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Chapter 78 Short-Bowel Syndrome Jon S. Thompson Alan N. Langnas Intestinal failure refers to a condition that results in inadequate digestion or absorption of nutrients, or both, so that an individual becomes malnourished and requires specialized medical and nutritional support.[1] Short-bowel syndrome is a type of intestinal failure caused by a shortened remnant after intestinal resection. The pathophysiologic changes that occur in short-bowel syndrome relate primarily to the loss of intestinal absorptive surface and more rapid intestinal transit ( Box 781 ). The consequences of malabsorption of nutrients include malnutrition, diarrhea, steatorrhea, specific nutrient deficiencies, and fluid and electrolyte abnormalities. These patients are at risk for other specific complications, including an increased incidence of nephrolithiasis, cholelithiasis, and gastric hypersecretion. The clinical manifestations of short-bowel syndrome vary greatly among patients and depend on intestinal remnant length, location, and function; the status of the remaining digestive organs; the presence or absence of the ileocecal valve; and the adaptive capacity of the intestinal remnant. Thus, short-bowel syndrome is not entirely dependent on a given length of remaining intestine. Box 78-1 Pathophysiologic Consequences of Massive Resection General Malnutrition and weight loss

Diarrhea and steatorrhea

Vitamin and mineral deficiencies

Fluid and electrolyte abnormalities Specific Cholelithiasis

Gastric hypersecretion

Liver disease

Nephrolithiasis

The prevalence of short-bowel syndrome is 3 to 4 per million, and thousands of patients are now surviving with short-bowel syndrome.[1] This condition occurs in about 15% of adult patients who undergo intestinal resection, with three fourths of these cases resulting from massive intestinal resection and one fourth from multiple sequential resections.[2] Massive intestinal resection continues to be associated with significant morbidity and mortality, primarily related to the underlying diseases necessitating resection. [8] [9] About 70% of patients in whom short-bowel syndrome develops are discharged from the hospital, and a similar percentage are alive 1 year later.[4] This improved survival rate has been achieved primarily by the ability to deliver long-term nutritional support. The long-term outcome of these patients is often determined not only by their age and underlying disease but also by complications related to the management of short-bowel syndrome. FACTORS INFLUENCING OUTCOME Intestinal remnant length is the primary determinant of outcome in patients with short-bowel syndrome. The length of the small intestine in adults varies between 12 and 20 ft (360 to 600 cm), depending on how it is measured and the height and sex of the individual. The duodenum measures 10 to 12 inches (25 to 30 cm). The length of the small intestine from the ligament of Treitz to the ileocecal junction is about 16 ft (480 cm), with the proximal two fifths being jejunum and the distal three fifths being ileum. Resection of up to half of the small intestine is generally well tolerated. Although short-bowel syndrome may develop in patients with less than 180 cm of small intestine, or about a third the normal length, permanent parenteral nutrition (PN) support is likely to be needed in patients with less than 120 cm of intestine remaining without colon in continuity and less than 60 cm remaining with colonic continuity ( Table 78-1 ). [10] [11] Table 78-1 -- Intestinal Length and Nutritional Prognosis Intestinal Anatomy Intestinal Length to Avoid Permanent Parenteral Nutrition End-jejunostomy (type 1) 100 cm

Intestinal Anatomy

Intestinal Length to Avoid Permanent Parenteral Nutrition Jejunocolic anastomosis (type 2) 65 cm

Jejunoileocolic anastomosis 30 cm (type 3) Adapted from Messing B, Crenn P, Beau P, et al: Long term survival and parenteral nutrition dependence in adult patients with the short bowel syndrome. Gastroenterology 117:1043, 1999.

The site of resection is also an important factor. Patients with an ileal remnant generally fare better than those with a jejunal remnant. The ileum has specialized absorptive properties for bile salts and vitamin B12, unique motor properties, a hormone profile different from that of the jejunum, and a greater capacity for intestinal adaptation. [12] [13] The presence of the ileocecal junction improves the functional capacity of the intestinal remnant.[7] Although previously this had been attributed to a barrier function and transit-prolonging property of the ileocecal valve, this advantage may actually be related to the specialized property of the terminal ileum itself. The status of the other digestive organs also contributes to outcome. The stomach influences oral intake, mixing of nutrients, transit time, pancreatic secretion, and protein absorption. Pancreatic enzymes are important in the digestive process and particularly influence fat absorption. The colon absorbs fluid and electrolytes, slows transit, and participates in the absorption of energy from malabsorbed carbohydrates. When compared with an end-jejunostomy (type 1 anatomy), a jejunoileal anastomosis with an intact colon (type 3 anatomy) is equivalent to 60 cm of additional small intestine, and a jejunocolic anastomosis (type 2 anatomy) is equivalent to about 30 cm of small intestine.[5] A variety of conditions requiring intestinal resection lead to short-bowel syndrome ( Table 78-2 ).[8] Patients with underlying inflammatory disease may have impaired intestinal function. The cause of resection will also influence the outcome because of the effect on other digestive organs. Long-term treatment and survival are influenced by the patient's age and other morbid conditions. Underlying disease will also influence these parameters. Table 78-2 -- Causes of Short-Bowel Syndrome Postoperative 52 (25%) Irradiation/cancer 51 (24%)

Mesenteric vascular disease 46 (22%) Crohn's disease Other benign causes 34 (16%) 27 (13%)

Total 210 From Thompson JS, DiBaise JK, Iver KR, et al: Short bowel syndrome as a postoperative complication. J Am Coll Surg 201:85, 2005.

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INTESTINAL ADAPTATION The small intestine is able to adapt to compensate for the reduction in absorptive surface area caused by intestinal resection. [15] [16] [17] This process occurs within the first year or two after resection and improves intestinal absorptive capacity ( Fig. 78-1 ). [11] Whether the adaptive response can be significantly accelerated or augmented is not clear. The overall intestinal adaptive response results from changes in intestinal structure, function, and motility.

Figure 78-1 Schematic presentation of intestinal adaptation. AA, accelerated adaptation; AHA, accelerated hyperadaptation; HA, hyperadaptation; SA, spontaneous adaptation. (From Jeppesen PB: Clinical significance of GLP-2 in short bowel syndrome. J Nutr 133:3721, 2003.)

Structural adaptation after intestinal resection involves all layers of the intestine. [15] [16] Mucosal DNA and protein synthesis and crypt cell proliferation are increased within hours after resection. Both the total number of cells and the proportion of proliferating cells are increased in the crypt. Enterocytes migrate at a faster rate along the villus. Villus lengthening occurs by an overall increased number of cells. Rates of apoptosis, or programmed cell death, increase in both crypt and villus enterocytes after resection. However, the proliferative stimulus dominates, so adaptation occurs. The ratio of crypts to villi may also increase. Microvilli along the epithelial surface increase as well. Overall, mucosal weight increases. The thickness and length of the muscle layers also increase after resection, primarily as a result of hyperplasia rather than hypertrophy of the muscle cells.[9] Muscle adaptation, however, occurs at a later time than mucosal adaptation and only after more extensive resection. These changes in the components of the intestinal wall

result in marked thickening of the intestinal wall, as well as increased intestinal circumference and length. Thus, there is an overall increase in mucosal surface area because of both villus hypertrophy and the increases in length and circumference of the remnant. Intestinal motor activity is also altered by intestinal resection.[6] The canine small intestine demonstrates a biphasic motor response to varying degrees of distal resection. There is initial disruption of motor activity, followed by adaptation. In the distal segment of the intestinal remnant after limited resection and more generally after 75% resection, motility recordings are initially dominated by recurring bursts of clustered contractions.[12] With extensive resection, these clusters are prolonged and associated with baseline tonic changes. With limited resection, there is evidence of progressive motor adaptation with eventual slowing of transit and return of migrating motor complex (MMC) cycling. This adaptation is less apparent after massive resection. Motor adaptation is more prominent in the jejunum than in the ileum. These changes are accompanied by modest alterations in smooth muscle contractility. Clinical reports also demonstrate a biphasic adaptive motor response during the first year after resection. There is disrupted motor activity in the first few months after resection, but these changes occur only after extensive resection (remnant shorter than 100 cm). Long-term human studies demonstrate a shorter duration of the MMC cycle and fed pattern after resection.[13] Functional adaptation has been well documented after resection, [7] [11] [20] [21] and structural adaptation increases intestinal absorptive surface area. Both structural adaptation and motor adaptation lead to prolonged transit time. Although the formerly accepted theory of improved absorption by individual enterocytes was discounted, more recent studies suggest that certain transport capabilities do improve. Within months of resection, diarrhea diminishes and nutritional status improves. The mechanism of intestinal adaptation has been studied extensively but is still not entirely understood. The degree of structural adaptation is related to the extent and site of resection. [15] [16] Adaptation is greater with more extensive resection, and the ileum has a greater adaptive capacity than the jejunum does. Subsequent resection elicits a further adaptive response. Luminal nutrients and secretions and growth factors are important for achieving the maximal response but are not essential for adaptation to occur ( Box 78-2 ). [15] [16] The early molecular events associated with this hyperplastic response are being investigated. [22] [23] Intestinal resection results in increased levels of a variety of gene products in enterocytes within hours. There is an immediate increase in genes that encode transcription factors, not only genes that influence cell proliferation but also those that augment nutrient trafficking, as well as heat shock genes, which maintain normal cellular function. Many of these are novel genes not normally present in intestinal epithelium. The specific triggers for these events are not clear, and there are obviously many candidates. Currently, there

is clinical interest in manipulating the adaptive response pharmacologically. Box 78-2 Factors Influencing Intestinal Adaptation

Gastrointestinal regulatory peptides

Luminal contents

Nutrients

Secretions

Systemic factors

Growth factors

Hormones

Cytokines

Tissue Factors

Immune system

Mesenchymal factors

Mesenteric blood flow

Neural influences
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MEDICAL MANAGEMENT The early management of a patient with short-bowel syndrome is that of a critically ill surgical patient who has recently undergone intestinal resection and other concomitant procedures. Thus, control of sepsis, maintenance of fluid and electrolyte balance, and initiation of nutritional support are important in the early management of these patients. For patients who have survived this early phase, the primary goals of management are to maintain adequate nutritional status, maximize the absorptive capacity of the remaining intestine, and prevent the development of complications related to both the underlying pathophysiology and the nutritional therapy. Maintain Nutritional Status The most important therapeutic objective in the management of short-bowel syndrome is to maintain the patient's nutritional status. This usually requires PN support in the early period after surgery. Fluid and electrolyte losses from the gastrointestinal tract may be great during the early postoperative period and must be monitored and replaced as soon as possible. Enteral nutritional support should be started as soon as possible when the ileus has resolved. With time, an increasing amount of nutrients are absorbed by the enteral route. This is important for maximizing intestinal adaptation and preventing complications related to PN. As their condition improves and intestinal adaptation occurs, many patients can absorb the necessary nutrients entirely by the enteral route. The length of the intestinal remnant and the status of the colon have important prognostic implications in this regard (see Table 78-1 ). The ability of patients with short-bowel syndrome to maintain adequate caloric intake enterally is determined by a variety of factors, including intestinal remnant length and location, any underlying intestinal disease, and the status of the remaining digestive organs. [21] [24] Whether there is continuity in the intestinal tract or

a stoma is also an important consideration. Diarrhea and perianal complications may markedly diminish oral intake. Patients with stomas are more likely to have a greater percentage of their calories taken enterally. Hyperphagia develops in many patients with short-bowel syndrome to overcome inefficient absorption.[19] Many patients with short-bowel syndrome require long-term PN for survival, and this therapy has considerable expense and morbidity. Patients without malignancy have 1-, 3-, and 5-year survival rates of about 90%, 70%, and 60%, respectively.[4] One third of deaths are related to the underlying disease, 50% to other supervening disease, and 10% to 15% to PN therapy. Sepsis and liver disease related to PN are important factors in long-term survival. The incidence of sepsis varies from 0.1 to 0.3 episodes per patient year of PN. Sepsis may be associated with catheter thrombosis. The need for prolonged therapy makes vascular access a long-term problem, and catheters may eventually need to be placed in the azygos, hepatic, or inferior vena cava veins. End-stage liver disease develops in about 15% of long-term adult PN patients and is associated with a survival time of about 1 year without liver transplantation. [26] [27] Although the etiology of the liver disease is not completely understood, it appears to be a multifactorial process that is initially reversible but ultimately leads to severe steatosis, cholestasis, and cirrhosis. Liver disease occurs more frequently in children than adults. Provision of enteral nutrients may prevent this problem, but overfeeding is a predisposing factor. Control of sepsis and bacterial overgrowth is important to minimize this liver disease. Patients with abnormal liver function test results while receiving PN should undergo abdominal ultrasound for evaluation of the gallbladder and bile ducts and should have a liver biopsy performed as appropriate.
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Maximize Enteral Nutrient Absorption Because the morbidity associated with nutritional support in patients with shortbowel syndrome is related primarily to PN, maximizing enteral absorption of nutrients is important for long-term survival. Furthermore, diarrhea and stomal fluid losses can also be important clinical problems that affect the patient's quality of life. Thus, it is beneficial to ensure that the patient's intestinal remnant is functioning optimally and absorbing nutrients and fluid. The optimal diet for patients with short-bowel syndrome remains controversial.

Provision of nutrients in their simplest form to minimize digestion has been one strategic approach. Simple sugars and dipeptides and tripeptides are rapidly absorbed from the intestinal tract. However, partially hydrolyzed diets appear to be just as effective and are less expensive. Complex carbohydrates reduce the osmotic load, but concentrated sugars, such as fruit juices, should be avoided because they generate a high osmotic load. Whether the diet should have a high-fat or low-fat content is another issue. There appears to be increasing agreement that patients with colon should have a low-fat (20% to 30% of calories), high-carbohydrate (50% to 60% of calories) diet but that patients with an end-enterostomy do not require fat restriction (30% to 40% of calories). Fat absorption obviously requires more digestion unless the fat is supplied in the form of medium-chain triglycerides. The ability to absorb these nutrients improves with time, so the diet may need to be continually modified. Specific problems such as lactase deficiency are often present, and the diet should be altered appropriately. Ingestion of a glucose-electrolyte oral rehydration solution with a sodium concentration of at least 90 mmol/L will optimize water and sodium absorption in the proximal jejunum and prevent secretion into the lumen. Minimizing gastrointestinal secretions and controlling diarrhea are also important goals for maximizing absorption. Both histamine H2 receptor antagonists and proton pump inhibitors are effective in controlling gastric hypersecretion, correcting malabsorption, and improving nutritional status in patients with short-bowel syndrome. Furthermore, cimetidine may also increase intestinal adaptation. Somatostatin and its long-acting analogue octreotide have been investigated for the management of severe refractory diarrhea in short-bowel syndrome. They improve diarrhea by prolonging small intestinal transit time and reducing salt and water excretion. Part of the beneficial effect may also be related to a reduction in gastric hypersecretion. Although these therapeutic agents are beneficial in the short term, it is not clear whether they continue to be effective after a few months, and they may have some potential deleterious effects. Somatostatin may exacerbate steatorrhea because of impaired pancreatic exocrine function. Other potential adverse effects of octreotide are inhibition of intestinal adaptation and the development of cholelithiasis. Recent evidence supports the use of ox bile and cholylsarcosine, a synthetic conjugated bile acid, as replacement therapy because they improve fat absorption without exacerbating diarrhea. Another important aspect of dietary management is to provide a diet that will maximize the intestinal adaptive response. [15] [16] [24] Provision of fat and dietary fiber may be particularly important in this regard. Long-chain and short-chain fatty acids appear to have a greater trophic effect on the intestine than medium-chain fatty acids do. Although these nutrients directly stimulate intestinal adaptation, nutrients also stimulate intestinal adaptation through endocrine and paracrine effects. Pharmacologic therapy for short-bowel syndrome is a rapidly expanding area of

investigation. Recent evidence suggests that provision of the appropriate diet, nutritional supplements such as glutamine, and growth factors such as growth hormone improves intestinal absorption and perhaps modifies the adaptive response in patients with established short-bowel syndrome.[22] However, which of these components is actually responsible for improved absorption is controversial. Growth hormone and glutamine do not have a consistent beneficial effect. [29] [30] Currently, glucagon-like peptide-2 appears to have the most promise for promoting absorption and adaptation.[11] Epidermal growth factor also stimulates intestinal adaptation and may soon be studied in clinical trials.[25] An important clinical issue is whether to establish intestinal continuity in patients who have a colonic remnant. There are both advantages and disadvantages to restoring continuity ( Box 78-3 ). The colon may improve intestinal absorption by increasing the absorptive surface area, deriving energy from short-chain fatty acids, and prolonging transit time, particularly if the ileocecal valve is intact. Avoiding a stoma also improves quality of life. However, the response of the colon to luminal contents is somewhat unpredictable. Bile acids may cause a secretory diarrhea. Perianal problems can be quite disabling and decrease the patient's oral intake. Oxalate is absorbed primarily in the colon, and restoring continuity places the patient at increased risk for the formation of calcium oxalate stones. Serum and intestinal fluid markers have been investigated as a means of predicting the response of the individual patient to restoring continuity, but none is generally available and useful. Distal reinfusion of enteral contents into a mucus fistula to assess the functional outcome has some usefulness, but it is cumbersome. Not all patients who initially have a stoma created eventually have continuity restored with a satisfactory outcome.[26] This decision should be considered on an individual basis and depends on the length of the intestinal remnant, the status of the ileocecal valve and the colon, and the patient's overall condition. Generally, at least 3 ft of small intestine is required to prevent severe diarrhea and perianal complications. Restoring continuity, however, should always be given strong consideration because of possible improvement in absorption. Box 78-3 Restoration of Intestinal Continuity Advantages Absorptive capacity increased

Energy absorbed from short-chain fatty acids

Infectious complications reduced

Transit time prolonged

Stoma avoided Disadvantages Bile acid diarrhea

Dietary restrictions

Nephrolithiasis increased

Perianal complications From Thompson JS: Intestinal resection and the short bowel syndrome. In Quigley EMM, Sorrell MF (eds): Medical Management of the Gastrointestinal Surgery Patient. Baltimore, Williams & Wilkins, 1994, p 327.
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Prevent Complications Metabolic complications are common in patients with short-bowel syndrome because of their tremendous fluid and electrolyte losses and the need to replace these losses with specialized solutions. Intravascular volume has to be maintained to prevent dehydration and renal dysfunction. Hypocalcemia is a common problem related to poor absorption and binding by intraluminal fat. Maintaining adequate calcium and magnesium levels and vitamin D supplementation are important to minimize bone disease. Hyperglycemia and hypoglycemia are frequent

complications of patients receiving a large amount of their calories parenterally. Both metabolic acidosis and metabolic alkalosis can occur. A specific problem is dlactic acidosis, which results from bacterial fermentation of unabsorbed nutrients, particularly simple sugars. Lactate reduces colon pH, thereby permitting the growth of acid-resistant anaerobes capable of producing d-lactate. Impaired metabolism of d-lactic acid may also contribute to elevated serum d-lactic acid levels. This diagnosis is suggested by an unexplained metabolic acidosis and associated neurologic symptoms, such as confusion and somnolence. d-Lactic acid is not measured by standard laboratory techniques for lactic acid determination. Thus, an increased anion gap but normal lactate level in the appropriate clinical setting mandates measurement of d-lactic acid. d-Lactic acidosis is treated by minimizing overall caloric intake or by instituting a low-carbohydrate diet. Administration of intestinal antibiotics may be appropriate, but the optimal duration of such treatment is unclear, and recurrence rates are significant. Specific nutrient deficiencies need to be prevented and monitored closely, including iron and vitamin deficiencies, as well as deficiencies in micronutrients such as selenium, zinc, and copper. Because fat is poorly absorbed, fatty acid deficiency can also occur. Although medium-chain fatty acids can supplement the diet enterally, parenteral lipids are required in patients who depend primarily on that route. Serum free fatty acid levels and triene-to-tetraene ratios may need to be monitored periodically to determine the need for supplementation and response to treatment. In general, enteral intake must greatly exceed the absorptive needs to ensure that these needs are being met. Catheter-related sepsis is an important problem that often necessitates rehospitalization and replacement of catheters. Attention to technique and meticulous patient education are important to prevent this complication. Most infections are due to Staphylococcus species, but gram-negative bacteria and fungi are also associated with line sepsis. An attempt at line sterilization before removal is appropriate when infections are caused by coagulase-negative staphylococci and gram-negative bacteria. Repeated placement of catheters can lead to catheter thrombosis, which is the other common problem. In patients who require PN permanently, this may become an important factor in the patient's survival because vascular access may not be achievable indefinitely. PN-induced liver disease is another potential long-term problem. [26] [27] It can be minimized by providing as large a portion of the calories as possible enterally, avoiding overfeeding, using mixed fuels (less than 30% fat), and preventing specific nutrient deficiencies. Treating bacterial growth and preventing recurrent sepsis are also important. Ursodeoxycholic acid administration may likewise be beneficial. Bacterial overgrowth is another long-term complication associated with both intestinal disease and resection. It may result from impaired motility or stasis caused

by obstructive lesions ( Fig. 78-2 ). Achlorhydria is also a contributing factor. Bacterial deconjugation of luminal bile salts impairs bile salt reabsorption. Bacteria also metabolize intraluminal vitamin B12. Depending on the bacterial species present, secretory diarrhea may occur as well. Bacterial overgrowth requires a high degree of suspicion to make the diagnosis. This complication should be suspected when a patient's absorptive capacity and stool habits change acutely. It may result from a mechanical obstruction or a blind loop, which can be relieved by surgery. However, it is often a primary motor abnormality and requires intermittent therapy with antibiotics. Colonization of the lumen with acidophilus or other nonpathogenic organisms is another potential therapy.[27]

Figure 78-2 Contrast study of a patient with short-bowel syndrome. The shortened remnant lies primarily in the

left side of the abdomen with a large dilated segment in the pelvis. Contrast has passed into the right colon beyond this area.

Cholelithiasis occurs in 30% to 40% of patients with intestinal insufficiency. [34] [35] Factors that predispose these patients to gallstone formation include altered hepatic bile metabolism and secretion, gallbladder stasis, and malabsorption of bile acids. Depending on the dominant mechanism, either mixed pigment stones or cholesterol stones may occur. Long-term PN is an important contributing factor causing altered hepatic bile metabolism and gallbladder stasis. Patients receiving PN are susceptible to the development of cholelithiasis and hepatocellular dysfunction and thus require careful clinical evaluation. [34] [35] Biliary sludge forms within a few weeks of initiating PN if there is no enteral intake, but it rapidly disappears when enteral nutrition is resumed. Intestinal mucosal disease and resection, particularly of the ileum, cause bile acid malabsorption, which leads to lithogenic bile and the formation of cholesterol stones. The risk for cholelithiasis is significantly increased if less than 120 cm of intestine remains after resection, the terminal ileum has been resected, and PN is required. The incidence of cholelithiasis can be minimized by providing nutrients enterally whenever possible. Patients totally dependent on PN may be treated with intermittent cholecystokinin injections to prevent stasis and the formation of sludge. Administration of intravenous lipids also stimulates gallbladder emptying. Cholelithiasis may lead to complications in a higher number of patients with short-bowel syndrome than in the general population and also requires more complicated surgical treatment. Thus, several authors now recommend prophylactic cholecystectomy in these patients when laparotomy is being undertaken for other reasons.[29] Nephrolithiasis also occurs with some frequency. Calcium oxalate stones form as a result of increased oxalate absorption from the colon.[29] Oxalate is normally bound to calcium in the intestinal lumen and is not absorbed. Decreased availability of calcium secondary to reduced intake or binding by intraluminal fat leaves free oxalate in the lumen. Thus, the oxalate is absorbed in the colon and forms calcium oxalate in the urine. Nephrolithiasis is unusual in patients after intestinal resection and jejunostomy but occurred in a fourth of such patients with an intact colon within 2 years of resection. Nephrolithiasis can be prevented by maintaining a diet low in oxalate, minimizing intraluminal fat, supplementing the diet with calcium orally, and maintaining a high urinary volume. Foods with high oxalate content include chocolate, tea, cola, spinach, celery, carrots, and other fruits and vegetables. Cholestyramine, which binds oxalic acid in the colon, is another potential treatment. Gastric hypersecretion is a potential problem in patients with short-bowel syndrome. Massive intestinal resection can cause gastric hypersecretion as a result of parietal cell hyperplasia and hypergastrinemia. This phenomenon is usually transient and

lasts several months. The etiology has not been elucidated but may involve loss of an inhibitor from the resected intestine. The associated hyperacidity exacerbates malabsorption and diarrhea. Clinical development of peptic ulcer disease may also occur and is seen in about a fourth of patients undergoing massive resection.[26] Treatment of gastric acid secretion may improve absorption but also prevents peptic ulcer disease. Control of acid secretion by H2 receptor antagonists or proton pump inhibitors should be initiated in the perioperative period after resection and maintained until the increased acid production resolves. Some patients, however, continue to have symptoms of peptic ulcer disease that eventually require surgical intervention. Gastric resection therapy should be avoided when possible. A highly selective vagotomy may be the most desirable procedure if feasible.
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SURGICAL MANAGEMENT The primary goal of surgical therapy for short-bowel syndrome is to increase intestinal absorptive capacity, which can be achieved either by improving absorption by existing intestine or by increasing the area of absorption ( Box 78-4 ). Recruiting additional intestine into continuity, relieving obstruction, or slowing intestinal transit will often improve absorption. The intestinal lengthening procedure is feasible in selected patients. The most significant increase in length, however, is potentially achieved by intestinal transplantation. The choice of surgical therapy for shortbowel syndrome is influenced by intestinal remnant length and caliber and the clinical condition of the patient ( Table 78-3 ).[3] Box 78-4 Surgical Strategies for Short-Bowel Syndrome

Preserve and maximize remnant

Avoid resection

Restore continuity

Recruit additional intestine

Improve intestinal function

Relieve obstruction

Taper dilated bowel

Slow intestinal transit

Increase absorptive area

Intestinal lengthening

Intestinal transplantation

Table 78-3 -- Surgical Approach to Short-Bowel Syndrome Intestinal Remnant Clinical Condition Surgical Options Adequate length with Enteral nutrition (remnant Optimize intestinal function, normal diameter >120 cm in adults, >60 recruit additional length cm in children) Adequate length with dilated bowel Bacterial overgrowth, stasis Treat obstruction, intestinal tapering Recruit additional length, reversed intestinal segment, artificial valve, colon interposition Optimize intestinal function

Marginal length with normal Rapid transit, need for diameter (remnant 60-120 parenteral nutrition cm in adults, 30-60 cm in children) Short length with normal diameter (remnant <60 cm in adults, <30 cm in Need for parenteral nutrition

Intestinal Remnant children) Short length with dilated bowel Short length

Clinical Condition Need for parenteral nutrition

Surgical Options Intestinal lengthening

Complications of Intestinal transplantation parenteral nutrition From Thompson JS: Surgical approach to the short bowel syndrome: Procedures to slow intestinal transit. Eur J Pediatr Surg 9:263, 1999.

Preserve and Maximize the Intestinal Remnant An abdominal reoperation is required in about half the patients with short-bowel syndrome after discharge from the hospital.[26] Intestinal problems are the most common indication. An important goal with any reoperation in patients with shortbowel syndrome is to preserve the length of the intestinal remnant. Several strategies can be used when further intestinal disease requires surgery.[30] Resection can often be avoided by intestinal tapering to improve the function of dilated segments, performing stricturoplasty for benign strictures, and using serosal patching for certain strictures and chronic perforations ( Fig. 78-3 ). Resections should be limited in extent when they cannot be avoided. An end-to-end anastomosis is favored both to prevent blind loops and to maximize functional length of the intestine. Depending on the previous operations performed, patients occasionally have intestinal segments that can be recruited into continuity at the time of reoperation. This should always be given careful consideration. The length, location, and characteristics of the remnant should be carefully documented at the time of any operation.

Figure 78-3 Techniques for preserving intestinal length include tapering of dilated segments rather than resection (A), stricturoplasty for strictures (B), and serosal patches for strictures and perforation (C). (From Thompson JS: Recent advances in the surgical treatment of the short bowel syndrome. Surg Ann 22:110, 1990.)

Stricturoplasty is most often performed in the fashion of a Heineke-Mikulicz pyloroplasty. The stricture is incised longitudinally and closed transversely. The incision extends at least 1 cm proximal and distal to the stricture, but larger incisions may be required to achieve a satisfactory orifice. The enterotomy can be repaired with either a single-layer or a two-layer anastomosis. Longer strictures or multiple

closely associated strictures can be opened with a side-to-side stapled anastomosis. Blind loops should be avoided, however. Serosal patching is performed by apposition of an adjacent serosal surface, usually either small intestine or colon, to a nonhealing fistula, stricture, or other focal defect. A single-layer seromuscular-to-seromuscular anastomosis is created in either an interrupted or continuous fashion. The serosal patch becomes covered by normal mucosa from adjacent tissue. This technique is most applicable to smaller defects because contraction of the patch does occur and could lead to a stenotic segment.
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Improve Intestinal Function

Improve Motility

Patients with short-bowel syndrome have a propensity for the development of dilated intestine secondary to chronic obstruction or intestinal adaptation. Dilated intestine may lead to stasis and bacterial overgrowth, which can further aggravate the malabsorption associated with the short remnant. Mechanical obstruction at an anastomosis or from adhesions or strictures related to the underlying disease process should always be sought in these patients and corrected with the techniques mentioned previously. These dilated segments, however, are often not associated with distal obstruction. Tapering dilated segments improves motility by permitting closure of the lumen during contraction of the wall, which improves peristalsis. Simple imbrication of the redundant bowel is the preferred method, although longitudinal transection plus removal of intestine along the antimesenteric border has also been performed. A continuous nonabsorbable suture line is usually most expeditious, particularly for lengthy segments. Excisional techniques are easily performed with stapling devices but can also be performed with bowel clamps. Tapering enteroplasty improves intestinal function in patients with short-bowel syndrome.[3] Blind loops should be sought and eliminated, preferably by revision of the anastomosis rather than resection.
Prolong Intestinal Transit

Procedures designed to prolong intestinal transit time have been evaluated experimentally and performed clinically, but their efficacy remains questionable ( Fig. 78-4 ). [37] [38] Most of the reports are anecdotal. These adjunctive procedures are often performed during the adaptive phase; hence, it is difficult to determine whether the improvement in nutritional status and absorption was due to the surgical procedure or the normal adaptive process. Three procedures have been attempted in sufficient numbers to be considered, including reversed intestinal segments, colon

interposition, and artificial sphincters ( Table 78-4 ).

Figure 78-4 Techniques for slowing intestinal transit: intestinal valve (upper left), antiperistaltic segment (upper right), recirculating loop (lower left), and intestinal pacing (lower right). (From Thompson JS, Rikkers JS: Surgical alternatives for the short bowel syndrome. Am J Gastroenterol 82:97, 1987.)

Table 78-4 -- Clinical Experience with Procedures to Prolong Transit for ShortBowel Syndrome Procedure Number of Number of Number of Patients with Clinical Patients Children (%) Improvement (%) Reversed 55 6 (11) 45 (81) segment Intestinal valve 12 (6)[*] Colon interposition Pouch or loop 12 4 1 (16) 11 (92) 1 (25) 4 (67) 6 (50) 1 (25)

Procedure

Intestinal pacing From Thompson JS: Surgical approach to the short bowel syndrome: Procedures to slow intestinal transit. Eur J Pediatr Surg 9:263, 1999.
*

Number of Patients 1

Number of Children (%) 0 (0)

Number of Patients with Clinical Improvement (%) 0 (0)

Six procedures performed as a staged approach for intestinal lengthening.

Reversed Intestinal Segments

Reversing segments of intestine to slow intestinal transit is the surgical procedure that has been reported most extensively. The antiperistaltic segment functions by inducing retrograde peristalsis distally and disrupting the motility of the proximal part of the intestine. In addition, disruption of the intrinsic nerve plexus slows myoelectrical activity in the distal remnant. Reversed segments also alter the hormonal milieu after resection. Most experimental studies of antiperistaltic segments demonstrate slowed intestinal transit, improved absorption, reduced weight loss, and prolonged survival after intestinal resection, but some reports do not show a beneficial effect. [39] [40] The variable outcomes may be explained by several factors, including variation in the extent of resection, timing of the procedure, and the use of different lengths of antiperistaltic segments. Reversed segments performed simultaneously with 75% resection in canines blunted the normal adaptive response, which may be related to the altered hormonal response.[35] The ideal antiperistaltic segment slows transit without causing complete obstruction. Several technical details are important. The optimal length of the reversed segment would appear to be about 10 cm or less in adults and 3 cm in children. The reversed segment should be created as distal in the small intestinal remnant as feasible. Care must be taken to avoid complete rotation of the mesentery to prevent intestinal ischemia. Antiperistaltic segments have been reported clinically in more than 50 patients, about 90% of whom were adults.[31] In these anecdotal reports, clinical improvement with slowed intestinal transit and increased absorption has been reported in 80% of patients. Transient obstructive symptoms and anastomotic leak are potential problems. The length of the segment has varied from 5 to 15 cm in these reports. Performance of this procedure in patients with Crohn's disease does not appear to influence recurrence rates. Long-term function has been demonstrated.
Intestinal Valves

The effect of valves and sphincters on intestinal motility involves several different mechanisms. They create a partial mechanical obstruction, disrupt the normal motor pattern of the small intestine, and prevent retrograde reflux of colonic contents.[31] In experimental studies, intestinal valves and sphincters have been shown to prolong transit time, increase absorptive capacity, and extend survival, although the results have been inconsistent. Effective valves usually result in some dilation of the proximal part of the intestine and may cause, at least transiently, obstructive symptoms. Potential complications include necrosis of the valve, complete obstruction, and intussusception. Durability of the sphincter function of valves has been questioned. Several different techniques for creating intestinal valves and sphincters to replace the ileocecal valve have been reported, including external constriction of the intestine, segmental denervation, and intussusception of intestinal segments to increase intraluminal pressure, with the latter being used most frequently. Intussuscepted valves should be 2 cm in length if retrograde and 4 cm if the intussuscepted valve is prolapsed antegrade. We have generally created a retrograde sphincter similar to that used in the continent ileostomy procedure, but it is only 2 cm in length.[3] The reported clinical experience with intestinal valves and sphincters is less extensive than that with reversed segments. Nipple valves were recently used in six infants to cause dilation of the intestine so that subsequent intestinal lengthening could be performed.[33] Intussuscepted valves were reported as primary treatment in five adults and one infant with short-bowel syndrome.[31] Four patients improved markedly, one had questionable benefit, and takedown of the valve was required in the other. Ileocolic nipple valves were lost in a third of patients monitored for more than 5 years in one study, again raising the issue of durability.
Colon Interposition

Interposing a colonic segment in the small intestinal remnant in either an isoperistaltic or antiperistaltic fashion retards intestinal transit. Isoperistaltic interposition is performed proximally and functions by slowing down the rate at which nutrients are delivered to the distal portion of the small intestine.[31] The antiperistaltic colon interposition is placed distally, similar to the reversed small intestinal segment. Interposed colonic segments absorb water, electrolytes, and nutrients, in addition to their effect on intestinal transit. Although it has been suggested that interposed colon might develop structural and functional similarities to the small intestine, this has not been substantiated. In experimental studies, isoperistaltic colon interposition generally resulted in slower transit time, less weight loss, and improved survival after resection. Results with antiperistaltic colon interposition, however, have been less consistent. The length of colon interposed seems to be less critical than with reversed segments.

The use of colon interposition has been reported in 12 patients, 11 of whom underwent isoperistaltic interposition.[32] All but one of the patients were infants younger than 1 year. The interposed colon segment varied between 8 and 24 cm in length. All patients were PN dependent preoperatively. Six (50%) patients demonstrated sustained clinical improvement; the other six, including the one with the antiperistaltic colon, did not improve and subsequently died of sepsis or hepatic failure. Colonic stasis with bacterial overgrowth may have contributed. This experience suggests that isoperistaltic colon interposition may have some merit.
Other Approaches

Intestinal pouches and recirculating loops would theoretically prolong transit time by permitting prolonged exposure of luminal nutrients to the intestinal absorptive surface. In experimental studies, however, these procedures have not improved absorption or survival rates after massive resection. Four clinical reports involving recirculating loops have been disappointing as well. Intestinal pacing in a retrograde fashion has also been investigated as a means of prolonging transit time. Retrograde electrical pacing promotes peristalsis in a reverse direction but also alters proximal intestinal motility, possibly through a hormonal mechanism. Postprandial retrograde pacing in canines improved absorption and intestinal status. In the one reported attempt to achieve retrograde pacing in a patient with short-bowel syndrome, the pacemaker failed to stimulate the intestine.
Choice of Procedure

Procedures designed to slow intestinal transit should be applied cautiously in patients with nearly adequate remnant length and demonstrated rapid transit. They should be considered only after maximal adaptation has occurred. Reversed intestinal segments and artificial valves have the greatest appeal as procedures to slow intestinal transit. Antiperistaltic segments should be used in patients with longer remnants. The 10-cm segment still leaves sufficient remnant for absorption. Valves should be considered in patients with shorter remnants because less bowel is used. In one experimental study, an intestinal valve was more effective than an antiperistaltic segment in prolonging transit time after resection. The efficacy of these procedures remains questionable, however, and other approaches have been even less encouraging. Furthermore, these procedures are applicable to only a small proportion of patients with short-bowel syndrome.
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Increase Absorptive Area

Dilated intestinal segments may be amenable to an intestinal tapering and lengthening procedure. Theoretically, such a procedure has the advantage of not only improving motility and reducing stasis but also improving intestinal absorption by the increased absorptive area. The primary technique was initially described by Bianchi[34] ( Fig. 78-5 ). More recently, an alternative technique called serial transverse enteroplasty (STEP) has been reported [42] [43] ( Fig. 78-6 ). In these approaches the dilated segments are tapered, and the redundant intestine is preserved and restored into continuity for additional length.

Figure 78-5 The Bianchi procedure. Longitudinal dissection between the blood vessels on the mesenteric border (A) permits the use of staples to divide the intestine longitudinally (B and C). The two parallel segments are then anastomosed end to end (D). (From Thompson JS: Surgical rehabilitation of the intestine in short bowel syndrome. Surgery 135:465, 2004.)

Figure 78-6 The STEP procedure. Several transverse applications of a linear stapler from opposite directions on the bowel wall allow the intestine to lengthen with reduced diameter. (From Thompson JS: Surgical rehabilitation of the intestine in short bowel syndrome. Surgery 135:465, 2004.)

In experimental studies, intestinal lengthening by the Bianchi procedure prolongs transit time but does not clearly improve absorption in the short term.[38] Intestinal lengthening causes motor disruption in the proximal portion of the intestine and alters the hormonal response to resection. The jejunum may yield better results than lengthening of ileum. Improved nutrition has been demonstrated in an animal model after the STEP procedure.[36] The Bianchi procedure is performed by transecting distal to the dilated segment to

be tapered. Dissection is performed longitudinally for about 5 cm on the mesenteric edge of the bowel between the terminal branching vessels to create a space that permits longitudinal division of the bowel with a stapler. A hand-sewn anastomosis can also be used. If the diameter of the bowel permits, the staple line can be imbricated as well. This procedure is repeated until the desired length is achieved. The two parallel longitudinal segments can then be anastomosed end to end to halve the diameter and double the length of the segment. Intestinal lengthening of segments from 5 to 90 cm has been reported. Obviously, longer segments are at greater risk for complications. The STEP procedure involves serial transverse applications of a linear stapler from alternating directions to divide the bowel perpendicular to the long axis of the intestine. The length and spacing of the transverse division are determined by the diameter of the intestine. Multiple stapler applications are required. The net result is an increase in length and a reduction in diameter. This procedure is less complicated than the Bianchi procedure because it avoids the extensive mesenteric dissection and the additional anastomosis. It is feasible for very short segments and those near the ligament of Treitz. Intestinal lengthening, primarily the Bianchi procedure, has now been reported in more than 100 patients. [40] [43] [45] [46] After an initial prolonged ileus, significant improvement in absorptive capacity and nutritional status has been reported in 90% of these patients in the short term. Potential complications, however, such as necrosis of divided segments, anastomotic leak, and obstruction develop in up to 20% of patients. Gastrostomy tubes are often placed because of the prolonged dysmotility that occurs. Although short-term results have been encouraging, emerging long-term results suggest that about half the patients undergoing this procedure have a sustained benefit for up to 10 years.[39] The initial experience with the STEP procedure has been favorable. [43] [46] Initial short-term results in 10 patients have demonstrated the feasibility and safety of the technique in the clinical setting. The outcome of these procedures is heavily influenced by patient selection in terms of age, remnant length, hepatic function, and requirement for PN. Thus, these procedures should be applied cautiously. One of the limitations of lengthening procedures is that they can be applied only to a fairly select group of patients. Obviously, the procedures should be considered only if bacterial overgrowth or other signs of malabsorption are identified that appear to be related to the dilated segment. The intestinal diameter should be at least 4 cm to provide an adequate lumen size after tapering. Sequential operations, first using a procedure such as an artificial valve to produce intestinal dilation and then performing the lengthening at a later time, have been used to expand the applicability of this technique. The vascular anatomy must be favorable for the Bianchi procedure. Effort is also being directed at recruiting additional vascular supply to permit further lengthening. The STEP procedure should markedly increase

the applicability of intestinal lengthening.

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INTESTINAL TRANSPLANTATION The development of intestinal transplantation must be placed in the context of patients and physicians faced with catastrophic clinical circumstances in the absence of reliable alternatives. The mortality rate of patients requiring PN for benign disease has been estimated at 5% to 25% per year, or about 15% at 3 years.[41] For infants, the risk for PN-induced liver disease is especially great. It is estimated that half the deaths in children receiving PN are due to liver failure.[42] Currently, intestinal transplantation is applied as rescue therapy for patients with lifethreatening complications of intestinal failure. In 2001 the U.S. federal government through the Centers for Medicare and Medicaid Services (CMS) approved payment for intestinal transplantation at select centers. Indications Indications for intestinal transplantation are restricted to life-threatening complications of intestinal failure, with the most common complication being the development of liver disease. It is important to determine whether the liver disease is reversible. If the liver disease is found to be irreversible, based on either biopsy findings or clinical features such as massive splenomegaly, ascites, encephalopathy, or gastrointestinal bleeding, the patient should undergo combined liversmall bowel transplantation. Greater emphasis has recently been placed on considering isolated small bowel transplantation for patients with potentially reversible PN-induced liver disease. Regardless of the type of transplant required, early referral and listing are important to ensure the patient the greatest opportunity to obtain a transplant. The other common indications for intestinal transplantation are an irreversible permanent PN requirement along with episodes of sepsis or loss of venous access. Septic episodes that would prompt consideration for intestinal transplantation are typically catheter related. Patients who have undergone multiple hospitalizations related to catheter sepsis, often requiring intensive unit care with the need for vasopressors, fall in this category. Other indications for intestinal transplantation are multiantibiotic-resistant bacteremia or metastatic infection in sites such as the tricuspid valve or brain. Loss of venous access typically implies an inability to place a catheter in the subclavian or intrajugular veins and the use of extemporaneous sites such as the hepatic veins or the inferior vena cava. A transplant evaluation is strongly recommended in patients with known poor survival on PN, such as those with microvillus inclusion disease, intestinal aganglionosis, or desmoid tumors that

have previously been eviscerated. Today, with improved outcomes and large numbers of patients dying on the waiting list, greater responsibility is being placed on the treating physician to make earlier referral to a transplant center. The transplantation evaluation process for patients with intestinal failure requires a multidisciplinary group of health care professionals, including surgeons, gastroenterologists, dietitians, social workers, and nurse specialists. The evaluation process also incorporates an assessment of the feeding program that the patient is currently receiving. Contrast studies of the small and large bowel are frequently performed. A liver biopsy is performed in patients with evidence of liver dysfunction to help select the appropriate type of transplantation procedure. During the evaluation process, other problems are addressed, including worsening liver failure, sepsis, difficult vascular access, and septic episodes. After being identified as a potential candidate, the patient is placed on an active transplantation waiting list.
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Operative Procedure The donor operation begins similarly regardless of the organs being removed. Potential organ donors are matched with recipients according to blood type, size, and medical necessity. Most patients with short-bowel syndrome have a loss of peritoneal domain, thus requiring the donor to be about 50% smaller than the potential recipient. Recent success with reduced-size intestinal transplants has challenged these donor size guidelines. Donors should be ABO blood group identical, although exceptions to this rule have been reported.[43] Human leukocyte antigen matching and a negative T-cell crossmatch may be beneficial, particularly for recipients of isolated small bowel allograft. Donor logistics often prevent this type of testing from being performed prospectively. Removal of the intestine for isolated small bowel transplantation involves removal of the liver and small bowel together, after which they are separated on the back table.[44] The donor operation for a future liversmall bowel transplantation is relatively similar, but no hilar dissection is performed. The colon and stomach are mobilized out of the field, and the liversmall bowel composite is removed en bloc, with care taken to remove as much aorta proximal to the celiac axis as possible.[44] Back-table preparation for an isolated small bowel graft involves removing the duodenumhead of the pancreas from the portal vein and superior mesenteric artery.

For liver and small bowel grafting procedures, the preparation involves removal of the distal pancreas and spleen. The numerous intercostal arteries are ligated, and the distal end of the aorta is oversewn. Critical for a liversmall bowel graft is that no hepatic hilar dissection take place so that the hepatobiliary-duodenal complex remains undisturbed.[44] The recipient operation typically makes use of previous incisions. For isolated small bowel transplantation, the infrarenal aorta is isolated, and the arterial anastomosis for the small bowel graft is typically performed between the donor superior mesenteric artery and the infrarenal aorta.[44] Venous drainage can be systemic or portal, but systemic drainage is preferred whenever liver disease is present. An enterostomy is created to decompress the small bowel and to facilitate biopsy. A loop ileostomy is the most common type of stoma created for both liversmall bowel and isolated small bowel transplantation. The liversmall bowel transplantation surgical technique leaves the donor hepatic hilar structures undisturbed and the hepatic-duodenal-biliary system intact ( Fig. 787 ). The advantage of this approach is that it limits the necessary back-table dissection, prevents any torsion around the portal vein after implantation, and virtually eliminates any possible biliary tract complications after transplantation. The liversmall bowel composite allograft is implanted orthotopically. Arterial inflow is through the donor aortic conduit, and a native portacaval shunt is created to decompress the recipient's viscera. Under certain circumstances, particularly when the native foregut is diseased or dysfunctional, complete abdominal evisceration is performed before implantation of the donor organs. With evisceration of the native foregut the operation is often referred to as a multivisceral transplant. The proximal gastrointestinal anastomosis is frequently performed between the remnant proximal part of the stomach and the donor jejunum.

Figure 78-7 Diagram of a liversmall bowel allograft. This diagram demonstrates the intact hepatobiliary duodenal complex. AO, aorta; BD, bile duct; IVC, inferior vena cava; PV, portal vein; SV, splenic vein. (From Deroover A, Langnas A: Surgical methods of small bowel transplantation and liver-bowel transplantation. Curr Opin Organ Transplant 4:335, 1999.)

After transplantation, the cornerstone of immunosuppressive management is the administration of tacrolimus and steroids. The majority of intestinal transplant programs now make use of some form of induction therapy, either with biologic agents such as Thymoglobulin or with interleukin-2 receptor blocking agents. [51] [52] [53]

Reports have been made of other drugs being administered, including sirolimus, alemtuzumab (Campath 1H), and mycophenolate.[48] Numerous other agents are given as prophylaxis for infection, in particular, broad-spectrum antibiotics, antifungal agents, and antiviral drugs.
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Outcome Worldwide, based on published data from the 2003 Intestinal Transplant Registry (ITR) contributed by 61 programs in 10 countries, 989 transplants have been performed in 923 patients.[49] According to ITR data, isolated intestinal transplantation was performed 433 times and an intestinal allograft transplanted with a liver 556 times. Thirty-two grafts were obtained from living donors, including an identical twin and a triplet. In 2001 the CMS approved intestinal transplantation as therapy for patients with life-threatening complications of intestinal failure. A total of 484 of the 923 patients reported in the ITR who underwent any type of intestinal transplantation are alive. The longest survivor has been on an enteral diet for over 14 years. Patient and graft survival has also steadily increased over time ( Fig. 78-8 ). The ITR results also demonstrated factors important in improving patient and graft survival.[49] In a log-logistic model, factors associated with improved patient and graft survival included transplantation of a patient coming from home and the use of induction therapy ( Fig. 78-9 ). As expected, programs that have performed at least 10 transplantations have better patient survival rates than do programs that have performed less than 10 transplantations. Patients who are called in from home have much higher survival rates, which should encourage physicians to refer patients earlier. Clinical experience remains confined to a small number of programs, with 83% of the cases performed at 10 institutions. The most common causes of death after intestinal transplantation included sepsis, multiorgan system failure, graft thrombosis, rejection, and post-transplantation lymphoma.

Figure 78-8 Graft survival rates after intestinal transplantation have improved over time. (From Grant D, Abu-Elmagd K, Reyes J, et al: 2003 Report of the intestine transplant registry: A new era has dawned. Ann Surg 241:607, 2005.)

Figure 78-9 Graft-survival rates plotted according to the type of immunosuppressive protocol with particular reference to induction therapy. ATG, antithymocyte globulin; IL2, interleukin-2. (From Grant D, Abu-Elmagd K, Reyes J, et al: 2003 Report of the intestine transplant registry: A new era has dawned. Ann Surgery 241:607, 2005.)

Rejection episodes continue to be a major problem in small bowel transplantation, even with tacrolimus-based immunosuppression combined with some form of induction therapy. The incidence of transplant rejection remains variable. According to the ITR, graft rejection rates were 57% for intestine grafts, 39% for combined

intestine and liver grafts, and 48% for multivisceral grafts.[49] Contemporary singlecenter reports demonstrate even further reductions in rejection rates. At the University of Pittsburgh, Thymoglobulin induction combined with tacrolimus has resulted in a 44% rejection rate in the first month, whereas patients receiving interleukin-2 receptor blocking agents combined with tacrolimus at the University of Nebraska had a rate of about 5%. [51] [53] These rates of rejection are now similar to those seen in recipients of heart, liver, and kidney transplants. The diagnosis of rejection is based on histologic findings. Mild rejection is diagnosed by the findings of mild cryptitis, increased inflammatory infiltrated lamina propria, and apoptosis of crypt cells ( Fig. 78-10 ). A diagnosis of moderate rejection is made when villus blunting develops in addition to the findings associated with mild rejection. Serious rejection is diagnosed when there is not only severe blunting but also complete loss of mucosal lining and severe crypt cell destruction. Biopsy of the small bowel allograft is performed either on a protocol basis or when changes in clinical findings occur. Clinical findings that could be associated with rejection include diarrhea, increased stoma output, bloody diarrhea, abdominal pain, or an intolerance to feedings. Unfortunately, a noninvasive marker for the diagnosis of rejection episodes is not available.

Figure 78-10 Photomicrograph of intestinal rejection in a transplanted intestinal graft. Apoptosis is a prominent feature. A single crypt is seen in the center with multiple apoptotic cells.

Infections after organ transplantation are generally frequent as a result of two important factors in these patients: increased levels of immunosuppression and an allograft colonized with enteric organisms. Common sites of bacterial infection include the central line, surgical wound, and intraperitoneum. Bacteremia or fungemia may also develop as a consequence of allograft rejection, infectious enteritis, or preservation injury. With any of these bowel injuries, there can be loss of mucosa with eventual translocation of enteric organisms. The primary viruses that cause infections after intestinal transplantation include herpesviruses such as cytomegalovirus (CMV) and Epstein-Barr virus (EBV). A variety of strategies have been proposed to either prevent or diagnose both CMV and EBV infection. Molecular monitoring for both EBV and CMV DNA in blood is now routine in most transplant programs. Prophylactic measures include infusions of pooled immunoglobulins and antiviral drugs such as ganciclovir. The intestinal graft is the most common site of CMV infection. Treatment is based on the use of antiviral drugs such as ganciclovir or foscarnet. Post-transplant lymphoproliferative disease (PTLD) is an EBV-associated process that occurs after all solid organ transplantations. Intestinal transplant recipients appear to be at higher risk for PTLD than do recipients of other organ transplants, probably in part because of the high level of immunosuppression needed to control rejection, as well as the relatively young age of recipients. The reported incidence of PTLD after intestinal transplantation is between 7% and 29%. [51] [52] [53] [55] Treatment of PTLD often involves lowering of immunosuppression and the use of antiviral agents as a first line of therapy. Newer treatments being proposed include the use of a lowdose cyclophosphamide (Cytoxan) regimen to control PTLD without the side effects of more traditional chemotherapeutic regimens.[50] Rituximab, a monoclonal antibody directed at CD20-positive B cells, is now being used to treat PTLD. Recently, the use of blood tests to measure qualitative and quantitative amounts of EBV DNA in the peripheral blood of transplant recipients has been advocated.[51] Measurements of EBV DNA are used in the hope of identifying PTLD before it becomes clinically evident so that less toxic preemptive therapy can be administered. Graft-versus-host disease has been a relatively uncommon clinical event after intestinal transplantation. Its incidence was 7% in one series.[45] The diagnosis is based on traditional histopathologic criteria of skin, native gastrointestinal tract, or mucosa. Treatment of graft-versus-host disease is based primarily on increases in immunosuppression. The functional status of the small bowel allograft is the foremost factor in determining the long-term quality of life for recipients. According to the ITR, enteral autonomy develops in 81% of recipients. Data also demonstrate that

intestinal transplantation becomes cost-effective in comparison to PN at 2 years.[52] The increasing experience and improved outcome of intestinal transplantation support the clinical use of this treatment modality. Although the potential morbidity of transplantation is greater than that of nontransplant surgical procedures, so too is the benefit. Intestinal transplantation is potentially applicable to a greater number of patients with short-bowel syndrome than nontransplant procedures are. All these procedures should be performed only in carefully selected patients ( Fig. 78-11 ).

Figure 78-11 Surgical management of short-bowel syndrome. TPN, total parenteral nutrition. (From Thompson JS, Langnas AN, Pinch LW, et al: Surgical approach to the short bowel syndrome: Experience in a population of 160 patients. Ann Surg 22:600, 1995.)

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SUGGESTED READINGS

DiBaise JK, Young RJ, Vanderhoof JA: Intestinal rehabilitation and the short bowel syndrome: Part I. Am J Gastroenterol 2004; 99:1386.

DiBaise JK, Young RJ, Vanderhoof JA: Intestinal rehabilitation and the short bowel syndrome: Part 2. Am J Gastroenterol 2004; 99:1823. Grant P, Abu-Elmagd K, Reyes J, et al: 2003 Report of the intestine transplant registry: A new era has dawned. Ann Surg 2005; 241:607. Messing B, Crenn P, Beau P, et al: Long term survival and parenteral nutrition dependence in adult patients with the short bowel syndrome. Gastroenterology 1999; 117:1043. Sudan D, DiBaise J, Torres C, et al: A multidisciplinary approach to the treatment of intestinal failure. J Gastrointest Surg 2005; 9:165. Thompson JS, Langnas AN: Surgical approaches to improving intestinal function in short bowel syndrome. Arch Surg 1999; 134:706.
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Gastroenterology 1993; 105:791. 13. Schmidt T, Pfeiffer A, Hackelsberger N, et al: Effect of intestinal resection on human small bowel motility. Gut 1996; 38:859. 14. Cosnes J, Carbonnel F, Beaugerie L, et al: Functional adaptation after extensive small bowel resection in humans. Eur J Gastroenterol Hepatol 1994; 6:197. 15. AGA Technical Review on short bowel syndrome and intestinal transplantation. Gastroenterology 2003; 124:1111. 16. Ehrenfried JA, Townsend CM, Thompson JC, Evers BM: Increases in nup 475 and cjun are early molecular events that precede the adaptive hyperplastic response after small bowel resection. Ann Surg 1995; 225:51. 17. Rubin DC: Enterocyte gene expression in intestinal adaptation: Evidence for a specific cellular response. Am J Physiol 1996; 270:G143. 18. DiBaise JK, Young RJ, Vanderhoof JA: Intestinal rehabilitation and the short bowel syndrome: Part 2. Am J Gastroenterol 2004; 99:1823. 19. Cosnes J, Lamy P, Beaugerie L, et al: Adaptive hyperphagia in patients with post surgical malabsorption. Gastroenterology 1990; 99:1814. 20. Chan S, McCowen KC, Bistrian BR, et al: Incidence, prognosis and etiology of end stage liver disease in patients receiving home total parenteral nutrition. Surgery 1999; 126:28. 21. Cavicchi M, Beau P, Crenn P, et al: Prevalence of liver disease and contributing factors in patients receiving home parenteral nutrition for permanent intestinal failure. Ann Intern Med 2000; 132:525. 22. Wilmore T, Lacey JM, Soultanakis RP, et al: Factors predicting a successful outcome after pharmacologic bowel compensation. Ann Surg 1997; 226:228. 23. Skudlarek J, Jeppesen PB, Mortensen PB: Effect of high dose growth hormone with glutamine and no change in diet or intestinal absorption in short bowel patients: A randomized, double blind, crossover, placebo controlled trial. Gut 2000; 47:199. 24. Scolapio JS, Camilleri M, Fleming CR, et al: Effect of growth hormone, glutamine and diet on adaptation in short bowel syndrome: A randomized, controlled study. Gastroenterology 1997; 113:1074. 25. Thompson JS: EGF and the short bowel syndrome. JPEN J Parenter Enteral Nutr 1999; 23:S113. 26. Thompson JS, Langnas AN: Surgical approaches to improving intestinal function in short bowel syndrome. Arch Surg 1999; 134:706. 27. Vanderhoof JA, Young RJ, Murray N, et al: Treatment strategies for small bowel bacterial overgrowth in short bowel syndrome. J Pediatr Gastroenterol Nutr 1998; 27:155. 28. Thompson JS: The role of prophylactic cholecystectomy in the short bowel syndrome. Arch Surg 1996; 131:556. 29. Nightingale JMD, Lennard-Jones JE, Gerner DJ, et al: Colonic preservation reduces need of parenteral therapy, increases incidence of renal stones, but does not change high prevalence of gallstones in patients with a short bowel. Gut 1992; 33:1493. 30. Thompson JS: Strategies for preserving intestinal length in the short bowel syndrome. Dis Colon Rectum 1987; 30:208. 31. Thompson JS: Surgical approach to the short bowel syndrome: Procedures to slow intestinal transit. Eur J Pediatr Surg 1999; 9:263.

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