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caffeine and easily fermentable foods (lactose, disaccharides, raffinose, sorbitol, fructose),
and sufficient ingestion of fiber, as well as adequate skin hygiene, techniques for improving defecation, programmed use of the bathroom, pelvic-floor exercises to strengthen the
musculature, and concomitant disease management.
28
Fiber supplements such as psyllium are frequently recommended in an attempt to
increase stool volume and reduce liquid stools. This measure is useful, especially in relation to fecal seepage, which the majority of the time is secondary to constipation with
overflow incontinence.
Patients truly refractory to conservative measures should undergo anorectal tests to
evaluate pharmacologic therapy, surgical treatment, or the use of devices.
13.6.1.2 Pharmacologic Treatment
Various medications can improve defecation frequency and stool consistency and are
therefore recommended in FI management.
2,4,5
Loperamide 4 mg 3 times a day or
diphenoxylate-atropine 5 mg 4 times a day can be of temporary help in patients with
diarrhea and FI. Loperamide has been reported to improve rectal sensitivity. Other less
efficacious agents that have been tested are topical phenylephrine, lidamidine, amitriptyline, and sodium valproate. Patients with diarrhea and FI secondary to bile salt malabsorption can benefit from using ion-exchange resins such as cholestyramine or
colestipol.
29
13.6.1.3 Biofeedback Therapy
Biofeedback therapy (BFT) is a safe and effective treatment that improves FI symptoms,
restores quality of life, and objectively improves anorectal physiology. Therefore it should
be considered a first-line therapeutic measure in patients with FI secondary to weakness
of the sphincteric muscular apparatus, with or without sensory abn ormalities of the rectum.
30
The principle behind BFT is the acquisition of different behavior patterns through
the process of trial and error. If the new conduct is repeatedly reinforced, the possibility of
success increases considerably. The goals of BFT are (a) to improve anal sphincter
strength, (b) to improve the coordination between the muscles of the pelvic floor and anal
sphincter, and (c) to improve anorectal perception. Because each aim requires a specific
method of training, treatment should be individualized for each patient, according to the
corresponding predominant pathophysiologic mechanism. At least six 15-day sessions are
recommended, and after each session, the patient should be taught how to perform the
exercises at home (Kegel exercises). Most treatment responders report symptom improvement after the 4th session.
31–33
The effectiveness of BFT on FI symptoms varies from 40% to 85%.
31–33
Long-term studies have shown that BFT reduces defecation frequency and the number of FI episodes and
increases quality of life, as well as anal sphincter pressure and rectal capacity.
33
Based on
the strength of evidence, the American Neurogastroenterology and Motility Society and
the European Society of Neurogastroenterology and Motility task force recommend
BFT for the short-term and long-term treatment of FI (Level II, Grade B).
34
However, it
Chapter 13 • Fecal Incontinence 171
should be stated that the presence of severe FI, severe pudendal nerve neuropathy, extensive sphincteric defects, and the coexistence of systemic neurologic diseases are factors
associated with poor response to BFT.
13.6.1.4 Posterior Tibial Nerve Stimulation
Posterior tibial nerve stimulation (PTNS) is a simple, noninvasive, low-cost technique.
There are two methods of stimulation: percutaneous, using needle electrodes, and
transcutaneous, using adhesive surface electrodes. Two electrodes are placed in the nerve
pathway of the posterior tibial nerve and are connected to a neurostimulator that can be
controlled by the patient. The mechanism involved in the treatment is still not well understood, but undoubtedly it can be inferred that stimulation improves or affects somatosympathetic reflexes. Despite the fact that there are only a few published studies with relevant
results,
35,36
PTNS reduces episodes of FI in 63%–82% of the patients treated, with followup ranging from 1 to 30 months. At present, there is no consensus on treatment duration,
the stimulation frequency/rhythm, or the need to repeat treatment. Nevertheless, the
treatment can be recommended for patients who are nonresponders to other noninvasive
techniques or that have FI with no transit disorders.
13.6.1.5 Transanal Irrigation
Transanal irrigation (TAI) is currently recommended as second-line therapy, after dietetic
measures and medical treatment, in patients with chronic neurologic diseases.
37
The
aim is to empty the colon through regular irrigation, achieved through the use of a catheter
with an inflatable rectal balloon to form a hermetic syst em. TAI improves FI in 40%–75% of
patients presenting with chronic neurologic diseases.
37
It also aids in improving patient
quality of life, increasing patient independence, and appears to reduce the risk of urinary
infections. However, TAI can only function if the patient and his or her family are committed to it.
13.6.1.6 Anal Plugs
The placement of a device that functions as a “plug” at the level of the anus can be useful in
some patients with FI. The devices are made from different substances, are easy to use,
and their routine use can be of great help. The plugs can be employed as a complementary
treatment to other therapies. However, it can be difficult to tolerate an anal plug and the
abandonment rate is 68%.
38
13.6.2 Minimally Invasive Treatments
When conservative therapy has not provided sufficient results, minimally invasive
methods for improving FI can be proposed, which are outlined in the following section.
13.6.2.1 Sacral Nerve Stimulation
Continuous sacral nerve stimulation (SNS) has become a treatment for patients presenting with at least one FI episode a week that are nonresponders to conservative therapies or
172 ANORECTAL DISORDERS
BFT.39Indications for SNS are: idiopathic FI with no sphincteric injury, patients with FI
and urinary incontinence, postobstetric perineal injury (tears of the anal sphincter or
pudendal neuropathy), and FI of central or peripheral neurologic origin.
40
SNS apparently improves the somatosympathetic medullary reflexes, and it also has a
certain effect at the central level, improving neuronal plasticity.
40,41
The treatment continuously stimulates the sacral nerves through an implanted electrode in contact with the
nerve that leaves the sacral foramen.
39
The device is configured in two stages. The first
stage, called peripheral nerve evaluation, is a trial period and lasts for 2–3weeks. The electrode is implanted near the S3 root and connected to an external stimulator.
39
In that
stage, the stimulation parameters are adjusted (frequency and intensity) until they reach
the desired effects. The second stage involves the definitive implantation of the stimulator
beneath the skin. This is done only if there has been a 50% reduction in FI episodes during
the first stage. In general, and according to a recent literature review, SNS appears to be
efficacious in approximately 60%–70% of patients with FI that have experienced conservative management failure. The therapeutic effect persists over time, but there is a 10%
reduction in efficacy within the first 5 years.
41
The most common adverse events are pain
and infection at the insertion site, which occurs in 10% of the patients.
39–41
13.6.2.2 Antegrade Continence Enema (Malone Procedure)
Based on the same principle as TAI, the aim of anterograde irrigation is to restore continence by keeping the colon empty. The procedure consists of performing a cecos tomy and
placing a catheter that lets patients carry out irrigations themselves. “Pseudo” continence
has been reported in 80%–90% of patients with this technique, but device explantation is
one of the complications.
42
Antegrade continence enemas have also been shown to be
efficacious when combined with an artificial urinary sphincter in patients with double
incontinence. Endoscopic cecostomies hav e currently been described with similar results;
however, more studies on this novel approach are needed.
42
13.6.2.3 Anal Radiofrequency (SECCA Procedure)
The SECCA procedure consists of the application of temperature-controlled radiofrequency energ y (465 kHz, 2–5 W) to all the quadrants of the anal canal.
39
The heat that
is released is thought to induce tissue contraction and anal canal remodeling through
retractile fibrosis formation, with subsequent collagen deposition, thus inducing IAS contraction. Even though initial studies reported improvement in FI and quality of life, more
recent analyses have shown contradictory results, and the long-term efficacy of the
procedure appears to rapidly decrease.
39
13.6.2.4 Intersphincteric Injections
To increase the resting pressure of the anal canal, the injection of various substances
into the submucosa or the intersphincteric space has been tested in FI management. Efficacy has varied, depending on the tria l product, but few studies have compared the substances with other simulated injections. At present, only the injection of dextranomer
Chapter 13 • Fecal Incontinence 173
microspheres stabilized in hyaluronic acid (NASHA Dx®) has been approved by the Food
and Drug Administration (FDA) for FI and it is the only product that has shown a statistically significant difference when compared with placebo.
43
In fact, a reduction in the
number of FI episodes by >50% was reported in 52% of the patients who received NASHA
Dx, compared with 31% of those who received placebo.
43
A large number of adverse effects
have been described, but the majority are not severe (proctalgia, fever, and rectal
bleeding).
13.6.3 Surgical Treatment
Patients with severe FI that have experienced medical-treatment failure or large defects of
the sphincter benefit from surgical treatment. Nevertheless, careful and thorough preoperative evaluation to determine FI etiology is the cornerstone of selection of those
patients.
39
13.6.3.1 Overlapping Sphincteroplasty
Overlapping sphincteroplasty is the most common surgical procedure and its success
varies from 35% to 70%. Despite the fact that 70%–80% of patients report initial
improvem ent, there can be up to a 50% decrease in long-term improvement.
44
Postoperative complication rates are generally low and wound infection of 6%–35% is most
commonly reported.
39,44
13.6.3.2 Artificial Intestinal Sphincter and Dynamic Graciloplasty
If the anal sphincter is severely damaged, (1) a neosphincter can be constructed by
reinforcing the existing sphincter with autologous skeletal muscle, frequently the gracilis muscle (dynamic graciloplasty) and/or the gluteuses; or
(2) an artificial sphinc ter
can be used.
39,45
The success of these procedures varies from 42% to 85%, and the most
common adverse even ts are infection (2 8%), stimulator malfunction (15% ), and leg
pain (13%).
39
13.6.3.3 Colostomy
Stool diversion through the creation of a colostomy or ileostomy offers definitive treatment for FI in patients in whom conservative treatments have failed or are not applicable,
especially in patients with spinal cord injury. Despite the esthetic implications and popular opinion, quality of life improves significantly in many patients after the procedure. In
an illustrative study, 83% of 69 subjects felt that a stoma restricted their lives “a little” or
“not at all,” whereas 84% “probably” or “definitely” would chose having the stoma over
their previous treatment. Furthermore, overall satisfaction with the stoma was graded
as 9/10.
46
Nevertheless, the generalization of these findings could be limited due to patient
selection. Approximate colostomy mortality rates range from 2% to 5% and the associated
complications can include cardiopulmonary events related to the anesthesia and parastomal hernia.
46
174 ANORECTAL DISORDERS
13.6.3.4 Other Therapies
A recently described innovative therapy is the use of muscle-derived stem cells (MDSCs).
They have two primary forms of use: (1) direct injections into the sphincter, or (2) perianal implantation of a bioengineered sphincter.
47
The majority of studies have been conducted on animal models. The use of a magnetic anal sphincter (Fenix) has recently been
described in which a system of magnetic pearls creates negative pressure around the
structures surrounding the magnets, which in this case is the anal sphincter. The device
is made of magnetic balls held together by threads of titanium. During defecation, the
pearls separate, enabling the passage of stools. In 2012, Wong et al.
48
reported the results
of a nonrandomized study showing the magnetic device to be as efficacious as SNS in the
improvement of symptoms and quality of life. On the other hand, morbidity was similar
between the two techniques. Despite the promising results, magnetic anal sphincter efficacy must be confirmed in larger studies with better methodological designs.
References
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12. Sultan AH, Kamm MA, Hudson CN, et al. Anal-sphincter disruption during vaginal delivery. N Engl
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18. Remes-Troche JM, Rao SS. Neurophysiological testing in anorectal disorders. Expert Rev Gastroenterol
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30. Chiarioni G, Bassotti G, Stanganini S, et al. Sensory retraining is key to biofeedback therapy for formed
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96–103.
32. Rao SSC, Happel J, Welcher K. Can biofeedback therapy improve anorectal function in fecal inconti-
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176 ANORECTAL DISORDERS
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35. Thomas GP, Dudding TC, Rahbour G, Nicholls RJ, Vaizey CJ. A review of posterior tibial nerve stimu-
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36. Knowles CH, Horrocks EJ, Bremner SA, et al. Percutaneous tibial nerve stimulation versus sham elec-
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37. Rosen H, Robert-Yap J, Tentschert G, Lechner M, Roche B. Transanal irrigation improves quality of life
in patients with low anterior resection syndrome. Colorectal Dis. 2011;13:e335–e338.
38. Deutekom M, Dobben AC. Plugs for containing faecal incontinence. Cochrane Database Syst Rev.
2012;4CD005086.
39. Bharucha AE, Rao SSC, Shin A. Surgical interventions and the use of device-aided therapy for the treat-
ment of fecal incontinence and defecatory disorders. Clin Gastroenterol Hepatol. 2017;15(12):
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40. Leroi AM, Michot F, Grise P, Denis P. Effect of sacral ner ve stimulation in patients with fecal and urinary
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41. Thin NN, Horrocks EJ, Hotouras A, et al. Systematic review of the clinical effectiveness of neuromo-
dulation in the treatment of faecal incontinence. Br J Surg. 2013;100:1430–1447.
42. Hoekstra LT, Kuijper CF, Bakx R, Heij HA, Aronson DC, Benninga MA. The Malone Antegrade conti-
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43. Graf W, Mellgren A, Matzel KE, Hull T, Johansson C, Bernstein M. Efficacy of dextranomer in stabilised
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44. Glasgow SC, Lowry AC. Long-term outcomes of anal sphincter repair for fecal incontinence: a system-
atic review. Dis Colon Rectum. 2012;55:482–490.
45. Chapman AE, Geerdes B, Hewett P, et al. Systematic review of dynamic graciloplasty in the treatment
of faecal incontinence. Br J Surg. 2002;89:138–153.
46. Norton C, Burch J, Kamm MA. Patient’s views of a colostomy for fecal incontinence. Dis Colon Rectum.
2005;48:1062–1069.
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48. Wong MT, Meurette G, Wyart V, Lehur PA. Does the magnetic anal sphincter device compare favour-
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e323–e329.
Further Reading
49.
Lienemann A, Anthuber C, Baron A, Reiser M. Diagnosing enteroceles using dynamic magnetic resonance imaging. Dis Colon Rectum. 2000;43:205–213.
50. George AT, Kalmar K, Sala S, et al. Randomized controlled trial of percutaneous versus transcutaneous
posterior tibial nerve stimulation in faecal incontinence. Br J Surg. 2013;100:330–338.
Chapter 13 • Fecal Incontinence 177
14
Anorectal Malformations
Jorge Can˜as Acar
HOS P I T A L I N F AN T I L DE VER A C R U Z, V E R A C R U Z , MEX I C O
14.1 Introduction
Anorectal malformations (AMs) constitute a spectrum of defects that present during the
embryologic formation of the human being and they involve the distal part of the digestive
tract, affecting the rectum and the anal cana l. Their exact causes are unknown, but they
comprise both genetic and environmental factors. The approximate frequency of AMs is
one in every 5000 newborns, with a slight predominance in males. The most frequent
defects are rectovestibular fistula in girls and recto-urethral-bulbar fistula in boys
(
Fig. 14.1).
1
The first description of an AM dates back to Aristotle in the third century BC. In the
second century AD, the Greek physician, Soranus of Ephesus, described the removal of
an anal membrane as treatment for an AM, but it was not until 1835 that the French physician, Amussat, described anorectal repair as surgical management for that type of
abnormality.
2
For many years, descriptions of and the treatments for AMs were limited,
with heterogeneous results. A reproducible surgical treatment with satisfactory results,
posterior sagittal anorectoplasty (PSARP), was first reported in 1982 by Dr. Alberto Pen˜a.
3
Since then, PSARP has been the surgical technique most widely used worldwide. Variations of the original technique have enriched treatment options, such as the incorporation
of laparoscopic surgery.
4,5
14.2 Classification
In an attempt to use the same terminology for identifying AMs, different classifications
have been suggested through the ages. In 1970, the first international classification to
unify criteria was proposed,
6
but its length made it unsatisfactory and it did not aid in
the practical approach to patients. At a meeting of experts held at the Wingspread Conference Center in Racine, Wisconsin, in 1984, a more practical modification of the previous classification was proposed. However, it was in 2005 that a group of experts came
together at the Castle of Krickenbeck in Germany and developed the so-called Krickenbeck classification, which is currently the simplest and most widely accepted classification (
Table 14.1).
7,8
Anorectal Disorders. https://doi.org/10.1016/B978-0-12-815346-8.00014-X
© 2019 Elsevier Inc. All rights reserved.
179
14.3 Clinical Manifestations and Diagnostic Approach
It is essential to be perfectly familiarized with the anatomy and embryology of the anorectal region, as the slightest gap in the knowledge of the anatomy of that area can lead to
failed and catastrophic results for the patient (see
Chapters 1 and 2).
The clinical manifestations of AMs are evident from birth and therefore targeted
physical examination of the anorectal region is very important to determine whether the
alterations are isolated (isolated AM) or belong to a syndrome (syndromic AM). The most
frequent syndromic AMs are associated with urinary tract defects, dysraphism (a group of
pathologies that are characterized by an anomaly in the development of the neural
tube, such as myelomeningocele), and other gastrointestinal malformations (
Fig. 14.2).
Table 14.1 The Krickenbeck Classification of Anorectal Malformations
Major Clinical Groups Rare/Regional Variants
Perineal (cutaneous) fistula
Rectourethral fistula/atresia/stenosis
Bulbar fistula
Prostatic fistula
Rectovesical fistula
Vestibular fistula
Cloaca
AMs with no fistula
Anal stenosis
Pouch colon atresia/stenosis
Rectal atresia/stenosis
Rectovaginal fistula
H-type fistula
Others
FIG. 14.1 (A) Image showing a rectovestibular fistula, a pathology more frequent in girls. White arrow illustrates the
presence of the hymen. (B) Image showing a recto-urethral-bulbar fistula, a pathology more common in boys.
180 ANORECTAL DISORDERS
There is also an association with ocular coloboma9and Hirschsprung’s disease.10On occasion, more than one defect can present, giving rise to syndromes that include Currarino
syndrome (AM, sacrococcygeal bone defect, and presacral mass), VACTERL (vertebral
defects, anal atresia, cardiac defects, trachea-esophageal fistula, renal anomalies, and limb
abnormalities) association, MURCS (Mullerian duct aplasia, renal agenesis, cervicothoracic
somite dysplasia) association, or OEIS (omphalocele, exstrophy of the cloaca, imperforate
anus, and spinal defects) complex.
11,12
At birth, all children should be carefully examined, and the permeability of all orifices
verified, including the anus. The absence of the anal orifice or its ectopic presence are reasons to carry out a comprehensive diagnostic approach to define the type of malformation
and associated defects and to establish priorities for the initial treatment. A flat “bottom”
or flat perineum, as evidenced by the lack of a midline gluteal fold and the absence of an
anal dimple, indicates that the patient has very poor muscles in the perineum. These findings are associated with a high malformation and warrant colostomy. Patients with low
malformations present with perineal signs that include the presence of meconium at
the perineum, a prominent skin tag (that an instrument can be passed under) located
at the anal dimple, known as a “bucket-handle” malformation, and an anal membrane
(through which the meconium can be seen).
A lateral radiograph in the prone position with the pelvis elevated (modified invertogram), taken after the first 18 h of life, enables the distinction to be made of whether the
patient requires a bowel diversion (colostomy) or surgical anoplasty at that time. Important associated anomalies include genitourinary defects, which occur in approximately
FIG. 14.2 Anorectal malformation: fourchette fistula variant with benign perineal teratoma.
Chapter 14 • Anorectal Malformations 181
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