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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_1499_Библиотеки_им_академика_М_И_Перельмана.pdf
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Pathogenesis reflects complex interaction between genetic susceptibility, environmental triggers (diet, infections, etc), and
the immune system: initiating factors result in a leaky mucosa, which allows for antigen presentation and sensitization.
Perpetuation and amplification of immune response subsequently cause the autodestructive action.
Lacking a specific cause, treatment remains unspecific. In contrast to ulcerative colitis (UC), surgery is generally not curative
in Crohn disease and is therefore only indicated for complications of the disease. Nonetheless and paradoxically, > 50%
probability that patients will need surgery in first decade, > 70–90% lifetime risk. Repeated interventions result in significant
risk of secondary morbidity (ostomy, short bowel syndrome).
Epidemiology
Annual incidence in Western countries: 6–8 new cases per 100,000, prevalence 50–100 per 100,000. Bimodal age peak: 15–
30 and 60–80 years. North-south gradient: higher incidence in industrialized countries, urban areas > rural areas. No
severity differences among ethnicities.
Positive family history in 15–25%. Disease concordance among twins: 30–67% monozygotic vs 4% dizygotic. Smoking
results in increased primary risk, increased risk for disease relapse (contrast: nicotine beneficial in UC). Higher mortality of
Crohn patients compared with non-Crohn population. Crohn disease is associated with increased risk of colorectal and small
bowel cancer in areas of chronic inflammation.
Symptoms
Variations of the disease in severity and duration with alternating exacerbations and remissions:
General symptoms (particularly in childhood onset): anorexia, weight loss, malnutrition, anemia (blood loss, vitamin
B
12
deficiency), growth retardation.
Abdominal symptoms: diarrhea (from epithelial damage combined with decreased bile acid absorption, causing
cathartic effect in colon), bleeding, abdominal pain and cramping, inflammatory mass, fever, sepsis, obstructive
symptoms (stricture).
Perianal manifestations: edematous skin tags, suppurations/abscess, fistula, nonhealing fissures/ulcers, anal
stenosis.
Extraintestinal manifestations: cholelithiasis (decreased bile acid absorption in ileum), urolithiasis (bile acids binding
calcium
increased absorption of oxalate increased urinary oxalate concentration), sclerosing cholangitis, skin
pathology (erythema nodosum, pyoderma gangrenosum), ophthalmopathy (uveitis, conjunctivitis, iritis), rheumatologic
pathology (polyarteritis nodosa, arthralgias, rheumatoid spondylitis), bronchopulmonary disease.
Complications: massive bleeding, sepsis, retroperitoneal abscess, toxic megacolon, malignant transformation.
Differential Diagnosis
UC, indeterminate colitis (7–15%), ischemic colitis, appendicitis, diverticulitis (sigmoid, right-sided), drug-induced colitis (eg,
NSAIDs), infectious colitis (including pseudomembranous C difficile colitis), STD proctitis (eg, lymphogranuloma venereum,
gonorrhea), radiation proctitis, IBS, celiac disease, Whipple disease, Behçet disease.
Pathology
Distribution pattern: anywhere in the GI tract: 40–50% small and large bowel, 30% small bowel, 20% colon (Figure 4–14A),
15–40% perianal involvement (3–5% of patients have only perianal disease).
Figure 4–14A.
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Crohn colitis: acute inflammation with skip areas.
MACROSCOPIC
– Patchy discontinuous inflammation (skip lesions, Figure 4–14A), longitudinal ulcers (bear claw, rake), cobblestoning,
aphthoid ulcers, deep ulcers and fissures that may form into fistulae, mesenteric fat wrapping or creeping, mesenteric
thickening and lymphadenopathy.
– Perianal lesions: skin tags, hemorrhoids, fissures, anal ulcers, fistulas, rectovaginal fistulas, perianal abscesses,
anorectal strictures, anal cancer.
– Caveat: cancer developing in Crohn disease does not often form a mass!
MICROSCOPIC
Transmural acute and chronic inflammation (neutrophils, lymphocytes), ulcerations, formation of noncaseating granulomas
(overall present in 50–60%, but only rarely in superficial biopsy!), penetrating fissures fistulae, abscesses; chronic
inflammation fibrosis, stricturing.
Caveat: morphologic overlap with UC in 7–15% of patients (indeterminate colitis).
Evaluation
REQUIRED MINIMAL STANDARD
History: family history of IBD? Bowel function, perianal manifestations (current, past), course of symptoms, extraintestinal
manifestations, preexisting fecal incontinence, smoking? Risk factors for other differential diagnoses?
Clinical examination: stigmata of Crohn disease (perianal disconfiguration, fistulae, etc), strictures?
Endoscopy:
– Rigid or flexible sigmoidoscopy: rectal involvement, biopsy?
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– Colonoscopy: gold standard to evaluate extent and activity of large bowel disease.
Stool cultures, O&P, C difficile toxins:
rule out specific, infectious etiology.
Blood work: C-reactive protein (CRP), CBC, liver parameters, nutritional parameters.
Small bowel evaluation: small bowel follow-through, CT-enterography, or capsule endoscopy (caveat: presence of
strictures!).
Emergency presentation: plain abdominal x-rays
rule out perforation or colonic dilation.
ADDITIONAL TESTS (OPTIONAL)
Markers: ASCA-positive (60% positive in Crohn disease), pANCA-negative (60–80% positive in UC): combination ASCA-
positive/pANCA-negative 80% predictive of Crohn disease, definitive role remains controversial.
CT: > 70% sensitivity and > 90% accuracy for Crohn-specific changes.
MRI: evaluation of complex pelvic and perirectal fistulae.
Contrast studies (barium or Gastrografin enema): mucosal pattern, intestinal macroconfiguration, strictures, fissures, and
fistulae (Figure 4–14B); procedures are contraindicated in patients with acute disease (may precipitate deterioration with
toxic dilation).
Figure 4–14B.
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Crohn colitis: chronic strictures and perirectal fistulae.
Virtual colonoscopy: role not defined, risk of perforation.
PET, PET-CT: role not yet defined, may be useful for detection of fistulae or skip lesions, for assessment of disease activity,
and for differentiation between Crohn disease and UC.
Classification
Disease behavior: stricturing, nonstricturing, penetrating, nonpenetrating (inflammatory).
Anatomic location: terminal ileum (distal one-third of small bowel), colonic (no small bowel involvement), ileocolonic
(both small and large bowel), upper GI tract (proximal to distal one-third of small bowel), perianal.
Severity (reflected in Crohn activity index): mild, moderate, severe, fulminant, in remission.
Nonoperative Treatment
Conservative management = treatment of choice; defining goals: induction of remission (symptom control, disease activity
suppression), maintenance of remission, prevention of relapse after surgery; in severe cases: need for bowel rest and TPN.
Drug categories:
Salicylates: mild to moderate disease.
Corticosteroids: moderate to severe disease, rapid suppression of activity in 70–80%.
Antibiotics: moderate to severe disease, active suppurations and abscesses.
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Conventional immunosuppressants (azathioprine, 6-mercaptopurine (6-MP), methotrexate, cyclosporine, tacrolimus,
mycophenolate mofetil): maintenance for long-term activity suppression, avoidance of chronic steroid dependency;
may take 3–6 months until visible effect.
Biologic immunosuppressants (infliximab, natalizumab, adalimumab): rapid suppression of activity, steroid-resistant
disease (20–30%).
Operative Treatment
INDICATIONS
Symptomatic subacute/chronic disease complications: recurrent/persistent abscess (not manageable with
percutaneous drainage), fistulae, stricture.
Acute life-threatening complications: fulminant colitis, toxic megacolon, perforation, sepsis, massive bleeding lack
of response or deterioration within 3–5 days of conservative treatment.
Malignancy: established cancer, any level of dysplasia (low grade, high grade), nonsurveillable colonic strictures (5–
10% risk of malignancy).
Treatment-refractoriness of limited-extent disease: failure of or side effects from conservative treatment, (risk of)
steroid-dependency.
OPERATIVE APPROACH
Principles
Goal: optimized symptom control vs reconstruction with low morbidity/mortality and high quality of life (eg,
avoidance of ostomy).
Small intestine nonrenewable resource need for bowel conservation: no benefit from radical surgery no difference
in terms of recurrence between limited and wide resection margins.
Laparoscopic approach (if appropriate) decreased long-term risk of adhesive SBOs.
Elective/Semi-Elective
Limited resection with/without primary anastomosis, possible Hartmann-type resection, possible proximal diversion.
Single/multiple stricturoplasty/-ies (different techniques).
Isolated Crohn colitis: total colectomy or proctocolectomy. In that situation, IPAA may be offered at experienced
centers as an option (even if 5–10 times higher risk of failure) if both criteria fulfilled: (1) small bowel disease absent;
(2) perianal manifestations absent.
Urgent/Emergency
Fulminant/toxic colitis: total abdominal colectomy with end-ileostomy (ie, sparing rectum and pelvic dissection) for all high-
risk patients; alternative: complete excision (ie, proctocolectomy with permanent ileostomy).
Outcome
Relapse after discontinuation of steroids: 40–50%.
Risk of enterocutaneous fistula: open abscess drainage > percutaneous abscess drainage.
Postsurgical recurrence rate at 2 years for small bowel disease: 70–80% without medical treatment, 55–60% with 5-ASA,
50% with 6-MP.
Recurrence rate for colitis only: ~25% after proctocolectomy.
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Follow-Up
Careful guidance of patients, monitoring of disease activity, surveillance, teamwork with gastroenterologist.
Cross-Reference
Diarrhea (Ch. 1)
Fistula (Ch. 1)
Perianal/-rectal fistula (565.1) (Ch. 4)
Hidradenitis suppurativa (705.83) (Ch. 4)
IBD—ulcerative colitis (556.9) (Ch. 4)
Enterocutaneous fistula (569.81) (Ch. 4)
"Stomatology"—the science of ostomies (V44.2) (Ch. 4)
Total abdominal colectomy with end-ileostomy (open) (Ch. 5)
Proctocolectomy, ileal J-pouch anal anastomies (Ch. 5)
Medical management of Crohn disease (Ch. 6)
Medications against tumor or inflammation (App. 1)
"POUCHOLOGY"—THE SCIENCE OF POUCHES
Overview
Pouches reflect an important concept in continence-preserving procedures: creation of an internal reservoir from the
intestines, to serve as a low-pressure/high-volume storage area for feces or urine and/or to slow or interrupt propulsive
waves and hence decrease urgency.
Effects
Pouch Physics
Goal: low-pressure reservoir, increased volume, reversed peristalsis: higher compliance, reduced urgency/frequency.
Pouch Bacteriology
Colonic pouch: normal colonic flora unchanged.
Small bowel pouch: adaptation of microenvironment adaptation of bacterial density and composition such that it
lies in between normal small bowel or end-ileostomy and normal colonic flora.
Histology
Colonic pouch: unchanged.
Ileal pouch: adaptation of mucosa to more colon-like architecture; varying degrees of inflammatory changes.
Classification
Different aspects to be considered when classifying pouches:
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Organ of origin: colon, small bowel.
Specific location of anastomosis: ileoanal, coloanal, urinary, ileocutaneous.
Type of construction: J-pouch (ileal, colonic), transverse coloplasty, S-/W-/H-pouches, continent ileostomy pouch
(T-pouch, Kock pouch, BCIR [Barnett continent intestinal reservoir]). Urinary pouches (T-pouch, Kock/Studer/Indiana
pouches, ileal conduit).
Connection: internal anastomosis (anus, urethra), skin-level ostomy.
Common Problems (for All Types of Pouches)
Early
Patient anxiety/frustration, mismatch between expectations and true function: need for continued patient education
and support (ideally to start before surgery: patient support group, Internet chat rooms; caveat: clustering of negative
outcomes!).
Leak: correlation with overall length of anastomotic suturing, tension, blood supply (ischemic edges), nutritional
status, concomitant immunosuppression/steroid medication, malnutrition.
After Healing
Pouchitis: unclear etiology, bacteriologic imbalance, increase in sulfide concentration. Defined by either (1) clinical
criteria: increased frequency, diarrhea, gas formation, flulike symptoms; or (2) morphologic criteria: mucosal
inflammation, ulcerations.
Pouch prolapse: ileal J-pouch anal anastomosis, continent ileostomy pouchopexy, local trimming of redundancy,
complete pouch revision.
Stricture: result of chronic leak, ischemic segment, or postradiation treatment with serial dilations, stricturoplasty,
salvage pouch.
Fistula: result of anastomotic leak, underlying Crohn disease, cryptoglandular origin proximal diversion, drainage
procedure, pouch revision with axial rotation and reinsertion, collagen plug, advancement flap, muscle interposition.
Sinus: confined anastomotic leak marsupialization/incorporation into pouch vs pouch revision.
Pouch polyp risk for pouch cancer: definitive risk in FAP, low but not negligible risk in IBD. Prophylaxis: adequate
pouch surveillance; once cancer present
oncologic resection and management.
Pouch outlet obstruction: low-pressure reservoir anastomosed to high-pressure segment, ie, S-pouch with long
efferent limb, J-pouch to high rectum:
functional outlet obstruction (as opposed to morphologic stricture) surgical
revision vs supportive measures (tube insertion, stool management).
Assessment Tools
Clinical assessment: inspection, digitalization, etc.
Endoscopy: rigid or flexible, often narrow instruments needed.
Contrast studies (pouchogram): road map, leak, sinus, fistula, size, configuration, distance to the sacral curvature (on lateral
view).
Defecation proctogram/pouchogram.
CT/MRI
possible 3D reconstruction.
Specific Pouch Characteristics
COLONIC POUCH
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Example: LAR/TME.
Purpose: creation of larger storage area, reversal of peristaltic wave, reduction of urgency. Functional benefit mainly
in the first 6–12 months postoperatively; thereafter advantage compared with straight coloanal anastomosis is less
obvious.
Technical aspects:
– J-pouch: length 5–6 cm. Risk of outlet obstruction if too long limb or too long rectal stump. Rule of thumb:
creation of J-pouch is indicated if (1) possible and (2) proximal colon is too narrow to accept 33-mm stapler.
– Transverse coloplasty: ~3–4 cm vertical colotomy with transverse closure about 4 cm proximal to distal end of
colon to be anastomosed. Possible alternative to J-pouch if colon would otherwise not reach. Increased leak
rate. Less effective than J-pouch.
Output: generally formed, depending on length and condition of residual colon.
Advantage: decreased frequency, urgency, improved continence. Potentially lower anastomotic leak rate with J-pouch
(initial claim unproven).
Disadvantage: need for additional colon mobilization, depending on patient's anatomy insufficient space for loop (eg,
narrow pelvis after radiation or in obese male patient). Increased leak rate with transverse coloplasty.
Specific Problems
Incomplete separation of pouch septum from inadequately stapled J-pouch creation endoscopic linear stapler
division of the septum or pouch revision.
Functional outlet obstruction.
ILEAL J-POUCH
Example: ileoanal pull-through procedure (ileal J-pouch anal anastomosis [IPAA]): J-pouch most common form of
ileoanal anastomosis: simple, easy, predictable.
Purpose: after proctocolectomy creation of new storage area, reversal of peristaltic wave, reduction of urgency.
Technical aspects: J-pouch: length ~12 cm (2–2
1
/
2
firings of 75-mm linear stapling device). Risk of outlet
obstruction if too long limb or too long residual rectal stump.
Output: semiliquid (oatmeal-like consistency), rarely almost formed, more liquid stool not uncommon (baseline or
during episodes of pouchitis).
Advantage: decreased frequency, urgency, improved continence. Definitively preferable to straight ileoanal
anastomosis!
Disadvantage: occasionally difficult to achieve adequate small bowel mobilization; in very obese patients potentially
insufficient space for loop or difficulty to get sufficient length:
alternatives: S-pouch (risk of outlet obstruction, see
below) or straight ileoanal anastomosis (poor functional result).
Specific Problems
Pouch leak: order of frequency for J-pouch: (1) pouch–anal anastomosis; (2) staple line of most distal bowel
segment; (3) any of the anastomotic lines in between.
Incomplete separation of pouch septum: inadequately stapled J-pouch endoscopic linear stapler division or pouch
revision.
Functional outlet obstruction.
ATZ cuffitis: frequently inflammatory changes on pathology, but clinically not relevant. If patient symptomatic
mesalamine suppositories. If no success and unclear whether frequency caused by cuffitis or pouchitis transanal
mucosectomy and pouch advancement to dentate line.
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ATZ dysplasia: Prophylaxis: adequate ATZ surveillance with visualization and biopsies every 1–3 years transanal
mucosectomy and pouch advancement to dentate line.
CONTINENT ILEOSTOMY POUCH (T-POUCH, KOCK POUCH, BCIR/BARNETT
POUCH)
Example: alternative to IPAA or end-ileostomy after proctocolectomy, fallback option after failed IPAA.
Purpose: skin-level stoma (in lower location than regular ileostomy), consisting of low-pressure, high-volume internal
storage area, equipped with a continence mechanism (valve) to prevent inadvertent discharge of stool. Evacuation of
stool through insertion of ileostomy tube 3–5 times per day. Obviates the need for an external appliance.
Technical variations (in historical order):
– Kock pouch (1969): valve mechanism consisting of intussuscepted efferent bowel segment, reservoir
commonly as folded U. Functional pouches result in high patient satisfaction. But increased perioperative
morbidity (leak rate) and high probability of reoperation: valve slippage (desintussusception), most commonly
either within the first 6 months or after 15–20 years
kinking of efferent segment with increasing leakage and
difficulty to intubate the pouch, or valve prolapse with leakage.
– Comment: despite high reoperation rate, these patients are generally so convinced of the continent ileostomy
concept that they would rather undergo one or several reoperation(s) than consider an end-ileostomy.
– BCIR (Barnett continent intestinal reservoir; 1984): Expanded but more complex Kock pouch concept: Valve
mechanism consisting of intussuscepted efferent bowel segment in combination with additional bowel wrap at
the pouch outlet/neck to form collar with additional pressure on valve as pouch fills; reservoir like in Kock pouch
but front suture anastomosed to bowel wrap. Concept developed in response to problems with intussuscepted
valve; construction more complex and using more small bowel. Results comparable.
– T-pouch (Kaiser, 2002): Completely different valve concept using an interposed separate bowel segment with
preserved vascular arcades
concept of intussusception abandoned and risk of valve disintegration and slippage
decreased. Design of pouch reservoir of no importance and therefore variable according to individual needs:
folded U, S-pouch, long U-pouch configuration. Pouch may be constructed completely new from scratch, or
incorporate parts of preexisting pouches, or augment just a new T-pouch valve on an existing reservoir.
Output: like ileostomy—oatmeal-like, opaque, 600–1000 mL/24 hours.
Advantage: no need for external appliance with improved quality of life, permitting active physical lifestyle, improved
body image, fewer social and workplace restrictions, fewer sexual restrictions (patient and partner).
Disadvantage: need for patient selection, increased perioperative morbidity, long-term dysfunction, pouchitis. Rarity
of this type of stoma
other physicians (eg, in emergency department) are not familiar with concept. Food
restrictions: size of holes in tube limited
raw vegetables, seeds, etc, may be a problem.
Specific Problems
Pouch leak: regardless of type of continent ileostomy, there is a 10–15% leak rate (correlation with overall length of
anastomotic suturing).
Skin-level stoma need for minor "maintenance" operations: (1) tendency to form stricture widening in local
anesthesia; (2) mucosal redundancy
trimming with infrared coagulation or surgical shortening.
Stone formation: particularly in inadequately drainable pouch area.
Inability to intubate emergency because of risk that pouch ruptures use of narrow rigid sigmoidoscope to
intubate under visual control and decompress pouch, followed by sliding in small guiding tube over which larger tube
can be inserted.
OTHER POUCHES (S-/W-/H- POUCHES)
Example: design alternatives to J-pouch for ileoanal pull-through procedure.
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Purpose: S-pouch improves ability to reach pelvic floor, W-/H-pouches increase reservoir (usually not necessary!).
Disadvantage: S-pouch—too-long efferent limb low-pressure reservoir situated proximal to high-pressure segment
fecal outlet obstruction. S-/W-/H-pouches—technically much more complex and challenging construction than J-
pouch.
URINARY POUCHES (T-POUCH, KOCK/STUDER/INDIANA POUCHES, ILEAL
CONDUIT)
Purpose: neobladder or conduit formation after complete cystectomy, eg, during pelvic exenteration.
Details: see urology literature.
Cross-Reference
Familial adenomatous polyposis (FAP; 211.3, 235.2, V84.09) (Ch. 4)
IBD—ulcerative colitis (556.9) (Ch. 4)
Pouchitis—ileoanal dysfunction (569.60) (Ch. 4)
Kock pouch dysfunction (Ch. 4)
"Stomatology"—the science of ostomies (V44.2) (Ch. 4)
Proctocolectomy, ileal J-pouch anal anastomies (open) (Ch. 5)
Low anterior resection (LAR)/total mesorectal excision (TME) (Ch. 5)
POUCHITIS—ILEOANAL DYSFUNCTION (569.60)
Overview
Restorative proctocolectomy (IPAA) is the surgical treatment option of choice for patients with treatment refractoriness or
(pre-)malignant transformation in ulcerative colitis and FAP. Overall outcome and satisfaction are excellent with expected
benchmarks of 4–8 bowel movements per day, good fecal control, ability to defer defecation until socially convenient, ability
to work, and improved quality of life.
Ileoanal dysfunction is defined as failure to meet these goals. Acute vs recurrent episodes or chronic ileoanal dysfunction.
Pouchitis is the most frequent long-term complication after IPAA or continent ileostomy for ulcerative colitis, represents the
most common cause of ileoanal dysfunction of an intact pouch. Pathogenesis remains unclear (bacterial overgrowth or
imbalance, sulfide concentration, IBD autoimmunity, nutritional etiology, etc).
Poor correlation between morphologic (endoscopic) and functional parameters
use of clinical criteria for diagnosis of
pouchitis:
Intact pouch, not defunctionalized by diverting ileostomy.
Sudden change in the clinical course (onset of diarrhea, fever, malaise, urgency, incontinence).
No evidence of pouch stricture or pelvic symptoms from Crohn disease, pelvic abscess, pouch fistula.
Negative bacteriology.
Rapid response to antibiotics (eg, metronidazole, ciprofloxacin, rifaximin).
Pouchitis disease activity index (PDAI): scoring system based on clinical, endoscopic, and histologic parameters.