Kapur Raj P, Smith Caitlin, Ambartsumyan Lusine
Department of Pathology, Seattle Children's Hospital, University of Washington, Seattle, Washington.
Department of Pediatric Surgery, Seattle Children's Hospital, University of Washington, Seattle, Washington.
Pediatr Dev Pathol. 2020 Jan-Feb;23(1):40-59. doi: 10.1177/1093526619890735. Epub 2019 Nov 21.
Some patients continue to have obstructive symptoms and/or incontinence after pullthrough surgery for Hirschsprung disease. Incontinence can be due to injury to the anal sphincter and/or dentate line, abnormal colonic motility (nonretentive), or a chronic large stool burden (retentive). A diagnostic algorithm based on clinical and pathological evaluations can be applied to distinguish potential etiologies for obstructive symptoms, which segregate into anatomic (mechanical or histopathological) or physiologic subgroups. Valuable clinical information may be obtained by anorectal examination under anesthesia, radiographic studies, and anorectal or colonic manometry. In addition, histopathological review of a patient's original resection specimen(s) as well as postoperative biopsies of the neorectum usually are an important component of the diagnostic workup. Goals for the surgical pathologist are to exclude incomplete resection of the aganglionic segment or transition zone and to identify other neuromuscular pathology that might explain the patient's dysmotility. Diagnoses established from a combination of clinical and pathological data dramatically alter management strategies. In rare instances, reoperative pullthrough surgery is required, in which case the pathologist must be aware of histopathological features specific to redo pullthrough resection specimens.
一些患有先天性巨结肠症的患者在拖出式手术后仍有梗阻症状和/或失禁。失禁可能是由于肛门括约肌和/或齿状线损伤、结肠动力异常(非潴留性)或慢性大便负荷过大(潴留性)。基于临床和病理评估的诊断算法可用于区分梗阻症状的潜在病因,这些病因可分为解剖学(机械性或组织病理学)或生理学亚组。通过麻醉下的肛门直肠检查、影像学研究以及肛门直肠或结肠测压可获得有价值的临床信息。此外,对患者原始切除标本以及新直肠的术后活检进行组织病理学检查通常是诊断性检查的重要组成部分。手术病理学家的目标是排除无神经节段或过渡区的不完全切除,并识别可能解释患者运动障碍的其他神经肌肉病理学特征。结合临床和病理数据做出的诊断会显著改变治疗策略。在极少数情况下,需要再次进行拖出式手术,在这种情况下,病理学家必须了解再次拖出式切除标本特有的组织病理学特征。