A 35-year-old man, who had a rare genetic blood disease, underwent a combined surgery – a liver transplant and artificial large blood vessel reconstruction – at a private hospital in the city.
The man, who was from Jordan, had paroxysmal nocturnal haemoglobinuria (recurrent propensity for clotting and lysis of blood) and Budd-Chiari syndrome, a disorder characterised by obstruction of hepatic venous outflow and the inferior vena cava reaching up to the heart and end-stage liver failure, according to a press release.
Thiagarajan Srinivasan, director, Liver Transplant, MGM Healthcare, said among the population in the Middle East, there was a peculiar propensity for genetic disorders. In this case, the person’s body attacked the red blood cells and platelets. There were formation of clots in multiple places. “The blood vessels are jammed, and the liver cannot work, turning fibrotic. We can transplant a new liver but there are chances that the blood will clot again,” he said.
A team of specialists, including liver transplant surgeons, cardio-thoracic surgeons, haematologists, liver anaesthetists and critical care specialists and hepatologists, were involved in formulating a treatment protocol. “We devised our own methodology to treat the disease and control clot formation. We removed the inferior vena cava and replaced it with a synthetic Dacron graft. The patient’s brother donated a part of his liver,” he said.
Before and after surgery, the patient underwent multiple courses of monoclonal antibody therapy to prevent clotting and haemolysis. Karthik Mathivanan, associate director, Liver Transplant, said the surgery lasted for 14 hours. The patient, who has returned to Jordan, will come to the hospital to undergo bone marrow transplant later.
Among others, Harish Manian, the hospital’s chief executive officer, Ananth Mohan Pai, director of medical services, and Dinesh Babu, critical care specialist were present.
