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Short bowel syndrome is a congenital or acquired severe disorder and the optimal treatment strategy needs to be highly individualised with the adjustment of conservative and surgical options
Department of Paediatric Surgery,
University Children’s Hospital Basel (UKBB), Basel, Switzerland
Division of Paediatric Surgery,
Department of General, Visceral and Transplantation Surgery,
University Hospital Heidelberg, Germany
Short bowel syndrome (SBS) is defined as anatomical or functional loss of intestinal resorptive capacity leading to malabsorption, malnutrition and multiple complications,1 including liver failure, central line infections and failure to thrive. SBS in children occurs after congenital or acquired loss of part of the intestine,2 in most cases due to gastroschisis, necrotising enterocolitis, mid-gut volvulus and intestinal atresia.3 In the short-term, the therapeutic goals for SBS are to ensure alimentation by parenteral nutrition as well as early detection and therapy of any occurring complications. The long-term aim is resumption of enteral autonomy. The current therapeutic algorithm to achieve intestinal autonomy encompasses a sequential strategy.
With first-line therapy this can be achieved by conservative intestinal rehabilitation programmes, which include the promotion of enteral feeding, the use of various dietary regimes and antibiotics and the restoration of bowel continuity, closing all enterostomies as early as possible.4,5 In case of complete or partial failure of conservative therapy, different surgical interventions may be applicable as a second-line therapy.6 These can be subdivided into autologous intestinal reconstruction procedures (AIRS) and small bowel transplantation (SBTX).4,7 There is an ongoing strive for optimisation of these techniques. In this review we aim to present the key surgical options for SBS treatment.
Avoidance and reduction of SBS occurrence
In most cases SBS is caused by intestinal resection due to gastroschisis, necrotising enterocolitis, mid-gut volvulus and intestinal atresia.3 Therefore, the decision to proceed with intestinal resection needs to deliberate and sparing the intestine as much as possible has to be the primary concern during the initial intervention. As the total postoperative intestinal length is directly and significantly correlated with the degree of parenteral nutrition needed and the intestinal absorptional capacity within the first postoperative year,8 any intestinal segment that might recover, must be left in situ. A temporary ileostomy with a spare resection followed by a planned second look after one to two days might be helpful to possibly preserve intestinal length. This strategy is particularly successful in preterm and newborn babies, as most of these children tolerate this well and frequently show recovery of the intestine. The early re-establishment of the bowel continuity enables an effective adaptation of the dysfunctional distal intestine and reduces the necessity of parenteral nutrition.9 Therefore, it is recommended to recruit the entire resorption area available by closure of all enterostomies restoring bowel continuity.10–14
Autologous intestinal reconstructive surgery (AIRS)
AIRS procedures are designed to delay intestinal transit, improve intestinal absorption and facilitate enteral autonomy by surgical manipulation of the existing bowel. Different techniques are applicable. However, the primary methods for AIRS used today are longitudinal intestinal lengthening and tailoring (LILT), also known as the Bianchi procedure, and serial transverse enteroplasty (STEP). The spiral intestinal lengthening and tailoring (SILT) technique is a further newly published AIRS option.15 All of these are one-stage procedures that most successfully combine the benefits of intestinal tapering and decrease of transit time with the preservation of enterocyte mass. While SILT needs to be followed-up, LILT and STEP have gained wide acceptance among adult and paediatric surgeons.
The LILT procedure
LILT was published by Bianchi in 1980.16 After blunt dissection of the mesentery in two leaves, each supplying vessels for half of the diameter of the bowel, the intestine is separated longitudinally either by a stapler or by electrocautery. Two tapered intestinal tubes are created, each with half the diameter of the original dilated loop and supplied by the ipsilateral vasculature. By positioning the loops in a circular manner, the new hemi-loops are anastomosed together in an isoperistaltic manner, thereby lengthening the previously tapered bowel (Figure 1).
The STEP procedure
Kim et al. introduced STEP in 2003.17 For STEP, the bowel lumen is partially transected using a linear GIA stapler alternatingly and sequentially applied from side to side of the bowel at 180° from the prior firing. Therefore, a manipulation of the mesentery vessels or bowel anastomoses as for the LILT procedure is not necessary. The STEP procedure leads to a zigzag intestinal channel with a tapered lumen and increased length (Figure 2).
The SILT procedure
SILT was recently published in 2011 by Cserni et al.15 For SILT, the intestine is opened spirally at a 45° and 60° angle to the mesentery axis, then stretched longitudinally and retubularised by sutures, tailoring and lengthening the intestinal segment (Figure 3).
Small bowel transplantation (SBTX)
SBTX is usually performed using intestinal grafts obtained from cadaver donors. However, living donor bowel grafts are increasingly used.18 Living donor SBTX represents an important option to overcome the general organ paucity available for transplantation. Furthermore, it enables optimised planning, improved HLA-matching, reduction of the cold ischaemia time, leading to a reduction of waiting list time and mortality.19,20 Venous drainage of the intestinal graft can either be systemic or portal depending on technical ease and feasibility.21 SBTX can be performed in isolation, combined with other organs, or multivisceral, depending on the individual comorbidities of the patient. The leading type of transplantation in children is combined intestine-liver, while in adults transplantation of the intestine alone is usually performed.22
The outcome of every procedure is strictly correlated to the general status of the patient and the remnant functional intestinal length.8
AIRS is an accepted alternative to SBTX to surgically enhance enteral autonomy, due to less associated complications.10,13 We compared the LILT and STEP procedures by reviewing the literature.4 Both procedures have a similar extent of intestinal lengthening (approximately 70%) and result in improvement of enteral nutrition and reversal of complications in parenteral nutrition in about 60–70% of patients.
SBTX is the most physiologic option in patients with SBS, as a new functional intestine substitutes the lacking organ. The best long-term survival is achieved in children and by combined intestine-liver grafts and the average survival rate after SBTX is >80% after one year, >60% after five years and >40% after ten years.23 Weaning from parenteral nutrition can be achieved in >70% of patients within six weeks after transplantation and there is a reported improvement of quality of life after SBTX.23
However, SBTX is plagued by high morbidity, including the highest rates of rejections, infections, graft versus host disease and post-transplant lymphoproliferative disorders. Therefore, SBTX represents the last resort in case conservative therapy and AIRS procedures fail to improve the intestinal autonomy of the patient. As SBTX still has many complications, this option is reserved to be carried out in specialised centres and the indication for SBTX should be considered individually for each patient, weighing up the possible advantages against the high risk of complications.
An interdisciplinary cooperation in SBS therapy is the most promising setting and should be implemented whenever possible. First-line therapy for SBS is always conservative and surgery should be taken into account in cases of failed intestinal adaptation. The optimal time point for surgery is still under debate. Despite the fact that SBTX represents the ideal surgical therapy, the associated complications prevent it having broader clinical application.
AIRS are accepted procedures potentially enabling the complete weaning from parenteral nutrition and can improve patient intestinal autonomy. AIRS procedures are technically easier to perform and can avoid the necessity of subsequent SBTX.24 If they fail to do so, they might postpone the need for SBTX until a more favourable point in time, implying a crucial gain of time.25,26 The choice for one of the two surgical options is essentially driven by feasibility of the procedure and personal preference and experience. From the technical perspective, STEP has many clear advantages over LILT, as no manipulation of the mesentery vasculature or intestinal anastomoses is necessary. Furthermore, STEP can be applied to shorter intestinal segments with varying diameters and is repeatable on the same intestinal segment, which is not feasible with LILT.4
In the future more therapeutic options for SBS will emerge, some of which will be of a surgical nature. For example, the in vitro creation of an artificial bowel wall established by tissue engineering is rapidly gaining importance and will soon let the surgeon face new challenges. However, more emphasis should be put in an interdisciplinary management of SBS patients and a highly individualised therapeutical strategy, possibly conducted in specialised centres.
Different options are available for SBS treatment. A continuous adaptation of an individualised treatment concept is necessary, within which surgery plays an important role. However, as SBS leads to a broad spectrum of complications, different disciplines need to cooperate and implement multidisciplinary teams specialised in the therapy and follow-up of SBS patients. The commitment of all involved caregivers need to be focused in creating a network that enables the patient to live at home with a barrier-free connection to a specialised multidisciplinary team.