Doctors from the Pirogov Clinic of High Medical Technologies at St Petersburg University hold master-class for colleagues in Almaty and perform two operations on ‘butterfly children’
- Dr Vladimir Zavarukhin, Head of the Department of Paediatric Traumatology and Orthopaedics, Candidate of Science (Medicine), traumatologist-orthopaedist;
- Dr Anna Firsova, traumatologist-orthopaedist;
- Dr Oleg Kuleshov, Head of the Department of Anaesthesiology and Intensive Care, Candidate of Science (Medicine), anaesthesiologist-resuscitator;
- Dr Veronica Novikova, anaesthesiologist-resuscitator,
Benkur, 6, had been diagnosed just in a month after birth. By the age of 12 months, all his fingers had already grown together, and his hand looked like a cocoon. Another patient, Esentai, has already been combating the disease for nine years. His hands and feet are almost always bandaged. The boy is afraid of speaking a lot and even smiling - the skin on the lips can burst at any moment and turn into an open wound.
According to Vladimir Zavarukhin, who provides medical care to such children from all regions of Russia, mouth opening limitations and bandaged wounds throughout the body severely complicate the administering of anaesthesia and intubation. At the beginning, an endotracheal tube is inserted into the child using a special thin endoscope. Further, a protective bandage is applied to the entire surface of the skin area which would be exposed to the contact during the operation, as well as under the tourniquet.
To cut off the blood flow in a limb to avoid bleeding, a special pneumatic tourniquet should be installed on the patient’s brachium. After that, surgeons separate the cocoon that binds the fingers of the hand; the fingers are gently unbent and, if necessary, wires are installed. After the fingers have been separated, the delicate stage of grafting the skin, which is taken from the undamaged parts of the child’s body, then follows.
The operation lasts about two and a half hours. Only one hand can be operated on in one operation; in six month time the other hand will be operated on, and two operations on feet are scheduled for the next year.
Patients with epidormolysis bullosa are called ‘butterfly children’ since the child’s skin is very vulnerable and sensitive like wings of a butterfly. Exhausting pains and constant dressing accompany children through all their lives.
Endormolysis bullosa is a genetic disease that cannot be completely cured. There is only one way to provide care - an operation and a lifelong symptomatic therapy.
Regular dressing of fingers and avoiding injuries can contribute greatly to further recuperation. Thus, the child’s life will become significantly better after the wounds heal and it becomes possible to remove the bandage. The little patients will even be able to start drawing within several months after the operation.