Polycystic Liver Disease (PLD) can cause enlargement of liver and even liver failure. But this genetic disorder is usually asymptomatic.
Polycystic liver disease (PLD) is a rare, inherited condition leading to benign (noncancerous) cysts. The occurrence of this genetic disorder is estimated to be around 1 person per 10000. It can cause enlargement of liver and eventually liver failure.
Recently, doctors at Global Hospitals, Parel, Mumbai successfully removed an enlarged liver weighing 7 kgs and 40 cm in size from a 46-year-old woman with Polycystic Liver Disease (PLD). They then transplanted a healthy liver received from a deceased donor. Dr Gaurav Chaubal, Director of Liver, Pancreas, Intestine Transplant Program, and Hepato-Pancreato-Biliary Surgery at Global Hospitals, and his team successfully conducted the challenging liver transplant and saved the patient’s life.
Symptoms of Polycystic Liver Disease (PLD)
The woman, a resident of Surat, was unable to lead a normal life due to severe abdominal pain, bloating, and discomfort due to Polycystic Liver Disease (PLD). She had been going through these symptoms for about 15 years.
Most patients with Polycystic Liver Disease are asymptomatic and it is diagnosed incidentally on imaging such as ultrasonography, computed tomography (CT) scan, or magnetic resonance imaging (MRI). However, in a few cases, enlargement of the liver can lead to abdominal pain, distension, and compression of adjacent organs, shortness of breath, postprandial fullness, esophageal reflux and back pain, potentially affecting the quality of life.
Treatment of Polycystic Liver Disease include medical therapy, surgical therapy (such as cyst fenestration, hepatic resection), and liver transplant (as a last resort).
When liver transplant is required?
In most cases, Polycystic Liver Disease (PLD) doesn’t require a liver transplant. It is only needed in very few cases when the liver size is huge and there is liver failure, according to Dr Chaubal.
In this case, the patient was recommended a cadaveric liver transplant as her liver had enlarged due to multiple cysts, leading to expanding abdominal girth.
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It was essential to perform a timely transplant on her as her liver function was low owing to liver failure, which can have potentially fatal complications, if not treated in time, Dr Chaubal said.
When they did the open laparotomy, they found a 1 feet wide enlarged liver. Dr Chaubal stated, “This is the largest explant that has been removed across India and we replaced it with the healthy liver from the deceased donor.”
It was quite a challenging task as the size of the liver was huge and the chances of the patient bleeding profusely and dying were very high if the explant was not removed as a whole. The woman recovered well and was discharged in two weeks, he added.
He noted that the availability of state of art OTs and post operative ICUs along with the technical expertise is necessary to ensure smooth recovery of such a patient.
The patient feels fortunate that she got a timely transplant and expressed gratitude to the family of the donor who came forward for organ donation. “Remember that organ donation can save lives,” she stated.
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