Post written by Akshay Kulkarni, DM, from Midas Multi-Specialty Hospital, Nagpur, India.

This video case shows the procedure of per-rectal endoscopic myotomy (PREM) for a case of Hirchsprung’s disease. It was a particularly difficult case requiring a long (25 cm) myotomy, and a delayed perforation complicated recovery.

However, it was successfully managed endoscopically, and the patient showed long-lasting symptomatic improvement.     

This long myotomy, with a delayed adverse event, is not seen commonly. It was performed for a rare disease, and we observed a peculiar course of recovery, but we were able to manage this with a judiciously combined approach.

The learning lesson here would be to test your limits with endoscopic interventions while being acutely aware of the possible adverse events and prepared to be one step ahead as you anticipate and manage them.  

To our knowledge, this is the longest PREM in the published literature, pushing the horizons of endoscopic interventions further. It also is exemplary in the way it was managed comprehensively with all the loose ends covered. 

A, A barium enema showed a dilated proximal colon and a narrow distal colon and rectum. B, A colonoscopy showed the transition zone at 23 cm. The colonoscopic biopsies confirmed presence of aganglionosis (C) to be present up to 23 cm, beyond which ganglia were present (D) (H&E, orig. mag., ×100).

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