Langenbecks Arch Surg (2012) 397:10431051
DOI 10.1007/s00423-011-0874-8
REVIEW ARTICLE
Current practice and future perspectives in the treatment
of short bowel syndrome in childrena systematic review
S. Weih & M. Kessler & H. Fonouni & M. Golriz &
M. Hafezi & A. Mehrabi & S. Holland-Cunz
Received: 23 August 2011 / Accepted: 3 November 2011 / Published online: 22 November 2011
# Springer-Verlag 2011
Abstract
Purpose Short bowel syndrome (SBS) is a malabsorption
disorder of the intestine, which leads to an inadequate
alimentary supply. A number of therapeutic approaches are
already in use, but research advances may provide new
options in the future. The purpose of this paper was to
provide an overview of the established therapeutic
approaches together with a discussion of the future
perspectives in the treatment of patients with SBS. We
review those studies dealing with the treatment of SBS
patients and discuss both surgical and non-surgical
approaches together with tissue engineering.
Methods A systemic review of Medline-cited studies
dealing with current practice and future perspectives in
the treatment of short bowel in children was performed.
Results Surgical approaches, non-surgical approaches, and
tissue engineering which was used in the treatment of SBS
were analyzed. Among the surgical approaches, the bowel
lengthening procedures and small bowel transplantation are
prevalent. Stimulants are most important concerning non-
surgical approaches. Tissue engineering seems to be more
experimental and was also evaluated.
Conclusion The treatment of SBS patients remains very
complex. It is eminent to find the best therapeutic option for
A. Mehrabi and S. Holland-Cunz equally contributed.
S. Weih : M. Kessler : S. Holland-Cunz (*)
Division of Pediatric Surgery, University Hospital Heidelberg,
Im Neuenheimer Feld 110,
69120 Heidelberg, Germany
e-mail: stefan.holland-cunz@med.uni-heidelberg.de
H. Fonouni : M. Golriz : M. Hafezi : A. Mehrabi
Department for General, Visceral and Transplantation Surgery,
University Hospital Heidelberg,
Im Neuenheimer Feld 110,
69120 Heidelberg, Germany
each patient and to individualize and modify the different
possible types of applied techniques frequently.
Keywords Short bowel syndrome . Surgical treatment .
Children . Tissue engineering . Malabsorption
Introduction
Short bowel syndrome (SBS) is generally defined as a
disorder in which the patients resorptive capacity of the
intestine function is not sufficient to provide an adequate
alimentary supply. The incidence of SBS varies between 2
and 5/Million [1, 2]. Currently, there are no strict criteria
with which to define SBS. SBS is caused mostly by
extensive resection of the small bowel, which can occur for
a number of different reasons [1, 3]. The most common
diseases that cause SBS in children are bowel atresia,
necrotizing enterocolitis, and gastroschisis [35]. Necrotiz-
ing enterocolitis seems to be the leading cause for SBS in
neonates, but the frequency varies from 14% to 43% in
published studies [1]. The incidence of gastroschisis has
increased over the past decades [1] and is likely to be of
growing importance concerning SBS in the future.
Although SBS is basically a functional problem rather
than an anatomical one [1], the residual small bowel length
plays a pivotal role. Diamanti et al. defined SBS as the
length of the remaining bowel being shorter than 70% of
the normal [6]. However, many authors refrain from using
the residual small bowel length for diagnosis and prognosis
of SBS, as several other factors can influence the syndrome
such as absorptive capacity of the bowel, presence of
ileocecal valve or ileum, the number of previous infections
and intestinal surgeries, and the presence of liver disease [6]
and cholestasis [1]. Additionally, the cause of resection and
1044
the age of the patient when surgery was performed affect
the clinical course [1]. As SBS is defined as a condition in
which total enteral alimentation is not possible, nutrition
must be provided parenterally at least partially. Providing
parenteral nutrition (PN) helps children with SBS to
develop normally during the adaptation of the residual
small intestine [1]. The main and potentially fatal [7]
problem of PN is liver failure [811] which occurs in 40
70% of SBS patients [12], with an eventual need for liver
transplantation [1, 9]. These children usually present with
cholestasis [12]. Additionally, central line infections [911]
leading to vanishing venous access sites [9], thrombosis,
and recurrent catheter-related sepsis [7, 10] are further
eminent problems. Further complications include dehydra-
tion, electrolyte disorders [7], and a failure to thrive [10]. In
summary, the 5-year-survival rate for SBS patients receiving
PN is 63% [7].
The effect of SBS on children is hard to define. Wales
and Christison-Lagay found a mortality rate of 37.5% in
neonates with SBS [10]. Warner reported a long-term
survival rate of 45% in patients with a residual small bowel
of less then 50 cm [13]. Another study cited a 5-year
survival of 95% in SBS patients who were able to resume
enteral nutrition compared with a survival rate of 52% in
those patients who could not [3].
Until now, the medical treatment and care of children
with SBS remains challenging and requires a very
individual therapeutic strategy. This review aims to provide
a survey of the current therapeutic options and the future
perspectives in the treatment of children with SBS.
Materials and methods
An analysis of Medline-published therapeutic options in the
treatment of SBS was performed. Articles were found using
short bowel syndrome, children, therapy, parenteral
nutrition, surgical treatment, recirculating loops, re-
verse segments, Bianchi procedure, STEP, small
bowel transplantation, and tissue engineering as search
items. Concerning languages, our analysis included pub-
lications in English, German, Spanish, and French. Atten-
tion was particularly paid to the application of the different
treatment strategies in patients and experimental
approaches.
Results
Conducting the Medline-analysis using the mentioned main
search items yielded 787 publications. Selection criteria
consisted of (1) application of the treatment strategy in
humans or (2) application of the tissue engineering. After
Langenbecks Arch Surg (2012) 397:10431051
applying these criteria, only 81 publications were included
in the analysis.
There are three main approaches toward the treatment of
SBS. These are:
1. Surgical approaches
2. Non-surgical approaches
3. Tissue engineering
Surgical approaches to treat short bowel syndrome
Different surgical procedures exist to treat the problems that
arise from SBS. These problems include a bowel length
which is too short to provide an adequate alimentary
supply, which can be corrected by bowel-lengthening
procedures. A second problem is a fast transit time
associated with a high stool frequency and a loss of
functional bowel. Procedures to slow intestinal transit such
as the use of reverse segments can decrease transit time.
Table 1 gives an overview of the most commonly used
procedures. Small bowel transplantation (SBTx) is a
possible therapeutic strategy if the remnant bowel is not
functional. In the following, we describe these surgical
procedures in detail.
Bowel lengthening procedures In 1980, Bianchi described
a longitudinal intestinal lengthening and tailoring procedure
performed in a pig model as a surgical approach to treat
patients with dilated bowel [15]. After dissection of the
mesentery, the bowel is divided into the intervascular space
of the mesentery forming two bowel loops out of one.
Then, an isoperistaltic anastomosis between the hemiloops
is performed [15]. In the literature, anatomical criteria for
patient selection have been suggested. These are: (1) an
intestinal diameter greater than 3 cm; (2) a residual small
bowel length of greater than 40 cm; and (3) a dilated bowel
length of greater than 20 cm [1]. In contrast, Thompson and
Sudan recommend intestinal lengthening procedures when
intestinal failure and life-threatening complications with
parenteral nutrition or substantial bowel dilatation occurs,
regardless of the bowel length [16]. However, Thompson
and Sudan as well as Bianchi do recommend an intestinal
lengthening procedure only in patients with absence of liver
failure and therefore preclude its use in patients with
advanced stages of intestinal failure [16, 17]. Bianchi
described a morbidity of two hemiloop anastomotic
stenoses and one spontaneously resolving air and bile leak
in a group of 20 patients [17]. Survival in patients after the
Bianchi procedure ranges from 30% to 100% [12]. Also,
the effects of weaning of total parenteral nutrition (TPN)
differ widely and range from 28% to 100% [12, 14, 18, 19].
In adults [20] as well as in children [1], an improvement
concerning intestinal transit, stool frequency, intestinal
Langenbecks Arch Surg (2012) 397:10431051
Table 1 Surgical approaches to treat short bowel syndrome
absorption rate, (especially of D-xylose and fat) [19], and
weight gain have been described in more than 50% of the
patients after the Bianchi procedure. The outcome of surgery
is also influenced by age and clinical status, especially liver
diseases, of the patient at the time of surgery [1, 14, 21].
Providing an adequate blood supply is a major challenge
of the technique [22]. Kim et al. described an alternative
procedure for intestinal lengthening [23] in which excessive
blood loss can be avoided, as the mesenteric vessels remain
untouched. They called this technique the serial transverse
enteroplasty procedure (STEP). Lengthening of the dilated
bowel is conducted by serial transverse stapler applications
from opposite directions. By doing this, a zigzag channel is
created [23] without diminishing the mucosal surface area.
As the mesenteric vessels are not disturbed, it can also be
conducted using short dilated segments of the intestine
[22]. The STEP procedure is becoming increasingly
popular worldwide [24]. An important advantage of the
STEP procedure is that it is easier to perform than the
Bianchi technique [12] and can be applied repeatedly after
adaption and redilation of the bowel [12, 25]. Besides the
classical SBS, indications to perform the STEP procedure
include bacterial overgrowth and dilation of the intestine
following neonatal intestinal obstruction [12, 24]. The
1045
international STEP data registry published in 2007 reported
a morbidity rate of seven patients out of 38 and a mortality
rate of three out of 38 patients [25]. Nearly 50% of the
patients with SBS could be weaned from PN [12] after the
STEP procedure.
Procedures to slow intestinal transit Aside from lengthening
procedures, other surgical options exist to treat patients with
SBS. These include procedures to slow down the intestinal
transit. To achieve this, anti-peristaltic segments, artificial
valves, colonic interposition, performing a pouch or loop, or
intestinal pacing have been used [22].
Anti-peristaltic segments Using anti-peristaltic or re-
versed segments is suggested when the transit time is
too fast, but sufficient bowel mucosa for nutrient
supply is available [14]. Although this is the most
commonly used technique, only a few anecdotal reports
regarding the use in humans have been published. These
describe the collective results of 40 procedures [26]. In
80% of these patients, a slower intestinal transit and
increased absorption was described [27, 28]. Achieving
an optimal length of the reversed segment is critical
because otherwise the procedure may not be fully
1046
effective or may cause severe obstruction. Published
studies report an ideal length of 10 cm in adults and
3 cm in children [14, 27]. Complications arising from this
procedure include transient intestinal obstruction [28] and
anastomotic leakage. For example, Panis et al. described a
high morbidity in 6/8 patients after this procedure and
half of the patients suffered from transient intestinal
obstruction. However, there was no postoperative mortal-
ity. Weaning of PN could be achieved in 6/8 patients
within 4 years after performing the procedure [28]. In
animal models, the technique did not cause intestinal
obstruction and led to an increase of bowel length as well
as diameter of the bowel [29]. The effects on the body
weight were reported to be inhomogeneous [30, 31].
Histological analyses showed that the procedure causes
an increase of villus height and crypt depth [29, 30] and a
decrease in apoptosis [30].
Artificial valves Only about ten cases of the use of artificial
valves are described in literature [26]. All of them report a
partial mechanical obstruction and disruption of the
physiologic motility of the intestine [27]. The construction
of an intestinal valve requires the sacrifice of valuable small
bowel [14]. Results of the procedures differ widely, but
different experimental studies have shown that transit time
is prolonged and absorptive function and survival is
increased [3234]. Valve removal may be required in the
case of uncontrollable bowel obstruction associated with
stasis and enterocolitis [14]. In patients, the outcome after
using valves is not very predictable, therefore the procedure
is used less frequently [27, 35]. Interestingly, in children,
valves have been utilized to cause dilatation of the intestine
which allows intestinal lengthening procedures afterwards
[18].
Colon interposition Literature has reported positive effects
of colon interposition between the remaining parts of the
small intestine on transit time and absorptive capacity of the
bowel. The interposition of the colon can be performed iso-
or antiperistaltically [36]. The antiperistaltic colon interpo-
sition functions similar to the reversed small intestinal
segment but with the advantage that none of the valuable
small bowel remnant is used [36]. After interposition of the
colon, adaptive changes of the colon to the small intestine
have been shown in humans [37], and changes of the
contractile response similar to those of the remaining small
bowel have been shown in a rat model [38]. This is in
contrast to trials in the pig model where colon interposition
did not work convincingly, although the Bianchi procedure
led to weight gain in the pig [39]. Despite some reported
success, functional obstruction, dilatation of the bowel, and
enterocolitis occurred in some cases [14]. There is no
reported perioperative morbidity or mortality associated
Langenbecks Arch Surg (2012) 397:10431051
with colon interposition in the therapy of SBS [40].
Weaning of PN has been successful in 50% of the patients
[40].
Recycling loops and intestinal pouches The creation of
recycling loops involves the transection of the small bowel
and connection end-to-side to the proximal small bowel.
Following this procedure, continuity is restored side-to-end
from the built loop to the distal part of the small bowel.
Recycling loops and intestinal pouches have not been reported
to be beneficial in patients with short bowel syndrome
previously [14]. The few reported cases, mainly published
in the 1960s, reported discouraging results [26, 27, 41, 42].
Two thirds reported patients died [27]. Morbidity rates and
rates of weaning of PN have not been reported.
Tailoring and plication Tailoring and plication have proven
to be effective in improving transit time and avoiding stasis.
Although this approach may improve intestinal adaptation
by enhancing isopropulsion and reducing stasis, a disad-
vantage is that it involves resection of the bowel. Therefore,
this procedure is only recommended when sufficient
remaining residual bowel can be guaranteed [14]. As it
has only been performed in very few patients, morbidity
and mortality rates as well as rates of weaning of PN have
not been reported.
Intestinal pacing Although there are very few reports
regarding the treatment of SBS patients with intestinal
pacing [43], experimental investigations have shown
promising results. Motility and transit in the small intestine
are regulated physiologically by pacesetter potentials which
are generated in the duodenum. These pacesetters can be
entrained leading to an increased frequency and the
propagation of pacesetter potentials to more distant bowel
segments [44]. Retrograde pacing can also be generated,
inducing peristalsis in a reverse direction [36]. In 1979,
Collin et al. showed that both backward and forward pacing
enhanced intestinal absorption, but backward pacing had a
stronger impact on intestinal absorption by slowing transit
time [43, 44] and subsequently prolonging the contact of
nutrients with the intestinal epithelium [44]. In rats, reverse
electrical pacing increases transit time and nutrient and
fluid absorption [45]. Furthermore, a decreased weight loss
as well as increased absorption of water, glucose, and
sodium were reported in dogs [46] with SBS in which
retrograde intestinal pacing was performed [47]. Also, a
decrease in fecal fat and nitrogen losses was observed [48]
in this model. Side effects have been observed during in
experimental procedures of intestinal pacing. For example,
some dogs suffered from vomiting during pacing and others
from diarrhea upon cessation of pacing [36]. The few
studies performed on humans were conducted using a
Langenbecks Arch Surg (2012) 397:10431051
nasoduodenal feeding tube carrying the stimulating elec-
trode. However, it must be considered that the method is
invasive and implantable devices and stimulators suitable
for use in humans do not yet exist [43]. Therefore, it is not
certain that this method will be used in the treatment of
humans in the future, and we cannot, at the moment, define
what the outcome of such treatment would be.
Small bowel transplantation SBTx was first successfully
performed in 1987 in Pittsburgh, USA. One year later, the
first successful SBTx from a living donor was conducted in
Kiel, Germany [49]. Since then, SBTx has been further
developed through the improvement of immunosuppression
and surgical techniques to become a therapeutic option for
patients with irreversible intestinal failure when parenteral
nutrition is no longer possible [50, 51].
There are different ways to perform the SBTx. These
different approaches are: isolated SBTx, combined SBTx
liverTx, or multivisceral transplantation [50]. In children, a
SBTxliverTx is the leading type of transplantation (50%),
followed by the isolated SBTx (37%) and the multivisceral
transplantations (13%). In adults, transplantation of the
intestine alone is usually performed (55%), followed by the
multivisceral transplantation (24%) and the SBTxliverTx
combination (21%) [52].
In most cases, cadaveric grafts are used. In 2009, a living
donor transplantation was performed in only 2/189 SBTx
operations in the USA [5], although living donor trans-
plantations are reported to be successful [50, 5355] and
the long-term outcomes after using living donor grafts are
comparable to cadaver grafts [56]. The living donor
transplantation is viewed as an option to reduce death on
the waiting list [57] because, often, it is not possible to find
a size-matched cadaver graft [58].
Immunosuppression following transplantation is
tacrolimus-based. In recent studies, the use of induction
agents, especially thymoglobulin, has been promising [59].
Induction agents are used in approximately 90% of the
cases [49].
The latest results show that >80% of patients survive
1 year. This then decreases with only 50% of patients
surviving 5 years [5]. Patients between 2 and 18 years seem
to have the best long-term survival and combined liver
intestine grafts, the best long-term engraftment [59]. The
majority (>70%) of the transplanted patients could be
weaned completely of parenteral nutrition within 46 weeks
after transplantation [52], and some patients only needed it
temporarily during periods of illness [50]. The quality of
life after SBTx was comparable to patients receiving TPN
and seems to be better than before transplantation [50].
Sudan assumed a perceived higher risk for possibly life-
threatening complications in patients after SBTx compared
with those remaining on TPN [60]. Summarizing the
1047
improvements in outcome, quality of life, and long-term
survival, the indication for SBTx is under debate and
should be considered carefully and individually for each
patient. In addition, for each patient, it should be decided
when the best time point for SBTx is indicatedif SBTx
should be done electively or as a salvage procedure.
In reviewing the surgical approaches to SBS, it is
important to distinguish between established procedures
such as bowel lengthening procedures or SBTx as well as
experimental approaches such as procedures to slow
intestinal transit. The latter are often associated with a high
morbidity, mortality, and an uncertain outcome, therefore
they should be applied very carefully.
Non-surgical approaches to SBS
Multiple stimulants are used to improve intestinal capacity.
The most widely evaluated agents used in humans include
endothelial growth factor (EGF), growth hormone (GH),
and glucagon-like peptide 2 (GLP-2) [61].
Endothelial growth factor EGF has different effects on the
epithelium of the intestine. Endogenous EGF plays a
pivotal role in maintaining the intestinal structure [62]. It
has been shown to increase epithelial cell proliferation but
also a 30% decrease of crypt fission in rats [63]. Other
experimental studies suggest that EGF could be useful to
stimulate structural and functional adaption of the intestine
after massive intestinal resection. The most effective results
observed in rodents were apparent when it was adminis-
tered shortly after resection. Both systemic and enteral
application appeared to be effective [62]. After application
of EGF, an increase in crypt cell mitotic activity, cell size,
and rate of proliferation as well as restoration of villous
architecture was observed [64]. It also led to a decrease in
crypt cell apoptosis in a mouse model [65]. Warner [13]
also showed that stimulation of the EGF receptor in mouse
resulted in an increased adaption of the intestine.
Additionally, they reported that both EGF receptor
activation and expression are elevated in enterocytes
after resection [66]. In rats, carbohydrate and amino acid
absorption after massive intestinal resection was increased
[67]. In contrast, Lukish et al. did not observe any
significant changes in the small intestine epithelial
ultrastructure after massive small bowel resection and
application of EGF in a rat model [68]. Patient data are
rare, but, in children with SBS, the application of EGF
was shown to be followed by a significant improvement in
carbohydrate absorption and improved tolerance of
nutrients applied enterally [69].
Growth hormone GH stimulates growth of the intestinal
layers [61]. It also induces the production of IGF-1 [61]
1048
which has also been shown to affect intestinal adaption
in experimental trials. GH resulted in a reduction of PN
in children with SBS [70], but, after discontinuation of
the GH application, the effect could not be sustained [71].
The effect of GH in adults with SBS also seems to vary
[64, 70]. A recent publication reviewing the treatment of
bowel disorders with growth hormones described mainly
positive effects of GH on body weight and mass, tolerance
to enteral feeding, decrease of the frequency of stool, and
weaning of PN [61].
Glucagon-like peptide 2 GLP-2 is another intestinotrophic
factor that has been tested in humans. Patients treated with
Teduglutide, a GLP-2 analog with a longer half-life, had an
increased body weight, gastric emptying, and an improved
nutrient absorption, as well as an increased crypt depth and
villus height [61]. In a study in which patients were treated
with GLP-2 for 2 years, no effects on mucosal morphology,
energy intake, or absorption were reported, but a reduction
in fecal weight was observed. Patients were able to
maintain their intestinal fluid and electrolyte absorption at
lower oral intakes [72].
Of all the substances presently used in the non-surgical
treatment of SBS patients, GH appears to have the most
convincing beneficial effects. GLP-2 may also be of
increasing importance in the future. The benefits and
potential effects of EGF treatment remains unclear, therefore
it should be used carefully.
Tissue engineering
Tissue engineering could potentially provide another
approach towards treating patients with SBS. It involves a
scaffold of different materials, which is used as a substitute
for extracellular matrix. This scaffold is loaded with cells
and implanted into vascularized spaces in in vivo studies
[73]. Acellular dermal matrix, small intestinal submucosa,
and numerous other forms of sheets serve as scaffolds in
tissue engineering.
Acellular dermal matrix (ADM) has also been implanted
into the bowel continuity to try and encourage the ingrowth of
the surrounding intestinal tissue and subsequent synthesizing
of new intestinal tissue onto the scaffold. This approach was
attempted in a rabbit model, but the ADM simply vanished
without the forming of new intestinal tissue and caused severe
intestinal adhesions [74]. In a similar approach, all the
animals suffered from peritonitis when the ADM tube was
inserted into the continuity. However, when the ADM tube
was positioned as a blind pouch, it led to epithelial
regeneration, angioneogenesis, myofibroblast infiltrates, and
formation of intact mucosa [75].
Similar experiments were performed using a small
intestinal submucosa (SIS)after implantation of a SIS
Langenbecks Arch Surg (2012) 397:10431051
tube, a neointestinal wall showed three layers of mucosa,
smooth muscle, and serosa without any adhesions or
stenosis [76]. In contrast, Lee et al. described only minimal
epithelial and smooth muscle regeneration and significant
shrinkage of the SIS using the same approach [8]. They
propose that loading cells onto the SIS may cause an
appropriate regeneration of the small intestine. Different
types of cells can be used for loading the scaffold [8].
Stem cells are undifferentiated precursor cells with capacity
for self-renewal and differentiation into a lot of different cell
types [77]. As they are able to differentiate into all types of
bowel cells, stem cells are very promising for use in tissue
engineering [77]. Markel et al. proposed that the supply of
sufficient stem cells either by tissue engineering or stem cell
transplantation could possibly improve the enteric function
and intestinal restitution in patients with massive intestinal
loss [77]. Both bone marrow and mesenchymal stem cells
have been shown to develop into intestinal cells. Circulating
hematopoietic stem cells have also been differentiated into
cells of the gastrointestinal tract [77].
Patients with inflammatory bowel disease and malignan-
cies have been treated by stem cell transplantation and
achieved a complete remission. Significant histological and
clinical improvements have also been reported in a special
group of celiac patients after stem cell transplantation. In
experimental models, stem cell transplantation has been
shown to facilitate tissue repair and contribute to regener-
ation [78]. However, the long-term safety, tolerability, and
efficacy of stem-cell-based treatments and their carcinogenic
risk remain unclear [78].
Several tissue-engineered products are used for in vivo
models and in clinical use [11]. Concerning tissue-
engineered intestine, animal models have shown encourag-
ing results. Plugs of full thickness intestine called organoid
units have been placed on a scaffold and wrapped into the
omentum of rats, where the tissue became vascularized and
formed a cyst resembling a small bowel. In the cystic
structures, the epithelium developed into mature crypts and
villi [11]. When the tissue-engineered small intestine
(TESI) was placed in continuity with the gastrointestinal
tract after massive enterectomy, the animals rapidly
returned to their preoperative weight [13, 79]. Ganglion
cells and S-100 positive groupings in the distribution of
Meissner and Auerbachs plexus have also been demon-
strated in a similar model. Histological analyses revealed
appropriate tissue development including the nerve tissue
[79]. In contrast, Wang et al. did not observe regeneration
of nerve tissue in a TESI model [76]. Nakase et al. reported
the regeneration of endocrine cells and nerve fibers of
tissue-engineered intestine in a canine model but did not
observe any development of a myenteric or submucosal
plexus [80]. Sala et al. generated a TESI with all intestinal
cell types including innervated muscularis mucosae in pigs
Langenbecks Arch Surg (2012) 397:10431051
but did not implant into the bowel continuity [81]. Tissue
engineering may be an important approach for the future.
However, more research is necessary to overcome the
persisting problems in creating a TESI before this approach
can be used in humans.
Discussion
Although surgical and non-surgical therapeutic options
have improved significantly in the last decade, various
unsolved issues regarding the treatment of SBS persist.
Even when parenteral nutrition and catheter-preserving care
are conducted in an optimal way, the long-time survival rate
of patients with SBS remains low [7]. Consequently, there
have been many approaches to try and improve the
outcome of treatment. Among the surgical treatment
options, the intestinal lengthening procedures described by
Bianchi and Kim have had encouraging results but are
applicable only in a very special group of patients, and
surgical complications such as leakage should not be
ignored. Even in patients with optimal pre-operative
conditions and without any surgical complications, the rate
of weaning of the parenteral nutrition is variable. However,
this technique was conducted the most frequently. In
contrast to the Bianchi and STEP techniques which have
been used for years in patients with SBS, some procedures
to reduce the intestinal transit time are still in the
experimental stage. These involve the use of antiperistaltic
segments. Intestinal valves, colon interposition, intestinal
recirculating loops, and tailoring have been performed in
very few cases, and each has had different outcomes, some
being associated with a very high morbidity and even
mortality. Intestinal pacing also remains at the experimental
stage, although studies performed in animal models have
revealed promising results. Additionally, SBTx has been
improved in the last years from an experimental procedure
to a treatment option for patients with SBS. Besides
standardizing the surgical technique and refining immuno-
suppression, immense advances in improving the outcome
have been achieved, also with regards to the weaning of
TPN. Besides that, issues in the postoperative course
remain challenging and may lead to potential life-
threatening complications [60]. To date, it has been taken
into account that evidence of benefit exists only for bowel
lengthening procedures and SBTx. All further approaches
have failed or are still in an experimental stage.
To treat SBS patients, different types of stimulants have
been tested in various clinical studies. These tests have shown
that growth hormone and glucagon-like peptide 2 are the most
promising therapeutic agents, but neither is used as a
standardized procedure so far because the long-term outcome
and long-term complications remain undefined. Advances in
1049
the use of tissue engineering will be of great interest in the
treatment of SBS in the future. In the last years, preliminary
experimental studies have yielded promising results, but, until
now, no functional tissue-engineered intestine with an
appropriate structure and nervous function has been produced
and/or implanted successfully.
Conclusion
In conclusion, the treatment of SBS patients remains very
complex. A combination of different approaches may add
up the risks and issues of each procedure rather than
combine the possible benefits. Because of that, combining
different approaches should be avoided. Of course, the
optimal concept should be prevention of SBS, which means
to detect, to transfer, and treat patients at risk in centers of
perinatal care and pediatric surgery. If failure occurs, all
attempts should be made towards optimizing the function of
the native bowel. This can be achieved by cooperation of
pediatric surgeons and gastroenterologists. Bowel length-
ening procedures should only be performed in transplant
centers, or if close cooperation with them exists if
lengthening fails. It is eminent to find the best therapeutic
option for each patient and to individualize and modify the
different possible types of applied techniques frequently. A
combination of the different approaches may lead to
favorable results only for individual patients. In the future,
tissue engineering may be an additional component in the
treatment of SBS for supporting and optimizing the clinical
outcome and quality of life of the SBS patients.
Acknowledgments We are very grateful that this publication has
been supported by Heidelberger Stiftung Chirurgie.
Conflicts of interest None.
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