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 - reoperation - incontinence - constipation
