Life-saving liver transplant at Osmania Hospital saves 14-year-old from AP with Marfan Syndrome

Hyderabad: A liver transplant helped cure a chronic liver and lung condition caused by the hereditary disease, Marfan Syndrome, in a 14-year-old boy from Machilipatnam, Krishna District of Andhra Pradesh. The procedure was successfully done at Osmania General Hospital in Telangana.

“This is a medical milestone and we are the first hospital in the world to do this treatment for Marfan Syndrome,” said Dr CH Madhusudhan, liver transplant specialist from OGH.

What is Marfan Syndrome?

Marfan Syndrome is a genetic disorder where the connective tissues of the organs and skeletal structure of the body are affected. Due to this syndrome, the person is tall and thin with unusually long limbs, fingers and toes. The syndrome affects the heart, eyes, blood vessels and skeleton. This condition affects approximately 1 in 10,000 individuals and can lead to life-threatening cardiac complications in usual cases.

The 14-year-old, G Nikhil Reddy of Gokavaram in Machlipatnam from AP, was diagnosed with Marfan Syndrome in early childhood. In the case of Nikhil, the syndrome produced complications in the liver, too.

What happened to the child?

* At one and a half years of age, Nikhil Reddy developed problems in the left eye. He was diagnosed with congenital glaucoma and underwent surgery. After that, he developed cataract in the left eye and consequently lost vision in that eye too. He had no left eyeball due to the syndrome.

* At the age of 7, there was bluish discolouration in nails, palms and tongue for which he underwent treatment.

* At the age of 12, the abnormal conditions in the body continued, and he was diagnosed with severe jaundice.

Liver tests at CMC Vellore, treatment at OGH

At CMC Vellore, liver biopsy and other liver tests were carried out, which showed that he had developed chronic liver disease. CMC Vellore referred the family to Osmania General Hospital for further treatment. Nikhil arrived at OGH with severe complications and low oxygen levels. He is very thin and very tall with long hands and long fingers. There was also spine deformity, retracted chest and other conditions diagnosed by doctors.

Dr CH Madhusudhan said, “He also developed hepato-pulmonary syndrome, which is a rare but severe complication of the liver. Due to this liver condition, there is abnormal gas exchange in the lungs, and there is enlargement of the blood vessels of the lungs.”

The patient came in with Marfan Syndrome, which is genetic and acquired a severe liver complication due to the syndrome.

Dr CH Madhusudhan said, “We were dealing with two major issues. The priority was ensuring that the liver condition could be controlled. Liver transplantation is an established and definitive treatment for hepato-pulmonary syndrome (HPS). Liver transplant helps to deal with the underlying liver disease and helps to resolve the symptoms of the lungs.” Maryan Syndrome adds to the complication. To deal with it, a multi-disciplinary team of doctors and caregivers was readied at OGH.

Risk versus benefits

A team of cardiologists, pulmonologists, anaesthesiologists, radiologists and gastroenterologists got together for the procedure. Deliberations and review of Nikhil’s condition were carried out, and it was decided that the HPS will be addressed first.

A part of the liver was taken from the mother, G Swathi, on March 7. While the Maryan Syndrome posed challenges, the surgery went well, said Dr CH Madhusudhan.

Post-operative care and recovery

The post-operative care and the patient’s ability to recover after the liver transplant enthused the multi-disciplinary team and the caregivers' team.

The team involved in surgery and care are liver transplant specialists Prof Dr CH Madhusudhan, Dr Wasif Ali, Dr Sudarshan Reddy, head of anaesthesiology department Dr Abhimanyu Singh, anaesthesia Prof Chandrashekhar, professor of Anaesthesia Dr Madhavi, professor of Anaesthesia Dr Raghu, Dr Lakshmi Narayana, Dr N Sunil, Dr Manasa, Dr Uma Devi, Dr Ramesh Kumar, Dr Nadeem, radiology Dr Vijay Pavan, biochemist Dr Rupa, blood bank Dr Sai Sudha, Dr Nala Kishore and Dr Shankar. Manjula, Subba Laxmi, Madhavi, Krishna, Babu and other team members were also involved in this surgery.

Donations and CSR

Apart from the government health scheme, a Rs 10 lakh donation from the chief minister's relief fund helped the patient.