The art of the scalpel to 'return' Adriana's hands

Adriana (eight years old) saw the light before the expected date, at 35 weeks of gestation, and she did it with a shock added to her prematurity. "He had a prominent forehead and mitten-shaped hands. I knew something was wrong, something serious ... And the silence of the doctors," explains his mother, Arantxa Herrero. His daughter was born with a series of malformations in the head, hands and feet. The cause: a rare genetic disease known as Apert syndrome, which occurs in one in 100,000 live births; Adriana, one of them. For the parents, it was all uncertainties, for the health of the child, her development, functional limitations ... Could she grab a pencil or get on and hold on to a swing? The truth is that thanks to the skill of a team of surgeons at the Gregorio Marañón University Hospital in Madrid,The girl is not only perfectly capable of handling a pen, but also of "making necklaces by putting the beads on a thread, zipping up the zipper, opening bags of sweets or stickers ... And she plays the drums."

A 'box' of surprises that everyone in the family learns from, from Adriana's first day of life. To begin with, they had to familiarize themselves with a completely unfamiliar syndrome. Understand that it is an alteration (most of the time due to a spontaneous mutation) whereby

and this affects the shape of the head and face, according to Elena de Tomás, head of the Children's Plastic Surgery section at Gregorio Marañón. "The head grows upwards, as in the form of a tower, and they have little development of the middle part of the face."

To these striking malformations, those of the hands and feet are added.

The most serious degree corresponds to the hands with the five fingers glued together, "what we call the hand in a cocoon [that of a rose]", clarifies the surgeon.

Adriana's most affected hand was her left.

It had four fingers together.

Normally, the first surgery is done at six months.

It is when "we try to get our fingers out and make them as functional as possible."

This is not done in one operation, but in two.

The second, a year, approximately.

As children grow, "we keep them under review, to monitor their evolution because sometimes they require touch-ups, to straighten the first finger a little, widen the corners, fix the nails ...".

Adriana's foot before the operation H.

Gregorio Marañón

It is a

who perform it for optimal results. "I would dare to say that we have the largest series of patients in Spain," says de Tomás. She was part of the team of surgeons that intervened more than 20 years ago on the first patient with Apert syndrome. Since then, "we have done more than 40 cases, we have a lot of practice. We have been learning, innovating and our results are getting better and better."

Arantxa recalls that when "they released her four little fingers, she began to hold on to a swing, open the lid of a yogurt, paint better, write better ... As her sister, Lucía (six years old) adds," Adriana now you can fasten the buttons. "Ultimately, it affects daily functionality. If it weren't for the operation, I wouldn't be able to take a bike and brake, it would have been impossible." Besides, "it is known that grip and fine motor skills result in better cognitive development" and on the other hand, there is the aesthetic part, "social integration. One of the things that Adriana is asked the most, more than about her face , it is by his hands ".

Aware of the difficulty and the repercussion of this type of operations, Arantxa considers "key to have a reference center that brings together several specialists in these rare pathologies who can attend to all the cases that exist in Spain. This is what gives them knowledge and the management that a rare disease requires ".

(

Centers, Services and Units -CSUR-), which would facilitate (without medical or bureaucratic obstacles) the care of any patient , wherever it comes from.

Adriana's hand before the operation H.

Gregorio Marañón

Meanwhile, the team of surgeons that treats these cases continue to make progress in addressing this genetic alteration and now, as de Tomás explains, "we have introduced child traumatologists to make feet" in the same surgical act. It is precisely the last intervention that Adriana has undergone, when she was six years old. As her mother explains, "she walked poorly barefoot because she had a prominence of the metatarsals on the sole of her foot, it hurt, and she was already causing possible back and growth problems because her hip was out of balance."

The result was evident: "We stopped worrying about the type of footwear he needed, to buy him one more number, he stopped using insoles, in addition to

; and his integration in games because he he stumbled a lot, he fell. "

In total, he has undergone five interventions, three of the hands, one of the feet and one of the head, at eight months of life.

"It was very hard, one of the worst moments, since there was a risk that it would not succeed," says his mother.

"It was necessary to do a frontal-occipital reconstruction of the skull so that the brain had enough space and avoid hydrocephalus."

Now, as you grow older, your extremities may need to be touched up.

On the other hand, says the father, Javier Cantero, also awaits him, in adolescence, "another more complex intervention, the advancement of the midface, with the aim of improving their appearance and breathing."

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