Singh Niharika, Petrancosta James, O'Daniel Elizabeth, Nurko Samuel, Calabro Kristen
Department of Surgery, Stony Brook University Hospital, Stony Brook, NY 11794, USA.
Department of Surgery, Boston Children's Hospital, Boston, MA 02115, USA.
Reports (MDPI). 2025 Jul 15;8(3):108. doi: 10.3390/reports8030108.
Acquired myenteric hypoganglionosis is a rare dysmotility disorder that can present in childhood and adulthood, characterized by a reduced number of ganglion cells within Auerbach's plexus. Due to the rarity of the pathology, few case reports of acquired myenteric hypoganglionosis in adolescents have been described. This case report explores the presentation, risk factors, and surgical complications associated with the ultimate diagnosis of myenteric hypoganglionosis. We present a case of a 12-year-old male with a history of constipation and achalasia, presenting with constipation and abdominal distention, who underwent a colonoscopy, which was converted to an exploratory laparotomy with loop ileostomy creation due to persistent significant abdominal distention. This was complicated by colonic perforation, most likely secondary to stercoral colitis, requiring takeback to the operating room on postoperative day 11 for an exploratory laparotomy with bowel resection and mucous fistula creation. The patient was then referred to Boston Children's Hospital for motility studies, which revealed poor colonic motility and plans to reassess motility in 1 year. : Although rare, it is important to have high clinical suspicion for acquired myenteric hypoganglionosis in children, especially males, with severe constipation.
获得性肠肌间神经节减少症是一种罕见的动力障碍性疾病,可在儿童期和成年期出现,其特征是奥尔巴赫神经丛内神经节细胞数量减少。由于该病理学情况罕见,青少年获得性肠肌间神经节减少症的病例报告很少。本病例报告探讨了与肠肌间神经节减少症最终诊断相关的表现、危险因素和手术并发症。我们报告一例12岁男性,有便秘和贲门失弛缓症病史,表现为便秘和腹胀,接受了结肠镜检查,由于持续严重腹胀,该检查转为剖腹探查并造环形回肠造口术。这因结肠穿孔而复杂化,很可能继发于粪性结肠炎,术后第11天需要返回手术室进行剖腹探查并切除肠段和造黏液瘘。然后该患者被转诊至波士顿儿童医院进行动力研究,结果显示结肠动力差,并计划在1年内重新评估动力。:虽然罕见,但对于患有严重便秘的儿童,尤其是男性,高度怀疑获得性肠肌间神经节减少症很重要。