SURGEONS in Port Elizabeth will make history today when they perform the first mandible distraction surgery in the province on the jaw of a four-year-old boy born with an extremely rare genetic condition.
France Cumbana, from Humansdorp, was born with Pierre Robin sequence, a genetic condition so rare that doctors estimate only one in a million babies is ever diagnosed with it.
France has a very small lower jaw that makes it difficult for him to breathe, eat and speak.
He also has a cleft palate, but doctors cannot repair it until his jaw has been corrected.
France can only open his mouth about 1cm.
Provincial Hospital plastic surgery department head Dr Chris van der Walt said he and Dr Ismael Gopal, a maxillo-facial surgeon at Livingstone Hospital, would be doing the surgery, with the input of colleague Dr Ashwin Kassan.
“Our little patient was born with a very small jaw,” Van der Walt said.
“He has difficulty eating and speaking. His tongue also pushes up and keeps his cleft palate open.”
The surgeon said the hospital’s access to hi-tech scans had significantly boosted the capacity to perform the operation.
“To perform this surgery, we needed access to special CT scans with 3D reconstruction.
“We can do those now in Port Elizabeth,
“It is easier for us and the parents. It was a logistical nightmare to get these children to Cape Town for surgery. They also need long aftercare.”
Van der Walt, an alumni of the University of Stellenbosch, said he had called on fellow alumni Gopal to help him plan and carry out the surgery.
“As these surgeries are done at Tygerberg Hospital and we both studied there, we know what to do,” he said. Kassan, another Port Elizabeth maxillofacial surgeon, would be assisting them.
“This is a big surgery,” Van der Walt said.
“The goal is to stretch the jaw.
“We will use a device to stretch the jaw.
“Then we will surgically break the jaw with an ultrasonic bonecutter.
“We will let it heal for a couple of days and then start stretching it by 1mm a day.
“We will stretch the jaw up to 2.5cm.
“That will take about a month.
“Then we will leave it to consolidate for another month.”
France’s dad, Joao, said when his son was born, doctors at Humansdorp referred them to the Provincial Hospital to seek help. “We were very happy when they said they could do the operation here because it would have been very difficult for us to go to Cape Town with France,” he said. “We have other children too.” He said France struggled to eat but always tried his best.
“He is a real little character. He is always smiling.”
-Estelle Ellis
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