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Although fistulas arising from other regions of the gastrointestinal (GI) tract (eg,
stomach, esophagus) may sometimes be included in the definition of ECF, the
discussion in this article is limited to the conventional definition of ECF. A fistula-in-ano,
although anatomically an ECF, conventionally is not referred to as such, because its
presentation and management are different.
An ECF, which is classified as an external fistula (as opposed to an internal fistula, which
is an abnormal communication between 2 hollow viscera), is a complication that is
usually seen following surgery on the small or large bowel. Earlier study suggests that
about 95% of ECFs were postoperative and ileum was found to be the most common
site of ECF.[1] Forty-nine percent of fistulas were high output and 51% were low output.
ECFs are a common presentation in general surgical wards, and despite advances in
the management of these lesions, they are still responsible for a significant mortality
rate, ranging from 5-20%, due to associated sepsis, nutritional abnormalities, and
electrolyte imbalances.
Understanding the pathophysiology of, as well as the risk factors for, ECFs should help
to reduce their occurrence. Moreover, the well-established treatment guidelines for these
lesions, along with some newer treatment options, should help clinicians to achieve a
better outcome in patients with an ECF.
Output-based classification
The type of ECF, as based on the output of the enteric contents, also determines the
patient's health status and how the patient may respond to therapy. ECFs are usually
classified into 3 categories, as follows [2] :
Etiology
Enterocutaneous fistulas (ECFs) can occur as a complication following any type of
surgery on the GI tract. Indeed, more than 75% of all ECFs arise as a postoperative
complication, while about 15-25% of them result from abdominal trauma or occur
spontaneously in relation to cancer, irradiation, inflammatory bowel disease, or ischemic
or infective conditions. The etiology of ECFs can thus be characterized as postoperative,
traumatic, or spontaneous.
Postoperative causes
Postoperative causes of ECFs include the following:
Disruption of anastomosis
Inadvertent enterotomy - Especially occurs in patients with adhesions, when
dissection can cause multiple serosal tears and an occasional full-thickness tear
Inadvertent small bowel injury - Occurs during abdominal closure, especially after
ventral hernia repair
Disruption of anastomosis can result from inadequate blood flow due to an improper
vascular supply, especially when extensive mesenteric vessels have to be ligated.
Tension on anastomotic lines following colonic resection, restoration of continuity without
adequate mobilization, or a minimal leak or infection can lead to perianastomotic
abscess formation, resulting in disruption, as seen in patients with anterior resection for
rectal carcinoma. In addition, if anastomosis is performed in an unhealthy bowel (eg,
diseased, ischemic), it can lead to disruption and cause an ECF.
Inadvertent picking up of the bowel during abdominal closure can result in small-bowel
fistula; this especially can occur with the use of open inlay mesh or intraperitoneal onlay
mesh repair by laparoscopic method, when the viscera comes in contact with the mesh,
leading to adhesions and sometimes to disruption.
Gastroduodenal fistulas are seen most often following surgery for perforated peptic
ulcer, especially in developing countries, where perforated peptic ulcer is more common.
In patients with a perforated duodenal ulcer, when the perforation is large, extensive
contamination is present. When the duration between the perforation and the surgery is
long, there is a high possibility of a leak following surgery, leading to a lateral duodenal
fistula. This problem is difficult to treat, and the mortality rate is high. Other causes of
gastroduodenal leak include surgery for cancers of the stomach and the biliary tract.
A colocutaneous fistula can develop following colonic surgery, especially when the blood
supply to a low colorectal/anal anastomosis is compromised or when there is tension at
the anastomotic suture line. This type of fistula can also result from diseases of the
colon, such as inflammatory bowel disease or malignancy leading to perforation,
pericolic abscess formation, and ECF. Surgery for appendicitis, appendicular perforation
at the base, or drainage of an appendicular abscess can also lead to a colocutaneous
fistula. Radiation therapy is also another major cause of colonic fistula. [2]
Traumatic causes
Traumatic ECF results from iatrogenic surgical trauma to the bowel that may or may not
be recognized. Road traffic accidents with injury to the gut can also lead to an ECF.[4]
Spontaneous causes
Spontaneous causes of ECF, seen in about 20-30% of cases, include the following:
Malignancy
Radiation enteritis with perforation
Intra-abdominal sepsis
Inflammatory bowel disease - Such as Crohn disease [2]
Ulcerative colitis can also lead to spontaneous ECF, but most cases of ECF associated
with this inflammatory bowel disease occur as a postoperative complication of
restorative proctocolectomy.[5] Rarely malignant tumor incised inadvertently can lead to
an ECF (shown in figure below). In this patient, a urachal tumor was inadvertently
incised when the patient was operated for appendicectomy by midline incision. The
patient presented with ECF (colocutaneous fistula) as the urachal tumor that ulcerated
on the abdominal wall postoperatively had also infiltrated the sigmoid colon.
A duodenal fistula can occur in association with a perforated duodenal ulcer, but again, it
most often arises postoperatively, resulting from a leak.
Prognosis
ECF is a common condition in most general surgical wards. Mortality rates have
decreased significantly since the late 1980s, from as high as 40-65% to as low as 520%, largely as a result of advances in intensive care, nutritional support, antimicrobial
therapy, wound care, and operative techniques.[6, 7] Even so, the mortality rate is still
significantly high, in the range of 30-35%, in patients with high-output ECFs.
Once a patient develops an ECF, the morbidity associated with the surgical procedure or
the primary disease increases, affecting the patient's quality of life, lengthening his/her
hospital stay, and raising the overall treatment cost. Malnutrition, sepsis, and fluid
electrolyte imbalance are the primary causes of mortality in patients with an
enterocutaneous fistula (ECF).
If sepsis is not controlled, progressive deterioration occurs and patients succumb to
septicemia. Other sepsis-related complications include intra-abdominal abscess, softtissue infection, and generalized peritonitis. [8]
However, patients with an ECF with favorable factors for spontaneous closure have a
good prognosis and less mortality.
End fistulas - Such as those arising from leakage through a duodenal stump after
Plya gastrectomy
Jejunal fistulas
Colonic fistulas
Continuity-maintained fistulas - Allow the patient to pass stool
Small-defect fistulas
Long-tract fistulas
In addition, a fistulous tract of more than 2cm has a higher possibility of spontaneous
closure. Spontaneous closure is also possible if the bowel wall disruption is partial and
other factors are favorable. If the disruption is complete, surgical intervention is
necessary to restore intestinal continuity.
F oreign body
R adiation
I nflammation/infection/inflammatory bowel disease
E pithelialization of the fistula tract
N eoplasm
D istal obstruction - A distal obstruction prevents the spontaneous closure of an
ECF, even in the presence of other favorable factors; if present, surgical intervention is
needed to relieve the obstruction
In addition, lateral duodenal, ligament of Treitz, and ileal fistulas have less tendency to
spontaneously close.[8]
Excoriation
Skin excoriation, seen in the image below, is one of the complications that can lead to
significant morbidity in patients with ECF. When the enteric contents are more fluid than
solid, this becomes a difficult problem, as the skin excoriation makes it difficult to put a
collecting bag or dressings over the fistula, and more leak leads to an increase in the
excoriation.
Lab Studies
The following lab studies are performed in the evaluation of an enterocutaneous fistula
(ECF):
Imaging Studies
Fistulography
During fistulography (images from which are seen below), a water-soluble contrast is
injected into the fistulous tract. Fistulography is conventionally performed 7-10 days after
Fistulogram showing
enterocutaneous fistula.
Fistulogram showing a colocutaneous
fistula following anastomotic leak after colostomy closure.
CT scanning
Computed tomography (CT) scanning is useful for demonstrating intra-abdominal
abscess cavities. Such cavities can occur if an ECF has an indirect tract when it first
drains into an abscess cavity and then drains to the exterior cavity. If an ECF is
associated with an intra-abdominal sepsis, then interloop abscesses may be present.
Markers
Oral administration of a nonabsorbable marker (eg, charcoal, Congo red) can help to
confirm the presence of an enterocutaneous fistula (ECF).
Methylene blue diluted in saline can be administered through a nasogastric tube as a
simple bedside test to confirm the presence of an ECF, especially in patients with a
gastrocutaneous or lateral duodenal fistula. This test can also help to determine whether
the leak is from a segment that is in the continuity of the GI tract, especially in the case
of proximal fistulas. However, because methylene blue loses diagnostic efficacy as it
becomes diluted with intestinal secretions, its role in identifying distal ECFs is limited.
Rehydration
Administration of antibiotics
Correction of anemia
Electrolyte repletion
Drainage of obvious abscess
Nutritional support
Control of fistula drainage
Skin protection
With the above-mentioned supportive therapy, spontaneous closure occurs in almost
70% of patients. In a study of 186 patients, Reber et al found that 91% of small intestinal
fistulas that closed spontaneously did so within 1 month after sepsis was cured. The
remaining fistulas that closed spontaneously did so by the end of 3 months after sepsis
cure, with the rest of the lesions requiring surgical closure. [15]
Uba et al reported that the majority of ECFs in children closed spontaneously following
high-protein and high-carbohydrate nutrition. [16] They found that hypoalbuminemia and
jejunal location were important variables resulting in nonspontaneous closure, while
hypokalemia, sepsis, and hypoproteinemia/hypoalbuminemia were risk factors for high
mortality in children with ECF.
Drainage Control
The fistula tract is intubated with a drain, as seen in the image below. Volume depletion
from a proximal, high-output fistula can be controlled with the use of a long-acting
somatostatin analog, octreotide, which acts by inhibiting GI hormones.
Dehydration
Hyponatremia
Hypokalemia
Metabolic acidosis
The author uses parenteral nutrition more often in patients with a proximal small-bowel
ECF, especially if it is in the proximal jejunum, or with a high-output fistula. In patients
with a distal ECF, the author prefers to use enteral nutrition whenever possible.
Studies have shown that the provision of only 20% of calories fed enterally may protect
the integrity of the mucosal barrier, as well as the immunologic and hormonal function of
the gut.[8] Hence, a combination of parenteral and enteral nutrition can be used. In highoutput fistulas, the author uses this combination therapy.
In patients with a proximal fistula, if a nasojejunal tube can be introduced beyond the site
of the fistula, then these patients can be supported with enteral nutrition, provided that
there are at least 4-5 feet of small bowel distal to it and no distal obstruction. Chronic
small-bowel ECFs may need additional supplementation with copper, folic acid, and
vitamin B-12.[8]
Maintenance - 200-300:1
Moderate stress - 150:1
Severe stress - Less than 100:1
A standard, general purpose formula for TPN consists of the following:
75 g glucose
20 g amino acids
30 g lipids per 1000 mL
The introduction of ethyl vinyl acetate bags has made the admixture of fat emulsion with
dextrose and amino acids possible (3-in-1 concept). [23] This leads to a more uniform
administration of a balanced solution containing the 3 macronutrients plus micronutrients
over a 24-hour period.
Enteral nutrition
Enteral nutrition is the mainstay of treatment for patients with ECFs. In fistulas of the
distal ileum, colon, or duodenum, enteral nutrition should be considered and can be
administered by various routes. Conventionally, when a gastroduodenal anastomosis or
closure is needed in adverse conditions, a concomitant feeding jejunostomy is
performed, so that access is available for enteral nutritional support in case of an
anastomotic leak.
The other routes of administration can be via nasogastric/jejunal tubes or a gastrostomy.
High rates of feeding should be avoided to prevent hyperosmolar diarrhea. Elemental
diets, that is, nonresidue balanced diets with protein components reduced to their basic
elements, are preferred. When a tube enterostomy is performed, proper fixation is
Fistuloclysis
Enteral nutrition can also be administered in patients with high-output proximal
jejunocutaneous or ileocutaneous fistulas with good mucocutaneous continuity. Feeding
can be administered through a feeding tube inserted in the distal limb of the ECF.
Teubner et al and Ham et al have reported good results with this method in select
patients to improve the nutrition of the patient, which is helpful for subsequent fistula
closure and promotes healing of the fistula. [25, 26, 27]
Skin Management
Based on the following fistula characteristics, Irrgang et al developed a fistula
assessment guide that has aided skin management related to enterocutaneous fistulas
(ECFs)[28] :
Origin of fistula
Nature of effluent
Condition of skin
Location of fistula opening
For a high-output fistula, a pouch system is preferable to a conventional skin dressing.
For a low-output fistula, a skin barrier with dressing/pouch is advocated.
The degree of skin irritation present (from erythema to maceration to skin loss) guides
the type of skin-protecting agents that should be applied and the type of pouch system
that should be used. In addition, an important consideration is whether the opening is
flush with the skin, retracted and deep, close to bony prominences, or in an open wound.
Wound manager.
Skin barriers
Powder, paste, wafers, spray, and creams are used as skin barriers for the protection of
skin from the enteric effluents.
Pectin-based wafers that melt and seal with the skin provide a good barrier and offer
protection for a variable period before the skin breaks down and ulcerates. In low-output
fistulas, absorbent dressings can be put on top of the skin-barrier wafer to absorb any
effluent overflow. The skin wafer protects the adjoining skin from erythema and
maceration.
Pectin- or karaya-based powders and paste are used. Powders are preferred over a
paste in wet, weepy, perifistular skin when severe skin maceration is present. A
generous amount of powder should be used and continuously added for good results. In
patients with weepy skin and a high-output fistula, management becomes difficult.
A spray provides a protective film and is helpful for pouching, but it might not be
beneficial if used alone.
Zinc creams, as shown in the images below, are used to waterproof and protect the skin.
Again, a generous amount with continuous replacement is necessary, because the
cream is washed away with discharging enteric effluents.
Zinc
Surgical Indications
Patients with an enterocutaneous fistula (ECF) with adverse factors may require earlier
surgical intervention. These adverse factors include a lateral duodenal or ligament of
Treitz fistula, an ileal fistula, a high-output fistula, or a fistula associated with diseased
bowel, distal obstruction, or eversion of mucosa (shown below). Enteroatmospheric
fistula (EAF), a special subset of ECF, is defined as a communication between the
gastrointestinal (GI) tract and the atmosphere. [30] It can occur as a complication of
"damage control" laparotomy (DCL) and results in significant morbidity and mortality.
Their etiology is complex and ranges from persistent abdominal infection, anastomotic
dehiscence, and adhesions of the bowel to fascia with a laparostoma. As EAFs almost
never close spontaneously, definitive repair usually requires major surgical intervention.
Complex abdominal wall reconstruction immediately following fistula resection is
necessary for all EAFs once the infection has subsided, which may be 6-12 months after
the original insult.[31] Fistula patch technique has also been reported for protecting open
abdominal wounds from being contaminated by intestinal fistulae drainage, while and
simultaneously applying enteral nutrition.[32]
Preoperative Details
As previously mentioned, patients should be stable and free from sources of sepsis
before surgical correction of an enterocutaneous fistula (ECF) is undertaken. In these
patients, antibiotic prophylaxis and parenteral nutrition should be supplemented during
the preoperative and the perioperative periods to achieve good results. Enteral feeding
should be decreased to allow luminal antibiotic preparation. Antibiotic therapy should be
administered after checking the culture sensitivity of earlier-grown organisms. [8]
Intraoperative Details
Incision
When performing surgery for an enterocutaneous fistula (ECF), the author's policy is to
always enter the abdomen through a fresh incision, since there is a possibility of the gut
being adherent to the site of the incision of the index operation. If the native incision is
supraumbilical midline, then the author takes an infraumbilical midline route and then
extends it to the operative site.
If it is mid-midline, then the author makes an incision in the midline superior or inferior to
the native incision or a transverse incision to approach the abdomen. The author always
enters the peritoneal cavity in a relatively virgin area to lessen the chance of an
inadvertent enterotomy.
Postoperative Details
In the postoperative phase of surgical therapy for an enterocutaneous fistula (ECF),
good nutritional status is essential, because healing of the tissue and anastomosis
depends on it.
Antibiotic cover is needed if the operation is performed in the presence of sepsis. Any
flare-up of sepsis increases the possibility of breakdown of the anastomosis and of the
abdominal wall closure (leading to dehiscence). However, unnecessary use of antibiotics
can lead to resistance and should therefore be avoided.
Fluid and electrolyte balance with appropriate correction is also important, especially in
patients with adverse factors (eg, high-output fistula).
Patients who develop spontaneous fistula due to disease need appropriate therapy (eg,
infliximab for Crohn disease or antituberculous therapy for tuberculosis) during follow-up
to prevent disease recurrence or recurrence of the ECF.[35] In patients with a malignancyrelated ECF, appropriate chemotherapy and radiation, if required, are administered to
control the primary disease.
Gelfoam Embolization
Lisle et al described the successful treatment of 3 cases of enterocutaneous fistula
(ECF) with the embolization of Gelfoam at the enteric opening of the fistula. [39] In this
technique, the ECF was assessed by CT scan and fistulogram to rule out any intraabdominal abscess, distal bowel obstruction, active bowel inflammation, or foreign body
that would prevent the fistula from healing. A fistulogram also provided information about
the fistulous tract and the site of communication with the bowel.
A 5 French introducer sheath was passed along a guide wire into the tract under
fluoroscopy and then removed, after which, Gelfoam strips or pledgets soaked in
contrast material were introduced into the tract through the sheath and pushed down to
plug the enteric opening of the ECF. All of the patients healed completely, with no
recurrence of ECF in a 2- to 3-year follow-up period. [39]