Case Study

A male infant (15 months of age) with SGS secondary to a midgut volvulus diagnosed at 1 month of age presented at the transplant center for evaluation following referral from his local gastroenterologist because of worsening hepatic dysfunction, line sepsis, and total dependence on TPN. Following a 5-day inpatient evaluation, he was accepted as a candidate for a combined intestinal-liver transplant and was listed with UNOS. At the time of evaluation, the patient's parents had 3 children; he was the middle child. They were a stable, intact family with a strong support system. The father worked full-time and the mother stayed home with the children. They had an excellent understanding of the transplant process and its challenges as well as their responsibilities for his care.

The patient was fairly stable while waiting for his transplant and spent most of that period at home. Medical management and care consisted of daily TPN infusions, enteral feedings as tolerated, weekly labs, and routine follow-up appointments with his local physician. He received a transplant after waiting 3 months and tolerated the surgery well. The donor was CMV seropositive, but EBV serologies were not completed. He was seronegative for CMV and EBV.

The surgical procedure consisted of anastomoses of the native jejunum to the donor jejunum with a Roux-en-Y loop for the bile duct anastomosis and the donor ileum to the native transverse colon. Gastrostomy and jejunostomy tubes were placed. Ultrasound examinations of the liver were normal for 5 days with patent vessels, no biliary dilatation, and no fluid collections. A small right pleural effusion was detected, but this resolved without further complications. The first endoscopy and biopsy were performed on postoperative day (POD) #6 and revealed a normal graft without rejection. An upper GI and gastrografin swallow study was completed on POD #8 to verify that the intestinal anastomoses were intact. No leaks were detected, so enteral feedings were initiated on POD #9. He did not experience any surgical complications and was transferred to the Transplant Unit from the pediatric intensive care unit (PICU) on POD #10.

He was recovering well and tolerating jejunostomy feedings. However, on POD #12 he developed a fever of 39.4° C. A chest x-ray showed mild congestion and a kidney, ureter, and bladder x-ray was normal. Viral and bacterial cultures were negative. An endoscopy revealed friable mucosa and the biopsy showed severe acute rejection. Due to the severity of rejection, he was transferred to the PICU for administration of OKT3. Immunosuppression was also optimized with IV solumedrol and TAC to maintain a trough level of 20 ng/mL. He also received 5 doses of daclizumab. An endoscopy with biopsy was repeated during this therapy on POD #16 and POD #20. By POD #23, the graft had responded well to increased immunosuppression and there was no rejection present on biopsy. He received 10 doses of OKT3. Viral testing for EBV and CMV were negative. He received an extended course (26 days) of ganciclovir for CMV and EBV because of the donor-recipient mismatch and increased immunosuppression with OKT3.

He tolerated an advancement in enteral feedings with Peptamen Jr., and his oral intake also improved. TPN and bowel decontamination medications were discontinued on POD #25.

On POD #23, his serum bilirubin increased and mycophenolate mofetil (MMF) was added to his regimen. Cholangitis was suspected when he spiked a fever, and antibiotics were initiated. His serum bilirubin increased from 7.1 mg/dL to 14.1 mg/dL within 4 days. Although a liver biopsy was not obtained, an intestinal biopsy revealed no rejection. Phenobarbital was ordered and resulted in slow improvement of liver function tests.

On POD #26, his stomal output increased from about 400 cc daily to 700 cc daily. Since an endoscopy on the previous day was normal, loperamide was ordered to decrease intestinal motility. He responded well to this medication, and his stool output stabilized with 450 cc output from the ileostomy and approximately 6 stools per rectum daily.

On POD #28, he experienced some stomal bleeding. An ileoscopy revealed normal mucosa with bleeding from a previous biopsy site. He was subsequently given a platelet transfusion for a platelet count of 57,000, after which the bleeding resolved.

He was discharged from the hospital to the local area on POD #31 and was alert, active, and playful. He was afebrile and viral studies were negative. He was tolerating an oral diet and Peptamen Jr at 50 cc/hour for 20 hours daily. Stool output was stable. He received IV fluids for 12 hours daily to maintain electrolyte balance. His discharge medications are listed in Table 3 .

Following discharge to the local area, he continued to do well and was seen in the transplant clinic twice a week. Surveillance endoscopies and biopsies were normal with no further rejection noted, and his serum bilirubin continued to decline. About 3 weeks after discharge, he was readmitted to the hospital with fever and was found to have a line infection. There was no rejection on biopsy and he was treated with a 14-day course of IV antibiotics.

He was discharged to home 63 days after transplantation. Immunosuppressive management included TAC to maintain a trough level of 15 ng/mL, prednisone 10 mg daily, and MMF 100 mg twice daily. His serum bilirubin continued to be elevated, and a liver biopsy revealed cholestasis without rejection. It was subsequently thought that this was donor-related since he received neonatal organs.

He continued to do well at home. Over the next 3 months, the TAC level was maintained between 10 and 15 ng/mL, MMF was discontinued, and prednisone was decreased to 5 mg daily. He had 1 line infection requiring IV antibiotics. The cholestasis resolved and liver function tests returned to normal within 2 months. Viral studies remained negative.

He returned to the transplant center at 6 months posttransplantation for an elective closure of the ileostomy. He tolerated this procedure well with no complications and was hospitalized for 4 days. An endoscopy with biopsy at this time was normal. Tube feedings were restarted on POD #3 after GI motility resumed. At discharge, he was tolerating full feedings and did not require IV fluids.

At 13 months posttransplantation, his EBV-PCR was elevated to 100 viral copies/105 lymphocytes. No therapy was initiated although his TAC level was maintained at around 10 ng/mL and prednisone was decreased to 2.5 mg daily. A repeat EBV-PCR 1 month later was elevated to 200 viral copies/105 lymphocytes. An endoscopy revealed an ulcer that was EBER-positive and therapy was begun with Cytogam and ganciclovir. On a repeat biopsy 2 weeks later, it was noted that there was no rejection or evidence of EBV or PTLD. Antiviral therapy was continued for another 2 weeks until all viral studies were completed.

At 18 months posttransplantation, he is doing very well. Lab studies are within normal limits and he has had no rejection. The EBV-PCR is monitored monthly and has been 100 viral copies/105 lymphocytes. He is currently a very active and developmentally appropriate 4-year-old who is growing well. Labs are obtained monthly and routine endoscopies are completed every 6 to 12 months. He is taking 10 oral medications, and all medical appliances have been removed. He receives all his calories through a regular oral diet. His father has returned to work full time, and his mother remains at home with the children. She states that her son is doing well and she feels the family has adapted well to his routine.

Case Summary

From all parameters, this patient's outcome following intestine-liver transplantation is

regarded as successful. He was referred early to the transplant center, before his condition deteriorated due to sepsis, liver failure, and limited venous access. He has a stable, cooperative, and knowledgeable family with a strong support system. To his great advantage, his waiting time for transplantation was only 3 months, considerably less than the center's average waiting time. During the waiting period, he was medically and nutritionally stable and had infrequent infections. Another benefit to his stable condition was that he was able to be at home, where the environment significantly contributed to his cognitive, social, and physical development.

He tolerated the surgery well, without any major complications and was in the PICU for less than 2 weeks. Feedings were advanced without complications and he eventually received all calories by mouth. Oral aversion, a common impediment in infants such as this patient, was not a problem.

He did encounter 2 typical complications of intestine transplantation: severe acute rejection requiring increased immunosuppression and a mild EBV infection. In both cases, he responded well to standard therapy without further complications.

The initial hospitalization was 31 days, less than average, and he had only a few brief readmissions for line infections, endoscopies, and stoma closure. His long-term outcome at nearly 2 years posttransplantation has been excellent. Medical routines and daily care have decreased with respect to the number of medications, appliances, and interventions. He continues to develop normally.

Intestine transplantation is a challenging strategy for end-stage SGS. With improvements in medical management strategies and treatments, a greater number of recipients are surviving this procedure with acceptable outcomes.

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