Fuchs J R, Langer J C
Department of Surgery, Washington University School of Medicine, St Louis, Missouri, USA.
Pediatrics. 1998 Apr;101(4):E7. doi: 10.1542/peds.101.4.e7.
It is unclear whether children with cystic fibrosis (CF) who present with neonatal meconium ileus have a different long-term outcome from those presenting later in childhood with pulmonary complications or failure to thrive. We examined a cohort of patients with meconium ileus, and compared their long-term outcome with children who had CF without meconium ileus and neonates who had meconium obstruction without CF (meconium plug syndrome).
Comparative study using retrospective and follow-up interview data.
Group 1 consisted of 35 surviving CF patients who presented with meconium ileus between 1966 and 1992. Two control groups were also studied: 35 age- and sex-matched CF patients without meconium ileus (group 2), and 12 infants presenting with meconium plug syndrome during the same time period (group 3).
Pulmonary, gastrointestinal, nutritional, and functional status were reviewed, and surgical complications were recorded.
Mean follow-up was 12.6 +/- 6, 12.6 +/- 6, and 9. 3 +/- 4 years in groups 1, 2, and 3, respectively. Patients without CF (group 3) demonstrated better growth and functional status, and had a lower incidence of pulmonary and gastrointestinal problems. Although the presence of meconium ileus among CF patients was associated with an earlier diagnosis, there were no significant differences between groups 1 and 2 with respect to hepatobiliary, nutritional, functional, or respiratory status. Meconium ileus was associated with a higher risk of meconium ileus equivalent (20% vs 6%), although this difference was not statistically significant. Long-term surgical complications (adhesive small bowel obstruction and blind loop syndrome) were seen in 27% of children with meconium ileus; there were no long-term surgical complications in groups 2 or 3, because these infants did not have any neonatal surgical procedures. Children presenting with complicated meconium ileus had a higher rate of long-term surgical complications than those with uncomplicated meconium ileus (36% vs 17%), and those managed with resection or enterostomy had more complications than those treated by enterotomy and lavage (33% vs 0%).
Long-term outcome is similar in CF patients who present with meconium ileus and those who do not, except for a slightly higher incidence of meconium ileus equivalent, and a significantly higher rate of surgical complications. The risk of surgical complications is highest in those presenting with complicated meconium ileus and those undergoing resection or enterostomy. Patients with meconium obstruction who do not have CF have an excellent long-term prognosis. This information will be useful in counseling the families of infants presenting with neonatal meconium obstruction.
目前尚不清楚患有胎粪性肠梗阻的囊性纤维化（CF）患儿与那些在儿童期后期出现肺部并发症或生长发育不良的患儿相比，长期预后是否不同。我们研究了一组胎粪性肠梗阻患者，并将他们的长期预后与没有胎粪性肠梗阻的CF患儿以及没有CF的胎粪梗阻新生儿（胎粪栓综合征）进行比较。
采用回顾性和随访访谈数据的比较研究。
第1组由1966年至1992年间出现胎粪性肠梗阻的35名存活CF患者组成。还研究了两个对照组：35名年龄和性别匹配的无胎粪性肠梗阻的CF患者（第2组），以及同期出现胎粪栓综合征的12名婴儿（第3组）。
回顾肺部、胃肠道、营养和功能状况，并记录手术并发症。
第1、2、3组的平均随访时间分别为12.6±6年、12.6±6年和9.3±4年。没有CF的患者（第3组）生长和功能状况更好，肺部和胃肠道问题的发生率更低。虽然CF患者中胎粪性肠梗阻的存在与更早诊断相关，但第1组和第2组在肝胆、营养、功能或呼吸状况方面没有显著差异。胎粪性肠梗阻与胎粪性肠梗阻等效物的风险较高相关（20%对6%），尽管这种差异没有统计学意义。27%的胎粪性肠梗阻患儿出现长期手术并发症；第2组或第3组没有长期手术并发症，因为这些婴儿没有进行任何新生儿手术。出现复杂胎粪性肠梗阻的患儿长期手术并发症发生率高于单纯胎粪性肠梗阻患儿（36%对17%），接受切除或肠造口术治疗的患儿比接受肠切开术和灌洗治疗的患儿并发症更多（33%对0%）。
出现胎粪性肠梗阻的CF患者和未出现的患者长期预后相似，只是胎粪性肠梗阻等效物的发生率略高，手术并发症发生率显著更高。出现复杂胎粪性肠梗阻的患者和接受切除或肠造口术的患者手术并发症风险最高。没有CF的胎粪梗阻患者长期预后良好。这些信息将有助于为出现新生儿胎粪梗阻的婴儿家庭提供咨询。
