肠无神经节细胞症(Hirschsprung’s Disease,HD),又称先天性巨结肠,是小儿外科常见的严重消化道畸形,其病理特征是病变肠段中神经节完全缺如,导致慢性功能性肠梗阻,其发病率约为1/5000,男女比例为4:1,且有一定的遗传易感性。多数 HD 患儿早期可通过开塞露、回流灌肠等方式缓解腹胀等症状,但最终根治仍需手术治疗。近年来 HD 的手术治疗方式主要包括HD一期根治术和分期手术(及根治术前行肠造口术),也有学者研究神经干细胞移植应用于治疗HD。现就HD的手术治疗方式的现状及研究进展综述如下。
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.
