Hirschsprung Disease beyond Infancy

Abstract

Hirschsprung disease (HD) is a common cause of neonatal intestinal obstruction in which a variable segment of the distal intestinal tract lacks the normal enteric nervous system elements. Affected individuals present with varying degrees of obstructive symptoms, but today most patients are diagnosed within the first several months of life owing to the well-recognized symptoms and the ease of making the diagnosis by way of the bedside suction rectal biopsy. Thus, for the adult general or colorectal surgeon, the vast majority of patients who present for evaluation will have already undergone surgical treatment within the first year of life by a pediatric surgeon. Despite several safe operative interventions to treat patients with HD, the long-term results are far from perfect. These patients may reach adult life with ongoing defecation disorders that require a systematic evaluation by a multidisciplinary group that should be led by a surgeon with a thorough knowledge of HD operations and the potential problems. The evaluation of these patients will form the basis for the majority of this review-however, some patients manage to escape diagnosis beyond the infant and childhood period-and a section herein will briefly address the case of an older patient who is suspected of having HD.

Keywords: Hirschsprung; complications; constipation; incontinence; reoperation.

Fig. 1

The different types of reconstruction “pullthrough” operations employed for the correction of Hirschsprung Disease.

Fig. 2

The algorithm for work-up and management of a patient with obstructive symptoms after a pull-through procedure for Hirschsprung disease. Consideration for proximal intestinal diversion should be based on proximal intestinal dilation and dysfunction. Motility work-up includes assessment of the contrast enema, anorectal manometry, and intestinal motility by manometric evaluation.

Fig. 3

The algorithm for work-up and management of a patient with soiling symptoms after a pull-through procedure for Hirschsprung disease. The examination under anesthesia serves to document the location of the previous anastomosis and the integrity of the dentate line and sphincter mechanism. A low anastomosis below the dentate line or a damaged sphincteric mechanism impairs anal sensation and normal defecation. Bowel management is accomplished with either retrograde enemas or an antegrade washout program deployed with an appendicocecostomy or cecostomy.

Fig. 4

Long-standing outlet obstruction from a restrictive cuff after a Soave pull-through. Note the dilated proximal colon above the cuff.