From
Dr. Paul Wales
Surgical Management and Intestinal Rehabilitation in Short Bowel Syndrome
With Dr. Paul Wales · hosted by Dr. Jason Silverman & Dr. Jennifer Lee
Chapter 1 of 9
Introduction
Introduction and Guest Background
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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
Intestinal failure occurs when intestinal function is inadequate to absorb enough nutrients or fluids to support survival and, in children, also growth.
Short bowel syndrome makes up approximately 85% of pediatric intestinal failure cases, with most caused by neonatal diagnoses (congenital anomalies, NEC, volvulus).
Short bowel syndrome is a functional diagnosis defined by parenteral dependence after gut loss, conventionally 60 days or 2 months, not by a specific bowel length.
Ultra-short gut is defined as less than 10% of expected bowel length for age, approximately 15-16 centimeters in a term baby with 160cm expected small bowel.
Small bowel length measurement is from the ligament of Treitz (DJ flexure) to the cecum, excluding the duodenum.
A term baby has approximately 160 centimeters of small bowel, which nearly triples to 425-450 centimeters by age 5 years.
Reporting small bowel length in children as centimeters alone is meaningless; it must be expressed as percentage of expected bowel length for age due to inherent gut growth potential.
Type 1 short bowel (jejunostomy with no ileum or colon in continuity) is the most challenging subtype, with high output and no hormonal feedback from ileum.
Type 2 short bowel (jejuno-colonic anastomosis with lost ileum) is the most common anatomical subtype in children and harder to manage than type 3.
Type 3 short bowel (retained jejunum and ileum with intact colon) has the best prognosis because retained ileum and right colon provide endogenous trophic peptide hormone release like GLP-2.
Jejunal mucosa has net fluid loss compared to ileum which reclaims fluid, making jejunum-only anatomy particularly challenging for fluid management.
Type 1 patients (jejunostomy) would be expected to have the best response to teduglutide but may not achieve enteral autonomy, creating a philosophical tension between individual benefit and societal cost.
In gastroschisis with atresia, if the baby has inflammatory peel at birth, it may not be possible to repair the atresia at initial surgery; reduction and closure followed by return in 6 weeks is appropriate.
For gastroschisis with atresia and size discrepancy, options include tapering the proximal end with end-to-end anastomosis, or proximal diversion with distal feeding tube access if tissue quality is poor or baby is unstable.
Distal feeding tube placement through a stab incision (not formal mucous fistula) provides clean, easy access with conventional feeding tube connector, making nursing care much simpler.
G-tube placement should have a low threshold in complex multi-stage cases for versatility (venting, decompression, medication delivery, supplementation) and is easiest to place when everything is already open and mobilized.
Most children with shorter gut have some bowel dilatation as part of adaptive response to increase mucosal surface area, but there is a tipping point where dilatation becomes pathological with stasis and bacterial overgrowth.
Surgical intervention for dilated bowel should be based on clinical picture (feeding intolerance, plateau in advancement, regression, symptoms requiring bacterial overgrowth therapy) not imaging alone.
Autologous reconstruction requires complete adhesiolysis, ensuring mesentery lies flat in non-rotation, and addressing any strictures or adhesive bands causing the dilated segment.
Wales would not perform STEP procedure unless bowel length was at least 55 centimeters, as anything less provides no real gain.
The most important aspect of STEP or Bianchi procedures is the tapering component that restores normal caliber, improves peristalsis, and clears bacterial stasis, not the lengthening itself.
Wales has not performed a STEP procedure in 3-4 years, primarily due to complications of bleeding on the staple line which can be refractory to therapy and require significant bowel resection.
Wales has not found that tapering alone (without lengthening) leads to worse outcomes or longer time to full feeds compared to STEP procedures, though the heterogeneous population makes definitive comparison impossible.
STEP bleeding complications occur in approximately 15% of cases, can happen months or years after surgery, and are hypothesized to result from venous congestion on the mesenteric side combined with pro-inflammatory luminal environment.
Therapies for STEP bleeding are essentially anti-inflammatory: enteral omega-3, desonide, 5-ASA products, systemic steroids, and biologics like Remicade, with variable individual responses.
Wales had one patient who required transfusion weekly after STEP at 13 months and ultimately required resection of essentially all stepped bowel at age 16 due to refractory bleeding.
The Bianchi procedure is more technically involved than STEP or tapering, relies on hemi-circumferential blood supply with higher theoretical risk of ischemia/leak/fistula, and Wales does not see a benefit to performing it.
For patients with good bowel length (>50% expected) and a dilated segment, options include tapering that segment or resecting it with primary anastomosis.
For short remnant bowel, the principle is to preserve as much bowel as possible through tapering or lengthening procedures rather than resection, though quality of bowel may matter more than length.
STEP is a misnomer for lengthening; it redistributes mucosal surface area so fluid contacts bowel walls rather than running through the center of dilated loops, not true lengthening in the short term.
During laparotomy for dilated bowel, Wales flushes proximal and distal bowel with saline via Foley catheter, then runs an inflated balloon through the length to detect partial obstructions or webs that saline alone might miss.
When there is a change in caliber from dilated to normal in a patient in continuity, Hirschsprung disease should be ruled out with frozen section in the OR.
For patients with unexpectedly poor function after anastomosis, Wales uses proximal diversion with distal feeding tube to evaluate functional capacity of distal bowel and confirm stooling ability before re-establishing continuity.
There is a tendency for pediatric surgeons to want to fix everything in one operation, but for complex intestinal failure patients, a staged approach is better, investing in the patient's 80-90 year life expectancy.
A staged approach with a roadmap that moves the ball down the field with each operation is more likely to achieve enteral autonomy in the long run than rushing to complete reconstruction.
Approximately 80% of gastroschisis cases are uncomplicated and typically achieve enteral autonomy and hospital discharge around 6 weeks.
A third category of gastroschisis patients start uncomplicated but become complicated due to decisions made or events that occur, a group that should be completely avoided.
Many young adults with history of uncomplicated gastroschisis develop chronic pain and obstructive symptoms, but the gastroschisis diagnosis often falls off their medical record, leading to labels like IBS or functional abdominal pain.
Wales has explored many young adults with gastroschisis history and chronic symptoms, finding kinked mesentery with engorged venous and lymphatic structures suggesting chronic mesenteric congestion, with symptom improvement after mobilization and laying mesentery flat.
Vanishing gastroschisis occurs when the abdominal wall defect closes in utero, causing loss of all exteriorized bowel, leaving only 10-15cm of jejunum and approximately half the colon (the bowel that remained inside the peritoneal cavity).
Vanishing gastroschisis patients have ultra-short gut with all the complications of gastroschisis (short gut, dysmotility, potential hypersecretion), representing a phenotype with multiple categories of intestinal failure.
For vanishing gastroschisis with 10cm jejunum, STEP of such a short segment would not gain significant length; tapering or proximal diversion with distal feeding tube access are better options.
Wales previously published a delayed primary STEP approach for ultra-short gut using distention enterogenesis (sham feeding with G-tube decompression to grow dilated proximal segment before later STEP and anastomosis), showing accelerated gut lengthening beyond natural growth.
Counterarguments to delayed STEP approach include potentially worsening motility by leaving bowel obstructed and dilated longer, and driving cholestasis through inadequate foregut decompression and elevated luminal pressure.
Babies born with duodenal atresia are often born with elevated bilirubins because high luminal pressure in the duodenum slows bile flow and causes cholestasis earlier.
In 2026, Wales would be less likely to use the delayed STEP approach he described 12 years ago, instead favoring distal enteral access with retrograde proximal decompression tube and distal refeeding tube with G-tube, planning staged operations.
A 10 French Blake tube can be placed retrograde through a stab incision into the distal duodenal segment with tip passed proximally into second portion of duodenum to decompress foregut, functioning like a stoma while preserving bowel length.
Distal sigmoid colostomy to divert fecal stream from perineum can be considered selectively in short gut patients, providing benefits of colon in continuity while avoiding perineal soiling, easier fluid balance tracking, and prevention of perineal rash.
Wales uses distal sigmoid colostomy much less commonly now than when he published on it in 2009, but it remains a strategy for short gut with colonic remnant.
For a 28-week infant with vanishing gastroschisis presenting today, Wales would almost certainly not perform one-stage operation, instead using retrograde proximal decompression tube, distal feeding tube, and G-tube with planned return for staged procedures.
Twenty years ago, there was a race against time to get patients off TPN because intestinal failure-associated liver disease was the #1 cause of mortality and bowel transplant, with babies developing cholestasis at 2 months and liver failure by 1-1.5 years.
Continuous feeds may provide more enteral tolerance and faster TPN weaning, but produce children with oral aversion who never learn to eat, which has huge negative implications.
Currently, the ability to prevent liver failure is much better; kids rarely die of liver failure, and the #1 reason for bowel transplant listing is now loss of IV access in long-term TPN patients, not liver failure.
The current approach prioritizes establishing normal feeding behavior and introducing age-appropriate foods (purees at 5-6 months when head control is adequate) even in patients with complex anatomy or no bowel in continuity.
Children with short gut do better orally if eating solids rather than being stuck on tube feeds.
Sham feeding (oral feeding that drains out of tube or proximal stoma) should be used even in patients with very short gut, proximal stoma, or no bowel at all to develop feeding skills.
Oral feeding in complex short gut patients should be viewed as skill development, not nutritive, allowing children to learn skills that will pay off when continuity is eventually restored.
Wales is very aggressive about preserving even questionable ischemic bowel by diverting proximally and protecting distal bowel with feeding tube access, as much of that bowel regenerates and becomes usable rather than being permanently lost if resected.
Outcomes are better with multidisciplinary care where team members bring expertise from different disciplines and look at patients through different lenses.