Novel WNT2B Variants Associated with Congenital Enteropathy and Ocular Dysgenesis: a Multi-System Disorder

Background: Several techniques have been described to taper the dilated small bowel (SB), including LILT, STEP and SILT.In 2021 we proposed an alternative technique designed to improve intestinal motility and decrease complications related to over-dilated SB anastomosis.We herein present our current experience with MATE Methods: Technique: The dilated side-to-side (STS) anastomosis is identified and the mesentery leaves from both the proximal and distal SB loops are separated using blunt dissection.The previous anastomosis is divided longitudinally with a gastrointestinal stapler.Once the SB transection is completed, two separate blind loops of intestine are created, each one with half the circumference of the dilated STS anastomosis.The antimesenteric stapled line is then imbricated with an outer layer of running suture.The blind loops of the tapered SB are then trimmed and anastomosed in an end-to-end isoperistaltic fashion in two layers.Results: Seven patients underwent MATE between June 2028 and June 2024.There were no postoperative surgical complications.The length of the tapering ranged from 10 to 70 cm, corresponding to approximately 21% (range 13-35%) of the remaining SB length.Five out of seven patients presented significant improvement of their symptoms and were able to have their parenteral support discontinued.Conclusions: Modified antimesenteric tapering enteroplasty is an alternative technique to improve intestinal motility and treat patients with dysfunctional STS anastomosis without the need for further SB resection.This bowel-sparing technique represents a valuable option in the armamentarium of the surgeon who manages patients with intestinal failure.