Rescue of a Living Donor with Liver Transplantation

Abstract

Postoperative liver failure is a rare complication after living donor liver resection. This is a case report of a 22-year-old healthy donor who was rescued with liver transplantation 11 days after right hemihepatectomy. Nine months later the patient is alive, and has fully recovered from his multiple organ failure. According to a review of the literature, there are four additional living liver donors, who received a liver transplant. Our own patient is the only survivor, so far. This case demonstrates that even in supposedly healthy living donors postoperative complications cannot be completely prevented. Although liver failure is rare in these patients, timely transplantation may need to be considered as the only life-saving treatment.

Introduction

Living donor liver transplantation (LDLTx) has become a life-saving operation for end-stage liver disease patients who cannot receive an organ from a deceased donor. Since LDLTx was introduced into clinical practice almost 20 years ago, probably over 15 000 procedures have been performed worldwide. Even before the first living donor liver transplant program was initiated in the United States, the pro's and con's, including the complex ethical issues were addressed, and concerns for the health and safety of the donor have always been of paramount importance (1).

There seems to be consensus within the international transplant community that living donation is an accepted modality to increase the number of organs for transplantation. At the same time, it is important to understand for both transplant professionals and patients that the principle of nonmaleficence—‘primum non nocere’—is obviously at variance with the practice of using living donors for organ transplantation (2).

In order to maintain truly informed consent and public confidence in this procedure, it is imperative to report all adverse events—mortality and morbidity—of living donors. Unfortunately, accurate data about donor mortality is unavailable. As we have documented recently, only 36% of living liver donor deaths mentioned or referred to in the transplant community are confirmed, that is directly reported and published by the transplant program where the mortality had occurred (3). Also, published morbidity rates have a very wide range between 0% and 67%, as mentioned in a review about current trends in live liver donation (4).

The need for liver transplantation of a living donor is recognized as possible complication. Although this seems to be a very rare event that probably nobody would like to talk about, it is a reality. We want to report a living donor who was rescued with a liver transplant in our own program, and we also reviewed the current literature for similar cases.

Case Report

A 22-year-old female suffering from cystic fibrosis (CF) with established liver cirrhosis and portal hypertension was referred to our institution in August 2006 by her pulmonologist and CF specialist who had followed her for many years. Preemptive liver transplantation was considered, prior to the development of hepatic and pulmonary decompensation, and to improve her lung function and nutritional status. Since she had a low MELD score of 11, living donation was brought up by the family who had already lost another daughter from variceal hemorrhage secondary to CF. The patient's mother was evaluated, but excluded for incidental discovery of hereditary hemorrhagic teleangiectasia.

The long-time friend of the patient, a 22-year-old male, volunteered to complete the evaluation process, as published previously in more detail (5). He was felt a medically and psychologically suitable candidate by the team and two independent donor advocates. A celiac artery stenosis identified on angiography was not considered a contraindication for donation, and the plan was to correct this abnormality. Both patients were prepared for elective surgery in July 2007.

The donor underwent right hemihepatectomy including segments 5–8 without the middle hepatic vein, using the waterjet dissector. Following transection of the hepatogastric ligament, the celiac axis was dissected with release of the compression by dividing the muscular and fibrous bands surrounding the supraceliac aorta. Total operating time was 8 h and 50 min. The patient had no replacement of red blood cells or fresh frozen plasma. The remnant left lobe volume was 527 mL, equivalent to 40% of his calculated preoperative total liver volume. At the end of surgery there was no hepatic congestion or vascular compromise.

His immediate postoperative recovery was uneventful, with aspartate and alanine transaminase peaks at 263 U/L, and 284 U/L, respectively. An ultrasound performed on postoperative day 2 showed homogenous echotexture of the liver and a small right pleural effusion. Because of clinical signs of acute abdomen, he was taken back to the operating room for exploratory relaparotomy 4 days later. We found a gastric perforation right below the gastroesophageal junction with acute peritonitis. There was no ischemic necrosis of the stomach or the liver. Following gastric repair and abdominal lavage the patient developed septic shock with acute renal and subsequent liver failure, as indicated by creatinine of 2.2 mg/dL, aspartate aminotransferase of 3483 U/L and lactate of 13.6 mg/dL. He necessitated intensive care treatment, support with artificial ventilation, multiple vasopressive agents, continuous veno-venous hemodiafiltration and broad-spectrum antibiotic coverage. Following two more abdominal washouts the peritonitis improved.

During this time his hepatic function worsened, with rising total bilirubin of 19.65 mg/dL and INR of 5.27. A liver biopsy performed after 1 week demonstrated submassive liver necrosis. Intraoperative Doppler Ultrasound showed patent hepatic artery and portal vein, and flow in all vessels. Assuming that the liver failure had been caused by shock, secondary to gastric perforation and sepsis, the donor was listed for transplantation as a status 1A. He received a full-size liver transplant from a deceased donor 11 days after his liver resection. The explanted remnant left liver lobe had a weight of 850 g, and pathology confirmed massive coagulative necrosis (Figure 1).

Figure 1. Left liver lobe remnant (segments 1–4) with multiple areas of ischemic necrosis 11 days after right hemihepatectomy.

Although the multiple organ failure resolved within 3 weeks subsequent to his liver transplant, the patient had a very complex intensive care course with several operating room visits. He had signs of persistent leukemoid reaction with a peak white blood cell count of 81.2 × 10³/μL 40 days post transplant. One of the major surgical problems was the abdominal wall opening that could not be closed primarily. There was significant visceral edema, and a size discrepancy between the liver graft weighing 1750 g and the recipient's abdominal cavity. After an attempt to reduce the incision with a V.A.C.^® dressing (Vacuum Assisted Closure System, KCI Licensing Inc, San Antonio, TX), the patient developed subcapsular necrosis of the caudal portions of his transplant, necessitating debridement resection of segments 3, 4B, 5 and 6. Following temporary packing, the abdomen was finally covered with absorbable polyglactin mesh (Vicryl^® polyglactin 910 mesh, Johnson & Johnson Gateway, Piscataway, NJ), and left to secondary wound healing (Figure 2).

Figure 2. Abdominal wound closure 28 days (before and after V.A.C.^® dressing), 109 days and 186 days after liver transplantation.

The patient was discharged from the hospital 79 days after liver transplantation. Nine months later, he has fully recovered from his liver and kidney failure as well as peritonitis. He continues with physical therapy, since he had developed critical illness neuromyopathy with distal weakness and bilateral foot drop. This has been described as a complication of systemic inflammatory response syndrome (6). The CF recipient had been discharged after 19 days without any complications, and she is already back to school as a full-time student.

Discussion

This is the first detailed description of a living donor who received a liver transplant. After a review of the literature, we identified four additional cases mentioned briefly in previous publications: however, only two were directly reported by the transplant program where the fatality had occurred.

According to the information available from Essen, in 2002 a 38-year-old father who was a donor for his 18-year-old son with Berardinelli-Seip syndrome, developed progressive liver insufficiency, and died due to cardiac failure 4 weeks after right hemihepatectomy, during a technically uncomplicated transplantation attempt. Reportedly, the donor had a preoperatively unknown autosomal recessive lipodystrophy syndrome (7).

In 2003, a 46-year-old mother died in Kyoto 9 months after right lobe donation to her daughter. The donor had a fatty liver diagnosed by preoperative computed tomography and ultrasound: histology revealed nonalcoholic steatohepatitis. The residual liver volume after right lobectomy with the middle hepatic vein was 28%. She developed postoperative liver failure, received an unsuccessful domino liver transplant from a donor with a metabolic disorder, and died 4 months later without regaining consciousness (8).

A report of the European Liver Transplant Registry mentioned four donor deaths (9). One of these patients, a 57-year-old mother and right lobe donor, developed postoperative heart failure, and died after 32 days following an unsuccessful liver transplantation (Scheele J, Jena, personal communication). In a survey of liver transplantation from living donors in the United States, one donor was put on the waiting list 5 days after donation, and received a deceased donor liver 3 days later because of a diagnosis of Budd-Chiari syndrome. Further details and outcome were not reported (10).

What can be learned from these patients? So far, five living liver donors have received a liver transplant, including our own patient: four out of five reportedly after right lobe donation. All of the donors developed postoperative liver failure, which was apparently caused by preexisting liver abnormalities in two, and surgical complications in two other cases, respectively. In three donors, the attempted transplant failed. In one the outcome is unknown. At this time our own donor is the only survivor with a follow-up of 9 months.

According to our recent worldwide review on living liver donor mortality, the majority of donor deaths seem to be directly related to the procedure, including unidentified preoperative medical conditions and postoperative surgical complications (3). We are not suggesting that individual deaths were preventable, but one or the other of these unfortunate patients might eventually have been rescued with transplantation.

It appears that timely decision for the need of a liver transplant in these donors is crucial for its success. In two of the cases published, the transplant was done 1 and 5 months after resection, respectively, and both patients did not recover. Our patient was listed as a status 1 eight days after liver resection, and was transplanted 3 days later. Although he had a stormy course with a long intensive care stay, he fully recovered. As we had observed previously, patients with progressive acute hepatic failure, especially subsequent to liver resection or transplant, can develop ‘toxic liver syndrome’, and have a significant risk of infection leading to death from sepsis despite successful transplantation. The only chance to rescue patients in this extreme condition is to perform the hepatectomy and liver transplantation before the onset of terminal and irreversible multi-organ failure (11). Obviously, this is not only a medically, but also ethically very difficult decision to make in a supposedly healthy volunteer donor. In addition, acute hepatic failure secondary to sepsis is typically not considered an indication for liver transplantation.

Ideally, a living donor should be completely healthy, and have normal hepatic function and anatomy. As we and others have shown, not all candidate donors meet the expectation to be ‘perfect’ (12). With regard to this particular case, there are two questions that need to be addressed: should the donor have been rejected based on the anatomic condition, and did the additional surgery cause or contribute to his postoperative complication?

In fact, there are several living donors with median arcuate ligament syndrome reported in the literature that had uneventful liver resections (13,14). Our patient had a nearly complete celiac artery occlusion during expiration, and although he was asymptomatic, we decided to transect the lateral crura to prevent any compromise to the hepatic arterial flow or accentuation of the effects of celiac axis compression, as had been observed previously in liver transplant recipients (15,16). The access to the supraceliac aorta we used in this patient is our standard technique in liver transplantation whenever there is a need for complex arterial reconstruction or conduits to the abdominal aorta. Even in patients with multiple previous hepatobiliary surgeries or retransplants, gastric perforation has been extremely rare. Whether the injury may have been caused by a retractor or placement of the nasogastric tube is unclear. Although we cannot rule out that this complication was related to release of the arcuate ligament, it was completely unexpected and inconceivable.

It remains an open question how to balance the risk and benefit of LDLTx, and where to draw the line. There is agreement that a recipient of a living donor organ must have an indication for liver transplantation from a deceased donor, and LDLTx should not be performed in patients not meeting minimal listing criteria. Our recipient had end stage liver disease secondary to CF with a MELD score of 11. Preemptive liver transplantation is suggested as the ideal management in these patients to prevent the progression of lung and liver disease (17). Based on personal experience as well as data from other centers, we feel comfortable about the indication for transplant in this patient—despite her low MELD score. With regard to the living donor, who became an involuntary recipient, of course, our team is not proud of the need for liver transplantation.

Since our patient is not the only donor in the world who subsequently received a liver transplant, but evidently the only survivor, we believe that it is very important to report the circumstances and the details of this case. As emphasized before, all living donor complications must be fully documented, and disclosed to our community by the transplant centers involved, no matter how painful this may be. Potential recipients, candidate donors, their families and transplant professionals have to understand that there may be a price to pay for the ultimate act of altruism, and the most precious gift—life.

Acknowledgments

In compliance with the living donor adverse outcome reporting requirement implemented by the United Network for Organ Sharing (UNOS) as of June 26, 2007, this case was officially reported to UNOS. After review by three experts and the Membership and Professional Standards Committee, it was decided that there were no OPTN policy violations or patient safety issues identified.

The liver transplant team of Drexel University College of Medicine at Hahnemann University Hospital wants to acknowledge everyone involved in the challenging care of this patient, and thank all those who contributed to his fortunate outcome, especially the nurses of the surgical intensive care unit. We greatly appreciate the generosity and attitude of the donor, and also his family who was exceptionally supportive during the patient's incredible journey to the edge and back.