Surgical considerations in cystic fibrosis: A 32-year evaluation of outcomes
Surgical considerations in cystic fibrosis: A 32-year evaluation of outcomes
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DOI：
10.1016/j.surg.2005.06.049
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发表时间：
2005-10-01
期刊：
影响因子：
3.8
通讯作者：
Rescorla, FJ
中科院分区：
文献类型：
--
作者：
Escobar, MA;Grosfeld, JL;Rescorla, FJ
背景关于囊性纤维化（CF）患者长期手术结果的信息在手术文献中相对较少。回顾性分析了1972-2004年在一家三级儿童医院接受手术治疗的CF患者的预后，包括长期发病率和生存率。共有226例CF患者进行了手术诊断（113例男性，113例女性）。213例患者（94%）共进行了422次手术。手术时的平均年龄为4.1 ± 6.2岁（范围：1天至26岁），109例为新生儿。42例单纯性胎粪性肠梗阻患儿中15例（36%）采用高渗灌肠非手术治疗，42例复杂性胎粪性肠梗阻患儿中27例（17%）需手术治疗，其中15例合并空回肠闭锁。27例胎粪性肠梗阻患者中有17例（63%）在新生儿时发生MI; 27例中有7例（26%）需要手术。9例纤维化结肠病变中8例（89%）需要手术治疗。21例患者需要器官移植。213例患者中有204例（96%）可以进行随访评价。随访评价持续时间为14.9 ± 8.5年（范围：2个月至35年）。术后1年的手术发病率为11%，2 - 4年为2%，5 - 10年为1%，超过10年时小于1%。24例死亡（11%）; 22例继发CF相关肺部并发症，其中8/16例（50%）儿童气胸。CF患者的长期生存率显著提高（89%），许多患者存活至40岁。MI可能易发生晚期并发症，包括胎粪性肠梗阻和纤维化结肠病。CF患者的气胸是死亡率的不祥预测因素。CF患儿的生存时间较长，是手术的良好候选者，但由于持续的外分泌功能障碍，需要长期随访评估。
Background. Information concerning long-term operative outcomes in patients with cystic fibrosis (CF) is relatively sparse in the operative literature.Methods. A retrospective review of CF patients with operative conditions was performed (1972-2004) at a tertiary children's hospital to analyze outcomes including long-term morbidity and survival.Results. A total of 226 patients with CF presented with an operative diagnosis (113 men, 113 women). A total of 422 operations were performed in 213 patients (94%). The mean age at operation was 4.1 +/- 6.2 years (range, 1 d to 26 y) and 109 were neonates. Fifteen of 42 (36%) babies with simple meconium ileus (MI) were treated nonoperatively with hypertonic enemas, 27 of 42 and all 45 patients with complicated MI required operation, including 15 with jejunoileal atresia (17%). Seventeen of 27 (63%) patients with meconium ileus equivalent had MI as neonates; 7 of 27 (26%) required operation. Eight of 9 (89%) with fibrosing colonopathy required operation. Organ transplantation was required in 21 patients. Follow-up evaluation was possible in 204 of 213 (96%) patients. The duration of follow-up evaluation was 14.9 +/- 8.5 years (range, 2 mo to 35 y). Operative morbidity was 11% at 1 year, 2% at 2 to 4 years, 1% at 5 to 10 years, and less than 1% at more than 10 years. There were 24 deaths (11%); 22 followed CF-related pulmonary complications and included 8 of 16 (50%) children with pneumothorax.Conclusions. Long-term survival in CF patients has improved significantly (89%), with many surviving into the fourth decade. MI may predispose to late complications including meconium ileus equivalent and fibrosing colonopathy. Pneumothorax in CF patients is an ominous predictor of mortality. Children with CF are living longer and are good candidates for operation, but require long-term follow-up evaluation because of ongoing exocrine dysfunction.