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In a first, 5 year old cured of liver failure and associated lung failure by a rare liver transplant

May 4 2016 , Written by Ambika Sukumar

Initial Prognosis

Paridhi had jaundice since birth. She was diagnosed as Biliary Atresia. Biliary Atresia is a condition where the bile duct is not formed by birth; bile duct connects the liver to intestine. In Biliary atresia liver will gradually become cirrhotic and go in liver failure. She underwent Kasai surgery for biliary atreisa in Mumbai at 2.5 months of life, where in the liver was connected to intestine.

Unfortunately this surgery was not successful and her jaundice persisted and she developed cirrhosis. At 1.5 years of age she was also diagnosed with Kawasaki infection which affected her heart and her heart vessels got slightly dilated. She had 6 admissions thereafter for lung and liver infections. By 5 years, Paridhi became weak, got breathless - was not able to walk and would get easily tired. Besides growth failure, her finger nails and lips turned blue. The family contacted us for liver transplantation 3months back. Her Echo test (saline contrast test) showed that Paridhi had developed intra-pulmonary shunts that is the blood going from heart through pulmonary artery instead of going through lungs was getting into pulmonary veins which open in left side of the heart. This resulted in poorly oxygenated blood to left side of heart – which got circulated in body.

Her PO2 (oxygen in blood) levels which are normally near 100%, was only 48% and her shunt fraction on MAA scan was also 48%. This classified her as very severe HPS (hepatopulmonary syndrome).

The challenges intra-operatively include (a) We had to maintain O2. As her PO2 were not improving >60%, we gave her NO (Nitric Oxide) and kept ECMO which is lung bypass machine as standby.

March 5 would have to be a red-letter day for rare cases of liver failure with associated lung failure. It was on this day that in a first in the country, 5 year old Paridhi underwent a highly complex and treacherous liver transplant to cure her of both, the liver and lung failure.

Her mother Mamta, who donated 20% of her liver to save her darling, said, “ my husband Nitin and I had nearly given up all hope of getting Paridhi well as doctors in Mumbai had said that her case was far too advanced for a liver transplant. Then about 3 months ago, we met the Medanta liver team who offered to try and save our child provided we understood that the transplant would be very high risk due to her low oxygen state. We took the chance as a last resort and God heard our prayers.”

Dr AS Soin, Medanta’s Chief Liver Transplant Surgeon who led the team said, “we have a long experience of operating milder cases of hepatopulmonary syndrome (HPS - when liver failure affects the lung circulation and in extreme cases, results in respiratory or lung failure), but had never taken on a such an advanced case with nearly 50 % shunt fraction , before”.

Explaining the patient’s state prior to transplant, Dr Neelam Mohan, Director of Children’s Liver Diseases and Transplantation, said that Paridhi had been suffering since birth from biliary atresia for which an operation at 2 and a half months did not prove successful. She then deteriorated,had life threatening liver infections with 8 prolonged admissions in hospitals and when she came to us, she was deeply jaundiced, had marked growth failure, had severe HPS with oxygen saturation less than 70 % causing breathlessness,necessiatating continuous oxygen and had blueing and rounding of her nails typical of a low oxygen state.“We took her up as one of the most challenging cases, and carried out an intensive nutritional and medical preparation to take her through the transplant successfully, she remarked.”

Elaborating on the risks and challenges her operation posed, Dr Soin said, “liver transplant is complex as it is, and therefore, requires perfect function of all other organs. Here, there was a real danger of Paridhi not waking up after surgery due to brain, heart or kidney injury, or, for that matter the new liver not kicking in, if the already low levels of oxygen fell any lower. So we had to do a bloodless, zero error, quick surgery, not allowing any fluctuation in the blood pressure, and with 100% oxygen support.”

Dr Vohra, Head of Anesthesia, added that in order to prevent dangerous fall in oxygen levels, they had resort to continuous nitric oxide treatment during surgery, keep a watch on the heart by a special ultrasound probe inserted in the patient’s food pipe, and keep an artificial lung machine on standby.

According to Dr Neelam Mohan, Paridhi’s postoperative treatment was also a challenge as the low oxygen state takes several weeks to correct. Nitric oxide, high external oxygen, ventilator support, temporary breathing route (tracheostomy) were necessary for many days. Infact , she was on ventilatory support for four weeks post operatively followed by oxygen support . Eventually , we discharged Paridhi home after 33 days of surgery. She now breathes normally without extra oxygen, and will grow like a normal child and live a healthy life.

Dr Naresh Trehan, CMD and Chief of the Heart Institute, said “Paridhi’s case was easier to handle at Medanta since we use nitric oxide therapy and artificial lung machine quite often on patients with serious heart diseases affecting the lungs”. Medanta’s strong multidisciplinary expertise came to the rescue here, he felt.

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