Unique case at Okhmatdyt: first surgery performed on a child with a rare syndrome that occurs once in 130,000
The surgeons at the National Children’s Specialized Hospital “Okhmatdyt” performed surgery on the chest of a child with asphyxial thoracic dystrophy. This was the first time such an intervention was performed in Ukraine, the hospital’s press service said.
It is noted that a boy with a rare syndrome that occurs in one in 130,000 newborns was admitted to Okhmatdyt at the age of 1 month.
Children with this syndrome are born with a small chest, which is bell-shaped and restricts the functioning of the lungs. This does not allow the child to breathe normally. The disease is very severe, especially if symptoms develop in children under 1 year of age. Often, such patients die of respiratory failure and recurrent pneumonia or become dependent on artificial respiration devices, doctors say.
In 1995, American surgeon Terrence Davis proposed a method of surgical treatment of this defect, which he called lateral thoracic expansion. This operation allows you to consistently increase the volume of both halves of the chest. Such interventions are rare and have been performed only in a few leading clinics in the United States, the United Kingdom, and Italy.
When such a patient was admitted to Okhmatdyt, the hospital’s specialists decided to apply the proposed approach for the first time in Ukraine.
“The essence of the operation is that 6 edges are staggered in a checkerboard pattern. And then they are connected with special metal structures in such a way that the long segments of two ribs are made into one rib twice as long. This increases the volume of the chest,” explains Professor Yevhen Rudenko, a surgeon at the Okhmatdyt Hospital.
The boy underwent surgery in two stages: at 1.5 months on the right side and at 2.5 months on the left. The hospital added that now his postoperative wounds have healed, the child is training to breathe on his own, and according to the examination, the volume of his chest has increased.
