The patient, a five-year-old girl had multiple health problems including advanced liver cirrhosis, congenital heart defects, severe growth retardation and rickets. Briefing media about this amazing accomplishment, Dr Manish C. Varma, Head of the Liver Transplant Department, Apollo Hospitals, Jubilee Hills; Dr Girish Warrier, Consultant Pediatric Cardiac Surgeon, Apollo Hospitals, Jubilee Hills and Ms Sangita Reddy, Joint Managing Director, Apollo Hospitals; said the baby was suffering from a rare genetic disorder. Though the ailment was diagnosed two years ago elsewhere, the complexity of performing a liver transplant and heart defect correction in one go and not having a precedence to this, prevented them from providing succour to the baby.
Five-year-old baby girl, Parvati Rohra, hailing from Hyderabad, was suffering from a rare genetic disorder called Alagille Syndrome. It occurs with a frequency of 1:1,00,000 children and can impact the liver, heart, lungs, kidneys, bones among others. However, the severity of the problem varies from patient to patient.
Because of Alagille Syndrome, Parvati had advanced liver cirrhosis, severe cardiac defects including Pulmonary Artery hypoplasia, Pulmonary valve dysplastic, Atrial and Ventricular septal defect, resulting in severe Pulmonary Artery Hypertension, severe growth retardation and rickets. Compounding the problem was her weight of just about ten kilograms. She was leading an extremely traumatic life, deprived of the normal childhood which kids of her age longed for. Although she was mentally developed, she was not going to school or out to play, as any attempt to walk would lead to fractures of the leg bones due to rickets. She was leading a very restricted life and was not able to achieve motor milestones.
Her medical history indicated that she presented initially with jaundice in the first few months after birth. Her parents consulted a hospital in Mumbai, which diagnosed this as biliary atresia and were counselled for a liver transplant. However due to the baby being underweight – 10 kgs, the transplant team was not confident of operating on such a small child. Though the problem was later diagnosed elsewhere in Hyderabad, two years ago, looking at the complexity of the patient with heart defects and advanced cirrhosis of liver occurring together and there being no precedence in treating such cases earlier, the hospitals she approached expressed its inability to treat. The family was desperately exploring all avenues known to them in the last 2 years, before they came to Apollo Hospitals.
Commenting on the high-risk nature of the case, Dr. Manish C. Varma said “Trying to deal with the problem one at a time would have been definitely fatal. If we attempted to perform liver transplant with a bad heart, she was at risk of dying in the operation theatre. On the other hand, if we tried to correct the heart alone, she was at a very high risk of dying after the heart surgery.”
A multidisciplinary team of doctors led by Dr. Manish Varma, consisting of specialists, Liver Transplant Surgeons, Pediatric Hepatologists, Liver Transplant Anesthetists, Pediatric Cardiac Surgeon, Pediatric Cardiologist, Pediatric Anesthetists and Pediatric critical care specialists, took up this case as a challenge.
The task ahead was daunting for the team, especially since there is no precedence of treatment for such a complex case being attempted in medical history. “We had no roadmap for us. We could find only one case in medical literature from a different country. However, that was a less complex case and the doctors in that case were able to correct the cardiac defect by endovascular balloon dilatation. We attempted that in our patient’s case but, unfortunately, that failed to correct the heart defect” explained Dr. Girish Warrier.
Left with no other option, the doctors at Apollo decided to do something that was never attempted before in medical history. They decided to perform an open-heart surgery for the cardiac defects and then a liver transplant in quick succession.
During an eight hour cardiac surgery performed on 23rd January 2017, by Dr. Girish Warrier and his team, the holes in the heart were closed, Pulmonary Artery was widened, Pulmonary valve was excised and an artificial flap valve was created. This was followed by an observation in the ICU for about 12 hours during which the baby was monitored closely for the improvement in heart function and for any deterioration in liver functions. Thereafter, a live donor liver transplant was performed by Dr. Manish Varma and his team, using a part of her mother’s liver, in the earliest available window of opportunity on 24th January 2017. The baby has since recovered and was discharged on February 7th, 2017.
Elaborating on the complexity of the case and the importance of clinical decision making, Dr. Manish Varma said, “All our investigations suggested that an open heart surgery would be able to achieve a cardiac function that would enable us to do the liver transplant. However, the most important thing was to identify the correct window of opportunity to do the liver transplant. Attempting a transplant too early would have meant that we would be stressing the heart which had still not recovered fully, and attempting it late meant that the failing liver would have made Baby Parvati so sick that a liver transplant would be impossible. We could attempt this only because we had a living donor transplantation option in her 32-year-old mother Prerna Rohra, as the mother’s liver was a perfect match for the baby.”
After this surgery, the baby will have a better growth curve and is likely to catch up on her growth over the next 2 years. The rickets will get treated with vitamin D supplements which will now work due to a functioning liver. The jaundice and itching will go away. She will need to be on some medicines for her heart and will need to be monitored for her heart status. After a few months, she will be able to walk, go to school, and do other age appropriate activities.
The multidisciplinary team which was part of this historical surgery consisted of Dr. Manish Varma, Dr. Girish Warrier, Dr. Anand Khakhar, Dr. Anand Ramamurthy, Dr. Mahesh Gopashetty, Dr. L. Sasidhar Reddy, Dr. Manjunath Balasubramaniam, Dr. Meena Trehan, Dr. Kavitha Chintala, Dr. Dinesh Babu, Dr. Vishnu Vardhan Reddy, Dr. Sunita Nareddy, Dr. Shweta Priyadarshini, Dr. Ramesh Srinivasan, Dr. Senthil, Dr. Kirubakaran, Nutritionists, Physiotherapists and pediatric nurses, among others.