Polycystic liver disease (PLD) is a genetic disorder that causes benign cysts in the liver. The disorder can lead to an enlargement of the liver and eventually even liver failure. While the occurrence of PLD is estimated to be around 1 in 10,000 people, most individuals who have the disorder are asymptomatic and it is only discovered incidentally through imaging tests such as ultrasonography, computed tomography (CT) scan, or magnetic resonance imaging (MRI). In rare cases, the enlargement of the liver due to PLD can cause abdominal pain, distension, and compression of adjacent organs, shortness of breath, postprandial fullness, esophageal reflux, and back pain.
Recently, doctors at Global Hospitals in Parel, Mumbai successfully removed an enlarged liver weighing 7 kgs and 40 cm in size from a 46-year-old woman with PLD. The woman had been suffering from severe abdominal pain, bloating, and discomfort for 15 years. Her liver had enlarged due to multiple cysts, leading to an expanding abdominal girth. The doctors then transplanted a healthy liver from a deceased donor and saved the patient’s life.
Dr Gaurav Chaubal, Director of Liver, Pancreas, Intestine Transplant Program, and Hepato-Pancreato-Biliary Surgery at Global Hospitals, led the team that conducted the challenging liver transplant. He noted that the availability of state-of-the-art operating theaters and postoperative intensive care units, along with technical expertise, is crucial to ensure the smooth recovery of such patients.
In most cases, PLD does not require a liver transplant. It is only needed in very few cases when the liver size is huge and there is liver failure. Individuals with PLD can receive medical and surgical therapies such as cyst fenestration and hepatic resection. Liver transplant is considered a last resort.
It is essential to perform a timely transplant on patients with PLD if their liver function is diminished due to liver failure, which can have potentially fatal complications if left untreated. When liver function is low, and the liver is severely enlarged due to multiple cysts, a cadaveric liver transplant may be necessary.
In the case of the woman from Surat, a timely transplant was essential as she was suffering from liver failure due to multiple cysts. When doctors performed the open laparotomy, they found a 1-foot-wide enlarged liver, which had to be replaced by a healthy liver from a deceased donor. Dr Chaubal stated that this was the largest explant that had been removed across India. Even though the size of the liver was huge, the team was able to remove it intact, and the woman recovered well. She was discharged from the hospital after two weeks.
The patient expressed her gratitude to the family of the donor who came forward for organ donation and feels fortunate that she received a timely transplant. She also emphasized the importance of organ donation and how it can save lives.
To sum up, while PLD is a rare disorder, it can cause severe complications and lead to liver failure. Liver transplant is only considered a last resort, but it can be life-saving for individuals suffering from PLD who have severe liver enlargement or are in liver failure. It is crucial to perform timely transplants on such individuals, and the availability of state-of-the-art medical facilities and technical expertise is essential to ensure a smooth recovery. Finally, organ donation can save lives and is critical to meeting the demand for lifesaving transplants.
