Dept. of Paediatric Surgery, Temple Street Children's University Hospital, Dublin, Ireland; National Children's Research Centre, Our Lady's Children's Hospital, Dublin, Ireland.
National Children's Research Centre, Our Lady's Children's Hospital, Dublin, Ireland.
J Pediatr Surg. 2019 Nov;54(11):2318-2324. doi: 10.1016/j.jpedsurg.2019.04.017. Epub 2019 Apr 26.
Retained transition zone is a leading cause of obstructive symptoms after pull-through operation in Hirschsprung's disease.
We aimed to evaluate the extent of the histological transition zone in patients with Hirschsprung's disease.
We performed an observational study. DAB+ immunohistochemistry for Protein Gene Product 9.5 was used to evaluate the neuronal networks in serial sections of pull-through specimens obtained from children with Hirschsprung's disease (n = 12). Reference ranges for ganglion size/density and nerve trunk diameter were statistically determined using healthy controls obtained from colostomy specimens from children with anorectal malformations (n = 8). The transition zone was defined as ganglionic bowel exhibiting ganglion hypoplasia, hypertrophic nerve trunks, or partial circumference aganglionosis.
The mean submucosal nerve trunk diameter in controls was 19.56 μm +/- 3.87 μm. The median age at pull-through for Hirschsprung's disease was 5 months (3-14 months). The median length of the transition zone across the population was 8 cm (4-22 cm). Median transition zone extent was significantly longer in patients with long-segment aganglionosis (n = 6) compared to rectosigmoid aganglionosis (n = 6, 13 cm vs 6 cm, p = 0.041). Due to the age of the patients enrolled, long-term follow-up of bowel function is not yet available.
Our data suggest that, in children with rectosigmoid Hirschsprung's disease, the transition zone can extend for up to 13 cm. In children with long-segment disease, a longer transition zone is possible. Extended resection at a minimum 5 cm beyond the most distal ganglionic intra-operative biopsy and intra-operative histological examination of the proximal resection margin are required to minimize transition zone pull-through.
在巨结肠病的经肛门拖出手术后，残留移行区是导致梗阻症状的主要原因。
我们旨在评估巨结肠病患者的组织学移行区范围。
我们进行了一项观察性研究。使用 DAB+免疫组织化学法检测蛋白基因产物 9.5，对取自巨结肠病患儿经肛门拖出标本的连续切片中的神经元网络进行评估（n=12）。使用取自肛门直肠畸形患儿结肠造口标本的健康对照（n=8）来统计确定神经节大小/密度和神经干直径的参考范围。将移行区定义为存在神经节发育不良、神经干肥大或部分周径无神经节的节段性肠段。
对照组的黏膜下神经干直径平均值为 19.56μm+/-3.87μm。巨结肠病患儿经肛门拖出的中位年龄为 5 个月（3-14 个月）。全人群的移行区长度中位数为 8cm（4-22cm）。长段无神经节细胞巨结肠（n=6）的移行区范围中位数明显长于直肠乙状结肠无神经节细胞巨结肠（n=6，13cm 比 6cm，p=0.041）。由于纳入患者的年龄，目前还无法获得长期的肠道功能随访结果。
我们的数据表明，在直肠乙状结肠巨结肠病患儿中，移行区可延伸至 13cm 长。在长段疾病患儿中，移行区可能更长。需要在至少距最远端术中神经节活检 5cm 处进行扩大切除，并在术中对近端切除缘进行组织学检查，以尽量减少移行区经肛门拖出。
2。
