Doctors at Yashoda Hospitals in Hyderabad performed a liver transplantation surgery on a 23-day-old male child who is suffering from a metabolic liver disease known as galactosemia.
Though the surgery was performed a month ago, the hospital disclosed the procedure on Friday, 2 December. They did this after concluding that the surgery was successful and evaluating the child’s condition.
Before the surgery, he was evaluated by the paediatric hepatology and transplantation surgery team of the hospital and was found nutritionally depleted.
The team found that his body weight was at the 25th percentile for his age.
What is galactosemia?
Galactosemia is a rare, hereditary disorder of carbohydrate metabolism that affects the body’s ability to convert galactose to glucose.
It is caused by a single enzyme defect known as Galactose-1-Phosphate Uridylyl Transferase (GALT).
An infant with galactosemia appears normal at birth, but loses his or her appetite within a few days or weeks and starts vomiting excessively.
Yellowing of the skin, mucous membranes, and whites of the eyes (jaundice), enlargement of the liver (hepatomegaly), the appearance of amino acids and protein in urine, growth failure, and ultimately the accumulation of fluids in the abdominal cavity (ascites) with swelling of the feet (edema) may also occur.
Often, a patient may die even without an established diagnosis.
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Risky procedure
In the case of the 23-day infant, biochemistry and genetic testing supported the diagnosis, leading experts to conclude that conservative management — the avoidance of surgery or other invasive procedures — would not be suitable or best for the infant.
The baby was diagnosed with respiratory distress, hypoglycemia (regularly low blood sugar), jaundice, and failure to thrive after birth. Phototherapy was used to treat these conditions for three days, but no improvement was noticed.
The family history of the child showed that he had two siblings who succumbed to a liver ailment at the ages of two months and nine months. Therefore, this was a precious child for the family.
His 31-year-old father came forward to donate a portion of his liver and was found suitable and fit for surgery.
Dr Venugopal, who led the surgical team, said, “It is a rare metabolic liver disease. We decided to perform a transplant after looking at the family history, the severity of the condition, and the high chance of mortality without the transplant.”
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‘Smallest paediatric transplant’
According to the hospital, in contrast with the standard living donor transplant surgery, these procedures require the engrafted liver to have two or three cut surfaces. This increases the potential for blood, lymph, and bile leakage from cut surfaces and complicates the postoperative course.
Additionally, children’s blood vessels are extremely thin, making anastomosing them difficult.
The child was discharged three weeks after surgery on stable immunosuppression and stable liver graft function.
“We carried out the procedure following detailed consultations with other members of the team and parental approval. We are happy to report that it was extremely successful and allowed the patient to resume his normal life,” said Venugopal.
“It was also a huge relief to the parents. This is arguably the smallest paediatric transplant reported from this part of the world,” he added.
