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Surgical Management and Autologous Intestinal Reconstruction in Short BowelSyndrome
Matthijs J. Hommel, Bsc, Medical Student, Robertine van Baren, MD, PaediatricSurgeon, Jan Willem Haveman, MD, PhD, Abdominal and Transplant Surgeon
PII: S1521-6918(16)00022-6
DOI: 10.1016/j.bpg.2016.03.006
Reference: YBEGA 1417
To appear in: Best Practice & Research Clinical Gastroenterology
Received Date: 15 January 2016
Accepted Date: 5 March 2016
Please cite this article as: Hommel MJ, van Baren R, Haveman JW, Surgical Management andAutologous Intestinal Reconstruction in Short Bowel Syndrome, Best Practice & Research ClinicalGastroenterology (2016), doi: 10.1016/j.bpg.2016.03.006.
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Surgical Management and Autologous Intestinal Reconstruction in Short Bowel Syndrome
Matthijs J Hommel, Bsc, Medical Student
Robertine van Baren, MD, Paediatric Surgeon
Corresponding author:
Jan Willem Haveman, MD, PhD, Abdominal and Transplant Surgeon
Department of Surgery
University Medical Center Groningen, University of Groningen
Hanzeplein 1, 9700 RB Groningen
The Netherlands
Phone: +31 50 361 2283
Fax: +31 50 361 1745
Keywords:
Intestinal failure, short bowel syndrome, longitudinal intestinal lengthening and tailoring,
serial transverse enteroplasty, spiral intestinal lengthening and tailoring, bariatric surgery
Total words used: 6861 (including abstract, summary bullet points, tables and figures)
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A. Abstract
Short bowel syndrome (SBS) is a serious condition with considerable morbidity and
mortality. When treatment with parenteral nutrition fails and life-threatening
complications occur, autologous intestinal reconstruction (AIR) should be considered
before intestinal transplantation (ITx). Single or combined ITx should be reserved for
patients with severe liver disease and as last resort in the treatment of SBS. Longitudinal
intestinal lengthening and tailoring (LILT) has proven its value in AIR, but its availability
depends on the expertise of the surgeons. Serial transverse enteroplasty (STEP) has
similar success rates as LILT and fewer patients progress to ITx. STEP is also applicable
at small bowel dilatation in ultra-short bowel syndrome. The scope may be widened when
duodenal dilatation can be treated as well. Spiral intestinal lengthening and tailoring
(SILT) is a promising alternative. More research is needed to confirm these findings.
Therefore we suggest an international data registry for all intestinal lengthening
procedures.
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A. Introduction
Parenteral nutrition (PN) is the standard treatment in short bowel syndrome (SBS). After
its introduction in the late 1960’s (1), several important improvements in the treatment of
PN have been introduced. Despite these improvements, PN-dependent patients are still
prone to develop severe complications contributing to a high mortality (2–4). Intestinal
transplantation is the standard treatment for patients with intestinal failure who develop
severe complications from PN or experience a low quality of life (5). The accepted
indications nowadays for intestinal transplantation (ITx) are recurrent catheter related
infections, thrombosis of the central veins, alteration in growth and development of
infants, severe dehydration with refractory electrolyte changes and liver failure and/or
liver cirrhosis with portal hypertension (5). The first successful isolated transplantation of
the intestine was performed in 1989 (6), 2 years after the first successful multivisceral
transplantation in 1987 (7). Improvement in surgical techniques, novel
immunosuppressive agents and advances in anaesthesia and critical care led to an overall
increase of intestinal transplantations worldwide. However, after the initial enthusiasm it
became evident that the long-term results were disappointing with a 5-year graft survival
of only 50% for patients transplanted after 2000 (8). As a consequence, the number of
intestinal transplantations has decreased from more than 200 procedures in 2008 to only
120 cases in 2012 (8). Given the severe complications and the disappointing survival of
ITx and low quality of life of patients on PN, all efforts should be made to prevent a SBS.
The ultimate goal of treating SBS is to achieve enteral autonomy through medical and
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surgical intestinal rehabilitation. The aim of this review is to discuss the initial surgical
management and autologous intestinal reconstruction (AIR) in patients with SBS.
A. Definition and aetiology of short-bowel syndrome
B. Definition
Short bowel syndrome (SBS) is a condition in which there is a congenital or an acquired
shortage of small bowel length and thus a lack of absorptive small bowel surface. This
leads to the incapability of retrieving enough nutrients, water and electrolytes to ensure
growth in infants and body maintenance in adults and therefore requiring long-term
administration of PN. The broader term ‘intestinal failure’ includes in addition to SBS,
mechanical abnormalities and intrinsic bowel diseases. These patients have a normal
bowel length, but experience severe bowel obstructions or functional problems caused by
motility disorders or mucosal abnormalities (9,10). In this chapter, we will limit ourselves
to SBS with an anatomical absence or loss of significant bowel length.
The total length of small bowel in a healthy person depends on the gestational age within
a natural spread. The younger, the more growth potential there is. Loss of for instance 50
cm is clearly more severe in a premature born child then in a mature neonate (11). From
the second year of life, bowel length does not significantly increase further and stays
around 340 cm. However, the diameter of the intestinal lumen still increases with age
(12).
There have been made functional and anatomic classifications, both in 3 types, which
enables us to compare treatments of SBS. The classification based on functional outcome
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was introduced by Shaffer in 2002 (13). The latest revision has been made recently by
Pironi et al (see Table 1): type I SBS being acute, short-term and usually self-limiting.
Type II stands for a prolonged acute condition, often in metabolically unstable patients
requiring complex multi-disciplinary care and intravenous supplementation over periods
of weeks or months and type III representing chronic intestinal failure in metabolically
stable patients, requiring intravenous supplementation over months or years; it may be
reversible or irreversible (10,14). In adults the minimal length needed for functional type
I or II SBS is at least 115 cm in patients with an endjejunostomy, 60 cm in case of a
jejunocolic anastomosis and 35 cm if a jejunoileal anastomosis with an intact colon is
present (Table 1 and Fig. 1) (15). Although the exact length is not part of the generally
accepted functional classification, we thus know that the remaining bowel length is an
important prognostic factor to estimate the amount of enteral autonomy that reasonably
can be expected. In addition, a detailed description of the remaining length of the
duodenum, jejunum, ileum and colon makes a comparison of the effectiveness of AIR
possible.
In the anatomical classification, type I represents the potentially severest condition being
an end-jejunostomy. In type II there is no ileocaecal valve present, but there is jejunocolic
continuity. In type III there is a jejunoileal anastomosis and the ileocaecal valve is
preserved (Fig. 1) (16).
It is clear from these classifications that small bowel length, presence of an ileocaecal
valve and colon length are important prognostic factors in SBS. Other factors that should
be taken into account are the age of the patient, diagnosis of an intrinsic bowel disease
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like Crohns’ disease or a systemic disease, the presence of duodenum and the length of
the remnant bowel (see Table 2) (17).
B. Aetiology
The aetiology of SBS differs between children and adults. In children SBS is most often
the result of extensive resections due to necrotizing enterocolitis (NEC), complicated
gastroschisis and midgut volvulus resulting from malrotation and severe intestinal atresia
(18). The most common causes of SBS in adults are bowel ischemia due to superior
mesenteric artery thrombosis, Crohns’ disease, volvulus and abdominal trauma (19). SBS
as a complication of bariatric surgery is relatively upcoming and therefore we consider it
worthwhile to include this in our review.
A. Initial Surgical Management
The initial management of SBS starts immediately after surgery. Abdominal sepsis and
organ failure must be treated if present. Enteral nutrition has to be initiated as soon as
possible to promote intestinal adaptation (20). Parenteral nutrition, management of
bacterial overgrowth and treatment of PN associated liver disease are all essential in
treating SBS patients, but are not within the scope of this chapter.
All these aspects and possibilities should be discussed in a multidisciplinary team (21).
Patients with intestinal failure must preferably be seen in an intestinal failure outpatient
clinic by a dedicated gastroenterologist, gastrointestinal surgeon, dietician, stoma or
wound nurse, psychologist and other specialists if necessary. A restorative surgical
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procedure has to be discussed with the patient in order to restore the intestinal continuity.
It is essential that the patient is in the best possible condition and that the surgical team is
fully prepared by reviewing the surgery reports and radiographic imaging. Patients must
be in a stable, anabolic state and infections and sepsis settled down. In a non-hostile
abdomen it should be considered to restore continuity as early as possible (20). In
addition, our own experience is that a relaparotomy performed more than 7-10 days after
the initial abdominal exploration has a high change of iatrogenic bowel perforations and
intra-operative complications. Most patients would benefit from a delayed intestinal
reconstruction procedure performed at least 3 to 6 months after the last surgical
procedure. Important principles of these operations are a complete adhesiolysis to get a
good overview of the complete intestine and identify all internal fistulas. Also, resections
have to be limited to a minimum with tension-free, well perfused anastomoses. All
anastomoses have to be covered by peritoneum, omentum if present or at least the
abdominal wall. Coverage of the intestine by abdominal wall reconstruction or biomesh
interposition when necessary is essential after reconstructive surgery.
A. Intestinal Failure after Bariatric Surgery
B. Introduction in bariatric surgery
Currently, bariatric surgery is the most effective ultimate treatment for severe obesity
(22). The dramatic increase in incidence of overweight, severe and morbid obesity
worldwide is a well known ongoing trend. Consequently, bariatric surgery has become
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one of the most performed abdominal surgical procedures worldwide. Two different
mechanisms contribute to weight loss after bariatric surgery, being restriction and
malabsorption, or a combination of both. Two restrictive procedures are commonly used:
gastric banding and most often a sleeve gastrectomy. Nowadays, combined restrictive
and malabsorptive surgery is most often performed. In this matter, the procedures of
choice are the Roux-Y gastric bypass, distal gastric bypass and the biliopancreatic
diversion with or without gastric sleeve (23,24).
B. Complications can lead to SBS
All these operation differ in effectiveness, but also in potential complications. The
incidence of severe complications that can potentially lead to SBS is very low. However,
due to the dramatic increase in the amount of procedures, complications of bariatric
surgery have become an important cause of SBS. There are several mechanisms that can
lead to intestinal failure after bariatric surgery. First of all, severe malnutrition from a
very short common channel <50 cm can lead to dramatic weight loss that results in severe
protein-caloric malnutrition, vitamin deficiencies and liver failure. The malabsorbtive
procedure should partially be reversed by increasing the bowel length of the ‘common
channel’ after a period on PN and before irreversible liver failure has occurred. Secondly,
catastrophic gut loss is the most severe complication possible and can lead to permanent
SBS. This may be the result of internal hernias and volvulus due to the altered anatomy,
incarcerated incisional hernias, mesenteric ischemia, obstruction and necrosis due to
severe adhesions. Finally, complications like anastomotic breakdown, perforations and
fistulas can lead to gut loss (22).
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Internal hernias are of specific concern after bariatric surgery. Internal hernias are
difficult to recognize clinically and radiographically and require specific knowledge of
the bariatric procedures (Fig. 2). Currently, most surgeons perform an antecolic gastric
bypass, as there is evidence of a lower incidence of internal hernia’s than with a
retrocolic gastric bypass (25). Although ischemia and necrosis from the herniated
segment as well as the small bowel bordering the defect can occur. Early recognition,
urgent laparoscopic exploration and closure of the defects are important aspects to the
success of treatment. Also, mesenteric ischemia due to thrombosis following altered
coagulation or a non-recognized clotting disorder can lead to dramatic gut loss (26).
B. Patient care
Patients should be referred to a centre with expertise in intestinal failure after the initial
dramatic event. Obviously, these patients need a thorough and multidisciplinary
evaluation. Clinical review of the patient medical history and operative notes are
essential. The anatomy and residual length of the intestine should be confirmed with
radiological studies and if necessary endoscopic evaluation. Malnutrition, electrolyte
disturbances, metabolic and vitamin deficiencies should all be corrected. When indicated,
coagulation blood testing, visceral angiograms and full liver function tests with or
without a liver biopsy should be performed. As mentioned before, we recommend
restorative surgery within 7-10 days or after 3 to 6 months. The aim of this procedure is
restoring the nutritional anatomy, reverse stomas and fistulas and avoid the recurrence of
severe obesity. In patients with critical bowel length, all efforts should be made to
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minimize additional bowel resection. Reversing bypassed segments and an additional
AIR can be offered if needed.
A. Antiperistaltic segments, colon interposition and nipple valve reconstruction
B. Antiperistaltic segments
In a select group of patients, interposition of an antiperistaltic segment might help to
wean from PN. The indications for such procedures are rapid transit diarrhoea and/or a
persistent high-output stoma despite conservative measures (27). The remaining bowel
should be at least 25 cm. An important advantage of this procedure is that there is no
need for a dilated bowel. Both the LILT and STEP procedure can only be performed on
dilated segments of bowel. There are several options in implementing a reversed
segment. The most used option is to reverse, thus not transposition, a 10-12 cm segment
of terminal ileum and to anastomose the distal end to the terminal ileum and the proximal
end to the colon (27). Another option is to isolate 10-12 cm of ileum and reverse
transposition this between the proximal jejunum loops. Continuity is then restored with 3
end-to-end anastomoses. The reversed segment decreases transition time, reduces the
myoelectric activity and consequently improves the absorption of nutrients. An important
disadvantage is the risk for obstruction by isolating a segment that is too long. Also,
reversed segments cannot be used in a very short remnant bowel (<15cm). Failure and
complications can lead to loss of the reversed small bowel segment.
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There are only a few series that describe the results of antiperistaltic segments in SBS.
The largest study is a retrospective analysis of 38 patients from the group of Panis (28).
After 5 years of follow-up 45% of their patients were completely weaned from PN. It has
to be mentioned that all these procedures were combined with continuity restoration by
stoma reversal, consequently inducing a significant gain in bowel length. Despite the
encouraging results and acceptable complication rate this method has not gained much
popularity (29,30).
B. Colon Interposition and nipple valve construction
The first publication on isoperistaltic colon interposition in dogs was done by Hutcher et
al in 1971 (31). Only very few reports have been published about colon interposition ever
since (32,33). Considered as important potential disadvantages are bowel obstruction,
bacterial overgrowth and significant loss of bowel in case of complications. The interest
in construction of artificial valves lies in the observation that a present ileocaecal valve
has a positive influence on achieving enteral autonomy. An artificial valve is constructed
at the intussusception of the distal ileum, thereby increasing the transit time and
improving the enteral absorption. There are only very few cases known in literature,
therefore we do not recommend to use this procedure as a treatment of SBS (34).
A. Longitudinal Intestinal Lengthening and Tailoring
B. Introduction and technique
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In 1980, Bianchi described the first longitudinal intestinal lengthening and tailoring or
LILT procedure in an experimental model using pigs (35). The first clinical application
was performed one year after (36). Bianchi isolated a segment of dilated bowel from the
rest of the bowel by dividing the proximal and distal end. Then, an avascular plane
between the double leaflets of the mesentery is created. After confirmation of this double
plane and having successfully separated these parts, the dilated bowel segment is divided
using a linear stapling device. Three years later, Bianchi described an alternative for the
stapling technique by cutting the bowel instead of stapling and close it afterwards with
use of a hand-sewn technique, because of interloop fistulas which occurred frequently
when the stapling technique was used (37). After creating an end-to-end anastomosis
using a hand-sewn technique, the isolated bowel segment is connected proximally and
distally to the small bowel or colon (Fig. 3). The net result is a doubling in length of the
isolated bowel segment and a reduction of the diameter by half the size.
Anatomical criteria for LILT are an intestinal diameter >3 cm, residual bowel length of >
40 cm and a length of dilated bowel loops >20 cm (38). Significant more complications
are described after performing a LILT in shorter bowel segments (39). LILT can lead to
less bacterial overgrowth and stasis. Although, evidence on treating bacterial overgrowth
with LILT is limited. Bianchi has described a protocol for the management of SBS with
an important role for intermittent clamping of the tube jejunostomy to expand the
diameter of the small bowel (40). Because the results of LILT are already well reviewed
elsewhere, we will only give a brief description of the outcome and complications (40–
42).
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B. Enteral autonomy and survival
The degree of intestinal lengthening is significant after LILT. Although the isolated
segment is doubled in length, the amount of intestinal lengthening of the entire bowel is
reported 48-55% (41,42). The most recent series report a success rate of 37-91% for
achieving enteral autonomy (40,43–45).
B. Complications
Initially, the reported complication rate was high with anastomotic stenosis (18%), staple
line leakage (13%), interloop abscesses (7%) and fistula formation (7%)(21,42,46–50).
Mortality in these series resulting from intra-abdominal sepsis and liver failure were
around 30%. However, recent series show that with more surgical experience an
acceptable complication rate and no surgical related mortality can be achieved (45). It
must be mentioned though that even the most recent series not always clearly describe
postoperative morbidity (40,45,51). Survival after LILT is comparable to other AIR
procedures between 87-95%. The need for ITx is relatively low between 10-26% after
LILT.
B. Perspective
A disadvantage of LILT is that it cannot be applied when the vessels in one of the
mesenteric leaves are compromised. Another limitation is LILT being a one-time surgery
that cannot be repeated on the same intestinal segment, as the double leaves of the
mesentery can only be separated once. LILT is considered a primary treatment in SBS
and is usually performed prior to any other AIR procedure. However, recently it was
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successfully applied in a 2-month old infant who experienced redilatation after a prior
STEP. Without major complications reported, the patient weaned from PN, however,
there is only a short-term follow-up of 7 months (52).
Due to the lack of expertise worldwide and upcoming alternative treatments, LILT is
nowadays performed only sporadically. Nevertheless, it is the only lengthening procedure
with over 30 years of clinical application in the field of SBS and novel treatments should
therefore be compared to the results achieved with LILT.
A. Serial Transverse Enteroplasty
B. Introduction and technique
Serial transverse enteroplasty or STEP is a surgical technique to taper and lengthen the
dilated small bowel. STEP was first described in 2003 in a porcine model as an
alternative to LILT because of its minimal manipulation of the mesenteric vessels (53).
That same year the procedure was performed at first in a 2-year old child with SBS (54).
Nowadays, STEP is next to LILT one of the two mainly accepted surgical approaches for
the treatment of SBS (40,41,55,56) and congenital dilatation and atresia (57).
STEP is based on the anatomical principle of the vessels rising from the mesenteric
border and enfolding the bowel perpendicularly to the long axis of the bowel. Therefore,
there will remain no segments devascularized afterwards. With the use of a linear stapler,
a zig-zag pattern is created with staples in opposite directions (Fig. 4), keeping a distance
of 2 to 2.5 cm and maintaining a luminal diameter of approximately 2 cm dependent on
the age and size of the patient (53). There is no lower limit in bowel length to perform
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STEP, but a minimal luminal diameter of 3.5 to 4 cm is required (53,54). One of the
characteristics of the procedure is a minimal manipulation of the mesentery. Also,
because there are no anastomoses required, the intestinal lumen remains closed,
consequently reducing bacterial contamination. STEP is considered to be a technically
less challenging procedure in comparison to LILT and has the possibility to customize
the tapering of the bowel (42,58), which is of importance when an asymmetrical
dilatation of the bowel is present (53).
B. Indications
The aim of STEP is similar to that of general AIR procedure. STEP has been used to treat
SBS caused by gastroschisis, proximal jejunal atresia, NEC and midgut volvulus
(55,59,60). STEP has proven its value as primary therapy in neonates with congenital
intestinal dilatation and atresia. It is possibly the only surgical intervention feasible for
patients with ultra-short bowel syndrome (USBS) or SBS with a remnant bowel length of
<20 cm (61–64). Also, STEP has successfully been performed in adult patients with a
90% survival rate and 47% of all patients who underwent STEP weaned from PN (44).
Furthermore, STEP has a clear long-lasting influence on bacterial overgrowth and the
associated D-lactic acidosis and therefore being a specific indication (65,66). STEP is
preferred in particular as an additive to previous AIR procedures. It is important to realize
that it is possible to perform STEP after LILT. Recently, Bueno et al reported a
successful attempt to treat the dilated duodenum of 3 patients using a modified STEP
procedure (67). To our knowledge, there are only few cases known where STEP was
applied to the duodenum (66). Their promising findings may widen the scope of STEP,
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whereas a significant amount of patients who underwent AIR still have to rely on PN
(55,56,68,69). Studies in larger populations are needed to confirm these effects on
dilatation of the duodenum.
B. Enteral autonomy
An important predictive factor for achieving enteral autonomy is the post-operative
length of the small bowel (41,56,68,69). Multiple studies report the possibility of
lengthening the small bowel over a 100% of its pre-operative length (56,60,61,69,70). In
a systemic review of 109 patients who underwent STEP, a median percentage of 63%
lengthening was reported (42). There is some variety in this finding, whereas other
studies report a lengthening of 46-69% of the small bowel (41,55,71). The majority of
patients who underwent STEP experience an overall improvement in nutritional status
and a decrease in requirement for PN (60,70,72,73). These findings are supported with
experimental evidence in animal and human studies. It is known that in patients with
SBS, the intestine is able to adapt to enlarge the uptake of nutrients after resection (74).
The absorption of xylose, which is a marker for the uptake of carbohydrates and mucosal
integrity, generally improves after STEP as also the faecal fat content which is related to
absorptive function of the bowel. Besides, motility patterns are preserved after STEP
(65,75), with better bowel ratios and stool consistency and frequency improving (55).
These improvements in absorption and function continue after both STEP and LILT and
takes around 1 – 2 years (42), but can take years to reach a final plateau (55). If weaning
from PN cannot be reached, STEP at least is able to postpone ITx (38). The number of
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patients who receive ultimately an isolated or combined intestinal transplantation varies
between 5% and 16% (41,42,73).
B. Survival and complications
Intestinal lengthening procedures of the last 15 years show a consistently high survival-
rate with reports of 89% by the STEP Data Registry and up to 95% in a single-centre
retrospective study. The main causes of death were liver failure and sepsis (41,42,58).
Recent smaller studies report similar survival rates up to 100% (60,61,70,72,76).
It is known that complications are common in STEP procedures, with a chance of
intestinal bleeding being up to 26%, which is higher compared to other lengthening
procedures (41,77). A recent study also identified a rather high infectious burden of 39%
in 14 patients who underwent STEP, which could be of importance considering that
sepsis is the second cause of death after STEP (78). Further research is required to
identify the relation between infection and STEP. Redilatation is considered a serious
complication effecting the nutritional status of the patient causing a lower weaning rate
and higher mortality (79). The exact mechanism behind redilatation is still unclear. In
literature, rates of redilatation requiring a re-STEP procedure go from 25% up to 49%
(42,58). Re-STEP is not an inevitable problem and has proven its feasibility in both
animals (80) and humans (81) with up to 50% of the patients weaned from PN. The
possibility of re-STEP is of specific importance in case of bacterial overgrowth which
can occur frequently as a complication of AIR (79).
B. Long term outcome and perspective
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Since the introduction in 2003, many studies have shown the positive effect of STEP.
Determining the long term outcome has been difficult due to a limited follow-up time of
only several years (41,42,55,70,82). More recently, Pakarinen et al reported the longest
follow-up time to date with a median follow-up time of 9.7 years (4.9-14 years) of 8
patients (59). Indications for intestinal lengthening were intestinal failure due to SBS,
dilated bowel segments and complications of bacterial overgrowth. Their conclusions
support the previous findings with a survival rate of 90% and a promising weaning rate
with 8 out of 9 patients remaining off PN at median 6.9 years after AIR. Interestingly,
they found the same survival and weaning rate for children with SBS without AIR. These
numbers and conclusions have to be interpreted carefully though, considering the sample
size and retrospective character of this study. Therefore, larger population studies are
needed, preferably including a control group, although due to ethical reasons such a study
will probably not be conducted. However, the International STEP Data Registry
(www.stepoperation.org) could confirm the promising findings with a next report (71).
The scope of STEP can possibly be widened further by using delayed primary STEP in
children with USBS, which remains a challenging condition and still has a poor outcome
(62). The principle of this approach rests in obstructing the small bowel intermediately
and facilitate the dilatation of the bowel with sham feeds to optimize the bowel for STEP.
This technique accelerates the growth potential of the bowel, with an observed increase
of the small bowel length up to 294%. Controlled bowel expansion is not a novel
concept, whereas it has already been applied previously in LILT (21,83–85). Further
research is needed to confirm these promising results of delayed primary STEP as
treatment for USBS and trace the mechanisms behind it.
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A. Spiral intestinal lengthening and tailoring
Spiral intestinal lengthening and tailoring (SILT), was introduced in 2011 by Cersni et al
in an experimental animal model as a possible alternative and additive to STEP and LILT
(86). With SILT, incisions in the mesentery are made carefully without damaging any
vessels. Next, the intestinal wall is cut spirally in an angle to the longitudinal axis.
Basically, the total length will increase and its diameter is reduced by stretching the
bowel over a tube preserving the adjust luminal diameter (Fig. 5) (87). SILT was then
applied in vivo to porcine small bowel in which the jejunum was lengthened with 75%
and the luminal diameter reduced by 56% (88) after inducing dilatation with controlled
bowel expansion. An advantage of SILT is the mild alteration of the position of the
intestinal muscle fibres and therefore should influence peristalsis less. A major
disadvantage of SILT is the requirement to open the lumen completely, whereas in a
STEP procedure the lumen remains closed, reducing bacterial contamination. Thereto, in
an experimental model a modified SILT procedure was developed where the mucosa is
remained intact with incisions made only in the muscular and submucosal layer so that
the lumen is only opened on the outer ends (89).
Recently, the first clinical application of SILT was successfully introduced on a 3-year
old patient after initial controlled bowel expansion (90). In this case, the dilated segment
was lengthened by 81% (from 11 to 20 cm) leading to an increase in total jejunal length
by 41% and a reduction of the diameter by 50% to 2 cm. The patient was weaned from
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PN 4 weeks after surgery and still is at 6 months follow-up. The second successful
application on a 10-month old neonate led to a lengthening of 125% (from 4 to 9 cm)
without controlled bowel expansion (91). This patient did not fully wean from PN, but
did well with a PN requirement of 18% at 12 months after surgery. The indication for
AIR in both cases was SBS due to midgut volvulus. In neither case serious complications
were reported. These case reports prove that SILT has potential as treatment in SBS. Its
remarkable technique is also already studied for other applications (92).
A. Patient selection and care
Specialized multidisciplinary programs to optimize the management of intestinal failure
have irrefutable improved the outcomes in the last decade (93). The chance of weaning
from PN and survival rates increased, whereas the cases of central line infections and
intestinal failure associated liver disease decreased. Also, patient selection is an important
key to successful intestinal lengthening procedures and to prevent unnecessary
procedures in patients who could wean from PN without surgery (38). STEP can be
performed at any age with cases in literature varying from the first day after delivery until
adulthood (41,57). Performing LILT in neonates is not without controversy because of
unfavourable outcome and complications (39), although LILT was performed
successfully at the age of 6 weeks. For SILT however, no conclusions can be drawn yet
due to its recent introduction.
Liver failure is an important contraindication to intestinal lengthening procedures (94).
Patients with advanced liver disease are facing poor prognoses after AIR and may benefit
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more from an isolated liver transplantation prior to AIR or a combined intestinal/liver
transplantation if enteral autonomy is not expected after AIR. (41). Nevertheless, there is
a lack of consensus in literature about when intestinal lengthening procedures can be
performed in patients developing liver disease (55).
A. Discussion and Conclusion
SBS has a dramatic impact on the quality of life of a patient and is associated with
significant morbidity and mortality. Therefore, it is obvious that every effort should be
made to prevent SBS by limiting bowel resections to an absolute minimum and treat the
underlying disease. Moreover, patient care for SBS must be optimized in a specialized
multidisciplinary intestinal failure program to prevent complications of PN and contain
intestinal failure associated liver disease, since this is associated with a higher morbidity
and mortality. Therefore, one of the keys to success of AIR in SBS is patient selection. If
AIR cannot lead to PN independence, it should be considered as an option to postpone
intestinal or combined transplantation, which is currently the last resort for these patients.
If AIR is indicated, the first step is restoration of small bowel continuity through
relaparotomy in case of unused intestinal segments. In a non-hostile abdomen this can be
done relatively early, meaning within 7-10 days, after the initial surgical procedure. The
alternative approach is a relaparotomy 3 to 6 months later. AIR can be performed as well
during this operation if the bowel is critically short. Important principles of AIR
procedures are knowledge of the exact altered gut anatomy and a thoroughly performed
adhesiolysis to recognize all fistulas, obstructions or strictures. Any anastomosis has to be
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well perfused, tension free and covered with peritoneum, omentum and at least the
abdominal wall.
As mentioned, AIR should be considered in every patient with SBS and persistent PN
dependence who reached a plateau in their intestinal adaptation. Over the last decades
several surgical procedures have been introduced. The least common performed
procedures are antiperistaltic segments and colon interposition. Because of complications
like obstruction, rapid transit time and disturbance of motility patterns, antiperistaltic
segments and colon interposition did not find its way into the clinic, despite initially
promising results. Generally, there are serious concerns on the effectiveness and
complications of these procedures. Therefore, we cannot recommend this as therapeutic
options in the treatment of SBS.
Since LILT is surgically a challenging procedure, the type of reconstruction will depend
first of all on the experience of the surgeon. If the expertise is not present, STEP should
be the first surgical treatment to consider. LILT doubles the length of the isolated
segment, whereas in STEP the gained length depends on the extend of the dilated bowel.
In clinical setting it is seen that with STEP more length is gained compared to LILT.
Both procedures show good survival rates of around 90%. In studies with larger
populations included, average rates are reported up to 69% for STEP vs. 55% for LILT.
PN independence is achieved in both LILT and STEP in up to 55-60% of the patients, but
there is a large spread of these numbers in literature, thus careful interpretation is
therefore needed.
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One should consider that after LILT, STEP can be performed relatively easy, but vice
versa will be challenging. However, despite what is generally assumed, it is technically
possible to perform a LILT procedure after STEP (52). Despite the short follow-up, this
is an interesting finding which teaches us that SBS is a dynamic condition that requires a
personalized medical and surgical approach.
Nevertheless, STEP remains the most performed AIR procedure nowadays, largely due to
the technically difficulties of LILT. There are clear advantages of STEP compared to
LILT (see Table 2). STEP is the only procedure where the lumen remains closed,
potentially reducing the chance of complications. Consequently, (late) interloop fistulas,
abscesses, necrosis and leakage are more often seen in LILT. This is of importance when
taken into account that these complications could lead to further gut loss. However, the
complications are, to our view, not always clearly described. Any postoperative
complication or mortality should be clearly described in future publications to get a better
view on what short and long term positive or side effects AIR procedures cause.
Furthermore, STEP has a wider scope whereas it can be applied to USBS tailored to
asymmetrical dilated bowel segments and dilated duodenum. Also, STEP has a clear
effect on bacterial overgrowth which is less seen after LILT. Despite this, redilatation is
seen more often in STEP, although it is easily reproducible and after a second or third
STEP >50% of the patients could still wean from PN after all or at least improve their
nutritional status.
SILT is novel lengthening procedure with promising clinical results. In unpublished data,
a lengthening of 75% is reported in three patients who all weaned from PN (91).
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SILT has similar advantages as STEP compared to LILT, being a technically less
challenging procedure with a minimal handling of the mesentery without compromising
the blood supply. However, STEP is considered a technically less challenging and
quicker procedure than SILT. With both SILT and STEP it is possible to reduce the
luminal diameter more than 50%, (91) whereas in LILT the luminal diameter can only be
reduced by half. Cserni et al state that in STEP the physiology of the muscle fibres is
dramatically altered and reverses the role of longitudinal fibres to circular and vice versa,
possibly compromising peristalsis (86,88,91). However, one could argue that
experimental evidence showed that motility in STEP is preserved (65,75), therefore the
relation between altered muscle fibres direction and peristalsis remains unclear and needs
to be studied further. Finally, whereas STEP has the potential of treating duodenal
dilatation (67), SILT could probably not be applied to the duodenum due to its relation
with the pancreas. Nevertheless, SILT has an undeniably potential in the treatment of
SBS. Promising results are encouraging further studies in a larger population to confirm
the effects of SILT and determine the long-term outcome. Also, SILT needs to be
compared to other AIR techniques like LILT and STEP. We are curious to see more data
and maybe a prospective randomized controlled trial could be conducted between STEP
and SILT in the future.
Based on the current literature, most patients who received AIR are treated with LILT or
STEP. The results are relatively good, but the considerable morbidity remains a point of
concern. Comparison between frequently used AIR procedures has been difficult due to
discrepancy in follow-up times, methods and lack of control groups. Only recently, long-
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term outcome of STEP has started to become clearer, but only in case reports or
retrospective studies. Currently, a randomized controlled trial between LILT and STEP is
in our opinion almost impossible to conduct due to the low patient numbers and limited
surgical expertise in both AIR procedures. In our opinion it might be worthwhile to
initiate a combined online data registry for all intestinal lengthening procedures,
including LILT, STEP and possibly SILT. This will give us more insights in all aspects
of the lengthening procedures.
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Summary
In general, AIR should be considered in SBS patients who are dependent on long-term
PN . Since there is no lengthening procedure without the risk of complications, all efforts
should be made to limit bowel resection wherever possible and prevent complications of
PN.
If AIR is needed after all, restoration of bowel continuity should be the first step.
Currently, in clinical practice two mainly applied surgical interventions are available;
being LILT and STEP. We think LILT, however being a technically difficult procedure
demanding specific expertise, should be performed if possible. If not, STEP can be
performed as both primary treatment and after prior AIR procedures. Of specific
importance is the right assessment of the indication. STEP is superior to LILT in treating
bacterial overgrowth and seems the only feasible option in treating USBS and duodenal
dilatation. Also, complications leading to gut loss are seldom seen after STEP. If PN
independence is not within reach, AIR can be considered as a bridge to intestinal
transplantation. Novel techniques like SILT need to be further investigated before
widespread clinical implication. Since there are no randomized controlled studies that
confirm these conclusions and it is unlike there is going to be one, it will be difficult to
determine an evidence based ‘golden standard’ because of the spread in results in the
currently available literature of the last decade. To create the best possible evidence based
clinical guidelines, we suggest that an international online data registry for LILT, STEP
and SILT should be founded.
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Bullet points:
Research agenda
• SILT
• Found online data registry and compare outcomes.
• Treatment of duodenal dilatation with STEP.
• Combination of controlled bowel expansion and STEP.
• Determine an international accepted golden standard for the surgical
management of short and ultra-short bowel syndrome.
Practice point
• If expertise is available, perform LILT as initial intestinal lengthening
procedure.
• A second or third STEP can still lead to independence of PN for >50% of
patients.
• STEP is the treatment of choice in bacterial overgrowth when medical
management fails.
• STEP should be considered in persistent PN dependence ultra-short bowel
syndrome without improvement.
• When PN independence is not to be expected, intestinal lengthening
procedures should be considered a bridge to intestinal transplantation.
Conflict of interest statement
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No conflict of interest has been declared by the authors.
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Table 1. Anatomical and functional classification of short bowel syndrome Anatomical Functional
Type I
End-jejunostomy
Acute, short-term, usually self limiting
Type II
Jejunocolonic continuitiy, ileocaecal valve
not present
Prolonged acute condition
Often metabolically unstable patients
Complex multidisciplinairy care
Intravenous supplementation for
weeks/months
Type III
Jejunoileal anastomosis, ileocaecal valve
present
Chronic condition
Metabolically stable patients
Intravenous supplementation for
months/years
Reversible or irreversable
The anatomical classification was derived from Feldman et al (16), the functional classification was derived from Pironi et al (10).
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Table 2 Prognostic Factors for Short Bowel Syndrome. Factor Favourable Unfavourable
Age Young Extremely young or old
Systemic disease Not present Present
Intrinsic Bowel Disease Not present Present
Duodenum Complete Bypassed or (partially) resected
Length small bowel > 150 cm < 150 cm
Part resected Jejunum Ileum
Ileocaecal valve Present Not present
Colon Present Not present
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Table 3 Comparison of technique and outcome of SILT, STEP and SILT.
LILT STEP SILT
Mesenteric handling Significant Minimum Minimum
Apply to duodenum No Yes No
Bowel length limitation 20-40 cm Any length Any length
Anatomical criteria Double leaflet present,
dilated bowel
Dilated bowel, also
asymmetrical
Dilated bowel
Surgical complexity Complex Not complex Less complex
World wide experience Low High Nihil
Risk of necrosis Considerable Low Unknown
Lumen Opened Closed Opened
Repeatable No Yes Unknown
Complications Bleeding, obstruction,
leakage, intestinal
necrosis, perforation,
fistula, abscess
Bleeding, obstruction,
leakage
Obstruction, leakage,
bleeding, possibly
fistula, abscess.
Average lengthening* 48 – 55% 43 – 69% -
PN independence* 55 – 71% 48 – 60% -
Estimated survival* 87-95% 77-89% -
ITx needed* 10 – 26% 5 -8% -
*average rates mentioned are based on recent systematic reviews (41,42,56,58,73).
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Fig. 1. Classification of short bowel syndrome. (Type I) endjejunostomy. (Type II) jejunocolic
continuity, no ileocecal valve present. (Type III) jejunoileal anastomosis, ileocecal valve present.
(Freely adapted after Feldman, Friedman & Sleisenger (16))
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Fig. 2. Internal hernia’s after Roux-Y gastric bypass. (1) Herniation of the small bowel
through the opening in the mesentery of the jejunojejunostomy. (2) Herniation of the
small bowel through the opening between the alimentary loop and the mesocolon
(Petersen Hernia). (Obtained from Bakker et al. (95))
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Fig. 3. Bianchi procedure or LILT. (A) separating the two leaves of the mesentery of the
isolated small bowel segment. (B) Creating a funnel on the mesenteric site for dividing
the small bowel. (C), (D) Separating the small bowel by introducing a surgical stapler.
This can also be done by cutting the bowel half. (E) The two bowel loops are then
anastomosed together in a isoperistaltic manner. (Obtained from Bianchi A. (35))
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Fig. 4. Schematic view of STEP. Perpendicular to the longitudinal axis, a stapler line is
made preserving a 2 cm luminal diameter. After multiple staples, the bowel is lengthened.
(Adapted to Kim et al. (53))
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Fig. 5. A model of the SILT technique. (A) The bowel is cut spirally in an angle to the
longitudinal axis. (B) maintaining orientation by using a silicon catheter, the bowel is
stretched. (C) When adjusted to the right length and diameter, the lumen is closed by
suturing. (D) The bowel in a longer and narrower shape. (Adapted to Cserni et al. (90))
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