Adult Hirschsprung's Disease: A Case Report and Literature Review

Introduction & Importance: Adult Hirschsprung's disease (AHD) is a difficult diagnosis to make due to its rarity, frequently after emergency interventions have been conducted. We present a case of possible AHD and sketch a classic presentation of Adult Hirschsprung's Disease. This would help recognize and include AHD in the differential diagnoses of chronic constipation where appropriate.

Case Presentation: The case involved a 41-year-old male with a history of multiple abdominal surgeries for volvuli and a ventral hernia repair complicated by post-operative SBO. Presenting symptoms were chronic constipation, abdominal colic, and dilation. SBO secondary to volvulus was discovered, decompressed, and emptied of 3000cc fecal material in the OR. His case was again complicated twice by SBO which led to the suspicion of AHD and instigated this systematic review. Papers were extracted from the EBSCO and PubMed databases. Papers were excluded if patients were younger than 10 years old.

Clinical Discussion: The classic patient will be a male over the age of 10 years old with an average age of 30 years old and a long history of chronic constipation, often complicated by an acute symptomatic obstruction. Patients may have had a history of multiple surgical or non-surgical interventions to relieve their constipation.

Conclusion: AHD is being seen more frequently with the increasing availability of healthcare in underserved areas of the world. Duhamel's procedure is the most effective procedure after diagnosis has been made. Barium enema and a biopsy show hypo- or a-ganglionic segments that are to be resected for curative purposes.