https://onlinelibrary.wiley.com/doi/pdf/10.1111/ajt.12979

Intestinal transplant registry report: global activity and trends.

Intestinal transplantation has evolved from an experimental procedure with limited success to standard of care for patients with intestinal and parenteral nutrition failure, achieving outcomes commensurate with other solid-organ transplants (Grant et al. 2005; Fishbein 2009). Paramount to achieving the success of intestinal and multivisceral organ transplant procedures has been the refinement of donor organ procurement and transplantation techniques (Fishbein et al. 2003a). Early graft failures and deaths due to technical and donor-related complications have been minimized using the techniques described here, leaving the current challenge largely of optimizing immunologic and infection management strategies (Fishbein 2004; Fishbein and Matsumoto 2005). The techniques depicted here are those of choice in our experience, though mention is made of alternative techniques that others may prefer (Abu-Elmagd et al. 2000; Fishbein et al. 2003b; Reyes et al. 1998; Tzakis et al. 1993). Because patients requiring intestinal transplantation often present with multiple organ failure and require multiorgan transplantation, modification of these techniques may be required to meet the needs of an individual patient. Some of the modifications that we have encountered include the addition of renal allotransplantation, inclusion of the colon in a small-bowel or multivisceral graft, and modified multivisceral transplantation with preservation of the native liver. Intimate description of all possible technical variations that might be required is beyond the scope of this chapter, and intestinal transplant surgeons should be prepared to draw from their experience in gastrointestinal and transplant surgery and employ generally accepted tenets as necessary.

Intestinal and Multivisceral Transplantation

Objectives:Chronic intestinal pseudo-obstructive (CIPO) conditions are considered the most severe disorders of gut motility. They continue to present significant challenges in clinical care despite considerable recent progress in our understanding of pathophysiology, resulting in unacceptabl

/pdf/paediatric-intestinal-pseudo-obstruction-evidence-and-380egqhet4.pdf

Paediatric Intestinal Pseudo-obstruction: Evidence and Consensus-based Recommendations From an ESPGHAN-Led Expert Group.

Intestinal epithelial dysplasia (IED), also known as tufting enteropathy, is a congenital enteropathy presenting with early-onset severe intractable diarrhea causing sometimes irreversible intestinal failure. To date, no epidemiological data are available, however, the prevalence can be estimated at around 1/50,000–100,000 live births in Western Europe. The prevalence seems higher in areas with high degree of consanguinity and in patients of Arabic origin. Infants develop within the first days after birth a watery diarrhea persistent in spite of bowel rest and parenteral nutrition. Some infants are reported to have associated choanal rectal or esophageal atresia. IED is thought to be related to abnormal enterocytes development and/or differentiation. Nonspecific punctuated keratitis was reported in more than 60% of patients. Histology shows various degree of villous atrophy, with low or without mononuclear cell infiltration of the lamina propria but specific histological abnormalities involving the epithelium with disorganization of surface enterocytes with focal crowding, resembling tufts. Several associated specific features were reported, including abnormal deposition of laminin and heparan sulfate proteoglycan (HSPG) in the basement membrane, increased expression of desmoglein and ultrastructural changes in the desmosomes, and abnormal distribution of α2β1 integrin adhesion molecules. One model of transgenic mice in which the gene encoding the transcription factor Elf3 is disrupted have morphologic features resembling IED. Parental consanguinity and/or affected siblings suggest an autosomal recessive transmission but the causative gene(s) have not been yet identified making prenatal diagnosis unavailable. Some infants have a milder phenotype than others but in most patients, the severity of the intestinal malabsorption even with enteral feeding make them totally dependent on daily long-term parenteral nutrition with a subsequent risk of complications. IED becomes an indication for intestinal transplantation, while timing of referral for it is crucial before the onset of severe complications.

/pdf/intestinal-epithelial-dysplasia-tufting-enteropathy-1h5bg59ulo.pdf

Intestinal epithelial dysplasia (tufting enteropathy).

https://onlinelibrary.wiley.com/doi/pdf/10.1111/ajt.12812

The current state of intestine transplantation: indications, techniques, outcomes and challenges.

Objective: Small bowel (SB) transplantation (Tx), long considered a rescue therapy for patients with intestinal failure, is now a well recognised alternative treatment strategy to parental nutrition (PN). In this retrospective study, we analysed graft functions in 31 children after SBTx with a follow-up of 2–18 years (median 7 years). Patients: Twelve children had isolated SBTx, 19 had combined liver–SBTx and 17 received an additional colon graft. Growth, nutritional markers, stool balance studies, endoscopy and graft histology were recorded every 2–3 years post-Tx. Results: All children were weaned from PN after Tx and 26 children remained PN-free. Enteral nutrition was required for 14/31 (45%) patients at 2 years post-Tx. All children had high dietary energy intakes. The degree of steatorrhoea was fairly constant, with fat and energy absorption rates of 84–89%. Growth parameters revealed at transplantation a mean height Z-score of –1.17. After Tx, two-thirds of children had normal growth, whereas in one-third, Z-scores remained lower than –2, concomitant to a delayed puberty. Adult height was normal in 5/6. Endoscopy and histology analyses were normal in asymptomatic patients. Chronic rejection occurred only in non-compliant patients. Five intestinal grafts were removed 2.5–8 years post-Tx for acute or chronic rejection. Conclusions: This series indicates that long-term intestinal autonomy for up to 18 years is possible in the majority of patients after SBTx. Subnormal energy absorption and moderate steatorrhoea were often compensated for by hyperphagia, allowing normal growth and attainment of adult height. Long-term compliance is an important pre-requisite for long-term graft function.

https://gut.bmj.com/content/gutjnl/57/4/455.full.pdf

Long-term outcome, growth and digestive function in children 2 to 18 years after intestinal transplantation

Results of the Paris program: ten years of pediatric intestinal transplantation.

We evaluated 131 patients (6 months–14 years) who experienced 21 deaths before listing, 11 continuing on the waiting list, 38 well on home parenteral nutrition, 6 off parenteral nutrition and 59 transplanted (20 girls) aged 2.5 to 15 years, (18 >7 years). They received cadaveric isolated intestine (ITx, n = 31) or liver-small bowel (LITx, n = 32), including right colon (n = 43; 23 LITx) for short bowel (n = 19), enteropathy (n = 20), Hirschsprung (n = 14), or pseudo-obstruction (n = 6). Treatment included tacrolimus, steroids, azathioprine, or interleukin-2 blockers. After 6 months to 10.5 years, the patient and graft survivals were 75% and 54%. Sixteen patients (10 LITx) died within 3 months from surgery (n = 3), bacterial (n = 5) or fungal (n = 6) sepsis, or posttransplant lymphoproliferative disorder (n = 2). Rejection occurred in 27 patients, including 10 steroid-resistant episodes requiring antilymphoglobulins. The grafts were removed due to uncontrolled rejection in seven ITx recipients. Surgical complications were observed in 38 recipients (25 LSBTx) within 2 months, including bacterial (n = 22) or fungal (n = 11) sepsis, cytomegalovirus disease (n = 12), adenovirus (n = 11), or posttransplant lymphoproliferative disorder (n = 12). Forty-two children (19 LSBTx) are alive. Weaning from parenteral nutrition was achieved after 42 days (median). Factors related to death or graft loss were pre-Tx surgery (P < .01), pseudo-obstruction (P < .01), age over 7 years (P < .03), fungal sepsis (P < .03), steroid resistant rejection (P < .05), hospitalized versus home patient (P < .01), and retransplantation (P < .05). Colon transplant did not affect the outcome. Interleukin-2 blockers improved isolated ITx (P < .05). Early referral and close monitoring of intestinal failure and related disorders are mandatory to achieve successful ITx.

Factors influencing outcome after intestinal transplantation in children

The chronic intestinal pseudo-obstruction syndrome (CIPOS) is characterized by repetitive episodes or continuous symptoms and signs of bowel obstruction, including radiographic documentation of dilated bowel, in the absence of a fixed lumen occluding lesion (1,2). This syndrome can occur in patients with underlying diseases associated with gastrointestinal manifestations (scleroderma, amyloidosis, hypothyroidism, etc.) or be secondary to water-electrolyte disorders (e.g. hypokalemia), and toxic, viral and parasitic causes. Most of the time, pediatricians deal with primary CIPOS, of which several histopathologic types and associated disorders have been described (3–8). Regardless of the histologic type (myopathy or neuropathy), CIPOS always involves alterations of smooth muscle contractile function, leading to abnormal intestinal tract peristalsis and finally nutritional disorders from both intestinal failure and recurrent sepsis. Accompanying uropathies must be sought in all patients with CIPOS (9). The clinical impact of these uropathies may be important requiring sometimes, vesicostomy. Some surveys of pediatric cases are published, including a large multi-center french study (10–11). From these studies one can consider that surgery is one of the mainstay of CIPOS therapeutic management. Indeed, surgery is performed in a variety of situations in pediatric patients with CIPOS, but indications must be appropriate and an excessive number of surgical procedures should be avoided. There is no consensus regarding indications and procedures. This short paper aims in discussing the main situations in which surgery may be required.

Surgery for pediatric patients with chronic intestinal pseudo-obstruction syndrome.

The first attempts at small bowel transplantation (SBT) in humans were disappointing1–3 and characterised by severe graft rejection in spite of initially encouraging experience in animals.3–5 Major difficulties were encountered as: (1) the intestine is an immune organ harbouring most immune competent cells of the body; and (2) it is particularly vulnerable to ischaemia, not allowing time consuming HLA matching prior to transplantation (Tx). Therefore, Tx of intestinal allografts into fully immune competent recipients results almost inescapably in terminal graft rejection. With the introduction of tacrolimus in the 1990s, this difficulty was partially overcome, improving small bowel graft survival rates (70% at one year post Tx).6–8 However, on follow up, a large number of grafts are still lost, raising the question of long term success and benefit after SBT. Here we present the first patient with a 17 year follow up after isolated …

/pdf/seventeen-years-after-successful-small-bowel-transplantation-4p870rp2eu.pdf

Seventeen years after successful small bowel transplantation : long term graft acceptance without immune tolerance

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