The unprecedented surgery that treated a rare disease of a Brazilian girl

  • André Biernath – @andre_biernath
  • From BBC News Brazil in London

22 September 2022

Credit, Little Prince Hospital

photo caption,

Yasmin Garcia, 7, underwent minimally invasive surgery that resolved a serious problem growing behind her right eye.

Yasmin Garcia’s story is one in which an ordinary episode, like the ones we experience every day, can change a whole life.

A tug-of-war game at school set off a series of events that, within hours, required the seven-year-old girl to travel more than 500 kilometers to undergo emergency surgery.

Fortunately, what could result in loss of vision and permanent aesthetic damage was resolved with a minimally invasive surgery, performed with a needle, performed in an unprecedented way in Brazil.

To understand this true epic, however, it is necessary to go back five years in time.

Attention: some images that appear throughout the report may be sensitive to some people.

first signs

Cleci Haerter, Yasmin’s grandmother-mother, reports that the first sign of something wrong with her eye appeared when the girl was just two years old.

At the time, the family lived in the city of Santo Ângelo, in the interior of Rio Grande do Sul.

“She woke up with her right eye slightly swollen and we thought it was conjunctivitis”, she recalls.

“As the day went on, we started to notice that the eyeball started to swell too much.”

Credit, Personal archive

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Yasmin’s first eye swelling started when she was two

In the morning of the following day, they were already in Porto Alegre, capital of Rio Grande do Sul, for a batch of exams.

The doctors’ first suspicion was that it was a case of lymphoma, a type of cancer that affects the lymphatic system, a network of vessels and nodes that are very important for the functioning of the immune system.

The tests showed, however, that the problem was different: Yasmin has lymphangioma, a disease marked by the abnormal formation of lymphatic vessels (and also blood vessels), which cluster in a certain region and can dilate, cause swelling and promote accumulation. of liquids.

According to experts heard by BBC News Brazil, this disease is rare and affects one individual in every 10,000 births.

But the seven-year-old girl’s condition was even more atypical: these lymphatic malformations usually develop in the head and neck region.

In Yasmin, the lymphangioma was located behind the eye — the eyeball is “pushed” forward because of the swelling of the vessels and the accumulation of fluid there.

This type of illness is so uncommon that there is not even an official statistic of how many cases are known in the world.

After that first episode at age two — which was treated with a drainage of the fluid — Yasmin’s life went on relatively normal.

“When we saw that her eye was starting to swell, we used ice packs to reduce the swelling,” says Haerter.

“At school, I always talked to the management and teachers so that Yasmin would not make too much physical effort, as this could be the trigger for a new crisis”, adds the mother-grandmother.

Exercise messes with your heart rate, changes your blood pressure, and dilates your blood vessels. This, in turn, could lead to an overflow of fluid in Yasmin’s lymphangioma.

In the meantime, the family moved to the city of Cascavel, Paraná.

And this is precisely where the fateful episode of the tug of war comes in: at school, the girl participated in the game with her classmates and, hours later, she already had the typical alteration of lymphangioma.

It all started with a small purple spot on the eyelid, which soon evolved into the projection of the eye forward.

Evolution of Yasmin's most recent eye injury

Credit, Personal archive

photo caption,

When Yasmin came home from school, her grandmother-mother noticed a smudge on her eyelid. In a short time, the swelling grew a lot.

“We tried to control the swelling at home and took her to two ophthalmologists, but no one knew what it was,” says Haerter.

“The eye started to get very bulging, it was pressed out of the skull. She was also in pain, she couldn’t eat and was just vomiting.”

Haerter then called the Pequeno Príncipe Hospital, which is about 500 km away, in Curitiba, and is a reference in the field of pediatrics.

She had already registered Yasmin for an evaluation at the institution and was waiting in the queue for care by the Unified Health System (SUS).

“They told us to take her urgently. We left here around noon and arrived in Curitiba at ten at night.”

“Yasmin was hospitalized and underwent radiography, tomography and magnetic resonance imaging while the doctors decided what to do”, he summarizes.

Yasmin's eye imaging exam

Credit, Little Prince Hospital

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One of the imaging tests showed how Yasmin’s right eye (the white spot on the left in the image) was sticking out.

delicate region

Pediatrician Rafaela Wagner, who works at the hospital and treated Yasmin, remembers how the situation had an instant impact.

“She had a deforming injury when we first saw her,” he says.

At that moment, the girl’s eye was so protruding out that her eyelids were no longer able to close completely.

“In addition to the physical alteration, the problem affected a noble and delicate region, where the optic nerve passes”, he details.

This nerve is responsible for transmitting light and visual elements from the eye to the brain, where this information will be interpreted.

The first step of treatment involved controlling symptoms, such as pain, and preventing other secondary problems, such as an infection, through the use of antibiotics.

But how to solve the problem itself and put the eye back in place?

Because it is a rare disease that involves multiple body systems, lymphangioma can be treated by several medical specialties, ranging from oncology and neurology to ophthalmology and surgery.

This situation required a veritable battalion of health professionals, who analyzed the case to find the best alternative.

Ultimately, the solution to Yasmin’s problem fell into the hands of interventional radiology, a relatively new area of ​​medicine that uses radiography equipment to analyze body parts in real time during a procedure.

a sting

The girl was referred to the hemodynamics room, which has the necessary equipment to perform such an operation, and was taken care of by pediatric interventional radiologists Pedro Santini and Helder Groenwold Campos.

After carrying out the surgical planning through some tests, the pair inserted a needle in the region between the upper base of the nose and the injured eye.

To guide the procedure, they had a machine that makes x-rays in real time, and allows them to visualize the exact place where they would intervene.

The duo of pediatric interventional radiologists perform the operation on Yasmin

Credit, Little Prince Hospital

photo caption,

The duo of pediatric interventional radiologists who performed the operation on Yasmin

“The first step was to drain the liquid that had accumulated there”, describes Campos.

The experts removed 12 milliliters of a sticky-looking dark red material.

This amount may seem like little, but when it accumulates in such a tight space behind the eye, it can cause a lot of problems.

The next step was to apply a chemotherapy drug, a drug that destroys only the defective cells that make up the lymphangioma.

“The idea is to stop the malformation from secreting the fluids that cause swelling and all the related complications”, summarizes Santini.

Doctors draining fluid into Yasmin's eye

Credit, Little Prince Hospital

photo caption,

The doctors drained the fluid that was causing the swelling…

Doctors infuse Yasmin's eye with medicine

Credit, Little Prince Hospital

photo caption,

…then they infused the chemotherapeutic that destroyed the diseased cells

‘It looks like magic’

After the procedure, Yasmin was under observation for a few hours.

When Haerter was finally able to see her granddaughter again, she couldn’t believe her eyes.

“It was wonderful to see how she was already looking 98% normal”, she estimates.

“I was super nervous, because the surgery took place in a very delicate region. But it didn’t even look like she had undergone a procedure there”, she confesses.

For Santini and Campos, quick and non-traumatic recoveries are not exactly new.

“We don’t make large incisions. All the material is guided by a needle, through small holes in the skin”, says Campos.

“People sometimes expect that the patient will leave with dressings and scars when, in fact, sometimes you can’t even see the hole in the needle afterwards”, he adds.

Such a treatment can even be done with local anesthesia when the patient is an adult — for children, specialists do sedation to prevent the child from moving or being too scared.

Cleci (left) with Yasmin shortly after surgery

Credit, Little Prince Hospital

photo caption,

Cleci Haertner with Yasmin shortly after surgery

But even for Wagner, the pediatrician who followed the entire case, the results are “stunning” and “seems like magic”.

“This is the beauty of the minimally invasive procedure. It was very significant to see the before and after of Yasmin”, he says.

Cure or control?

After the most serious moment of the crisis, specialists are now assessing what the future of the girl’s treatment will be.

She takes one pill every 12 hours to keep the inflammation under control and prevent further swelling.

The unprecedented surgical intervention was considered a success by interventional radiologists.

“We had a reduction of more than 75% and, after a month of follow-up, we did not have a significant amount of fluid formation again”, points out Santini.

But experts are wary of using the word cure for such a case.

“We need to follow up for a longer time to see if there are no changes and the problem returns in two, three or five years”, says Campos.

Rafaela Wagner and Yasmin

Credit, Personal archive

photo caption,

Pediatrician Rafaela Wagner said Yasmin is now ‘free to be a child’

From a practical point of view, Yasmin is free to follow a normal routine.

“Our recommendation is that she is a child and can play freely”, guides Wagner.

For Haertner, this news is a big relief.

She also believes that telling stories like this helps alert other parents who may be in a similar situation and still haven’t been able to make the proper diagnosis or find the most effective treatment for a problem.

“It feels like a ton came off my back,” he compares.

“There are no words that can sum up the feeling of seeing Yasmin happy and with quality of life again”, concludes the mother-grandmother.

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The article is in Portuguese

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