Surgeons who care for patients with short bowel syndrome need experience, creativity, and patience, as the first several months of life for these infants are often marked by frustrating trial and error, frequent complications, unexpected downturns, and extremely slow progress, with the added pressure of trying to “beat the clock” vis-à-vis parenteral-nutrition induced liver failure. Although a multi-disciplinary approach appears to be best, it is important that the team have a unifying philosophy regarding the care of these children. Frequent changes in course or abandoning a particular therapy before giving it a chance to work only worsens the frustration and can further harm the child.
As a general rule of thumb, newborns with more than 60 cm of intestine are likely to wean from parenteral nutrition, those with less than 40 cm are unlikely to become independent enteral feeders without intervention, and those between 40 and 60 cm have an intermediate prognosis. Children with intestinal failure due to NEC are the most difficult to predict as the bowel that remains after resection is often injured due to ischemia and therefore dysfunctional. Most also seem to be able to tolerate more feeds when bowel continuity has been established, making it desirable to close an enterostomy as soon as possible. While full-length bowel dilatation is a welcome sign of bowel adaptation and allows the child with short gut to have a bowel-lengthening procedure, segmental bowel dilatation can be the result of a specific problem (stricture, adhesion, segmental dysmotility) and the cause of a problem (bacterial overgrowth, pseudo-obstruction, pain), and upper GI contrast and motility studies are not especially accurate in distinguishing a true obstruction from a pseudo-obstruction. Given the pressure to advance enteric feeds, one should have a low threshold to explore the abdomen and perform a lysis of adhesions in these children when the possibility of a stricture or adhesion remains and antibiotics are not effective in relieving symptoms.
Most pediatric surgeons trained in a certain era are partial to the Bianchi bowel-lengthening procedure, and, in the right patient, it clearly works – children are often able to wean from parenteral nutrition and they generally do well. But it is becoming increasingly clear that the STEP operation is easier to perform, is probably better tolerated, and also has excellent results. The STEP has clearly become the bowel-lengthening procedure of choice for children with short bowel syndrome who have developed adequate adaptation-associated bowel dilatation.