Intestinal and multivisceral transplantation after abdominal trauma

SUMMARY: BACKGROUND Some trauma victims who survive acute illness develop lingering, debilitating syndromes that are incompatible with any semblance of normalcy. Intestinal failure, in particular, exacts a high price in terms of quality of life. Total parenteral nutrition (TPN) has served these patients well, but complications limit its long-term therapeutic effect. Consequently, transplantation is emerging as a life-saving therapy for some patients with the short gut syndrome.METHODS We reviewed eight adult and two pediatric recipients of intestinal and multivisceral transplants after severe abdominal trauma. Background demographics, type of abdominal trauma, transplant procedure, postoperative complications, and survival rates were appraised. This group was also compared with 47 nontrauma recipients of intestinal transplants performed during the same period.RESULTS Four patients (40%) died postoperatively (postoperative days 7, 53, 87, and 91) as a result of multiple organ failure after graft pancreatitis (n = 1), viral encephalitis (n = 1), and sepsis after severe rejection (n = 2). Six patients (60%) are alive (postoperative days 52-1,783). All are off TPN. The 4-year patient survival was 58%, with no significant difference between trauma and nontrauma patients.CONCLUSION Intestinal and multivisceral transplantation are viable options for the treatment of irreversible intestinal failure associated with severe trauma. Surviving patients are TPN independent and have a satisfactory quality of life.     

Surgical approach to complicated intestinal failure for benign disease in adult patients: transplantation or surgical rehabilitation?

Surgical approaches to complicated benign intestinal failure are gaining acceptance, especially in the pediatric population. Less international experience has been obtained in adult patients, who are usually treated with total parenteral nutrition (TPN). An intestinal rehabilitation program was started in our institution with comprehensive medical rehabilitation, surgical bowel rescue, and transplantation. Among 38 adult patients referred by our gastroenterologists for bowel rehabilitation and surgically treated in our institution, 92.2% received TPN on admission. After careful evaluation, 71% underwent transplantation. Five patients died, but 18 recipients were completely weaned off TPN at follow-up. Eleven patients underwent surgical resection of the affected bowel and a subsequent program of intestinal rehabilitation: they were all alive and weaned off TPN at discharge. At a 2-year mean follow-up, deaths occurred only in the transplant population. Therefore, intestinal surgical rescue, if successful, is optimal in adult patients.     

Isolated liver and multivisceral transplantation for total parenteral nutrition-related end-stage liver disease

Purpose

Total parenteral nutrition (TPN) has prolonged survival in children with intestinal failure; however, end-stage liver disease owing to TPN-induced cholestasis (ESLD-TPN) may preclude its use. ESLD-TPN is an indication for isolated liver transplantation (ILT) or multivisceral transplantation (MVT). Isolated liver transplantation for ESLD-TPN should only be considered in patients who have the potential for enteral autonomy.

Methods

We retrospectively reviewed the records of patients with ESLD-TPN who underwent ILT (n = 7) or MVT (n = 5) between 1994 and 2005. The median age at the time of transplantation was 10.0 months. Intestinal failure followed necrotizing enterocolitis (n = 3), gastroschisis (n = 3), gastroschisis with volvulus (n = 3), gastroschisis with atresia (n = 1), malrotation (n = 1), and megacystis microcolon intestinal hypoperistalsis syndrome (n = 1).

Results

Isolated liver transplant patients had a median length of small bowel of 70 cm and tolerated a median of 50% of enteral calories. The median length of small bowel in patients who underwent MVT was 29 cm, and none tolerated more than 30% of goal enteral feeds. Reduced-size (n = 5) and whole-liver (n = 2) allografts were used for patients undergoing ILT. Patients undergoing MVT received liver-small bowel-pancreas (n = 4) or liver-small bowel-pancreas-colon (n = 1). Overall patient survival was 57% in ILT (median follow-up = 25.1 months); 3 survivors are TPN independent, and the fourth patient requires TPN 3 days/wk. Patient survival was 40% after MVT (median follow-up = 13.0 months); 1 MVT patient died of abuse 16.9 months after transplant and was TPN independent at the time of death. Both survivors are TPN independent. Bilirubin levels are within normal range in all survivors.

Conclusion

Isolated liver transplantation for ESLD-TPN in the setting of intestinal failure is a viable option in patients who have the potential for enteral autonomy. Multivisceral transplantation is the only alternative in patients without the potential for intestinal recovery. Survival can be achieved in patients with ESLD-TPN, but mortality remains high for both procedures.     

Temporary elevation of serum transaminases after pediatric intestinal transplantation: incidence and clinical correlation in multivisceral transplant vs isolated intestinal transplant

Data were gathered from the records of 51 children of median age 1.5 years who survived more than 6 months after intestinal transplantation. Abnormal liver function tests (LFTs) were defined as serum aspartate aminotransferase (AST) greater than 100 IU/L or total bilirubin greater than 2.0 g/dL lasting more than 3 days. Temporary elevation was defined when LFTs returned to normal without graft loss or death. LFT elevation at the time of transplantation was not included as a temporary LFT elevation. Median follow-up was 36 months. In multivisceral transplant recipients, all patients (n = 34) showed abnormal LFTs at transplantation that normalized within a median period of 2 days. Temporary LFT elevations were seen in 20 of 34 (59%) in multivisceral transplantation and 5 of 17 (29%) in isolated intestinal transplantation. Median length of elevation was 14 days in multivisceral transplantation and 12 days in isolated intestinal transplantation. Peak AST was 353 +/- 190 IU/dL in multivisceral transplantation and 839 +/- 605 IU/dL in isolated intestinal transplantation (P = .0059). Events associated with temporary LFT elevations in multivisceral transplantation were total parental nutrition (TPN) (n = 8), dehydration (n = 2), viral infection (n = 2), others (n = 3), and nonspecific (n = 5). Events in isolated intestinal transplantation were posttransplant lymphoproliferative disorder (n = 2), TPN (n = 1), and nonspecific (n = 2). Temporary LFT elevations were commonly seen among pediatric intestinal recipients, which correlated with events other than rejection. Approximately half of the temporary LFT elevations were associated with no significant clinical events. They resolved spontaneously. Interestingly, the peak AST value was higher in isolated intestinal transplantation compared to multivisceral transplantation.     

Intestinal Transplantation in Children: The First Successful Italian Series

The preliminary experience of the first Italian program of pediatric intestinal transplantation is presented herein. A multidisciplinary group with broad experience in pediatric solid organ transplantation started the program. Nine children with complications of chronic intestinal failure were listed for transplantation. One child died on the waiting list; one received an isolated liver transplantation; three isolated intestinal; three multivisceral; and one, a combined liver/intestine transplantation. There was no in-hospital mortality, and all children were weaned from parenteral nutrition. The recipient of the multivisceral graft died after 14 months for unknown causes. All other recipients are alive after a median follow-up of 13 months. Patient and graft actuarial survivals for recipients of intestinal grafts were 100% at 1 year and 75% at 2 years.     

Combined living-related segmental liver and bowel transplantation for megacystis-microcolon-intestinal hypoperistalsis syndrome

Background

Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) is the most severe form of functional intestinal obstruction in the newborn. To date, multivisceral transplantation has been the only accepted treatment modality for these patients, and the results have met with marginal success. We report the first case of a patient affected by MMIHS and cholestatic liver failure treated by a combined living-related liver and intestinal transplant (CLRLITx).

Case Report

The patient was a 1-year-old Hispanic girl born with MMIHS and maintained on total parenteral nutrition since birth. Once liver failure developed, she was referred for evaluation for possible CLRLITx. The patient's mother volunteered as the donor. The left lateral segment was used for the liver transplant. The intestinal graft consisted of the terminal 180 cm of the ileum with a single vascular pedicle. Initially, the patient continued to have severe gastroparesis; however, by 8 months posttransplant, stomach function had returned to normal. Currently, at 2 years posttransplant, she is tolerating an oral diet with gastric tube supplementation. Results of absorption studies are within normal, and she has shown catch-up growth.

Conclusion

A CLRLITx can be a viable alternative for infants diagnosed with MMIHS. This procedure can help avoid the 25% wait-list mortality for children who are in need of a combined transplant.     

Intestinal and multivisceral transplantation

Intestinal and multivisceral transplantation represents an important treatment option for patients with intestinal failure. Early attempts were hindered by technical and immunological complications. However, significant developments in immunosuppressive therapy have led to marked improvements in outcomes in recent years. The main indications for intestinal transplantation are life-threatening complications or unacceptable quality of life on total parenteral nutrition (TPN), or following evisceration for extensive intra-abdominal tumours. In suitable patients, in the absence of significant liver disease, an isolated intestinal graft is appropriate. A combined liver and intestinal transplant is indicated in patients with significant liver disease, almost always as a result of long-term TPN. Pathology affecting the foregut may require more extensive grafts including the stomach, duodenum and pancreas. Multivisceral transplantation is technically demanding. The transplant recipient has frequently undergone multiple previous laparotomies and may present with multiple stomata, fistulae, collections, distortion of intra-abdominal anatomy and significant contraction of the abdominal cavity. The most important early complications are acute rejection and sepsis, which frequently occur together. In the long-term, chronic rejection and malignancy are the leading causes of graft loss and mortality. It is hoped that ongoing improvements in intestinal and multivisceral transplantation may eventually justify its use as a primary alternative to long-term TPN.     

Intestinal and multivisceral transplantation

Intestinal and multivisceral transplantation represents an important treatment option for patients with intestinal failure. Early attempts were hindered by technical and immunological complications. However, significant developments in immunosuppressive therapy have led to marked improvements in outcomes in recent years. The main indications for intestinal transplantation are life-threatening complications or unacceptable quality of life on total parenteral nutrition (TPN), or following evisceration for extensive intra-abdominal tumours. In suitable patients, in the absence of significant liver disease, an isolated intestinal graft is appropriate. A combined liver and intestinal transplant is indicated in patients with significant liver disease, almost always as a result of long-term TPN. Pathology affecting the foregut may require more extensive grafts including the stomach, duodenum and pancreas. Multivisceral transplantation is technically demanding. The transplant recipient has frequently undergone multiple previous laparotomies and may present with multiple stomata, fistulae, collections, distortion of intra-abdominal anatomy and significant contraction of the abdominal cavity. The most important early complications are acute rejection and sepsis, which frequently occur together. In the long-term, chronic rejection and malignancy are the leading causes of graft loss and mortality and immunosuppression related renal impairment a major source of morbidity. It is hoped that ongoing improvements in intestinal and multivisceral transplantation may eventually justify its use as a primary alternative to long-term TPN.     

Intestinal and multivisceral transplantation

Intestinal and multivisceral transplantation represents an important treatment option for patients with intestinal failure. Early attempts were hindered by technical and immunological complications. However, significant developments in immunosuppressive therapy have led to marked improvements in outcomes in recent years. The main indications for intestinal transplantation are life-threatening complications or unacceptable quality of life on total parenteral nutrition (TPN), or following evisceration for extensive intra-abdominal tumours. In suitable patients, in the absence of significant liver disease, an isolated intestinal graft is appropriate. A combined liver and intestinal transplant is indicated in patients with significant liver disease, almost always as a result of long-term TPN. Pathology affecting the foregut may require more extensive grafts, including the stomach, duodenum and pancreas. Multivisceral transplantation is technically demanding. The transplant recipient has frequently undergone multiple previous laparotomies and may present with multiple stomata, fistulae, collections, distortion of intra-abdominal anatomy and significant contraction of the abdominal cavity. The most important early complications are acute rejection and sepsis, which frequently occur together. In the long term, chronic rejection and malignancy are the leading causes of graft loss and mortality and immunosuppression related renal impairment a major source of morbidity. It is hoped that ongoing improvements in intestinal and multivisceral transplantation may eventually justify its use as a primary alternative to long-term TPN.     

Comprehensive Surgical Intestinal Rescue and Transplantation Program in Adult Patients: Bologna Experience

Introduction

Surgical approaches to complicated benign intestinal failure are accepted worldwide, especially in the pediatric population. Intestinal transplant surgery is thought to rescue patients in whom complications of total parenteral nutrition (TPN) develop.

Objective

To report our experience with surgical intestinal rescue in an adult population with intestinal failure.

Patients and Methods

An intestinal rehabilitation program initiated at our institution included comprehensive medical rehabilitation, surgical bowel rescue, and transplantation. From 2000 to 2009, of 81 adult patients referred by our gastroenterologists for bowel rehabilitation, 42 (51,8%) underwent 43 transplantations (32 isolated intestinal grafts and 11 multivisceral grafts). Underlying diseases were primarily short-bowel syndrome, Gardner syndrome, and intestinal pseudo-obstruction. Thirty-nine patients (48,2%) underwent surgical rescue (40 cases) consisting of bowel resection, adhesiolysis, stricturoplasty, liver transplantation with portocaval hemitransposition (6 cases in 5 patients). Underlying diseases were primarily intestinal fistulas, stenosis, or perforations, short-bowel syndrome, cocoon syndrome, and complete portal thrombosis.

Results

After a mean (SD) follow-up of 1043 (1016) days, in the transplantation population, 21 patients (50%) are alive, with a 1-, 3-, 5-year patient survival of 76%, 59%, and 52%, respectively, and graft survival of 66%, 54%, and 48%, respectively. After 901 (404) days in the rescue population, 32 patients (82%) are alive (2 died, and 5 were lost to follow-up); in 75%, TPN 25% was discontinued, and are receiving oral feeding with TPN support. The 1- and 3-year survival rate was 100% and 83%, respectively.

Conclusions

Deaths occurred primarily in the transplantation population. Intestinal surgical rescue, when possible, is optimal.     

Isolated Intestinal Transplant for Chronic Intestinal Pseudo-Obstruction in Adults: Long-Term Outcome

Background

Chronic intestinal pseudo-obstruction (CIPO) has been treated in adults by total parenteral nutrition (TPN) or, if complications arise, by multivisceral transplantation because the stomach is often involved. Eleven adults with CIPO were transplanted by intestinal graft in our center from 2000 to 2011.

Methods

Nine patients underwent isolated intestinal transplant and 2 patients had multivisceral transplant. Immunosuppression was represented by FK and steroids plus induction with alemtuzumab, daclizumab, or thymoglobulin. Average age at transplant was 33.5 years. We reported 1 graftectomy, followed by retransplantation.

Results

Seven patients are currently alive with working small bowel; cause of death was infection in the 4 remaining cases. In 9 isolated intestinal transplants, we performed different digestive reconstructions to allow gastric emptying. In 2 cases we were forced, after transplant, to perform ileostomy to improve intestinal motility. Graft and patient survival after 5 years are 60% and 70%, respectively, while after 10 years, 45% and 56%, respectively.

Conclusions

Adults with CIPO and irreversible TPN complications benefit from isolated intestinal transplant with different surgical techniques to empty the native stomach: this strategy achieves good gastric emptying, with effective establishment of oral feeding and graft and patient survivals comparable to isolated intestinal transplant for short bowel syndrome.     

Growth after intestinal transplant in children

Intestinal transplantation has been more frequent in children with intestinal failure. However, the growth after intestinal transplantation has not been well documented. The demographics, transplant information, postoperative complications, heights, and weights were obtained retrospectively from medical records on 23 children who underwent intestinal transplantation. Z-scores were calculated from the STAT Growth-BP, based on Centers for Disease Control and Prevention growth chart (2000). Transplantations were performed between 1999 and 2004. Patient median age was 1.1 years (range 0.5 to 6.9 years). Twelve were boys and 11 girls. Seventeen children received multivisceral transplantations, one modified multivisceral transplantation, and five isolated intestinal transplantations. Baseline immunosuppression consisted of tacrolimus and corticosteroids. Daclizumab was used as induction agent in 18 patients; alemtuzumab, in five patients. Median pretransplant Z-scores were median -1.67 (n = 23) in weight, and median -3.36 (n = 21) in height. Pretransplant growth was significantly retarded. We analyzed significantly retarded patients with Z-score <-2.0. The change of weight Z-score from pretransplant was: 1.25 at 6 months (n = 11), 1.46 at 12 months (n = 10), and 2.21 at 24 months (n = 7). The change of height Z-score: 1.9 at 6 months (n = 16), 1.42 at 12 months (n = 13), and 1.51 at 24 months (n = 10). Z-score significantly improved (P < .002, ANOVA). Among the analyzed factors sex, age at transplant, length of stay, and rejection within 6 months, were not associated with catch-up growth. Children with retarded growth showed significant catch-up after successful intestinal transplantation.     

Intestinal transplantation for the treatment of desmoid tumors associated with familial adenomatous polyposis

BACKGROUND: Desmoid tumors associated with familial adenomatous polyposis (FAP) are locally invasive. Often occurring in the mesentery of the intestine, they sometimes recur after resection. Complications can include intestinal failure and dependence on parenteral nutrition. We describe 9 patients who underwent intestinal transplantation for the treatment of desmoid tumors associated with FAP. METHODS: Records of patients undergoing intestinal transplantation for desmoid tumors at 2 transplant centers were reviewed for patient age, sex, type of graft, procedure date, tumor site, desmoid complications, medications, extracolonic manifestations, status at follow-up, and length of survival. RESULTS: Nine patients with FAP and intestinal failure caused by desmoid tumors were treated with isolated intestinal (n = 6), multivisceral (n = 2), or combined liver-intestinal transplantation (n = 1). Desmoid tumors recurred in the abdominal walls of 2 patients. Two patients died: one as a result of sepsis, the other because of a rupture of a mycotic aneurysm of the aortic anastomosis. One graft lost to severe rejection was replaced with a second intestinal graft. Eleven to 53 months after transplantation, 7 patients were alive, well, independent of parenteral treatment, and leading apparently normal lifestyles. CONCLUSIONS: Transplantation of the intestine alone or as part of a multivisceral transplantation may help rescue otherwise untreatable patients with complicated desmoid tumors.     

Transplantation for the treatment of intra-abdominal fibromatosis

MATERIALS AND METHODS: During the last 9 years we treated 14 patients with a diagnosis of intra-abdominal fibromatosis. The 11 patients who received an intestinal allograft included isolated intestine (n = 6), liver-intestine (n = 1), intestine-kidney (n = 1), multivisceral (n = 1), multivisceral-kidney (n = 1), multivisceral-no liver (n = 1). Three patients received an intestinal autograft after partial abdominal evisceration and ex vivo tumor resection. Three patients additionally underwent an abdominal wall allograft. RESULTS: At follow-up until August 2004, all autotransplant patients are alive. Four intestinal transplant patients died within the first postoperative month. There were three graft losses. A patient who lost his graft early postoperatively was retransplanted but died of sepsis shortly there after. Two more patients lost their graft due to severe rejection and were retransplanted successfully. Two patients developed desmoid tumor recurrence in their abdominal or thoracic wall. Ten patients are alive 1 to 9 years posttransplantation. Nine have fully functioning grafts and one patient requires TPN supplementation at night due to dysmotility of her autograft. CONCLUSION: Intestinal allo-, or autotransplantation combined with transplantation of the abdominal wall can be lifesaving for patients suffering from extensive intra-abdominal fibromatosis.     

An integrated approach to intestinal failure: Results of a new program with total parenteral nutrition, bowel rehabilitation, and transplantation

Intestinal failure can be treated with bowel rehabilitation, total parenteral nutrition, or intestinal transplantation. Little has been done to integrate these therapies for patients with intestinal insufficiency or failure and to develop an algorithm for appropriate use and timing. We established a multidisciplinary program using bowel rehabilitation, total parenteral nutrition, or intestinal transplantation as appropriate in a large population. Evaluation included clinical, pathlogic, and psychosocial assessments and assignment to therapy based on the results of this evaluation. Of 59 patients evaluated for life-threatening complications of intestinal failure, 68% were considered appropriate candidates for transplantation, 10% were managed with rehabilitation, and 17% were maintained on optimized long-term parenteral nutrition. Nineteen transplants were performed, with 78% patient survival and 66% graft survival. Patient survival among isolated intestine recipients was 90%. All patients managed with rehabilitation were weaned from parenteral nutrition within 6 months. Long-term management with parenteral nutrition resulted in a significant number of deaths both among patients waiting for a transplant and those who were poor candidates for transplant. Intestinal rehabilitation, when successful, is optimal. For patients with irreversible intestinal failure, isolated intestinal transplantation holds particular promise. Parenteral nutrition is plagued by high failure rates among this population of debilitated patients compared with the general parenteral nutrition population. Integration of these therapies, with individualization of care based on a multidisciplinary approach and perhaps with earlier isolated intestinal transplantation for patients with irreversible intestinal failure, should optimize survival. Presented in part at the Forty-First Annual Meeting of The Society for Surgery of the Alimentary Tract, San Diego, California, May 21–24, 2000 (oral presentation).     

The clinical spectrum of neurologic disorders after intestinal and multivisceral transplantation

?ivkovi? SA, Eidelman BH, Bond G, Costa G, Abu‐Elmagd KM. The clinical spectrum of neurologic disorders after intestinal and multivisceral transplantation.
Clin Transplant 2009: DOI: 10.1111/j.1399‐0012.2009.01065.x
© 2009 John Wiley & Sons A/S. Abstract: Background: Intestinal transplantation has evolved into an effective therapy for patients with intestinal failure and the inability to be maintained on total parenteral nutrition. Long‐term heavy immunosuppression and complex systemic disturbances increase the risk of the neurologic complications. Methods: This retrospective analysis identified the post‐transplant neurologic complications in adult patients who underwent intestinal transplantation at the University of Pittsburgh Medical Center between May 1990 and August 1998. The recipients received 28 isolated intestine, 17 composite liver‐intestine, and nine multivisceral allografts. Results: With a median follow‐up of 25 months, 46 of 54 recipients (68%) developed headaches (n = 27; 50%), encephalopathy (n = 23; 43%), seizures (n = 9; 17%), neuromuscular disorders (n = 4; 7%), opportunistic CNS infections (n = 4; 7%), and ischemic stroke (n = 2; 4%). Conclusions: Under high maintenance immunosuppression, intestinal transplant recipients were at high risk for neurologic complications. Future studies are needed to describe post‐transplant neurologic complications with modern immunosuppression protocols.     

Intestinal transplantation: 1997 report of the international registry. Intestinal Transplant Registry

BACKGROUND: Small bowel transplantation is an evolving procedure. We reviewed the world experience since 1985 to determine the current status of this procedure. METHODS: All of the known intestinal transplant programs were invited to contribute to an international registry using a standardized report form. RESULTS: Thirty-three intestinal transplant programs provided data on 273 transplants in 260 patients who were transplanted on or before February 28, 1997. The number of procedures per year has increased at a linear rate since 1990, with 58 transplants performed in 1996. Two-thirds of the recipients were children or teenagers. The short gut syndrome was the most common indication for transplantation. The types of transplants included the small bowel with or without the colon (41%); the intestine and liver (48%); and multivisceral grafts (11%). The 1-year graft/patient survival for transplants performed after February 1995 was 55%/69% for intestinal grafts; 63%/66% for small bowel and liver grafts; and 63%/63% for multivisceral grafts. Transplants since 1991 and programs that had performed at least 10 transplants had significantly higher graft survival rates. Seventy-seven percent of the current survivors had stopped total parenteral nutrition (TPN) and resumed oral nutrition. CONCLUSIONS: Transplantation has become a lifesaving procedure for (1) patients with intestinal failure who cannot be maintained on total parenteral nutrution and (2) patients who require abdominal evisceration to completely remove locally aggressive tumors. The 5-year survival rate of intestinal transplantation with large series is comparable to lung transplantation.     

Long-term parenteral nutrition in pediatric autoimmune enteropathies

Severe and protracted or persistent diarrhea (SPD) is the most severe form of diarrhea in infancy and has also been defined as intractable diarrhea when it leads to dependence on total parenteral nutrition (TPN). One of the rare causes of SPD is represented by autoimmune enteropathy that is characterized by life-threatening diarrhea mainly occurring within the first years of life, persistent villous atrophy in consecutive biopsies, resistance to bowel rest, and evidence of antigut autoantibodies. We evaluated 10 patients (seven boys, mean age at diagnosis 18 months; range: 0 to 160 months) fulfilling criteria of autoimmune enteropathy to assess dependence on TPN. TPN was first required in all patients to avoid dehydration and electrolytic imbalance. All patients were dependent on immunosuppressive therapy (steroid, azothioprine, cyclosporine, tacrolimus). Three patients died of sepsis: two during TPN while in the hospital, and one at home after he was weaned off TPN. Five patients are weaned off TPN after a mean period of 18 months; they are actually on oral alimentation with a cow milk-free diet after a period of enteral nutrition with elemental formula. One underwent total colectomy and bone marrow transplantation and one developed an IPEX syndrome. One patient is still dependent on TPN for 24 months. She is on home parenteral nutrition. Patients with diagnosis of IPEX syndrome require parenteral support with three or four infusion per week. TPN represents a fixed step in the management of autoimmune enteropathy, but it may be considered as an interim treatment while waiting for intestinal adaptation, at least in some selectioned case of autoimmune enteropathy. Bone marrow transplantation should be considered and reserved for those patients with severe complications due to home parenteral nutrition, or in those that are really dependent on parenteral nutrition.     

Intestinal and multivisceral transplantation in children

OBJECTIVE: To describe a single-center experience of pediatric intestinal transplantation (Itx) and to provide an overview of the children who underwent this procedure along with their outcomes. SUMMARY BACKGROUND DATA: Pediatric Itx presents multiple challenges because of the very young ages at which patients require transplantation and their higher susceptibility to infectious complications. METHODS: We have performed 141 Itx in 123 children with a median age of 1.37 years. Primary grafts included isolated intestine (n = 28), liver and intestine (n = 27), multivisceral (n = 61), and multivisceral without the liver (n = 7). Two protocol modifications were introduced in 1998: daclizumab induction and frequent rejection surveillance. In 2001, indications for multivisceral transplantation were expanded, and induction with Campath-1H was introduced. RESULTS: Actuarial patient survival at 1 and 3 years for group 1 (January 1994 to December 1997, n = 25), group 2 (January 1998 to March 2001, n = 29), group 3a (April 2001 to present, daclizumab, n = 51), and group 3b (April 2001 to present, Campath-1H, n = 18) was 44%/32%, 52%/38%, 83%/60%, and 44%/44%, respectively (P = 0.0003 in favor of group 3a). Severe rejection implied a dismal prognosis (65% mortality at 6 months). Observed incidence of severe rejection in groups 1, 2, 3a, and 3b was 32%, 24%, 14%, and 11%, respectively. In multivariable analysis, use of a multivisceral (with or without liver) transplant (P = 0.002), induction with daclizumab (P = 0.005), patient at home prior to transplant (P = 0.007), and age at transplant > or =1 year (P = 0.02) favorably influenced patient survival. Multivisceral transplant was protective with respect to the mortality rate due to rejection, while an older age at transplant was associated with both a lower incidence rate of developing respiratory infection and lower risk of mortality following the respiratory infection. Survivors are off parenteral nutrition and have demonstrated significant growth catch-up. CONCLUSIONS: Itx in children still is a high-risk procedure but has now become a viable option for children who otherwise have no hope for survival. Control of respiratory infection is of particular importance in the younger children.     

A multidisciplinary approach to the treatment of intestinal failure

Intestinal failure is most commonly treated by the administration of total parenteral nutrition (TPN). In some patients, however, surgical therapy may increase the ability to use the intestine for nutrition and thereby decrease the complications of TPN therapy. A multidisciplinary comprehensive intestinal failure program was initiated at the University of Nebraska Medical Center in October 2000. Here we describe the surgical approaches to patients with short bowel syndrome and the subsequent impact on the need for TPN and on survival. Fifty patients (children = 30, adults = 20) underwent surgical procedures to restore intestinal continuity (n = 5), repair enterocutaneous fistulas (n = 5), resect dysmotile or strictured/obstructed bowel segments or mesenteric desmoid tumors (n = 7), stricturoplasty (n = 2), Bianchi tapering and lengthening (n = 20), serial transverse enteroplasty (n = 8), and other operations (n = 8). Of these 50 patients, three patients did not require TPN after surgical intervention and seven had remnant small bowel anatomy that precluded TPN weaning (e.g., end duodenostomy) and were listed for transplantation or continued on full TPN support. Of the 40 remaining patients, most received the majority of calories fromTPNat the time of referral, i.e., mean calories fromTPN _ 90%. Subsequent to the surgical and medical therapy, 26 (65%) have been completely weaned off TPN. In addition, 10 had substantial decreases in their TPN requirements (i.e., from 85% of calories from TPN at onset decreased to a median 35% of required calories at most recent follow-up). Four patients remained on the same amount of TPN support. Four of the seven patients listed for transplantation underwent successful transplantation. Despite the complications of short bowel syndrome, 86% (n = 43) of the patients are alive and well at a mean follow-up of 2 years. Patient deaths occurred primarily in those listed or eligible for transplantation and were related to advanced liver disease (n = 3), gastrointestinal hemorrhage (n = 1), or line sepsis (n = 1). Two other patients died, one from influenza A infection and one from unknown cause at home, months after complete discontinuation of TPN. In this series of patients with short bowel syndrome, surgical intervention led to weaning or discontinuation of TPN support in 85% of patients. An organized multidisciplinary approach to the patient with short bowel syndrome is recommended. Presented at the Forty-Fifth Annual Meeting of The Society for Surgery of the Alimentary Tract, New Orleans, Louisiana, May 15–19, 2004 (oral presentation).