Obata Satoshi, Yoshimaru Koichiro, Kirino Kosuke, Izaki Tomoko, Ieiri Satoshi, Yamataka Atsuyuki, Koshinaga Tsugumichi, Iwai Jun, Ikeda Hitoshi, Matsufuji Hiroshi, Oda Yoshinao, Taguchi Tomoaki
Japanese Study Group for Allied Disorders of Hirschsprung's Disease, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan.
Department of Pediatric Surgery, Reproductive and Developmental Medicine, Faculty of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan.
Pediatr Surg Int. 2019 Feb;35(2):215-220. doi: 10.1007/s00383-018-4398-y. Epub 2018 Nov 19.
Acquired isolated hypoganglionosis (A-IH) is a late-onset intestinal pseudo-obstruction disorder and shows different pathophysiological findings from congenital isolated hypoganglionosis (C-IH). In this study, we retrospectively examined five cases of A-IH and investigated the features of A-IH.
Five cases of A-IH were extracted from a nationwide retrospective cohort study in 10 years, from which totally 355 cases of Allied Disorders of Hirschsprung's Disease (ADHD) were collected.
Ages of onset were between 13 and 17 years in three cases, and 4 years and 4 months in ones. Initial symptoms were abdominal distension and/or chronic constipation in 4 cases, whereas one exhibited intestinal perforation. Affected lesions varied from case to case, extending various length of intestinal tracts. All cases underwent multiple operations (average: 4.6 times), such as enterostomy, resection of dilated intestines, and/or pull-through. Pathological findings showed the decreased numbers of ganglion cells and degeneration of ganglion cells, whereas the size of the plexus was normal. Currently, all cases were alive and almost all eat regular food without requiring parenteral feeding.
A-IH is rare, but distinct entity characterized by different clinical courses and pathological findings from those of C-IH. The outcome is considered to be favorable after a resection of affected intestine.
获得性孤立性神经节减少症（A-IH）是一种迟发性肠道假性梗阻疾病，其病理生理表现与先天性孤立性神经节减少症（C-IH）不同。在本研究中，我们回顾性研究了5例A-IH病例，并探讨了A-IH的特征。
从一项为期10年的全国性回顾性队列研究中提取5例A-IH病例，该研究共收集了355例先天性巨结肠相关疾病（ADHD）病例。
3例患者的发病年龄在13至17岁之间，1例为4岁4个月。4例患者的初始症状为腹胀和/或慢性便秘，1例表现为肠穿孔。受累病变因病例而异，累及不同长度的肠道。所有病例均接受了多次手术（平均4.6次），如肠造口术、扩张肠段切除术和/或拖出术。病理结果显示神经节细胞数量减少和神经节细胞变性，而神经丛大小正常。目前，所有病例均存活，几乎所有患者都能正常饮食，无需肠外营养。
A-IH较为罕见，但具有独特的特征，其临床病程和病理表现与C-IH不同。切除受累肠段后，预后被认为良好。
