S3691 Hirschsprung's Disease in a 91-Year-Old Male: Oldest Documented Case to Date

Introduction: Hirschsprung's disease (HD) is a congenital disorder defined by the absence of ganglion cells in the distal colon. Its prevalence varies from 1 to 1.63 per 10,000 births. Surgical treatment has reduced disease mortality to 3% in developed countries in younger populations, however mortality remains high in the elderly population. We present a rare case of a 91-year-old man with a long-standing history of diagnosed HD who was managed surgically leading to severe postoperative complications leading to his demise. Case Description/Methods: A 91-year-old man, with biopsy-proven Hirschsprung's at age 46, presented with 2 weeks of constipation and 3 days of obstipation. He managed his disease conservatively with magnesium, docusate sodium, and polyethylene glycol for many decades, requiring colonic decompression only once in his lifetime. On presentation, imaging revealed a completely collapsed rectum and a moderately dilated colon. Initially, colonic decompression was performed with rectal tube placement with no relief. Following this, he was treated with neostigmine however associated complications of sinus bradycardia and hypotension led to prompt ICU transfer. After stabilization, an appropriate bowel regimen was initiated with minimal improvement. When conservative measures were exhausted, a surgical consult was made. The patient underwent a preoperational decompression sigmoidoscopy followed by a partial colectomy with colostomy. Soon after, he developed hypotension and acute hypoxic respiratory failure and ultimately passed away. Discussion: With fewer than 300 adult cases reported over 5 decades, adult Hirschsprung's disease, defined as a diagnosis at the age of 10 years or older, is exceedingly rare. The average age at diagnosis is 24, with the oldest documented case reported being 74 years. It is usually managed conservatively with an appropriate bowel regimen; however, surgery remains the ultimate treatment for refractory cases. In elderly patients, less invasive techniques such as the modified Duhamel or Rehbein procedure may provide benefits, yet the risk of postoperative morbidity and mortality remains high. This case identifies the challenges of managing HD in the geriatric population and poses the question of whether surgery is the right decision to make in refractory cases or not. This case highlights the need for greater awareness and research needed to develop tailored management strategies for this population.