Multivisceral transplantation for pediatric intestinal pseudo-obstruction: single center's experience of 16 cases

doi:10.1016/j.transproceed.2004.01.084

Transplantation Proceedings

Volume 36, Issue 2, March 2004, Pages 312-313

https://doi.org/10.1016/j.transproceed.2004.01.084 Get rights and content

Abstract

Chronic intestinal pseudo-obstruction (CIPO) in children may be life-threatening due to the complications of parenteral nutrition (PN) or catheter-related sepsis. Multivisceral transplantation (MVTx) is a lifesaving option but limited experience is available. We report our experience with MVTx in pediatric CIPO patients. Sixteen children with CIPO underwent MVTx at median age of 4 years. Indications for MVTx were liver failure (n = 10), loss of venous access (n = 3), or sepsis (n = 3). Modified MVTx without the liver was performed in six patients. Induction immunosuppression included tacrolimus, steroid with adjunctive agent in period I (April 1996 to December 2000), namely, OKT3 (n = 1), mycophenolate mofetil (n = 4), or daclizumab (n = 2); and in period II (January 2001 to present), Campath 1H (n = 4) or daclizumab (n = 5). The grade of rejection was severe in 12.5% and mild to moderate in 87.5% of cases. Isolated rejection of the transplanted stomach or pancreas was not diagnosed during clinical course or on autopsy. Actuarial patient survival for 1 year/2 years for period, I and II were 57.1%/42.9% and 88.9%/77.8%. None of the long-term survivors is on PN and all tolerate enteral feedings. Pancreatic enzyme supplementation or insulin therapy is not needed in survivors. Gastric emptying was substantially affected in one case. Bladder function did not improve in those with urinary retention problems. MVTx for CIPO offers a lifesaving option with excellent function of the transplanted pancreas and stomach among survivors.

Section snippets

Patients and methods

The surgical technique for MVT has been previously described.⁵ The en bloc graft includes stomach, pancreaticoduodenal complex, and small intestine. Liver and kidney were included in the en bloc graft for patients with liver disease and renal failure. Induction immunosuppression included tacrolimus and steroids with an additional agent. In period I (April 1996 to December 2000) this agent was OKT3 (n = 1), mycophenolate mofetil (n = 4) or daclizumab (n = 2); in period II (January 2001 to

Results

Sixteen children with CIPO had 17 MVTx (one retransplantation) at a median age of 4 years. Eight of them had a clinical and pathological picture compatible with MMIHS, with a younger mean age of MVTx at 1 year. Intestine explants from MMIHS patients showed a thinner outer longitudinal muscule layer and enhanced immunochemical staining of synaptophysin in the inner layer. The indications for transplantation were liver disease (n = 10), loss of venous access (n = 3), or sepsis (n = 3). A modified

Conclusions

MVTx offers a lifesaving option for CIPO cases with intestinal failure destined to die of PN complications. Excellent function of the transplanted stomach and pancreas is seen in MVTx survivors. MVTx does not improve bladder function in CIPO patients with large dilated bladders.

References (5)

C. Di Lorenzo
Pseudo-obstructioncurrent approaches
Gastroenterology
(1999)
W.E. Berdon et al.
Megacystis-microcolon-intestinal hypomotility syndrome. A new cause of intestinal obstruction in the newborn. Report of radiologic findings in five newborn girls
Am J Roentgenol
(1976)

There are more references available in the full text version of this article.

Cited by (32)

Indications for Multivisceral Transplantation: A Systematic Review
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Isolated intestinal transplant for chronic intestinal pseudo-obstruction in adults: Long-term outcome
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Citation Excerpt :
Other reports from the Pittsburgh group, instead, support the need for multivisceral transplant for CIPO patients, with removal of affected organs such as the colon and creation of an end ostomy,9 because the native remnant digestive tract is often involved in the motility disease. The Miami group in 2004 also confirmed the need for multivisceral transplant, which could offer a lifesaving option for CIPO cases.10 In our experience with adult patients, we generally privilege isolated intestinal grafts, due to our better results with isolated intestinal transplant rather than multivisceral transplant (which, in our series, is essentially indicated in the case of liver cirrhosis or celiac axis involvement by native disease).
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.
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.
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.
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.
Intestinal and multivisceral retransplantation results: Literature review
2013, Transplantation Proceedings
Intestinal/multivisceral transplantation (IT/MVT) is the gold standard treatment for patients with intestinal failure and complications related to total parenteral nutrition, gastrointestinal inoperable indolent tumors, or diffuse portal trombosis. Currently, the reported 1-year patient survival rate is around 80%, similar to other solid organ abdominal transplantations. Unfortunately, the patient survival decreases after the first year with the 5-year rate not close to 70% yet. Acute cellular rejection is the main cause of graft loss. Its early diagnosis may make it possible to improve survival of retransplantations.
To analyze the reported results published in the last 5 years by leading transplant centers to evaluate IT/MVT retransplantation results.
We performed a literature review using PubMed focusing on multivisceral and intestinal retransplantation in articles published between 2006 and 2012. In relation to the first transplantation, we analyzed demographics, imunosuppression, rejection, infection as well as graft and patient survival rates.
Two centers reported results on intestinal and multivisceral retransplantations. Mazariegos et al reported their experience with 15 intestinal retransplantations in 14 pediatric recipients. Four patients died from posttransplant lymphoperliferative disease, severe acute cellular rejection, fungal sepsis, or bleeding from a pseudoaneurysm at a mean time of 5.7 months post-transplantation. Total parenteral nutrition was weaned at a median time of 32 days. Abu-Elmaged et al reported 47 cases with a 5-year survival of 47% for all retransplant modalities. Retransplantation with liver-contained visceral allograft achieved a 5-year survival rate of 61% compared with 16% for liver-free visceral grafts.
Despite those huge improvements, some transplanted patients develop severe acute cellular rejection, culminating in graft loss and retransplantation. Repots on multivisceral and intestinal retransplantation outcomes suggest that it is a viable procedure with appropriate patient survival after primary graft loss.
Motility Disorders of the Stomach and Small Intestine
2012, Shackelford's Surgery of the Alimentary Tract: Volume 1-2, Seventh Edition
Domino liver transplantation: Indications, techniques, and outcomes
2011, Transplantation Reviews
Citation Excerpt :
Neurogenic intestinal pseudo-obstruction is an incompletely understood condition often involving all hollow abdominal organs in affected patients, leaving them entirely reliant on parenteral nutrition. Multivisceral transplantation of the affected abdominal organs is sometimes curative in these patients [83]. Although the hepatic function of pediatric patients affected by this condition is often preserved, the difficulty of reconstructing the small-caliber hilar structures in these pediatric recipients often requires that an en bloc multivisceral transplant (including the liver) be performed.
The long-term shortage of livers available for transplantation has spurred the development of many strategies to bolster the donor organ supply. One particularly innovative strategy is domino liver transplantation in which a select group of liver transplant recipients can donate their explanted native livers for use as liver grafts in other patients. Several hereditary metabolic diseases (such as familial amyloid polyneuropathy, maple syrup urine disease, and familial hypercholesterolemia) are caused by aberrant or deficient protein production in the liver, and these conditions can be cured with an orthotopic liver transplant. Although their native livers eventually caused severe systemic disease in these patients, these livers are otherwise structurally and functionally normal, and they have been used successfully in domino liver transplants for the past 15 years. This article will review the indications for donating or receiving a domino liver transplant, the surgical techniques necessary to perform these transplants, as well as the recently revealed long-term outcomes and risks of domino transplantation.
Chronic intestinal pseudo-obstruction
2006, Gastroenterologie Clinique et Biologique
La pseudo-obstruction intestinale chronique (POIC) désigne un syndrome clinique évoquant une obstruction mécanique de l’intestin grêle, mais pour laquelle aucun obstacle n’est mis en évidence. Il s’agit d’une affection rare et grave, d’origine primitive (40 %) ou secondaire (60 %).
Les principaux symptômes de pseudo-obstruction sont nausées, vomissements, météorisme, douleurs abdominales et troubles du transit à type de constipation et/ou diarrhée. Les symptômes précèdent souvent pendant des années le diagnostic positif de POIC. Ils aboutissent à terme à une dénutrition et des troubles hydro-électrolytiques parfois majeurs.
La prise en charge des malades nécessite une démarche rigoureuse : suspecter le diagnostic et éliminer un obstacle organique ; rechercher sur les examens cliniques et paracliniques les anomalies en rapport avec les segments atteints ; rechercher des atteintes extra-intestinales, en particulier de nature neurogène ou myogène ; discuter au cas par cas la réalisation de biopsies intestinales chirurgicales et/ou une biopsie neuromusculaire si une cytopathie mitochondriale est suspectée.
La prise en charge est centrée sur le contrôle des symptômes et l’assistance nutritionnelle. Les prokinétiques peuvent améliorer les symptômes digestifs. La nutrition entérale doit être préférée à la nutrition parentérale. La mise en place d’une double-sonde permettant une gastrostomie d’aspiration et une jéjunostomie d’alimentation peut améliorer le confort. La nutrition parentérale est généralement définitive. La transplantation intestinale peut-être proposée dans certaines situations.
Chronic intestinal pseudo-obstruction (CIPO) is a disease characterized by episodes resembling mechanical obstruction in the absence of organic, systemic, or metabolic disorders. Pseudo-obstruction is an uncommon condition and can result from primary (40 %) or secondary (60 %) causes.
The most common symptoms are nausea, vomiting, abdominal distension, abdominal pain and constipation or diarrhea. These symptoms are usually present many years before CIPO diagnosis. They can lead to severe electrolyte disorders and malnutrition.
Principles for management of patients with CIPO are: to establish a correct clinical diagnosis in excluding mechanical obstruction; to perform a symptomatic and physiologic assessment of the gastrointestinal tract involved; to look for extra-intestinal manifestations, especially for myopathy and neuropathy; to discuss in some cases a surgery for full-thickness intestinal biopsies, and/or a neuromuscular biopsy in case of mitochondrial cytopathy suspicion.
The management is primarily focused on symptom control and nutritional support to prevent weight loss and malnutrition. Treatment of CIPO includes prokinetic agents which may help to reduce gastrointestinal symptoms Courses of antibiotics may be needed in patients with symptoms suggestive of bacterial overgrowth. When necessary, enteral nutrition is preferred. In carefully selected patients, feeding jejunostomy with or without decompression gastrostomy may be tried. Long term parenteral nutrition should be reserved for patients who can not tolerate enteral nutrition. Intestinal transplantation can be discussed in selected patients.

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^☆: C.L. is the recipient of a grant from the Spanish Ministry of Health, FIS 01/5014.

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Clinical outcomesMultivisceral transplantation for pediatric intestinal pseudo-obstruction: single center's experience of 16 cases☆

Abstract

Section snippets

Patients and methods

Results

Conclusions

Pseudo-obstructioncurrent approaches

Gastroenterology

Megacystis-microcolon-intestinal hypomotility syndrome. A new cause of intestinal obstruction in the newborn. Report of radiologic findings in five newborn girls

Am J Roentgenol

Clinical outcomes
Multivisceral transplantation for pediatric intestinal pseudo-obstruction: single center's experience of 16 cases☆