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Current Anesthetic Management in a 20-Month-Old Pediatric Patient With Intestinal Transplantation Due to Microvillous Inclusion Disease

Intestinal transplantation is a complex procedure both in terms of anesthesia and surgery. In particular, pediatric anesthesia management during intestinal transplant surgery can become even more complicated. It has been stated that propofol, remifentanil, and sevoflurane reduce patient mortality by reducing the incidence of intestinal ischemia-reperfusion injury. Although studies of these agents continue to be conducted in vivo or in vitro, these anesthetics are currently used for specific procedures that have a high risk of incurring ischemia-reperfusion injury. Herein, we present the case of a male child, aged 20 months, who was dependent on total parenteral nutrition and was found to have intestinal failure associated with liver disease type 1. Hematologic tests showed findings of anemia and metabolic acidosis. Propofol was administered for induction of anesthesia. Anesthesia maintenance was achieved using sevoflurane with remifentanil infusion. We ensured safe and adequate vascular access in the patient and performed hematologic and biochemical tests with detailed system controls. Before the procedure, we prepared a leukocyte-poor erythrocyte suspension, leukocyte-poor random or apheresis platelets, and ABO- and Rh-compatible fresh frozen plasma. We monitored for signs of acidosis, hypotension, coagulation disorders, and hyperkalemia during the reperfusion period. We maintained patient normothermia. In this case report on the anesthetic management of a pediatric patient aged 20 months who received a small bowel transplant due to microvillous inclusion disease, we found that the selection of anesthetic agents may affect the prognosis of future surgical procedures.

Key words : Intestinal failure, Pediatric anesthesia, Total parenteral nutrition


In patients with intestinal failure due to congenital or acquired causes, their life is completely dependent on total parenteral nutrition (TPN). Because the use of TPN is associated with increased morbidity and mortality, a small bowel transplant is considered a means of treatment for patients with intestinal failure.1,2

As in any other type of surgery, the anesthetic agents used in small bowel transplant procedures are generally not specific to the procedure type. However,  the potential positive effect of anesthetic agents used in particular surgical procedures, as shown in in vitro and in vivo studies, have resulted in a proliferation of research on this subject. Remifentanil, propofol, and sevoflurane are 3 anesthetic agents thought to prevent or reduce ischemia-reperfusion injury in small bowel transplant procedures. These anesthetics are considered to be effective, through complex mechanisms and pathways, in preventing ischemia-reperfusion injury.

Case Report

A male child was born by caesarian section during his 36th gestational week with a weight of 2960 grams. The boy was diagnosed with polyhydramnios and microvillous inclusion disease in the antenatal period. As a result, he was placed on TPN. He was monitored in the intensive care unit for 239 days because of recurrent and persistent diarrhea after birth, and he experienced 3 episodes of septic shock in addition to multiple Klebsiella pneumoniae-induced uroseptic shock episodes for approximately 1.5 months.

Biochemical tests revealed an alanine amino­transferase of 130 IU/L, an aspartate aminotransferase of 53 IU/L, an alkaline phosphatase of 535 IU/L, and a gamma glutamyl transferase of 85 U/L, which were all elevated values. Hematologic tests revealed findings consistent with anemia and metabolic acidosis: hemoglobin 8.5 g/dL, pH 7.35, partial pressure of carbon dioxide 26.7 mm Hg, partial pressure of oxygen 173 mm Hg, bicarbonate (HCO3) 16.6 mEq/L, and base excess of -10.1 mmol/L. The patient was diagnosed with intestinal failure-associated liver disease type 1.

A transplant surgery was performed, and the patient received an intestinal organ graft from a deceased donor of similar weight and height who had brain death due to trauma. During the procedure, the patient was monitored via pulse oximetry, 3-way electrocardiogram, and noninvasive arterial blood pressure measurements. A single-lumen Hickman port catheter was inserted into the left subclavian vein. Because the single-lumen Hickman port catheter would not be of sufficient size for anesthesia administration during the postinduction phase, a 4F, 8-cm, double-lumen, central venous catheter was inserted from the right vena jugularis interna using ultrasonography guidance. Before the induction of anesthesia, the patient was administered 50 μg/kg of midazolam through the central venous catheter. After induction with 2.5 mg/kg propofol, 0.6 mg/kg rocuronium, and 1 μg/kg fentanyl, intubation was established with a size 4 uncuffed endotracheal tube. Maintenance of anesthesia was achieved with infusion of a 50-50% oxygen-air mixture, 2% to 3% sevoflurane, 0.25 μg/kg/min remifentanil infusion, and intermittent 0.2 mg/kg rocuronium. Throughout the operation, ventilation was adjusted to maintain end-tidal carbon dioxide at 33 to 35 mm Hg. Ultrasonography-guided right femoral artery cannula­tion was achieved for invasive arterial pressure measurement.

During the surgery, the patient was given antithymocyte globulin infusions at a rate of 20 mL/h and a concentration of 25 mg/100 mL, 20% human albumin at a rate of 100 mL/h and a concentration of 20 g/500 mL, teicoplanin at an infusion rate of 100 mg/h, methylprednisolone at 30 mg every 2 hours, and ganciclovir at 35 mg every 30 minutes. As a maintenance fluid, 85 mL/h of 5% dextrose in 0.9% sodium chloride solution was provided. When a hemoglobin level of 8.3 g/dL was detected via arterial blood gas, a leukocyte-poor erythrocyte suspension infusion of 10 mL/kg was initiated. At the end of the surgery, the patient’s hemoglobin was 9.3 g/dL. The patient developed metabolic acidosis during revascularization; a base excess of -6.4 mmol/L with a pH of 7.28 was detected. As a result, the patient was treated with 15 mEq of sodium bicarbonate. There was no hyperkalemia detected. At that point, after arterial pressure dropped to 70/40 mm Hg, 5 μg/kg/min of dopamine and
0.05 μg/kg/min of noradrenaline infusion were initiated. When the patient had hemodynamically stabilized, noradrenaline infusion was stopped, and dopamine infusion was continued. The surgery lasted for 4 hours and 35 minutes from the time of anesthesia induction to the patient’s exit from the surgical suite. The patient was transferred to the pediatric intensive care unit under endotracheal intubation. He was hemodynamically stable with the support of a mechanical transport ventilator.


In patients who are scheduled to receive a small bowel transplant, it is necessary to perform hematologic and biochemical tests and to prepare blood and blood products before surgery. For blood and blood product preparation, a leukocyte-poor erythrocyte suspension, random or apheresis platelets, and ABO- and Rh-compatible fresh frozen plasma are preferred. Human leukocyte antigen class I and II moleculesfound in human T leukocytes can cause a severe immune response to develop in the recipient.3,4

When random or apheresis platelets are exposed to a single donor antigen, the risks of alloimmunization are the same. In pooled platelets, the risk of alloim­munization increases in direct proportion to the number of donors of the pooled platelets; the higher the number of platelet donors, the greater the exposure of the recipient to various platelet antigens. If random platelets are chosen for patients receiving a small bowel transplant, reduction of leukocytes (leukofiltration) should be performed. If an apheresis platelet suspension is chosen, however, leukocytes are also filtered; therefore, there is no need for leukofiltration.5

Adequate and safe venous access is required for fluid, drug, blood, and blood products to be transfused during surgery. Invasive arterial blood pressure monitoring via arterial catheterization is performed for instantaneous arterial pressure and blood gas measurements. However, in pediatric patients, particularly those receiving TPN, there are often limitations to arterial and venous access. As a result, it is often necessary to use ultrasonography to ensure venous access. Pediatric patients also experience greater heat loss during surgery than adults. Temperature monitoring should be done, heaters should be used, and fluid, blood, and blood products should be heated as well. Efforts should be made to maintain normothermia in order to avoid the cardiac, coagulation, and hemodynamic depressive effects of hypothermia. Throughout the procedure, the patient should be maintained on antibiotic, antifungal, immunosuppressant, and albumin regimens. Acid-base and electrolyte imbalances should be monitored with regular arterial blood gas evaluations.

Before reperfusion, mean arterial pressure should be greater than 60 mm Hg, central venous pressure should be approximately 12 to 15 mm Hg, and urine output should be 0.5 mL/kg/h. During reperfusion, mean arterial pressure should be greater than 60 mm Hg and central venous pressure approximately 8 to 12 mm Hg. During this period, blood volume, inotropic (dopamine, noradrenaline) support, erythrocyte suspension, fresh frozen plasma, and platelet infusion may be required; furthermore, albumin support may be increased. Hyperkalemia and coagulation disorders should be rectified with the introduction of the graft preservation solution into the circulation. Coagulation disorders may develop due to hemodilution, bleeding, or ischemia-reperfusion or because of the type of immunosup­pressive agents used. Ideally, the cause of the disorder is determined specifically by viscoelastic tests such as thromboelastography androtational thromboelastometry. However, standard tests can also be applied.

Anesthetic agents used in small bowel transplant procedures are not specific to these surgeries. However, the research on procedure-specific anesthetic agents is increasing. Some studies have found that propofol reduces mortality by reducing ischemia-reperfusion injury through the inhibition of nicotinamide adenine dinucleotide phosphate oxidase and nuclear factor κB and the activation of phosphoinositide 3-kinase.6-8

In small bowel transplant surgeries, remifentanil is thought to have positive effects in preventing or reducing ischemia-reperfusion injury. Study results have suggested that remifentanil can prevent ischemia-reperfusion injury by lowering serum levels of interleukin 6, interleukin 8, C-reactive protein, tumor necrosis factor α, and oxidative stress.9,10

Sevoflurane protects against ischemia-reperfusion injury through the activation of phosphoinositide 3-kinase and peroxisome proliferator-activated receptor γ. These anesthetic agents have anti-inflammatory and antioxidant effects; furthermore, they have the potential to produce positive effects on the prognosis of ischemia-reperfusion injury.11,12


In this case report, the choice of anesthetic agents used in the small bowel transplant surgery of a male child aged 20 months is explained. The indication for the transplant procedure was a diagnosis of microvillous inclusion disease; the transplant graft originated from a deceased donor. The use of propofol, remifentanil, and sevoflurane during intestinal transplant surgery may be associated with a reduction in intestinal ischemia-reperfusion injury. Future randomized, prospective, and large-scale studies are needed to investigate this potential association.


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DOI : 10.6002/ect.2020.0385


From the University of Health Sciences, Izmir Tepecik Training and Research Hospital, Department of Anesthesiology and Reanimation, Izmir, Turkey
Acknowledgements: The authors have not received any funding or grants in support of the presented research or for the preparation of this work and have no declarations of potential conflicts of interest.
Corresponding author: Yucel Karaman, University of Health Sciences, Izmir Tepecik Training and Research Hospital, Guney Mah. 1140/1 Sok. No:1 Yenisehir, Izmir, 35120, Turkey
Phone: +90 533 5617137

Experimental and Clinical Transplantation (2021)