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Monday, June 25, 2018

After rare skull surgery at AIIMS, twins from Kerala look like each other

Heads of both babies had grown vertically rather than horizontally.

Written by Pritha Chatterjee | New Delhi | Updated: April 12, 2014 8:15:06 am
Adhi and Adhri with their parents after the surgery. PRITHA CHATTERJEE Adhi and Adhri with their parents after the surgery. (IE photo: Pritha Chatterjee)

It was about four months after their birth that an aunt noticed Adhi and Adhri’s heads looked “abnormal” in shape.

When they came to AIIMS in March from their home in Kerala a few days after their first birthday, the heads of both babies had grown vertically rather than horizontally — a condition known as craniosynostosis, where sutures or natural divisions in the skull bones fused in the womb, leaving little space for their brain to grow.

The condition is rare, with 1 in 5,000 cases reported in international literature, and the AIIMS neurosurgery department getting about 10 cases a year. But for the institute, this was the first time that monozygotic twins, who looked identical otherwise, had this abnormal growth, with one twin having a more prominent vertical growth than the other.

“Adhi’s head was swollen more than Adhri’s, and the shapes were different,” said Dr. Deepak Gupta, additional professor of neurosurgery at AIIMS who operated on the babies.

“In surgeries for this condition, we remove the bones and resize and reshape them by artificially breaking them and inducing fractures. But since these were identical twins, we had to resize the bones and shape the heads, to make them look exactly the same,” he said.

The mid-line of the skull, known as centre ridging, which divides the left side and the right side of the brain, were missing in both babies, leading to a “perpendicular bulge” in the skulls. “The intracranial pressure or the pressure in the brain was very high in both babies. Such high pressure leads to headaches, abnormal brain development and can result in loss of vision,” Gupta added.

The fear of their babies having different shaped heads haunted the parents, till a day after the surgery.

“We named our children after Lord Shiva, because they looked the same. We dressed them the same way, barring the fact that Adhi smiled at his doctors and Adhri started howling, our kids looked the same. It was a huge shock when we first started noticing their heads were odd, and then gradually when Adhri’s head swelled more than Adhi’s,” said their father Binu Mon P R, a businessman based in Alleppey.

In the surgery that lasted about eight hours — four hours for each baby, one after the other, “to ensure we maintain continuity of shape” — doctors opened their skulls, extracted the primary bones and then cut and broke them inducing multiple fractures to resize the bones to decrease the brain’s vertical growth and increase horizontal growth.

“We had to ensure that for every re-alignment, we left sufficient space to allow the brain to grow in the new direction. The growth of the brain is maximum till about three years of age, so we had to ensure we did not obstruct that, because that would lead to developmental disorders,” Gupta explained.

That both babies had a rare blood group of AB negative also complicated matters, but doctors said the surgery was done without the kids requiring blood transfusion.

Two weeks after the surgery on March 27, both babies have been discharged and follow-up scans have shown their brains are now growing normally. Doctors said since chromosomal analysis of the babies has not revealed any causes, the abnormal growth of the skull was possibly due to the fact that they were twins.

“The space in the uterus is obviously restricted when there are twins. Possibly due to this reason the skulls had fused at a premature stage during the pregnancy,” Gupta said.

The mother, Sandhya, said her babies now looked like “real twins”.

“I was obviously worried putting my babies through surgery when they are so young. But doctors said if we delayed the matter their heads would grow bigger and even more different in shape, and their brain development may be flawed. I am glad they have normal heads now, they look like real twins,” she said.

Doctors said the need for future surgery is unlikely since there does not seem to be any genetic reason for this growth. “Surgeries may have been needed in the mandible and around the ears later in life had this been due to genetic reasons. The twins should grow normally now,” Gupta said.

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