By Mr A.Morabito MD FRCS (Ed), FRCS (Eng) FICS

Consultant Specialist Neonatal and Paediatric Surgeon
Honorary Senior Lecturer University of Manchester - School of Medicine  

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Children with short bowel (SB) are some of the most challenging patients. We acknowledge the fact that the definition of short bowel should be revised as it groups patients with significantly different length of bowel and this can be a limitation when discussing outcomes. What sometimes is referred to as short bowel should be better addressed as dysfunctional bowel where collapsed and or distended bowel loops are responsible for its symptoms. Patients with more than 30 cm of bowel are likely to have a better outcome and therefore it is sensible to wait (within a logical time frame) for adaptation once bowel continuity is restored. These patients can do well if appropriately managed. 

Patients with 5 to 30 cm of bowel require careful evaluation in order to establish a robust plan of action. This group should be referred to as Extreme Short Bowel (ESB) and their management is a complex multidisciplinary process over a prolonged period of time. 

The presence of bowel dilatation leading to vomiting and faltering growth is an indication for surgery in order to restore physiology. 

The main aim of surgery is to re-establish condition as close to a normal physiology as possible regardless of the length of the remaining bowel.  This can be achieved through a “wisely staged process” starting with bowel preservation, expansion and stimulation, thereby preventing the need for central line catheters and ensuring liver function is preserved. Once our patient is ready Autologous Gastro Intestinal Reconstruction (AGIR) should be considered. Parents should be aware that autologous bowel reconstruction may not stand for an operation only but could mean “combined procedures” to help patients towards a life without parenteral nutrition PN.

As no such care coordination is commonly undertaken, the normal pattern established over the years to assess ESB patients has been to:  a) request an “urgent referral“ to a transplant Unit or  b) try to stabilize their clinical condition in order to “wait“ for intestinal adaptation on a long, and sometimes damagingly long time on parenteral nutrition (PN).

Patients rely on PN to survive but unfortunately the neonatal and paediatric liver is more prone to PN related complications and therefore requires careful management in order to avoid fatal liver failure. The majority of these PN patients are managed in centres lacking of the time and expertise with consequent uncoordinated and sub-optimal management.

The integral role and what surgery to be performed in SB is not well understood. 

Surgery is sometimes performed as an “emergency” (on an unprepared patient) with consequent poor results. A normal but not justifiable reaction to poor surgical results is to blame “the condition”, “the patient” or “the procedure”.  This myth should not be accepted.  Better communication and the internet, have lead to higher expectations by the public in the treatment of ESB. Severe short bowel patients should be given the best chance to achieve enteral autonomy or safely undergo transplantation. This process should begin at initial diagnosis.

Paediatric SB patients should be enrolled in a structured programme of Intestinal Rehabilitation (IR) this is a management system not based on operations only, it is focused on what can be done for the patients rather than to the patients. The programme should take into account psychosocial and medical aspects of the disease as well. It should be centred on the patient, the family, liver-sparing PN, central line preservation, sepsis prevention, early institution of oral feeding, minimal initial surgery, tissue expansion and finally definitive surgery or surgeries ( combined techniques)  when patients are in good health and  adequately prepared.