Hyderabad, January 10th, 2019: Gleneagles Global Hospitals (GGH), Lakdi-Ka-Pul successfully performed a liver transplant surgery on a 6-month-old baby who was suffering from Maple syrup urine disease. Baby Mythilli is the 12 infant to undergo a liver transplant last year with 100% recovery.
The liver transplant was performed by a team of doctors from Gleneagles Global Hospitals.
Baby Mythilli was born on 18-06-2018 and suffered multiple seizures from day eight which could not be controlled through medication. Initial evaluations showed that she has increased levels of branched chain amino acids (BCAA) and the MRI scan revealed diffuse brain damage consistent with Maple Syrup Urine Disease (MSUD).
In spite of putting Mythilli on special diet which is called MSUD diet and providing her medication, the 6-Month-Old baby continued to have seizures. She even developed having vision and hearing defects.
In view of poor metabolic control and severe phenotype, doctors at Gleneagles Global Hospitals decided to undertake a liver transplant. She underwent a pre-op work up and was seen by specialists from various departments who gave clearance for the surgery.
Mentioning about the disease, DrBalbir Singh, Senior Consultant - HPB & Liver surgery said,
“MSUD is a genetic disease and occurs due to BCKD gene. Certain amino acids called branched chain amino acids are not metabolized and cause damage to the nervous system of the child. The same happened to Mythilli. In those affected by this MSUD, the urine smells like maple syrup or burnt syrup.
Doctors mentioned that if the child was not treated, her nervous system could have been completely damaged. “In Baby Mythilli’s case, she was unable to sustain the normal diet given to her. And the damage to her nervous system progresses as she grows and it’s irreversible. Keeping this in view, we decided to initiate the transplant and we are happy that it was diagnosed at an early stage,” Dr K Venu Gopal, Senior Consultant Surgeon HPB Surgery & Liver Transplantation.
Dr Prashant Bachina, Pediatric Gastroenterologist & Hepatologist stated that 100 % recovery can be expected in spite of its high complications. “In a pediatric transplant, the size of the abdomen is small, the blood vessels and ducts are also tiny. Joining the donor vessels and ducts with the baby’s vessels and ducts is tough. Depending on the child’s size, one or two segments of the donor liver may be used. In adults, preferably whole or lobes of liver are used. Outcomes after surgery are similar in both adults and children, in spite of its complexity.
The latest reports of Baby Mythilli show that her liver is functioning well and her Amino acid levels in the blood have come down. The doctors informed that she did not have further seizures after the surgery and the developmental milestones are improving.