Postoperative Pullthrough Obstruction in Hirschsprung Disease: Etiologies and Diagnosis

Abstract

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.