Enterocutaneous Fistula
60
Jennifer D. Stanger and Andreas H. Meier
Enterocutaneous fistula (ECF) is an uncommon but complex
problem faced by pediatric surgeons. While there have been
significant improvements in the management and care of
patients with ECF, they still carry a high morbidity and the
associated mortality remains high (up to 20 %). The majority
(approximately 80 %) of ECFs in children arise as a compli-
cation of surgical or radiologic intervention. Causes of spon-
taneous ECF include inflammatory bowel disease, infectious
causes (especially in areas where tuberculosis, actinomyco-
sis, and typhoid are endemic), trauma, and malignancy.
There is a paucity of data on ECF in the pediatric population,
and the majority of the management strategies are extrapo-
lated from experience in adult populations, which is itself
largely based mostly on case series and expert opinion.
Effective treatment of ECF requires comprehensive, multi-
disciplinary management and the disease often has a pro-
longed clinical course.
The management of ECF can be broken down into two
phases: the initial acute phase followed by the planning and
execution of definitive management (Table 60.1). During the
acute phase, the goals of treatment are to control sepsis, cor-
rect fluid and electrolyte abnormalities, support the patient’s
nutrition, and provide wound care and skin protection.
Presentation and Initial Management
Sepsis frequently accompanies the development of an ECF. In
postoperative patients, ECFs result from anastomotic leak,
missed enterotomy, bowel ischemia, or injury during fascial
closure. Spontaneous ECFs result from underlying pathology
that leads to a bowel perforation and subsequent fistula
development. All patients presenting with an ECF should be
investigated for an associated abdominal abscess. CT is the
J.D. Stanger, MD, MSc (*) • A.H. Meier, MD, MEd
Division of Pediatric Surgery, Upstate Medical University,
725 Irving Avenue, Suite 401, Syracuse, NY 13210, USA
e-mail: stangerj@upstate.edu; meieran@upstate.edu
© Springer International Publishing AG 2017
P. Mattei et al. (eds.), Fundamentals of Pediatric Surgery, DOI 10.1007/978-3-319-27443-0_60
investigation of choice in older children. In premature infants
and neonates, an US is often sufficient for detection of an
abscess.
Initial control of abdominal infection should include per-
cutaneous drainage procedures when possible, as early surgi-
cal intervention is associated with increased morbidity.
However, patients presenting with peritonitis or frank sepsis
may require emergent surgical intervention. Rarely can an
ECF be closed in the acute setting and exteriorization and
proximal diverting stoma are preferred operative strategies.
Antibiotic therapy is typically required in the acute manage-
ment of patients with ECF. The choice of antibiotics should
be directed toward the etiology and location of the fistula and
guided by local bacterial nomograms and sensitivities. In
some patients, antifungal therapy may be required. Patients
that have had recent hospitalizations or live in assisted living
facilities should be screened for drug-resistant pathogens.
Dehydration and acid–base and electrolyte abnormalities,
especially hypokalemia, frequently accompany an ECF. Upon
presentation the patient’s fluid status should be assessed and
their intravascular volume replaced with intravenous fluid.
Fistula output should be closely monitored and recorded so
that fluid administration can be adjusted to losses. Fistula
losses are typically high in potassium and patients should be
given an IV solution containing supplemental potassium.
Patients with proximal duodenal or pancreatic fistulas may
require replacement of bicarbonate losses. During the initial
work-up and management of an ECF, patients should have
frequent monitoring of serum electrolytes in order to adjust
fluid management. Younger infants and neonates have dimin-
ished ability to regulate their serum sodium and should be
monitored for hyponatremia, especially in the setting of a
high-output fistula. Sodium supplementation of feeds may be
needed to support maintenance of fluid and electrolyte bal-
ance and promote weight gain.
Somatostatin analog (octreotide) has been shown to
decrease GI fluid secretion. It also diminishes splanchnic
blood flow and intestinal transit time. Somatostatin and
octreotide have been used to facilitate spontaneous closure
493
494 J.D. Stanger and A.H. Meier

Table 60.1 Principles for the management of enterocutaneous fistula in children

Acute phase
Sepsis control ▪ Assess for associated intra-abdominal abscess/contamination
▪ Radiology-guided control when possible in the acute setting to avoid further
bowel injury
▪ Broad-spectrum antibiotic coverage
Fluids and electrolytes ▪ Close monitoring of fistula output and serum electrolytes
▪ Replacement of losses to euvolemia and normal serum electrolytes
▪ Consider somatostatin and somatostatin analogs to decrease fistula output
Nutrition support ▪ Initially minimal oral intake to assess fistula output/volume
▪ Enteral nutrition preferred if the patient tolerates and there is minimal change
in fistula output
▪ If TPN is required, consider early introduction of liver protective strategies
▪ A combination of enteral and parenteral nutrition is often required
Wound care ▪ Essential to facilitate fluid and electrolyte management
▪ Ostomy appliance is helpful
▪ Negative-pressure wound therapy is safe in children with appropriate
modifications
Definitive care
Anatomic definition ▪ Combination of imaging modalities often required
▪ Essential to rule out distal obstruction
Predictors of non-healing ECF ▪ Distal obstruction
▪ Foreign body
▪ Short fistula tract
▪ High-output fistula tract
▪ Previous radiation
▪ Nonsurgical cause
Surgical repair ▪ Defer until abdominal infections have resolved and patient is nutritionally
optimized
▪ Dedicate sufficient operative time
▪ Careful lysis of adhesions
▪ Resection of the fistula associated with better outcome than suture repair
▪ Improved outcomes with abdominal wall closure

of ECF in adults. These agents are associated with increased
likelihood of spontaneous ECF closure and decreased time to
closure. There have been few case reports on the use of these
agents in the management of ECF in children. Most patients
tolerate somatostatin well; however it must be used with cau-
tion in infants with a history of bronchopulmonary dysplasia
as its nonselective vasoconstrictive effects can worsen pul-
monary hypertension. Other agents that may be effective in
decreasing fistula output include proton pump inhibitors, H2
blockers, and antimotility agents such as loperamide and
codeine.
Malnutrition frequently accompanies an ECF, and correc-
tion of nutritional deficiency is essential to promote healing.
Many patients will have a marginal nutritional status prior to
the development of the ECF. High-output fistulas and proxi-
mal small bowel fistulas can result in significant protein loss
in the fistula effluent. They can also result in the patient hav-
ing a functional short bowel in which there is insufficient
intestinal length for nutrient digestion and absorption. Patients
are also frequently in a catabolic state due to sepsis or inflam-
matory conditions. Energy and protein requirements must not
only meet the patient’s immediate metabolic demands but
also provide adequate substrate to support ongoing growth
and development.
The decision to provide enteral versus parenteral nutrition
should be individualized to each patient based on their fistula
output, ability to tolerate oral/enteral nutrition, and their spe-
cific anatomy and intestinal length in continuity. There is
insufficient evidence to promote one modality over the other
in terms of facilitating ECF healing and closure. Correction
and prevention of malnutrition is the primary goal of nutri-
tional support, and this can be achieved by several modali-
ties. Total parenteral nutrition has traditionally been used, as
bowel rest was advocated to slow fistula losses and encour-
age closure. While TPN has been shown to reduce the vol-
ume of gastrointestinal secretions, there is a lack of evidence
to support this translating to improve ECF closure.
Enteral nutrition has the benefit of preserving intestinal
mucosal integrity and avoiding the complications associated
with TPN (mechanical line issues, electrolyte abnormalities,
infectious complications). Enteral nutrition is also easier for
parents and caregivers to provide out of hospital. In some
60 Enterocutaneous Fistula
situations, it may be possible to collect the fistula effluent
and refeed it into the distal intestine, essentially simulating
intestinal continuity, thereby minimizing fluid losses and
maximizing enteral function. While distal intestinal refeed-
ing can be beneficial, it requires nursing staff that are famil-
iar with the process and acceptance and compliance from
both nursing staff and parents. Infants and neonates are at
increased risk of TPN-induced cholestasis, should long-term
TPN be required. Septic episodes such as those seen with the
development of an ECF can also worsen cholestasis. Liver
protective strategies, including lipid minimization, early ini-
tiation of enteral nutrition, and alternative lipid sources,
should be used when available. Partial enteral nutritional
support should be considered, even when parenteral nutrition
is required to meet the patient’s nutritional needs, as the
enteral intake will serve to mitigate the potential hepatotoxic
effects of TPN. Whichever nutritional modality is chosen,
these patients have complex nutritional care needs and are
best managed by a multidisciplinary team with experience in
intestinal rehabilitation.
Control of the fistula output and wound management are
important early priorities for patients with ECF. The goal of
wound management is to collect the fistula effluent in a way
that preserves the integrity of the patient’s skin, protecting it
from the corrosive effects of the effluent, and promotes heal-
ing of the fistula. Detailed recording of losses can help direct
replacement therapies for fluids and electrolytes. The
approach used must be tailored to each patient, their size,
and the anatomy of their fistula. Two commonly used strate-
gies include wound drainage bags (a modified ostomy appli-
ance) and negative-pressure wound therapy (NPWT). A
variety of barrier creams, dressings, and wound–stoma man-
agement devices are available. The successful care of
patients with ECF requires a nurse or enterostomal therapist
familiar with their use and application.
Negative-pressure wound therapy is a useful addition to
the management strategies available for patients with
ECF. Application of NPWT has several advantages includ-
ing promotion of spontaneous closure, decreased frequency
of dressing changes, and portability, which facilitates mobil-
ity and patient’s participation in care. NPWT can also be
managed in an outpatient setting allowing children to con-
tinue to attend school and other extracurricular activities.
There have been some concerns about the safety of NPWT in
young children; however several centers have successful
used NPWT to manage open abdomens and ECF in infants
and premature neonates. The devices require adaptation to
the patient’s size, and in premature and low birth weight
infants, the suction pressure should be reduced (typically to
−75 mmHg or lower if applied directly to bowel). If being
used in the setting of an open abdomen, care needs to be
taken to place a protective layer of plastic against the bowel
to prevent the formation of further fistulas.
495
Enteroatmospheric fistula (EAF) is a subset of ECF in
which the bowel communicates with the air. These typically
arise in the setting of an open abdomen in patients who are
critically ill following abdominal sepsis or trauma. EAF can
be classified as superficial if the opening in the intestine
drains at the surface of the abdominal cavity and deep if the
fistula drains somewhere deep within the peritoneal cavity.
Superficial EAF can be managed by either direct suture clo-
sure or conversion of the EAF to a stoma. Direct suture clo-
sure is accomplished by limited dissection of the fistula,
primary suture repair, and then protection of the repair with
a skin graft or biologic mesh. Suture closure has a low suc-
cess rate and multiple attempts at closure are often required
but can be attempted with low risk. Alternatively a wound
care or NPWT system can be devised that converts the super-
ficial EAF into a functional stoma. This requires the avail-
ability of an experienced enterostomal therapist.
Deep EAF are much more difficult to manage. They typi-
cally present with peritonitis in a critically ill child. The initial
goal is resuscitation, followed by operative intervention for
source control and drainage of the peritoneal contamination.
NPWT can then be used to provide continuous drainage of
the peritoneal cavity and encourage wound granulation. One
useful technique for the management of a deep or multiple
EAF is to intubate the fistula (Foley catheter, T-tube, Malecot)
and to bring the tube out through the vacuum sponge. With
time the goal is to convert a deep EAF to a superficial EAF
that can then be managed with closure or an ostomy device.
Caution needs to be applied when using an NPWT system in
the setting of an open abdomen as it has been associated with
the formation of new EAF. A protective layer should always
be placed between the bowel and the sponge, care should be
taken when changing the system to avoid abrading the bowel,
and in smaller children and infants, the negative pressure
should be reduced (typically to −50 mmHg).
Surgical Planning
Following the acute phase of resuscitation, sepsis control,
and stabilization, attention is turned to the planning and exe-
cution of definitive management of the ECF. This process
involves careful and detailed demonstration of the specific
anatomy of the ECF, identification of factors that will either
encourage or preclude spontaneous closure, initiation of
medical therapy for those patients with underlying inflam-
matory or infectious conditions, and planning definitive sur-
gical care should it be required.
In both the resuscitation phase and definitive stage of
management, the patient will require imaging studies to look
for associated abscesses and to determine the origin, length,
and complexity of the ECF. The choice of study will depend
on the patient’s age, size, and stability, and often more than
496
one modality is required to fully delineate the ECF. A contrast
fistulogram can confirm the diagnosis of the ECF may be suf-
ficient to demonstrate the anatomy of the tract and involves
less radiation exposure than a CT scan. However in the set-
ting of ongoing abdominal sepsis or a complex ECF, a fistu-
logram will not identify associated fluid collections. CT with
oral or rectal contrast will provide detailed information
regarding the ECF and on associated abscess and other
pathologies such as distal obstruction. In premature infants
and neonates, the radiation exposure of a CT scan should be
avoided and consideration should be given to the use of
MRI. MR enterography with contrast can provide excellent
anatomic detail; however it does require patient cooperation
and younger children require sedation or anesthesia. In chil-
dren with chronic diseases such as inflammatory bowel dis-
ease (IBD) who will require numerous imaging studies,
non-radiation imaging modalities, such as US and MRI,
should be used when appropriate in order to limit their life-
time radiation exposure.
Rates of spontaneous closure of ECF vary widely depend-
ing on the population studied, and the expectation of sponta-
neous closure needs to be individualized to each patient.
Factors that have traditionally been thought to preclude spon-
taneous closure include distal obstruction, foreign body, a
well-established epithelialized tract, a high-output fistula,
ongoing inflammation or infection, previous radiation expo-
sure, and a short fistula tracts.
In adults, a high-output fistula is defined as one that drains
more than 500 mL in 24 h. No specific definition exists in
children; however extrapolating from the adult definition, an
output of more than 5–10 mL/kg/24 h in a child would be
considered high output.
Patient-specific factors also need to be considered in the
prediction of spontaneous closure of an ECF. Patients who
are malnourished, have underlying inflammatory etiology, or
have ongoing or recurrent septic episodes are unlikely to
have spontaneous closure of the fistula. Patients who develop
an ECF secondary to Crohn’s disease may have spontaneous
closure of the fistula with the initiation of biologic therapy.
Patients with IBD and ECF should be managed collabora-
tively with both a surgeon and a gastroenterologist in order
to provide both optimal medical and surgical care and maxi-
mize potential for spontaneous ECF closure.
Surgical Treatment
Definitive surgical intervention is required for non-healing
fistulas. The timing of surgery must be individualized. Prior
to surgical intervention, the patient should be free from infec-
tion, nutritionally optimized (based on both clinical and bio-
chemical assessments), and far enough out from their most
recent operation (6–8 weeks) so that peritoneal inflammation
J.D. Stanger and A.H. Meier
and vascularized adhesions are minimized. Goals of surgical
intervention are safe entry into the abdominal cavity with
preservation of the abdominal wall in a manner that provides
adequate tissue for closure, mobilization of the entire GI tract
to identify and address areas of obstruction, resection of the
ECF and involved bowel, and reestablishment of intestinal
continuity. Adequate operative time should be allotted, as
safe conduct of the operation requires patient, careful adhe-
siolysis, and avoidance of secondary bowel injury.
Bowel anastomoses should be separated from each other
where possible to avoid re-fistualization. In the setting of
multiple anastomoses, proximal intestinal diversion (enter-
ostomy) should be considered to protect the distal recon-
structed bowel. If mesh is required to reconstruct the
abdominal wall, a biological or absorbable product should be
used to minimize the risk of re-fistualization. Despite opti-
mal medical and surgical care, there remains a significant
risk of recurrence.
Outcomes
In adults and possibly in children as well, mortality in this
population remains high at 10–15 %, with a successful clo-
sure rate ranging from 70 to 90 %. Spontaneous closure is
achieved in a minority of patients, up to about a third.
Morbidity associated with an ECF remains high, with
reports of close to 90 % of patients experiencing a signifi-
cant complication. Morbidity arises from both the disease
process itself and the high rate of comorbidity among
those with ECF. Operative complications associated with
repair of ECF approach 85 % in most series and include
anemia, sepsis, wound problems, and pulmonary compli-
cations with up to 25 % of patients requiring prolonged
(>48 h) ventilation.
Particular attention is being paid to assessing factors that
are predictive of spontaneous closure, recurrence, and mortal-
ity. Spontaneous closure appears to be more likely in patients
who have a surgical cause of the ECF, have low-output fistu-
las, and have an intact abdominal wall. Recurrence after sur-
gical repair is predicted by operative complications, including
organ space infection, mechanical ventilation >48 h, periop-
erative blood transfusion, and sepsis. Some find that recur-
rence is lower after resection and primary anastomosis than if
the fistula is just oversewn. Multiple predictors of mortality
from ECF have been described including an admission serum
albumin less than 3.0 g/dL, high-output fistula, sepsis, and
multiple or complex fistulas.
ECF is an uncommon but complex and potentially devas-
tating problem encountered by pediatric surgeons. There is a
paucity of data on the management of this problem in pediatric
patients. Future work needs to focus on the reduction of the
associated morbidity and mortality and to refine the modalities
60 Enterocutaneous Fistula
currently in use in adult populations to make them safe and
applicable in children. As ECFs in children are infrequent and
their management complex, they should be managed in a mul-
tidisciplinary fashion including input from surgery, gastroen-
terology, dietitians, social work/psychologists, pharmacy, and
nurses comfortable with complex wound management.
Editor’s Comment
Enterocutaneous fistula remains a rare but vexing problem
for the pediatric surgeon. Source control of sepsis and efflu-
ent is critical for early management, and long-term outcomes
are dependent on excellent nutritional replenishment. The
surgeon should keep in mind the possibility of underlying
IBD when these occur in older children. We tend to use MRI
more frequently to define the anatomy of the fistula, thereby
avoiding exposure to ionizing radiation.
When faced with an enterocutaneous fistula that was
unexpected or one that will not close spontaneously despite
optimal nutrition and sufficient delay, the surgeon would do
well to recall the traditional factors that are well known to
prevent fistula closure: foreign body, radiation, infection/
inflammation, epithelialization, neoplasm, distal obstruction,
and a short fistula. These need to be ruled out or addressed
systematically every time, and it should be kept in mind that
more than one could be applicable in a given patient. It is
also important to consider variations on these common
themes: The foreign body could be silk suture material, an
anastomotic staple line, a fecalith, or ingested vegetable mat-
ter. Inflammation might refer to the normal inflammatory
phlegmon that accompanies healing from an anastomosis or
occult IBD. Distal obstruction could be from a segment of
dysmotile bowel, undiagnosed Hirschsprung disease, or
some other forms of pseudo-obstruction. A short fistula that
arises adjacent to the fascial wound can often be closed with
absorbable mattress sutures in the fascia itself, assuming the
edges of the fistula are adherent to the fascial opening all
around—although the epithelialized tract should be excised
or debrided, no attempt should be made to mobilize the
bowel away from the fascia.
The operation for closure of an enterocutaneous fistula
should be delayed as long as possible and can be expected in
most cases to take many hours to complete. The parents
should be warned of a high risk of recurrence. A contrast
enema should be performed preoperatively to rule out
colonic stricture, dysmotility, IBD, and Hirschsprung dis-
ease. Every adhesion must be taken down and enterotomies
avoided. Repairs should be made with absorbable suture, and
anastomoses need to be separated from each other and from
the fascial closure by viable tissue, healthy bowel, or omen-
tum. Staple lines should be buried with absorbable Lembert
sutures.
497
Vacuum dressings are a huge advance and can help protect
the skin and control fluid losses. However if used improperly,
they can be dangerous. The degree of suction needs to be the
absolute lowest necessary to maintain flow. This is to mini-
mize the gradient across the fistula, prevent small ischemic
areas in the exposed bowel which then become secondary fis-
tulas, and avoid a bean-bag phenomenon that can occur in the
abdomen when excessive negative pressure is applied in small
children resulting in bowel ischemia and vessel thrombosis
(compartment syndrome caused by excessively negative pres-
sure and subsequent collapse of the intra-abdominal organs
into a tightly compacted mass).
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