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Olsztyn. They operated on little Lilianka, who suffers from an extremely rare genetic defect

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Doctors from the Children’s Hospital in Olsztyn operated on a 7.5-month-old girl who suffers from achondroplasia. A complicated neurosurgical procedure, which was carried out under the supervision of prof. dr. hab. n. med. of Dawid Larysz, ended successfully.

A 7.5-month-old girl suffers from an ultra-rare genetic defect – achondroplasia. Failure to perform this operation as the disease progresses could result in severe neurological deficits, including internal hydrocephalus, respiratory disorders, cardiac regulation disorders and motor disorders, e.g. problems with moving the limbs.

“I feel very relieved that the operation was successful. I really wanted Prof. Dawid Larysz to perform the operation, because I wanted my daughter to be in the best hands,” said Magdalena Muracka, Liliana’s mother, quoted in the hospital release.

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In a hospital in Olsztyn, an operation was successfully performed on an infant suffering from a rare genetic defectTVN24

Every year in Poland, two or three children are born with achondroplasia. What kind of illness is that?

Achondroplasia is a defect occurring with a frequency of 1:25,000-50,000 live births. On average, two or three children are born with it every year in Poland. The essence of this disease is abnormal bone growth, e.g. skull, spine and limbs, and short stature. Very often in achondroplasia the largest opening of the skull base, i.e. the opening of the great occipital bone, is narrowed. This opening is where the brain ends and the spinal cord begins. The medulla oblongata located in this area is a structure responsible for the most important activities of our body, such as e.g. breathing, controlling the work of the heart, moving or regulating the sleep-wake cycle.

The narrowing of the lumen of the foramen magnum additionally causes disturbances in the flow of cerebrospinal fluid between the cranial cavity and the spine. These disorders most often lead to the appearance of hydrocephalus, which is a life-threatening condition for the child.

The defect is progressive, so over time the foramen magnum will grow more slowly than the rest of the skull and will become relatively smaller. Such a situation may cause serious clinical problems in the child, e.g. breathing problems (eg sleep apnea) and mobility problems.

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They operated on a 7.5-month-old child. This is another such patient in the Olsztyn hospital

Surgery was necessary to prevent the progression of the disease. “It consisted in the so-called atlanto-occipital decompression, i.e. widening the great foramen of the occipital bone of the child. Thanks to the procedure, the medulla oblongata will have more space for further growth and development” – explained Prof. dr hab. n. med. Dawid Larysz, head of the Department of Head and Neck Surgery for Children and Adolescents at the Children’s Hospital in Olsztyn.

He emphasized that it was quite a complicated neurosurgical procedure, because the child is very small and additionally has an abnormal skull structure and surrounding vascularity.

“The opening is narrow, so there is a higher than standard risk of neural compression, which is extremely dangerous. In order for us to safely perform this procedure, we used virtual reality planning, i.e. we performed this procedure earlier in the computer. The operation was performed with the use of intraoperative neuromonitoring and microscope for neurosurgical operations” – added the specialist.

So far, several such patients from all over Poland have been operated on at the Children’s Hospital in Olsztyn. The smallest of them was three months old.

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The only such facility in Poland

The Voivodeship Specialist Children’s Hospital in Olsztyn is an Expert Center for Rare Diseases established by the Minister of Health. As the only medical facility in the Warmian-Masurian Voivodeship, it has obtained the status of the Center of Experts in Rare Diseases, granted from this year by the Minister of Health. The Children’s Hospital in Olsztyn is an expert center in two specialist areas: ITHACA (rare neurogenetic diseases) and CRANIO (rare head and neck diseases). In the scope of CRANIO, the Provincial Specialist Children’s Hospital in Olsztyn became the Center of Experts in Rare Diseases as the only medical facility in Poland.

Main photo source: TVN24



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