Surgical Techniques for the Treatment of Hirschsprung's Disease-A Historical Systematic Review and Current Status Quo

Hirschsprung's disease, or aganglionosis of the colon, is a congenital disease characterized by a lack of neurons in the enteric plexus of the large bowel due to either migration failure or defective differentiation of neural crest cells. We aimed to conduct a historical systematic review of available literature and personal experience to exhibit the surgical techniques performed in the past and the current status quo of surgery for Hirschsprung's disease.

We searched PubMed, EMBASE, Google Scholar databases for studies examining surgery in aganglionosis or Hirschsprung's disease. The search included all studies published from inception until 9 November 2024. Eligible criteria included all peer-reviewed articles, not necessarily written in English, but also German, French, Italian, and Spanish items dealing with surgery and the prognosis of patients with Hirschsprung's disease after surgical intervention.

A total of 2527 studies were reviewed, and the studies relevant to the surgery were extracted for this historical review. This article delivers a unique historical path and an analysis of some of the most critical surgical techniques for the approach to Hirschsprung's disease, with two outstanding sections on innovative robotic-assisted surgery and life quality after surgery. Currently, surgeons often tailor their approach to Hirschsprung's disease by combining their knowledge and expertise on several surgical procedures and incorporating subtle adjustments based on each patient's imaging, inspection, and pathological findings. The conventional wisdom held that surgeons should perform whichever surgery they felt most comfortable with, regardless of whether a particular technique produced better results. Although the core elements of surgical care have been identified and surgery has been shown to improve outcomes in patients affected with Hirschsprung's disease, the precise etiology and correct treatment for reconstitution of ganglion cells in the aganglionic portions of the bowel are lacking. The surgical treatment options for Hirschsprung's disease have been conceived and implemented after the etiology of Hirschsprung's disease was successfully clarified in the 1940s.

There is still no internationally valid agreement on which techniques should be used for the various forms of intestinal aganglionosis, especially for total colonic and ultra-short intestinal forms. On the other hand, minimally invasive surgery, artificial intelligence, and machine learning are quickly entering medicine and surgery. These approaches will probably modify the surgical approaches to Hirschsprung's disease. In the future, surgeons may integrate new knowledge derived from proteomics and genomics into current surgical procedures. This integration pinpoints a therapeutic approach that may eventually entail aspects of personalized medicine.