Search

Purpose of Review: Recent studies have evaluated intestinal physiology following bowel resection; understanding changes in small bowel physiology after intestinal transplantation has received less attention. In this review, we will examine recent studies focused on changes in intestinal physiology following resection and intestinal transplantation., Recent Findings: Absorption, immunity, and motility are fundamental components of small bowel physiology. Absorption after resection or transplantation is mediated by adaptation and enterocyte function. After resection, adaptation results in increased villus height and crypt depth. Hepatocyte growth factor and epidermal growth factors cause enterocyte hypertrophy and hyperplasia, allowing greater peptide uptake. Little is known about intestinal adaptation after transplant, but enteral autonomy is attainable. Immunity in small bowel after transplantation relies on a balance of innate and adaptive immune responses in the presence of the luminal microbiota. Intraepithelial lymphocytes are decreased following small bowel resection. After small bowel transplant, the number and the ratio of intraepithelial lymphocytes to enterocytes, as well as changes in the microbiota, can be used to identify rejection. After intestinal transplant, immune-mediated dysmotility is common. Perioperative infliximab in addition to tacrolimus may decrease the inflammation that contributes to dysmotility., Summary: As intestinal transplantation becomes more successful, understanding how absorption, immunity, and motility changes will allow for optimization of bowel function.

Intestinal transplantation (IT) can involve small bowel transplantation alone, or be associated with liver or multivisceral transplantation. Although IT is the radical treatment for intestinal failure, home parenteral nutrition (PN) remains the treatment of choice for this disease. Indications for IT are still debated. A recent study showed that early referral for IT is recommended for patients with life-threatening combined liver and intestinal failure or for patients with invasive intra-abdominal desmoid tumors. In the same study, no survival benefit was shown for patients undergoing IT for ultra-short bowel or major complications related to the PN catheter; indications still need to be fully assessed. While short-term outcomes for IT have improved dramatically (one-year survival for small bowel-alone IT is now 80% versus 0-28% in the 1980s), long-term outcomes have not improved much since the introduction of Tacrolimus in the 1990s: five-year survival still does not exceed 60%. Some prospective developments could improve these results: the use of multivisceral grafts, the use of Sirolimus and Thymoglobulins in the immunosuppressive treatment, or the use of new biochemical markers for early diagnosis of graft rejection., (Copyright © 2012. Published by Elsevier Masson SAS.)

Background: Infants with short bowel syndrome (SBS) frequently have proximal enterostomies with the colon excluded from intestinal continuity. Restoring intestinal continuity may create difficulty with perineal soiling. We propose creation of a distal sigmoid colostomy as an option for select infants with SBS., Methods: This study involves the descriptive case series of all children with SBS who received a distal sigmoid colostomy., Results: Eight infants (mean age, 70 days) received creation of a distal colostomy. In 1, placement of the colostomy was done at the initial surgery for SBS, 2 at the time of intestinal lengthening, and 5 during another procedure. Median length of small bowel was 58 cm. At 60 days postprocedure, median gain in enteral tolerance was 21% (P = .09), with median stoma output of 125% enteral intake. There were no complications directly attributable to the distal colostomy., Conclusion: Establishment of a distal sigmoid colostomy is an excellent management strategy for select children with SBS. It provides the benefits of restoring bowel continuity such as increased fluid and electrolyte absorption, improved energy absorption from fermentation of carbohydrate to short-chain fatty acids, and the theoretical enhancement of glucagonlike peptide 2 secretion that may optimize intestinal adaptation-all while protecting the perineal skin and simplifying quantification and collection of stool.

Intestinal failure (IF) can be defined as the reduction of functional gut mass below the minimal amount necessary for digestion and absorption adequate to satisfy the nutrient and fluid requirements for maintenance in adults or growth in children. In developed countries, IF mainly includes individuals with the congenital or early onset of conditions requiring protracted or indefinite parenteral nutrition (PN). Short bowel syndrome was the first commonly recognized cause of protracted IF. The normal physiologic process of intestinal adaptation after extensive resection usually allows for recovery of sufficient intestinal function within weeks to months. During this time, patients can be sustained on parenteral nutrition. Only a few children have permanent intestinal insufficiency and life-long dependency on PN. Non-transplant surgery including small bowel tapering and lengthening may allow weaning from PN in some cases. Hormonal therapy with recombinant human growth hormone has produced poor results while therapy with glucagon-like peptide-2 holds promise. Congenital diseases of enterocyte development such as microvillus inclusion disease or intestinal epithelial dysplasia cause permanent IF for which no curative medical treatment is currently available. Severe and extensive motility disorders such as total or subtotal intestinal aganglionosis (long segment Hirschsprung disease) or chronic intestinal pseudo-obstruction syndrome may also cause permanent IF. PN and home-PN remain are the mainstays of therapy regardless of the cause of IF. Some patients develop complications while receiving long-term PN for IF especially catheter related complications (thrombosis, sepsis) and liver disease. These patients may be candidates for intestinal transplantation. This review discusses the causes of irreversible IF and emphasizes the specific medico-surgical strategies for prevention and treatment of these conditions at several stages of IF.

In this review the main clinico-laboratory and morphological changes of a liver in the patients with the syndrome of suction disorder are shown on an example of the patients with celiac disease and the short bowel syndrome (SBS). The data on recognition of symptoms of liver affections in this groups of the patients are presented. The expediency of correction of the liver pathological changes in the patients with celiac and SBS is discussed.

In contrast to other solid organ transplantations slow progress has been seen in introducing intestinal transplantation (IT) from an experimental level to clinical practice. In nine years less than 200 transplantations have been performed worldwide with a three-years, survival of approximately 40%. The main problem of IT is immunological. Large amounts of lymphatic tissues transplanted along with the intestinal graft increase the risk of acute rejection and necessitate high doses of immunosuppressive regimens liable of inducing serious side-effects. The immunocompromized recipient is vulnerable to various infections, particularly cytomegalovirus (CMV) enteritis of the graft. However, improved results are expected after introducing modern potent immunosuppressive drugs such as combination of tacrolimus and mycophenolic acid. Emphasized antiviral prophylaxis and treatment, improved preservation and prevention of ischaemia reperfusion injury are other means presently available to obtain better results after intestinal transplantation in the near future. Intestinal transplantation is becoming the treatment of choice in intestinal failure when total parenteral nutrition (TPN) fails for one reason or an other.

The behavior of jejunal to colonic mucosal autografts was studied in an experimental animal model of short bowel syndrome (SBS). Histological appearances, enterocyte enzyme activities, and in vitro glucose transport were studied at the donor and recipient graft sites in control, short-bowel syndrome, and gastrocolic fistula 5-week-old Sprague-Dawley rats. Small intestinal function was maintained in the jejunocolonic graft after 80% small bowel resection; animals in which small bowel was not resected showed loss of graft function and enzyme activity. This effect is dependent on the presence of jejunal chyme: after gastrocolic fistulae, the jejunum to colon grafts lost jejunal functional activities. Total parenteral nutrition did not alter graft behavior but improved the postoperative mortality of the procedures. The results provide additional information on intestinal adaptation in SBS.

A patient with the short-gut syndrome and antithrombin III deficiency underwent small bowel and liver grafting a year ago. Transient, mild graft-versus-host disease and intestinal rejection occurred within 2 months of grafting and were easily managed. Parenteral nutrition was discontinued 8 weeks after surgery. The patient has maintained normal nutritional indices while on an unrestricted oral diet. Small-bowel/liver grafting is feasible for patients with the short-gut syndrome and associated liver disorders. Further experience is needed to determine the specific risks, benefits, and general applicability of this procedure.

We report a case of short bowel syndrome (60 cm of jejunum anastomosed to the left colon) with reversal of the distal 15 cm of jejunum in a 21-year-old man. The nutritional absorptive capacity and digestive motility was studied for 18 months postoperatively. His absorptive capacity reached subnormal values allowing him oral nutritive autonomy and normal social life. The results of the manometric study suggested that the reversed segment delayed intestinal transit time. The prolonged contact of the chyme with the intestinal absorptive mucosa possibly increased its absorptive capacity. Our data and the literature reports suggest that reversal of a bowel loop could help wean patients from their dependence on parenteral nutrition.

An operation according to Bianchi in a 2-year-old girl is described and indications as well as technical procedure are discussed. The girl was born with a gastroschisis. There was a jejunal perforation 10 cm below the ligament of Treitz caused by a volvulus. Only 20 cm of the jejunum remained. Moreover, only the left part of the colon was present. Total parenteral nutrition for 2 years was necessary. The principle of the operation is based on a longitudinal division of the remaining bowel and a creation of two separate bowel tubes out of the divided bowel halves, thus effecting an isoperistaltic serial connection by means of two anastomoses. This is technically possible since each half of the bowel wall has its own blood supply. The vessels originating from the mesenterium branch off before they reach the bowel wall so that the mesenteric dissection line can be anastomosed longitudinally with the antimesenteric border. This results in doubling of the bowel length, narrowing of the preoperatively dilated bowel diameter, closer contact of bowel contents with the mucosa, prolonged transit time and a Bacteroides colonization which is reduced by more effective peristalsis. Indications, time of operation and our own experiences are discussed and three cases are described. All children are alive and show marked improvement in nutrition.

Extensive small bowel resection may become necessary for several reasons in children and adults. The only causal therapy of short bowel syndrome is small bowel transplantation. So far severe immunological problems have caused deleterious results despite technically successful transplantation. A clinical case of small bowel transplantation in a child is reported. The 3-year-old boy had been operated on for volvulus which had led to nearly total gangrene of the whole small bowel. Finally, only 4 cm of jejunum could be saved. Total parenteral nutrition (TPN) therefore became necessary. Small bowel transplantation was carried out with the mother as donor; transplantation technique is described in detail. Postoperative immunosuppression was performed by administration of cyclosporin A and prednisolone. Because of graft rejection, the graft had to be removed on the 12th postoperative day. At present, the child is well and on TPN again. This case shows that small bowel transplantation by living related organ donation is technically possible without impairment of the donor's quality of life. Further experimental and clinical work should be encouraged.

Massive (85%) resection of the small bowel was performed in 24 dogs to study a new surgical technique. In 12 dogs, two valves were created in the remaining small bowel at the same time, one proximal and the other distal to the anastomosis; the other 12 dogs constituted the control group. Biochemical tests, clinical evolution, and the weight curves show satisfactory results, with high survival, high lipid absorption, and low impairment of the overall condition. The valves can be created at the same time as the resection without any increase in the surgical risk. This new technique shows some possible advantages, and should be used in combination with total parenteral nutrition during the early stages and with basic diets thereafter. Ultrastructural alterations in the liver and pancreas have been studied in both groups.

In 10 mongrel dogs, massive resection of small intestine was performed by removing 90% of its length. Following a period of approximately 2 months, the animals presented excessive steatorrhea and severe cachexia. Construction of a valve using a local transposition flap within the intestinal lumen near the colon resulted in a detainment of weight loss and steatorrhea. The animals were kept under observation over a period of 4 months.

A small bowel transplant was performed on a five-year-old boy with a short bowel syndrome. The donor was the child's mother. Despite immunosuppressive therapy, the transplant was acutely rejected and had to be explanted on the twelfth day. Morphologic and immunohistochemical investigations on subsequent biopsies taken from the small bowel transplant were carried out. Besides typical changes in epithelial cells and the presence of T-cell infiltrates and Ig-deposits in vessels, many macrophages were seen. The submucosa in particular was invaded before rejection by numerous macrophages with positive results of antimonocyte/macrophage antibodies Ki-M6 and Ki-M7. The number of the macrophage antibodies Ki-M6 and Ki-M7. The number of the monocyte/macrophage cells and the immunohistochemical characteristics of the same may be important parameters for monitoring small bowel transplantations.

The purpose of this study was to determine whether small intestinal transplantation could be considered as an alternative in the treatment of patients suffering from the short-bowel syndrome. The site of absorption of oral cyclosporine A was determined as were the changes that follow small intestinal transplantation. The interactions between the lipophilic cyclosporine A molecule and fat emulsion solutions used for total parenteral nutrition were investigated. Finally, a technique for harvesting the entire small bowel in man was developed. The absorption of oral cyclosporine A in normal dogs, and in bowel-resected, autotransplanted, and allotransplanted dogs was determined. Cyclosporine A levels were monitored in all animals. This demonstrated that cyclosporine A is absorbed through the small bowel and carried through the lymphatics; that absorption is decreased to 40 percent of normal after autotransplantation or allotransplantation without rejection. Rejection further hampers cyclosporine A absorption. Administration of olive oil alone enhances absorption of cyclosporine A. We also administered cyclosporine A IV to five dogs, with and without a concomitant infusion of fat emulsion solution (Intralipid). No changes in plasma cyclosporine A levels, in the clearance of cyclosporine A, or in the in vivo distribution of cyclosporine A were noted. Finally, dissections in six cadavers and in four brain-dead organ donors were performed, and a reproducible technique for harvesting the small bowel in man was established. In selected patients with the short-bowel syndrome, small intestinal transplant may be considered as an alternative therapy to home total parenteral nutrition.

To determine the frequency of gastric acid hypersecretion in infants with chronic malabsorption due to short bowel syndrome, acid secretory function was determined in 23 infants with malabsorption 2-22 months following small bowel resection and in a control group of 14 chronically ill, age- and weight-matched infants who did not undergo bowel resection. The prevalence of basal acid hypersecretion (defined as acid output 2 SD above the mean for the control group) was 17% (4 of 23). Basal gastric acid hypersecretion was associated with two factors: massive small bowel resection and initiation of enteral feeding. Basal acid hypersecretion was present on the initial study in 3 of 7 infants with less than one-third of the small bowel remaining, but in only 1 of 16 with more than one-third intact (p less than 0.05). Hypergastrinemia was present in 3 of 6 infants following massive bowel resection, but in only 1 of 15 with more than one-third intact (p less than 0.05), but hypergastrinemia was not consistently associated with hypersecretion. In each of six previously unfed infants, a trial of enteral feeding resulted in increased basal and maximal acid output. Three infants developed basal acid hypersecretion during initiation of enteral feeding. There was no evidence of pentagastrin-stimulated maximal acid hypersecretion in any of the infants.

During the past nine years period, we managed 7 cases with short bowel syndrome resulting from massive intestinal resection in the neonatal period. Six children, aged 2 to 8 years are alive, and one died from cardiomyopathy caused by selenium deficiency at the age of one year. The carbohydrate-free milk was used for initial enteral nutrition, and the result was encouraging in 4 cases. In 3 cases in which residual small bowel varied between 6.5 to 18 cm, the enteral feeding was not incapable. These cases underwent adjunctive surgical therapy. Tailoring jejunoplasty was performed in the first case, and bowel lengthening in the second case. In both cases enteral nutrition was advanced postoperatively. The third patient who had rapid intestinal transit time underwent isoperistaltic colon interposition. Intestinal transit time was increased and enteral nutrition was advanced gradually.

Six infants with short-gut syndrome refractory to medical management underwent isoperistaltic colon interposition (length 11.7 +/- 3.1 cm.). The abdominal catastrophes that required extensive intestinal resection were: volvulus (3), necrotizing enterocolitis (2), and gastroschisis with intestinal atresia (1). No infant had another major congenital anomaly. The average trial of attempted medical therapy prior to colon interposition was 5.5 +/- 3.6 months. There was no perioperative mortality or morbidity associated with the colon interposition. Following the colon interposition, three infants were weaned from total parenteral nutrition (TPN) in 3 +/- 1 months and all survived. In contrast, three infants could not be withdrawn from TPN and they died secondary to complications of TPN (2 from sepsis and 1 from hepatic failure). Long-term survival was associated with a greater length of small bowel remaining after the initial resection (51 +/- 12 cm v 35 +/- 24 cm), colon interposition at a younger age (3 +/- 1 months v 8 +/- 3.5 months), and a shorter duration of medical management prior to colon interposition (2.8 +/- 0.8 months v 6.7 +/- 5.0 months). All survivors are now tolerating a regular diet and having one to four formed stools per day. Normal somatic growth and developmental milestones are being achieved. The follow-up period is from 24 to 84 months. Our experience with the colon interposition in the patient with short gut syndrome has led us to conclude that when a reasonable trial of medical management has failed, a colon interposition is a safe and effective adjuvant to treatment.

A full term girl survived nonresected, subtotal small-bowel gangrene. The length of the small-bowel remnant (only jejunum) measured 11 cm. Anastomosis was performed with insertion of a 3-cm-long antiperistaltic segment. The ileocecal valve remained intact. The jejunal remnant showed an increase in length of at least 1 m. The resorptive functions of the bowel residue recovered incompletely, particularly the ileal functions. Disturbances of calcium and phosphorus metabolism are now in the foreground; nevertheless, the child now manages to live without parenteral feeding.

This article defines short bowel syndrome and reviews the pathophysiology, medical management, and surgical manipulations proposed to improve intestinal absorption. Emphasis is also placed on possible future methods of management.

The effect of diet on jejunostomy output of fluid, fat, sodium, potassium, calcium, magnesium, zinc, and copper was studied in five metabolically stable, home parenteral nutrition patients. Three isocaloric diets were compared; one low in fat (30% kcal) but high in complex carbohydrate (55% kcal), and two high in fat (60% kcal) but low in carbohydrate (25% kcal). The polyunsaturated/saturated fatty acid ratios of the two high fat diets were 1:4 and 1:1. Although increasing the percentage of fat in the diet increased the amount of steatorrhea, altering the polyunsaturated/saturated fatty acid ratio had no clearly beneficial effect on the amount of fat absorbed. Neither the amount of fat, nor the type of fat, had any consistent influence on jejunostomy volume. The sodium and potassium concentration of the jejunostomy fluid stayed remarkably constant and hence net monovalent cation losses reflected jejunostomy volume rather than the fatcarbohydrate content of the diet eaten. The most consistent effect of the high fat diet was a marked increase in ostomy losses of divalent cations; calcium, magnesium, zinc, and copper. Most of the time a net divalent cation secretion on the high fat diet was converted into a net absorption on the low fat, high carbohydrate diet. Altering the polyunsaturated/saturated fatty acid ratio had no consistent effect on divalent cation losses. In conclusion, the proportion of fat versus carbohydrate calories does not appear to influence ostomy volume or monovalent cation loss in extreme short bowel, end jejunostomy patients; however, a high fat intake causes a significant net secretion of divalent cations.

The value of an interposed antiperistaltic colon segment in the management of short-bowel syndrome was assessed in four adult dogs. Following 90 per cent small-bowel resection, and an interposition of 20-cm length of antiperistaltic colon segment, several parameters of small-bowel function were measured. These included regular serum chemistries, including serum glucose, electrolytes, total protein, and the usual liver function studies. Between the test group of four dogs and a control group of three dogs, there was no difference in xylose absorption curves, 24-hour fat excretion, bowel transit time, and average weight loss. On the basis of these findings, colon reversal would not appear to be of benefit in the surgical management of short bowel syndrome.

The introduction of total parenteral nutrition has resulted in more patients surviving massive intestinal resection. Long-term parenteral nutrition is expensive, has potential complications, and causes inconvenience for the patient. Therefore, interest persists in surgical therapy for the short bowel syndrome. The goals of surgical therapy in the short bowel syndrome are to slow intestinal transit, increase the area of absorption, and reduce gastric hyperacidity. Patients with sufficient absorptive area, but rapid intestinal transit, benefit from antiperistaltic segments or colon interposition. Intestinal valves yield inconsistent results. Recirculating loops are associated with prohibitive morbidity and mortality. Experience with intestinal pacing is limited. Patients with dilated bowel segments may benefit from intestinal tapering or lengthening. Growing neomucosa holds promise but has not been evaluated clinically. Recent improvement in the results of intestinal transplantation in animals may warrant clinical trials. The efficacy of H2 receptor antagonists makes procedures for reducing gastric hyperacidity less necessary. None of the operations to treat the short bowel syndrome are sufficiently safe and effective to recommend their routine use. Operations should be performed only on selected patients to achieve specific goals. Although investigation continues, our emphasis should continue to be conservation of as much of the intestine as possible when massive resection is necessary.

Short-bowel syndrome results in malabsorption and malnutrition producing profound metabolic disorders. Attempts to correct this problem with various surgical procedures have so far proved unsuccessful. Studies made in our laboratory using experimental animals, showed that a modified jejunocolic intussusception valve appears to be effective in prolonging the small-bowel transit time and the absorptive capacity of the intestines in short-bowel syndrome. Furthermore, construction of the valve is uncomplicated and unaccompanied by any mortality or morbidity.

Surgical approach to short bowel syndrome has been dealing with two major problems: lack in absorptive surface and dysfunction of the peristalsis of the widely distended loop above the anastomosis. In those children having a very short intestine, one is reluctant to either resect or reduce the diameter of this loop. Bianchi, followed by Boeckman and Traylor, described a procedure of loop lengthening by dividing it longitudinally. Their procedure has the advantage of restoring normal peristalsis without losing any absorptive surface. A modification of the original procedure of Bianchi is described. We report on its application in a child born with laparoschisis and intestinal atresia; she had in fact 25 centimetres of duodenum and proximal jejunum anastomosed with left colonic angle. This child was referred to us with functional occlusion related to distension above an intact and unobstructed anastomosis. She was operated on at six weeks of age. Postoperatively oral feeding could be started after one month. Broviak's catheter for parenteral nutrition was removed at six months. In conclusion we believe that this technique offers a chance of better and faster adaptation to children born with short bowel syndrome.

Small intestinal lengthening by the Bianchi procedure has now had successful clinical application in children and neonates with the short-bowel syndrome. This paper reviews the background experimental work and clinical cases so far treated. A personal case of intestinal lengthening in a 7-week-old baby with 35 cm jejunum is described in detail. Intestinal lengthening appears to reduce dependence on parenteral nutrition, thus allowing earlier establishment of total enteral alimentation. The procedure may therefore have a useful place in the overall management of the short-bowel syndrome.

Significant advances in the use of enteral and parenteral nutrition in patients with either enteric fistulas or short bowel syndrome include increased usage of enteral nutrition because of its trophic effects on the gut and increased usage of both enteral and parenteral nutrition in the home setting. Current investigations are directed toward identifying gut-specific fuels and dietary and pharmacologic enhancement of nutrient utilization.

Combined parenteral and peroral nutrition is necessary when a large small bowel resection leads to inadequate intestinal absorption and starvation. We report the case of a 17-year old schoolgirl in whom, following a rupture of the spleen and splenectomy, extensive small bowel necrosis necessitated small bowel resection from the lig. Treitz to the terminal ileum saving the last ten centimeters of the ileum. The serious starvation which developed after the operation was first treated with a one-month course of parenteral nutrition in hospital. Thereafter the patient took care of the infusions herself. Home parenteral nutrition took place during a 10-12 h overnight period 5 times a week. The proportion of peroral dietary intake was increased gradually during the first year, after which a 1 to 1 ratio between peroral and parenteral intake of energy and protein was maintained. The patient's subjective and objective well-being after two-years' of follow-up is good. Regular examinations at 3-6 month intervals are needed for the maintenance of adequate nutritional replacement.

Successful use of colon interposition has not been previously reported in an adult with short bowel syndrome. This article describes the course of a 34-year-old woman who had interposition of an 18 cm segment of sigmoid colon between 5 cm of proximal jejunum and 7 cm of terminal ileum after midgut volvulus. The rationale for use of colon interposition in cases of extreme short syndrome is discussed.

The functional integrity of transplanted bowel would most convincingly be demonstrated if transplantation after an otherwise lethal intestinal resection permitted survival and growth. In this experiment, we proposed to define the extent of bowel resection necessary for lethality and to show that transplantation allows salvage of these animals. Adult Brown-Norway rats (250 g) underwent extensive small-bowel resection (SBR) (ligament of Treitz to ileocecal valve) (n = 5), cecectomy only (CEC) (n = 3), SBR plus CEC (n = 6), or SBR plus CEC and syngeneic transplantation of 25 cm of jejunum (n = 6). All animals with SBR or CEC alone survived and grew; all animals with SBR plus CEC died within 2 weeks (mean, 6.4 days); and 6 of 6 animals with SBR plus CEC and transplant survived and grew. The difference in survival between the lethally resected rats and those with an intestinal transplant was highly significant (P less than 0.01). Dietary intake was similar in all groups. We conclude that in the rat, extensive small-bowel resection alone is not sufficient to be lethal; most of the small bowel and the cecum must be resected to obtain a lethal model. We have also shown that transplantation of small bowel into rats with an otherwise lethal extent of bowel resection will allow the animals to survive and grow. These results suggest the need for caution in correlating survival after intestinal transplantation in the rat with transplant function, since the preservation of too much native gut might permit survival even if the transplant has no nutritional function.

Total parenteral nutrition now permits long-term survival in patients after massive intestinal resection. Surgical therapy for the short-bowel syndrome is still largely experimental and cannot be recommended routinely. Thus, prevention of intestinal resection and conservation of intestinal length, when resection is necessary, should be emphasized. Strategies are presented that can be employed to preserve intestinal length when surgery is required in patients with a shortened bowel. These include strictureplasty, minimal resection, serosal patching, and intestinal tapering. In suitable candidates strictureplasty can relieve obstruction from strictures while avoiding resection. Minimal resection of involved intestine can be performed safely in selected patients with radiation injury or Crohn's disease. Serosal patching is an alternative to resection for the treatment of perforation or strictures of the intestine. Intestinal tapering can improve the function of dilated intestinal segments and eliminate the need for resection in intestinal atresia. The judicious use of these procedures can preserve intestinal length and obviate the need for long-term parenteral nutrition in patients after massive intestinal resection.

We report a technique, appendiceal interposition, which permitted preservation of the ileocecal valve in an infant with a congenitally short intestine (jejunum, 12 cm; ileum, 1 cm). The procedure was performed on the first day of life in conjunction with jejunal lengthening by the Bianchi technique. The result was a small intestine of 21 cm in length with an intact ileocecal valve.

A 1-day-old male infant with congenital short small bowel was transferred to our hospital because of bilious vomiting and abdominal distension. An exploratory laparotomy performed at the age of 8 days revealed malrotation without any volvulus or stenosis of the small intestine, which was 45 cm long. There were no other anomalies except absence of the appendix. Postoperative nutritional support was by intravenous hyperalimentation and administration of elemental diet. However, parenteral nutrition was discontinued because of hyperbilirubinaemia. The patient died from marked emaciation at 2 months of age despite the administration of elemental diet. Therefore, careful total parenteral nutrition is the only management for successful therapy until the child's intestinal absorptive function develops.

Segmental intestinal transplantation was studied in a rat model of severe short gut syndrome across major histoincompatibility barriers. Lewis (RT1l) recipient rats whose entire small bowel (approximately 80 cm), ileocecal valve, and cecum were resected and who had no transplant, uniformly died of malabsorption on Day 9.8 +/- 0.4 (n = 11). Without cyclosporine, allograft recipients (n = 2), died of rejection on Days 8 and 10. Recipient animals with 20-cm jejunum and 40-cm jejunal transplants from Buffalo (RT1b) rats and treated daily with cyclosporine (5 mg/kg/day) intramuscularly (Days 0-28) and vitamin B12 (every other week) enjoyed significantly prolonged survival to Day 58.2 +/- 13.7, P less than 0.003, n = 10, and Day 129.1 +/- 7.4, P less than 0.001, n = 10, respectively. While 7 of 10 rats in the 20-cm jejunal transplant group died of malabsorption between Days 14 and 58, none of 10 animals in the 40-cm jejunal transplant group died of this complication. Four of 10 rats in the 40-cm jejunal transplant group thrived at 150 days after the operation, at which time they were sacrificed. Morphologically, the grafts demonstrated hypertrophic changes. The data from this study suggest that intestinal allografts have pronounced intestinal adaptative characteristics. Using segmental jejunal grafts, intestinal transplantation is an effective surgical modality for the short gut syndrome in the rat.

Despite the recent advent of total parenteral nutrition (TPN), the long-term survival of the patients with short gut syndrome is not satisfactory with complications of catheter-related sepsis and cholestasis. The causes of short gut syndrome in pediatric surgery are multiple intestinal atresias, necrotizing enterocolitis (NEC), and midgut volvulus. For the multiple atresias, multiple anastomosis without resection has been successfully undertaken to avoid developing short gut syndrome for the last 17 years. For the massive involvement of the intestines due to NEC or volvulus, high jejunostomy and peritoneal drainage with TPN support have been tried for the last two patients and successfully weaned from the TPN within two months after closure of jejunostomy. However, in case 2 malabsorption has been persistent presumably due to a severe degree of mucosal damage occurring in the remaining intestines. Whether this eventually gets back to the normal or not is unknown. For the patients with short gut syndrome, small bowel reversal procedure was successfully done and now doing well 10 years after surgery. This paper reports details of these 3 cases.