摘要
Background Reoperation for total colonic aganglionosis(TCA)may be required for residual aganglionosis after an initial radical operation.We aimed to investigate the symptoms,management,and outcomes of patients who required a redo pullthrough(Redo PT).Methods Nine TCA patients underwent Redo PT at our center between 2007 and 2017.Their medical records were reviewed.Parental telephone interviews that included disease-specific clinical outcomes were conducted,and post-operative complications and long-termoutcomes(including height-for-age/weight-for-age and bowel-function score)were compared to those of single-pull-through(Single PT)patients(n=21).Results All the nine Redo PT patients suffered obstruction within 1 month after the initial operation that could not be alleviated by conservative treatment.All abdominal X-ray/contrast barium enemas showed proximal bowel dilatation,indicating residual aganglionosis.The median ages at the initial operation and Redo PT were 200 and 509 days,respectively.Reoperation consisted of an intraoperative frozen biopsy and a modified laparotomic Soave procedure in all patients.Postoperative complications included perianal excoriation(n=3),intestinal obstruction(n=2),enterocolitis(n=2),and rectovestibular fistula(n=1).Seven Redo PT patients were followed up for a mean time of 7.162.3 years;six(85.7%)had good growth and four(57.1%)had good bowel-function recovery.Post-operative complications and long-term outcomes were almost equal between the Redo PT and Single PT groups(all P>0.05).Conclusion TCA patients with recurrent obstructive symptoms and dilated proximal bowel may have residual aganglionosis after an initial operation.Redo PT is effective and provides good long-termoutcomes comparable to those of patients who benefited from Single PT.
背景:全结肠无神经节细胞症(TCA)在初次根治性手术后,可能因病变残留需再次手术。我们旨在分析需行再次拖出手术治疗的TCA患者的症状、治疗及临床结局。方法:2007-2017年间,9例TCA患者在本中心接受再次拖出手术治疗。复习患儿医疗记录,并通过家长电话随访了解患儿临床结局。将本组9例再次手术患儿的术后并发症和长期结局与21例接受单次拖出手术的患儿进行比较。结果:本组9例再次手术患儿均于初次手术治疗后1个月内出现保守治疗无法缓解的肠梗阻。腹部X线和/或钡餐检查显示近端肠管扩张,提示病变残留。初次手术和再次手术时患儿中位年龄分别为200天和509天。再次手术时所有患儿均先行术中冰冻活检,证实为病变残留后行改良开腹Soave手术。术后并发症包括肛周皮肤脱落(3例)、肠梗阻(2例)、小肠结肠炎(2例)和直肠前庭瘘(1例)。7例再次手术患儿接受了(7.1˘2.3)年的随访,其中6例(85.7%)生长发育良好,4例(57.1%)肠道功能恢复良好。再次手术组与单次手术组患儿术后并发症和长期结局相当(均P>0.05)结论:TCA手术患儿出现复发性梗阻症状和近端肠管扩长,可能是因为病变残留。再次拖出手术是有效的,可以获得与单次拖出手术相当的长期结局。
