Team approach to Ehlers – Danlo

A 13-year-old female patient underwent a one-stage operation. A mitral valve was prosthetically inspected and the funnel-like deformation of the thorax was eliminated.

A week after the intervention, Vika sees the first changes: there is no cosmetic defect anymore, it’s easier to breathe and to move. The confidence of the girl and her parents is supplemented by the support of the attending physician, a cardiac surgeon Mikhail Shved (on the photo), and all the staff of the Cardiac Surgical Department No. 2 of the Children’s Surgery National Applied Research Hospital. They have known each other for more than 7 years.

“The first operation was correction of the open arterial duct,” says Zhanna Gutko, the girl’s mother. “Seizures of paroxysmal tachycardia began at the age of 6 years. We are from Yelsk, we were directed to Mozyr, then here.”

“Of course, this is scary. In the moments of attack, you do not let the child out of sight for a minute. We bought the phone to our daughter first in our classroom, because we need to constantly monitor if she was not bad. We were sitting on a powder keg.”

“We visited an arrhythmologis of the Children’s Surgery National Applied Research Hospital. We are very thankful to her. The seizures stopped after radiofrequency ablation.”

“Now Vika underwent the biggest operation. She is recovering, and we know that everything will be good.”

Vitaly Dedovich, Head of the Cardiac Surgery Department No. 2 of the Children’s Surgery National Applied Research Hospital:

The experience is interesting because we worked simultaneously with the colleagues from the NARH of Traumatology and Orthopedics for the first time, specifically – with Georgy Brodko, the head of the Pediatric Traumatologic and Orthopedic Department.

If you divide the operation into two stages – cardiosurgical and traumatological, then each is a routine enough. But atypical diagnosis caused some peculiarities of approaches to treatment.

Ehlers-Danlo syndrome is quite rare: hereditary systemic pathology of connective tissue; depending on the type, it occurs in one of 10.000-100.000 people. Defect in collagen synthesis led to mitral valve isufficiency and chest deformation.

The cardiac surgeon had to resort to prosthetics of the mitral valve. In childhood it is a forced measure, in our Hospital there are no more than 5 such cases per year. Usually we make plastic, because children grow, and the heart grows with them. But because of hyper-elasticity of tissues, it was impossible to carry out such a correction for Vika. Our patient is 13 years old, so we decided to operate her as an adult (from the age of 18, valve prosthesis is a standard intervention).

The traumatologists performed Nass operation. Usually only the funnel-shaped deformation is corrected, the sternum is not opened. Straightening steel is introduced through small cuts. But in this case, as the operation on the heart was performed, the sternum was dissected.

The operation was prepared for several months, we worked out the actions minute by minute. We have faced a kind of surgical paradox: when prosthetics of the mitral valve, cardiac surgeons are afraid of bleeding. However, when traumatologists, correcting the deformation, reposition the bone fragments, it is almost inevitable. As a result, everything has passed in a normal mode, the postoperative period proceeds well.

This intervention is difficult to call unique. But the circumstances and conditions were unusual. Every patient for cardiac surgeons is special, because every heart is unique.

Taken from the site www.MedVestnik.by