Liver Weighing 12kg... Severance Hospital Successfully Performs Liver Transplant on Rare Disease Patient

60s Patient with Enlarged Liver Due to 'Polycystic Liver Disease'
Recovers Normal Function Through Transplantation

A patient with polycystic liver disease whose liver weighed more than 12 kg, over 10 times the normal liver weight, successfully underwent a transplant surgery and regained health.

Professor Lee Jae-geun (second from left), Department of Transplant Surgery at Severance Hospital, and Ms. Kim Ok-hee (third from left) are undergoing the final test of liver transplant function and taking a commemorative photo with the medical staff.
[Photo by Yonsei Medical Center]

Professor Lee Jae-geun (Transplant Surgery) of the Liver Transplant Team at Severance Hospital Organ Transplant Center announced on the 12th that the living-donor liver transplant surgery was successfully completed for Kim Ok-hee (61), a patient with polycystic liver disease. The results of this surgery were presented at the international academic conference "HPB Surgery Week 2023," hosted by the Korean Association of Hepato-Biliary-Pancreatic Surgery held in Busan.

Kim was diagnosed with liver cysts over 10 years ago, and her condition worsened in 2020, leading her to visit Severance Hospital. Her abdomen was protruding, and the liver was abnormally enlarged to a visible extent. Her complexion was poor, and except for her abdomen, she appeared noticeably thin. After examination, Kim was diagnosed with polycystic liver disease.

Polycystic liver disease is a rare disorder in which waste products in the body are not expelled and cluster to form cyst-like masses, with more than 20 such cysts developing throughout the liver. These cysts continue to grow, impairing liver function. The liver weight of a healthy adult is about 1.2 to 1.8 kg, but in polycystic liver disease, cysts attach to the liver, increasing its weight more than tenfold. As symptoms worsen, ascites, abdominal pain, and vomiting may occur.

Due to the excessively enlarged cysts, Kim was unable to eat and experienced difficulty breathing, leading the medical team to decide on transplantation. However, since Kim’s condition was poor to wait for a donor, they checked the possibility of living-donor liver transplantation with her children. Her son carried the gene for polycystic liver disease and could not donate, while her daughter was eligible for living donation but had a different blood type. To perform a blood type-incompatible liver transplant, the infectious disease and diagnostic laboratory medicine departments collaborated to administer various vaccinations and reduce antibody secretion to lower the risk of transplant rejection.

Professor Lee Jae-geun is holding the liver removed from the patient during surgery. [Photo by Yonsei Medical Center]

Still, immediate transplantation was impossible due to poor vascular conditions caused by cysts scattered throughout the liver. Typically, liver transplantation involves clamping the inferior vena cava (the vein carrying blood from the legs) connected to the liver and removing the liver. However, in Kim’s case, clamping the inferior vena cava could cause unstable blood pressure and heart rate, and in severe cases, rupture of blood vessels leading to death. Therefore, Professor Lee used ECMO (extracorporeal membrane oxygenation) to directly divert blood from the inferior vena cava to the heart. The use of ECMO in liver transplantation is very rare because inserting cannulas carries a risk of vascular damage, increasing surgical difficulty. Fortunately, the surgery was completed safely, Kim was discharged in December last year, and recent check-ups confirmed that the transplanted liver is functioning normally.

Liver transplantation cases for polycystic liver disease are known to be extremely rare. Last year, Keio University School of Medicine in Japan successfully performed a liver transplant on a patient with polycystic liver disease. At that time, the patient’s liver weighed 10 kg, and the surgery took 18 hours. The amount of blood used reached 48,800 cc. In contrast, Kim’s liver weighed 12.1 kg, accounting for 25% of her body weight, but Professor Lee’s surgery lasted 11 hours and required only about 200 cc of blood transfusion. Compared to Keio University, the surgery time was reduced by 40%, and the amount of blood used decreased by 99.6%, reducing the patient’s burden and increasing surgical safety.

Professor Lee said, "Polycystic liver disease, a rare condition where the liver abnormally enlarges, has few surgical cases domestically. This successful surgery involved challenges such as different blood types between donor and recipient and the use of ECMO, but it was achieved through multidisciplinary cooperation among medical staff and the trust and cooperation of the patient and her family."

This content was produced with the assistance of AI translation services.