management, and motor development besides defecation problems.
背景/目的：评估肛门直肠畸形患儿的生长发育情况，并分析畸形类型和合并症的影响。
对非综合征型肛门直肠畸形患儿进行前瞻性评估，分别在 0.5 岁、1 岁、2 岁和 5 岁时进行。所有就诊时均进行生物测量。确定精神和运动功能发育情况。
共纳入 108 例患儿（59%为男性）。49%的患儿畸形程度较高，46%的患儿存在≥1 种主要合并症。所有生长参数在所有年龄段均低于正常值（p<0.01），与畸形类型无关。存在≥1 种主要异常的患儿在所有年龄段的身高均较低；5 岁时，合并症患儿的平均（95%CI）身高为-1.83（-2.7 至-1.1），无合并症患儿的平均身高为-0.70（-1.3 至-0.1）（p=0.019）。无论畸形类型或合并症如何，精神发育均正常。运动发育在所有年龄段均延迟。在 5 岁时，运动发育（n=30）正常的患儿占 70%，边缘型的患儿占 23%，有明确运动问题的患儿占 7%（p=0.043）。
非综合征型肛门直肠畸形患儿存在生长受损风险，尤其是存在其他主要合并症的患儿。精神发育正常。运动发育略有受损。支持性护理应重点关注生长、饮食管理和运动发育，同时还应关注排便问题。
