典型(直肠乙状结肠)疾病的结果很好。小肠结肠炎在术后发生,但是通常在 1 岁之后消失。通过正确的医学治疗通常可以治疗遗粪。[51]Levitt MA, Dickie B, Peña A. The Hirschsprungs patient who is soiling after what was considered a "successful" pull-through. Semin Pediatr Surg. 2012;21:344-353.http://www.ncbi.nlm.nih.gov/pubmed/22985840?tool=bestpractice.com可以使用恰当的技术保留括约肌和肛管从而避免真正的大便失禁。[46]Levitt MA, Dickie B, Peña A. Evaluation and treatment of the patient with Hirschsprung disease who is not doing well after a pull-through procedure. Semin Pediatr Surg. 2010;19:146-153.http://www.ncbi.nlm.nih.gov/pubmed/20307851?tool=bestpractice.com[52]Levitt MA, Martin CA, Olesevich M, et al. Hirschsprung disease and fecal incontinence: diagnostic and management strategies. J Pediatr Surg. 2009;44:271-277.http://www.ncbi.nlm.nih.gov/pubmed/19159755?tool=bestpractice.com已经行拖出术但仍不能排空结肠的患者,可以保留一段有移行区的结肠或扭曲拖出。[46]Levitt MA, Dickie B, Peña A. Evaluation and treatment of the patient with Hirschsprung disease who is not doing well after a pull-through procedure. Semin Pediatr Surg. 2010;19:146-153.http://www.ncbi.nlm.nih.gov/pubmed/20307851?tool=bestpractice.com
全结肠无神经节细胞症
对全结肠无神经节细胞症患者的长期随访指出尚未确定对此非常严重疾病的理想治疗。[4]Ruttenstock E, Puri P. A meta-analysis of clinical outcome in patients with total intestinal aganglionosis. Pediatr Surg Int. 2009;25:833-839.http://www.ncbi.nlm.nih.gov/pubmed/19669647?tool=bestpractice.com将一部分的无神经节结肠与正常的有神经节肠道相整合以造袋(Duhamel 手术)的概念会允许液态粪便淤滞、吸收液体、减少排便次数并形成固态粪便(从而改善生活质量),这是一种常常无效的概念,因为造袋引起淤滞。小肠中粪便淤滞产生细菌增值和炎症过程。肠道常常将水分泌到腔中(而不是吸收水分)导致本质上的分泌性腹泻。为了应对营养不良和体液损失需要切除袋,这种情况并不少见。[46]Levitt MA, Dickie B, Peña A. Evaluation and treatment of the patient with Hirschsprung disease who is not doing well after a pull-through procedure. Semin Pediatr Surg. 2010;19:146-153.http://www.ncbi.nlm.nih.gov/pubmed/20307851?tool=bestpractice.com