Biliary Atresia Case Study

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 the intestine.  In Biliary atresia, the liver will gradually become cirrhotic and go towards failure. Paridhi underwent Kasai surgery for Biliary atreisa in Mumbai at 2.5 months of life, when the liver was connected to the 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 Medanta, the Medicity Hospital, Gurugram for liver transplantation 3 months back. Her Echo test (with saline contrast) 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 in normal cases near 100%, was only 48% and her shunt fraction on Macroaggregated Albumin Lung Perfusion Scan (MAA scan) was also 48%. This classified her as with very severe Hepatopulmonary syndrome (HPS).

They decided to transplant 20% of her mother’s liver to her. The challenges intra-operatively included – they had to maintain O₂. As her PO₂ were not improving more than 60%, they gave her NO (Nitric Oxide) and kept ECMO which is lung bypass machine as standby.

They ensured that surgery time was reduced to 4.5 hours and the surgery would be with minimal blood loss and blood pressures were tightly controlled not allowing to fall much. They also monitored her heart though transesophageal echo during surgery. Post operative care was also a major challenge. The new liver would need good oxygenation for adequate working. They kept her on ventilator for 4 weeks along with Nitric Oxide therapy for 3 weeks. They did tracheostomy to give the above treatment. They had to maintain good balance of fluids, blood pressure, O₂ saturation and platelet levels. Low platelets could lead to intracranial bleed while high platelets with good coagulation could block the newly stitched small vessels of the liver.

It was after 4 weeks that she was shifted to the ward where she started accepting food orally and was discharged with oxygen support 33 days after surgery. Gradually over the next 10 days they could wean her off oxygen and remove her tracheostomy. She is now active and playing around within room, air maintaining both normal oxygenation and normal liver tests.

Thus the transplant cured her both of liver and lung issues.