Highly complicated End Stage Liver Disease Budd-Chari Syndrome patient given new lease of life by Fortis doctors


Patient, residing in New Zealand, opts for treatment at Fortis Hospital, Mohali

NewZNew (Mohali) : Fortis Hospital, Mohali today announced that it had successfully conducted a very complicated and technically advanced surgery to give a new lease of life to Nirmal Singh, a 28 year resident of New Zealand (native of Patiala). In New Zealand itself, he was diagnosed with Budd-Chiari Syndrome (BCS), a rare and life-threatening vascular disease.

Subsequently, with the diagnosis of end stage liver disease due to BCS, he was advised for liver transplantation. However, he would have to wait for up to 2-3 years before he could get a deceased donor liver transplant in New Zealand. Due to rapid deterioration in his condition, he contacted Dr. Vivek Vij, Director – Liver Transplant & GI Surgery, Fortis Hospital Mohali & Noida and Dr. Ashish Singhal, Consultant – Liver Transplantation & Hepatobiliary Surgery, Fortis Hospital Mohali in August 2014 for an option of living donor liver transplant (LDLT) and made an initial visit to Fortis Hospital, Mohali in September 2014.

Worldwide, management of patients with BCS remains challenging. A stepwise therapeutic approach is commonly accepted. Concerning therapeutic options, medical therapy alone is generally associated with a high mortality up to 90%. When all other therapeutic options (shunt surgery, TIPSS) are unsuccessful, or patient has end-stage liver disease, transplant has to be considered.

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According to the Fortis doctors, when the patient came to Fortis, he was in a critical state. His main symptoms were fluid in abdomen (ascites, diuretics resistant), gross pedal edema, pain in abdomen, fatigue, anorexia, weight loss (up to 15 kg), episodes of confusion and renal dysfunction. The workup for the etiology of BCS did not demonstrate a specificcause. Multislice computed tomography of the abdomen revealed a series of complications (thrombosis of all three hepatic veins, narrowing of retrohepatic IVC with partial thrombosis of infrarenal IVC. He also had thrombosis of bilateral internal jugular veins.)

Prior to transplant, he required multiple paracentesis (drainage of abdominal fluid) along with medical optimization of kidney function under the care of Hepatologist (liver medicine specialist) Dr. Arvind Sahni, Director – Gastroenterology, Fortis Hospital Mohali.

His 36 year old sister volunteered for liver donation. The donor and the recipient underwent surgery in October 2014. The recipient native liver was removed and the vena cava was observed to be thickened and compressed. Technically, in these kinds of cases, the mobilization of the liver and the piggy-back maneuver are not easy. The Fortis doctors, however, succeeded in conducting the surgery.

[blockquote author=”stated Mr. Abhijit Singh, Facility Director, Fortis Hospital, Mohali. ” pull=”pullright”]“Our team has successfully performed LDLT in 5 patients (including Mr. Nirmal Singh) with BCS. This has been possible mainly because of the skill and expertise of the surgeons, the warm and professional approach of the paramedical and nursing staff and the state-of-the-art equipment at Fortis Hospital, Mohali,” [/blockquote]

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Thanks to the paramedics and the nursing team, the peri-operative and post-operative period were uneventful and Nirmal was discharged from Fortis  on post-operative day 16. His liver function tests recovered gradually after the transplant. Multislice computed tomography obtained 2 weeks after surgery demonstrated patent IVC and all anastomoses. Currently, his liver function tests are normal.

A highly relieved and happy Nirmal said, “I am glad that I opted to come to Fortis Hospital, Mohali from New Zealand. I got world class medical care here and my life was saved by the expertise of the entire Fortis team. My family and I are very grateful to them.”

Dr. Singhal said “LDLT is a safe option for patients with end-stage liver disease associated with BCS, especially in countries in which deceased donor grafts are of limited availability”.


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