PER RECTAL ENDOSCOPIC MYOTOMY (PREM) FOR INFANTILE HIRSCHSPRUNG'S DISEASE

Background:

Hirschsprung's disease is a congenital disorder characterized by absence of intrinsic ganglion cells in myenteric plexuses of the hindgut. Patients present with constipation, intestinal obstruction and/or megacolon. Standard treatment includes pull-through (short segment) or posterior anorectal surgical myotomy (ultrashort). Per rectal endoscopic myotomy (PREM) for adult and pediatric HD has been reported earlier. This video demonstrates PREM performed successfully for infantile HD.

Patients and methods:

2-years male child reported refractory constipation since neonatal period; stool frequency – 2/week on combination of 2 laxatives. Barium enema and manometry demonstrated HD. Sigmoidoscopy guided serial deep mucosal biopsies revealed absence of ganglion cells. Transition zone was measured at 5 – 9 cm.

PREM was planned after informed parental consent. Procedure was performed in prone position under general anesthesia. HD gastroscope (GIF-H190, Olympus) with distal transparent attachment and CO₂ insufflation. PREM technique was similar as for POEM. Mucosal incision was taken within 1 cm of dentate line. Tunnel length was 10 cm. Full-thickness myotomy 9 cm was performed in oral to anal direction. Mucosal incision was closed using endoclips.

Patient was maintained NPO for 12-hours post-procedure and diet was gradually started thereafter. IV antibiotics administered for 48-hours and oral lactulose stared once oral feeds resumed and continued for 1 week. Follow up was at 2 weeks.

Results:

At 2-week follow up, patient reports daily 1 – 2 bowel movements on 2.5gm fiber supplement. He does not report any incontinence or constipation.

Conclusions:

Current video case demonstrates safety, feasibility and technique of PREM in infantile Hirschsprung's disease. Procedure requires further evaluation and validation.