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Intestinal Transplantation in Children With Intestinal Failure


The introduction of tacrolimus and the development of the abdominal multiorgan procurement techniques allowed the tailoring of various types of intestine grafts that can contain other intraabdominal organs, such as the liver, pancreas, and stomach. The understanding that the liver protects the intestine against rejection demonstrates the interaction between recipient and donor immunocytes (host-versus-graft and graft-versus-host) which under the cover of immunosuppression allows varying degrees of graft acceptance and eventual minimization of drug therapy. Over the past several years the number of patients placed on the list for and those undergoing intestinal transplantation has decreased, which may be a result of (1) improvements in the care of patients with intestinal failure under a multidisciplinary intestinal care team management, (2) the introduction of new lipid management strategies for the treatment of cholestatic liver disease, and (3) corrective surgery enhancing absorptive surface and motility, which has led to increased survival and decreased morbidity.

Indications for Intestinal Transplant

Intestinal failure describes a patient who has lost the ability to maintain nutritional support and adequate fluid requirements, needed to sustain growth, with their own intestine and is permanently dependent on total parenteral nutrition (TPN). The majority of these patients have short bowels as a result of a congenital deficiency or acquired condition (see Chapter 364.07 ). In others, the cause of intestinal failure is a functional disorder of motility or absorption ( Table 365.1 ). Rarely do patients receive intestinal transplants for benign neoplasms. The complications of intestinal failure include loss of venous access, life-threatening infections, and TPN-induced cholestatic liver disease.

Table 365.1
Causes of Intestinal Failure in Children Requiring Transplantation
SHORT BOWEL

  • Congenital disorders

  • Volvulus

  • Gastroschisis

  • Necrotizing enterocolitis

  • Intestinal atresia

  • Trauma

INTESTINAL DYSMOTILITY

  • Intestinal pseudoobstruction

  • Intestinal aganglionosis (Hirschsprung disease)

ENTEROCYTE DYSFUNCTION

  • Microvillus inclusion disease

  • Tufting enteropathy

  • Autoimmune disorders

  • Crohn disease

TUMORS

  • Familial polyposis

  • Inflammatory pseudotumor

Paucity of Venous Access

Administration of TPN requires the insertion of a centrally placed venous catheter, there being only 6 readily accessible sites (bilateral internal jugulars, subclavians, iliac veins). The loss of venous access generally occurs in the setting of recurrent catheter sepsis and thrombosis; clinical convention suggests that loss of 50% of these venous access sites places the patient at risk of not being able to be treated with TPN.

Life-Threatening Infections

Life-threatening infections are usually catheter-related; the absence of significant lengths of intestine may be associated with abnormal motility of the residual bowel (producing both delayed or rapid emptying), with varying degrees of bacterial overgrowth and possible bacterial or fungal translocation as a consequence of loss of intestinal barrier function and/or loss of gut immunity. This situation can produce cholestatic liver disease, multisystem organ failure, and metastatic infectious foci in lungs, kidneys, liver, and the brain.

Liver Disease

The development of cholestatic liver disease is the most serious complication of intestinal failure and may be a consequence of the toxic drug effects of TPN on hepatocytes, a disruption of bile flow and bile acid metabolism, and the frequent occurrence of bacterial translocation and sepsis with endotoxin release into the portal circulation. This complication varies in frequency depending on the patient's age and the etiology of the intestinal failure; it is most common in neonates with extreme short gut. The effects on the liver include fatty transformation, steatohepatitis and necrosis, fibrosis, and then cholestasis. The development of clinical jaundice (total bilirubin > 3 mg/dL) and thrombocytopenia are significant risk factors for poor outcome, because these changes portend the development of portal hypertensive gastroenteropathy, hypersplenism, coagulopathy, and uncontrollable bleeding.

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