INITIAL EXPERIENCE WITH PER-RECTAL ENDOSCOPIC MYOTOMY (PREM) FOR HIRSCHSPRUNG’S DISEASE: LONG-TERM OUTCOMES OF THE FIRST CASE SERIES OF A NOVEL THIRD SPACE ENDOSCOPIC PROCEDURE

Aims Per-rectal endoscopic myotomy (PREM) has been recently described as a minimally invasive treatment for Hirschsprung’s Disease (HD). This case-series reports safety and long-term outcomes after PREM.

Methods Retrospective analysis of prospectively maintained database of PREM for HD at single center over 4 years. HD was diagnosed& mapped using – barium enema, anorectal manometry (ARM) and sigmoidoscopic cap EMR biopsies. PREM was performed with myotomy extending beyond length of aganglionic segment. Abstracted parameters included demographics, clinical data including laxative use index (LUI = No of laxatives x times the standard dose + use of enema), procedure related parameters and follow-up data.

Results N = 8(6 males, Mean age = 7.1 ± 5.2 years [1–24]).All had rectosigmoid HD. Mean aganglionic segment length-6.8 ± 3.0 cm (2–15).PREM technically succeeded in all. Mean myotomy length-11.3 ± 2.9 cm (8–20).Median procedure time-80 min (IQR 70–120). No immediate adverse events encountered.Mean time to first post-PREM bowel movement −2 days (1–3). Longest follow-up = 48 months; median = 15 months (IQR 2.5–30). All had improvement in stool frequency(1 in 4.5 ± 1.1 vs 1 in 1.2 ± 0.3 days [p = 0.0004, Wilcoxon signed-rank test]). Mean LUI reduction −6.62 (4–11) to 0.25 (0–2) (p = 0.0002).6(75%) did not require laxative. No incontinence noted. Post-PREM high-resolution ARM in 3/8 revealed normal basal and squeeze pressures. First patient had mild anal stenosis 8 months post procedure-single session digital anal dilatation.No other delayed AE.

Conclusions PREM is safe and feasible procedure. Results of this case-series demonstrate excellent long-term clinical outcomes. PREM could be considered a minimally invasive therapeutic option for patients with HD. Studies with larger sample size are required to confirm these findings.