A Rare Case of Left Hemicolectomy Masking the Signs and Symptoms of Underlying Hirschsprung's Disease in an Adult

Abstract

Hirschsprung's disease (HD) is a congenital gastrointestinal condition characterized by the lack of ganglion cells within the submucosal and myenteric nervous plexuses in the large intestine. This results in a dysfunctional segment of the large colon, resulting in symptoms such as failure to pass meconium, constipation, and dilated loops of the bowel. The vast majority of patients are diagnosed during the neonatal period, but a handful can be diagnosed later into childhood and adolescence. A rare subset is diagnosed during adulthood, in which the section of the aganglionic colon is minimal yet symptomatic. We report the case of a 54-year-old female presenting with dilated loops of bowel and a remote history of severe constipation, recurrent bowel obstructions, previous left hemicolectomy, and an improvement of symptoms following the procedure. Upon further workup, she was diagnosed with HD, raising the question of whether there should be increased testing for this condition in adults. This case can serve as an example of the need for a more in-depth workup of severe constipation in adults, as the finding for HD in adults is rare but still possible.

Keywords: colon; constipation; hirschsprung's disease; left hemicolectomy; rectoanal inhibitory reflex.

Figure 1. Dilation of the distal transverse colon

Figure 2. Dilation of the descending colon

Figure 3. No volvulus in this area distal to the descending colon dilation seen in Figure 2.