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Biliary Atresia : A Tale of a Little Hero

    Kavya Vivek Raut, a 9 month old baby, weighing only 5.6 kg is one of the smallest babies to undergo a successful liver transplant surgery. He received a hyper reduced left lateral lobe living donor liver transplant on June 14, 2018.

This is a tale of nine months, a life threatening disease, two traumatic surgeries and a miracle called life! A tale of grit and courage of parents who fought the battle for their son’s life, a brave mother who donated part of her liver to save her son’s life.

This also is a tale of an exceptional liver transplant surgery, performed on a tiny and underweight baby, a surgery where the odds were so heavily stacked against even the best of the transplant surgeons.

 

Little Kavya after the liver transplant surgery.
This Is How It Began

At birth Kavya appeared to be a healthy baby weighing 3.2 kg, a bundle of joy to Vivek and Nisha. They started getting worried as there were no signs of his neonatal jaundice going away. His stools were clay white, his eyes yellow and he was not chirpy like other babies of his age. After a few frantic visits to hospitals and multiple investigations the parents were told that Kavya was suffering from Biliary Atresia . They were shocked and shattered.

Kavya’s mother, Nisha says, “It was all so sudden and difficult for us. To deal with the medical phrases that we had never heard, never seen such illnesses, so trying to get accustomed to see my baby all strapped up with tubes and gasping for life”.

A Little About Biliary Atresia

Biliary Atresia is a chronic, progressive liver problem that becomes evident shortly after birth. Tubes in the liver, called bile ducts, normally allow a liquid produced by the liver called bile to drain into the intestines and kidneys. Bile aids in digestion and carries waste products from the liver to the intestine and kidneys for excretion. In biliary atresia , bile ducts in the liver are blocked. As bile accumulates in the liver, it damages the liver and many vital body functions are affected. In most cases liver transplant is the only treatment for this fatal condition.

The initial treatment recommended for children with biliary atresia is an operation known as the Kasai procedure known as Porto-enterostomy. This correction gives time before a transplant becomes possible, and in some cases, fixes the bile flow problem well enough that a transplant isn’t necessary.

Coming to Terms and Finding a Way Out

When diagnosed of Biliary atresia, Kavya was barely 2 months. At this time he was too small to even think of a liver transplant. At this unthinkable age of 2 months Kavya underwent porto-enterostomy. This surgery didn’t succeed and Kavya ended up with permanent irreversible damage of liver – Cirrhosis of Liver. He stopped growing, his weight was stagnant at 5 kg, developed deep jaundice and fluid in his belly. He had couple of life threatening infections that needed hospitalization in ICU. By the time his parents Vivek and Nisha and were not just emotionally drained, but had exhausted all their financial resources.

Doctors recommended liver transplant to be the only way out. Kavya’s health was still too fragile to withstand a major trauma of a liver transplant. However there was no time to wait any longer. Dr Anurag Shrimal, Consultant Abdominal Organ Transplantation and HPB Surgeon, led the team of doctors who performed this complex operation. Kavya’s mother Nisha came forward and donated a small part of her liver – left lateral segment of liver. Kavya’s complex surgery happened at the same time.

Liver Transplant an Uphill Battle

Kavya was too small and liver transplant for such a tiny baby is fraught with risks during the surgery. However with careful planning the team of doctors led by Dr Anurag Shrimal performed this very high risk surgery. Dr Anurag explained the details of this case further.

Due to the first surgery of porto-enterostomy that Kavya has undergone,  the entire small and large intestine was densely stuck to liver. While carefully separating these adhesions the surgeons ensured that there was no damage to the intestines and minimal blood loss, as infants cannot tolerate even 300-400 ml of blood loss. The entire diseased liver was removed.

The left lateral portion of the mother’s liver was harvested, it weighed 260gms. Even this small segment would not fit in Kavya’s small abdomen. The harvested liver was then further reduced on the back-bench in cold condition using special techniques to 210gms. Then this small piece of liver was implanted in Kavya’s body by joining tiny blood vessels of 3-5 mm diameter.

On reperfusion (restarting the blood flow) the liver turned pink and started working immediately. The surgery lasted for 14 hrs. After completion of operation Kavya was shifted to the PICU in stable but critical condition. A great attention to details in the peri-operative period ensured smooth recovery. Kavya stayed in the hospital for 3 weeks and recovered well.

Kavya with mother after 5 months of his liver transplant

In these 5 months Kavya has transformed to a healthy normal child. He is developing well, gaining weight, has started standing with support, new teeth have erupted and his skin and eyes are no longer yellow. His parents are ever so grateful to all the help they received.