Intestinal Rehabilitation, Episode 4: Surgical Management, Part 1
With Dr. Paul Wales & Dr. Michael Helmrath · hosted by Dr. Ellen Encisco & Dr. Tom Ponsky · StayCurrentMD
Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
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What the experts said
Adrian Bianchi first reported the longitudinal intestinal lengthening procedure (Bianchi procedure) that divides bowel along its two leaves and tubularizes it.
Rising direct bilirubin was recognized as a sign that children with intestinal failure would not do well.
The gut doubles in length during the last trimester and the first year of life, and this maturation process occurs when the baby is fed.
Healthy growth of the intestine requires nutrition; anything that disrupts this affects maturation of both absorptive/digestive capacity and peristalsis.
Lengthening bowel that doesn't have peristalsis does not increase absorption; very short bowel children can sometimes come off TPN because their motility is good.
The majority of pediatric short bowel syndrome cases present at birth due to neonatal causes: congenital GI anomalies or acquired conditions like necrotizing enterocolitis.
When reestablishing bowel continuity, size discrepancy between bowel ends must be addressed or a functional obstruction will result even if the anastomosis is patent.
Size discrepancy can be managed by resection to a more appropriate caliber, tapering enteroplasty, or serial transverse enteroplasty (STEP) depending on available bowel length.
Gastroschisis patients tend to not do as well with STEP procedures regardless of whether atresia is present; among STEP procedures performed over the years, gastroschisis cases consistently had worse outcomes.
The enteric nervous system in gastroschisis is damaged from exposure to amniotic fluid and requires a healing and recovery phase; this regeneration is attenuated by dysmotility and stasis, not enhanced.
In atresia without gastroschisis, the bowel may have good peristalsis from working against an obstruction, and when done correctly with proper orientation, a longitudinal lengthening procedure may benefit the child.
Children who undergo STEP procedures in their first year of life sometimes never progress and are labeled as poor motility patients unable to tolerate enteral feeds.
Among patients with limited gut, necrotizing enterocolitis patients do best because they have been fed before, have established GI motility, and the maturation phase has already been initiated.
STEP procedure is not usually performed unless bowel is at least 5 centimeters dilated to make it worthwhile.
In scenarios where bowel is not adequately dilated, options include resecting bulbs with tapering enteroplasty, establishing continuity if possible, or leaving bowel disconnected with distal feeding tube access to grow the distal bowel before reconnection.
The STEP procedure was originally described by HP Kim and Tom Jackson.
For STEP, a transverse supraumbilical abdominal incision is used, all adhesions are lysed, and the entire intestinal tract is laid out from top to bottom to understand anatomy and maximize mobility.
The anti-mesenteric surface of the bowel is marked with surgical pen after drying with a lap sponge; maintaining this alignment prevents longitudinal twisting of the bowel during stapling.
Bowel length is measured before the procedure using 30 silk dipped in mineral oil; caliber and length of the dilated segment to be stepped are also recorded.
An endo-GIA stapler is preferred over an open GIA stapler because the smaller cartridge head is more amenable in smaller patients.
Staple lines are applied perpendicular to the mesentery at 90° and 270° (3 o'clock and 9 o'clock positions if mesenteric vessels are at 0°).
A vascular (white) cartridge with 2.5 mm staples that crimp to 1 mm is used; this is more appropriate for younger patients' bowel thickness and prevents leaks that occurred with classic blue loads in early experience.
Target caliber for STEP is 1.5 cm in babies and 2 to 2.5 cm in older infants or children; making it too narrow risks obstruction, especially in patients with borderline motility.
Before firing the stapler, measurements are taken to both the end of the bowel and to the side to ensure proper caliber is maintained throughout the length.
A U-stitch with 4-0 PDS is placed in the crotch of each staple line to prevent potential leaks at that point.
The procedure is performed with a partner, alternating sides of the bowel, working down the length; 45 mm cartridges are most versatile, but 30 mm and 60 mm cartridges may be needed depending on bowel diameter.
At the top and bottom of the STEP segment, an additional oblique staple line is fired to taper the transition and avoid a 'dog ear' blind loop that can dilate over time.
The STEP procedure is performed with staple lines perpendicular to the mesentery (90° and 270°), not in the anti-mesenteric to mesenteric orientation (180° and 0°).
The duodenum is not stepped; the STEP procedure starts where the bowel begins to dilate, usually distal to the duodenum.
Avoiding staple lines in the duodenum allows proximal access if emergency reoperation is needed for a leak.
If duodenal narrowing is needed, a stapler is used to partially narrow the lateral side away from the bile duct and ampulla; plication sutures are not used because they usually fail.
The duodenum has no mesentery, making it impossible to orient 90° and 270° positions; surgeons who STEP the duodenum end up entering the bowel at inconsistent orientations.
In the 1980s and 1990s, babies with intestinal failure had poor outcomes primarily due to liver progression to inflammation and fibrosis associated with parenteral nutrition, lipids, and phytosterols.
The first 4 months of life is when care for intestinal failure patients is most uncoordinated, and surgical decisions made during this period have the most profound effect on long-term outcome.
Absorptive surface area comes from the waves of peristalsis moving over the villi, not from the exposed length of bowel.
Gastroschisis patients have inherent dysmotility.
At birth, neonatal bowel is usually not dilated enough to apply the STEP procedure.
If one part of the stepped bowel becomes dilated, the STEP segments can rotate away from each other separately, causing twisting and functional obstruction.
After completing the STEP, the length of stepped bowel, new total bowel length, and final caliber (2-2.5 cm) are recorded.