Abstract：
BACKGROUND: Immaturity of ganglia (IG) is an extremely rare disease and always requires surgical intervention in the neonatal period, but without guidelines to choose the ideal enterostomy procedure, the timing of stoma closure remains controversial. The aim of this study was to report our experience using Santulli enterostomy for the treatment of nine infants diagnosed with IG.
METHODS: Patients who underwent Santulli enterostomy and were diagnosed with IG in our center between 2016 and 2021 were retrospectively studied. Temporary stoma occlusion and a 24-h delayed film of barium enema (BE) were performed to evaluate intestinal peristalsis function to determine the timing of stoma closure. The demographic data, clinical and radiological findings, stoma occlusion and stoma closure results were explored.
RESULTS: A total of 9 infants underwent Santulli enterostomy and were diagnosed with IG postoperatively. Their median gestational age at birth was 36 weeks (range 31-42), and their median birth weight was 2765 g (range 1300-3400). All patients had symptom onset in the neonatal period, including abdominal distension and biliary vomiting. Eight patients showed obvious small bowel dilatation in the plain films, except for one patient\'s films that suggested gastrointestinal perforation with free gas downstream of the diaphragm. BE was performed in 6 patients, all of which had microcolons. The median age at operation was 3 days (range 1-23). Seven patients had an obvious transitional zone (TZ) during laparotomy, and the position of the TZ was 25-100 cm proximal above the ileocecal (IC) valve. Immature ganglion cells were present in the colon in 7 patients and the terminal ileum in 6 patients. The median age of successful stoma occlusion was 5 M (range 2-17) and 8 M (range 4-22) at ostomy closure. There was little or no barium residue in the 24-h delayed film of BE before stoma closure, and all patients were free of constipation symptoms during the follow-up.
CONCLUSIONS: Santulli enterostomy appears to be a suitable and efficient procedure for IG, combined with temporary stoma occlusion and 24-h delayed film of BE to evaluate the recovery of intestinal peristalsis function.
METHODS: Patients who underwent Santulli enterostomy and were diagnosed with IG in our center between 2016 and 2021 were retrospectively studied. Temporary stoma occlusion and a 24-h delayed film of barium enema (BE) were performed to evaluate intestinal peristalsis function to determine the timing of stoma closure. The demographic data, clinical and radiological findings, stoma occlusion and stoma closure results were explored.
RESULTS: A total of 9 infants underwent Santulli enterostomy and were diagnosed with IG postoperatively. Their median gestational age at birth was 36 weeks (range 31-42), and their median birth weight was 2765 g (range 1300-3400). All patients had symptom onset in the neonatal period, including abdominal distension and biliary vomiting. Eight patients showed obvious small bowel dilatation in the plain films, except for one patient\'s films that suggested gastrointestinal perforation with free gas downstream of the diaphragm. BE was performed in 6 patients, all of which had microcolons. The median age at operation was 3 days (range 1-23). Seven patients had an obvious transitional zone (TZ) during laparotomy, and the position of the TZ was 25-100 cm proximal above the ileocecal (IC) valve. Immature ganglion cells were present in the colon in 7 patients and the terminal ileum in 6 patients. The median age of successful stoma occlusion was 5 M (range 2-17) and 8 M (range 4-22) at ostomy closure. There was little or no barium residue in the 24-h delayed film of BE before stoma closure, and all patients were free of constipation symptoms during the follow-up.
CONCLUSIONS: Santulli enterostomy appears to be a suitable and efficient procedure for IG, combined with temporary stoma occlusion and 24-h delayed film of BE to evaluate the recovery of intestinal peristalsis function.
摘要：
背景：神经节未成熟（IG）是一种极为罕见的疾病，在新生儿期总是需要手术干预，但是没有指南来选择理想的肠造口程序，造口关闭的时间仍然存在争议。这项研究的目的是报告我们使用Santulli肠造口术治疗9名被诊断为IG的婴儿的经验。
方法：回顾性研究了2016年至2021年在我们中心接受了Santulli肠造口术并被诊断为IG的患者。进行了临时造口闭塞和钡灌肠（BE）的24小时延迟膜，以评估肠蠕动功能，以确定造口闭合的时机。人口统计数据，临床和放射学发现，探讨了造口闭塞和造口闭合的结果。
结果：共有9名婴儿接受了Santulli肠造口术，术后被诊断为IG。他们出生时的中位胎龄为36周（范围31-42），他们的中位出生体重为2765g（范围1300-3400）。所有患者均在新生儿期出现症状，包括腹胀和胆道呕吐。8例患者在平片中表现出明显的小肠扩张，除了一个病人的电影，建议胃肠道穿孔与游离气体在隔膜下游。在6例患者中进行了BE，都有微结肠。手术年龄中位数为3天（范围1-23）。7例患者在剖腹手术中有明显的过渡区（TZ），TZ的位置位于回盲（IC）瓣膜上方25-100cm。7例患者的结肠中存在未成熟的神经节细胞，6例患者的末端回肠中存在未成熟的神经节细胞。造口术闭合时成功造口闭塞的中位年龄为5M（范围2-17）和8M（范围4-22）。造口关闭前，BE的24小时延迟膜中几乎没有钡残留，随访期间所有患者均无便秘症状。
结论：Santulli肠造口术似乎是IG的一种合适且有效的方法，结合暂时性造口闭塞和24h延迟贴膜评价肠蠕动功能的恢复。
方法：回顾性研究了2016年至2021年在我们中心接受了Santulli肠造口术并被诊断为IG的患者。进行了临时造口闭塞和钡灌肠（BE）的24小时延迟膜，以评估肠蠕动功能，以确定造口闭合的时机。人口统计数据，临床和放射学发现，探讨了造口闭塞和造口闭合的结果。
结果：共有9名婴儿接受了Santulli肠造口术，术后被诊断为IG。他们出生时的中位胎龄为36周（范围31-42），他们的中位出生体重为2765g（范围1300-3400）。所有患者均在新生儿期出现症状，包括腹胀和胆道呕吐。8例患者在平片中表现出明显的小肠扩张，除了一个病人的电影，建议胃肠道穿孔与游离气体在隔膜下游。在6例患者中进行了BE，都有微结肠。手术年龄中位数为3天（范围1-23）。7例患者在剖腹手术中有明显的过渡区（TZ），TZ的位置位于回盲（IC）瓣膜上方25-100cm。7例患者的结肠中存在未成熟的神经节细胞，6例患者的末端回肠中存在未成熟的神经节细胞。造口术闭合时成功造口闭塞的中位年龄为5M（范围2-17）和8M（范围4-22）。造口关闭前，BE的24小时延迟膜中几乎没有钡残留，随访期间所有患者均无便秘症状。
结论：Santulli肠造口术似乎是IG的一种合适且有效的方法，结合暂时性造口闭塞和24h延迟贴膜评价肠蠕动功能的恢复。
