Cape Town - After years of sleeping in a sitting position and keeping her mouth open 24 hours a day due to breathing problems associated with her facial deformity.
The seven-year-old Akikere Bassey can now breathe easier.
Following major surgery at Tygerberg Hospital about 10 months ago, not only can she sleep in a lying position with her mouth closed, but she will soon attend a mainstream school for the first time after more than a year of being at home.
Her heart condition, associated with the syndrome has also improved that she is now off cardiac medication.
Akikere’s mother, Joy Bassey, said her daughter’s breathing and developmental problems were becoming so bad that the family had decided to home-school her as she could not cope at school.
Her breathing and developmental problems, caused by Crouzon syndrome – a genetic disorder characterised by the premature fusion of the skull bones and cranial base – had resulted in Akikere developing other life-threatening conditions, including pulmonary hypertension and heart problems.
Because of severe midface hypoplasia, a condition where the centre of the face develops more slowly than the eyes, forehead, and lower jaw, her upper airway was obstructed, resulting in right heart damage.
In June, Akikere, from Nigeria, came to Cape Town to have a marathon operation in which surgeons, led by a leading plastic surgeon Professor Frank Graewe, detached her midface and forehead from the skull and gradually shifted it forward with special pins, which they inserted into her head.
After the hospital teamed up with the World Craniofacial Foundation and Smile Foundation, the team of doctors used 3D technology to print models of her face and skull.
CT images were taken at Tygerberg and were sent to the US, where a model of her skull was made using 3D printers.
The 3D technology helped to select the right pins or “distractors”, and where to position these before the seven-hour surgery.
This month, Akikere is back in Cape Town to have the distraction devices removed and for doctors to follow up on her progress.
Graewe said while Akikere might need more minor procedures, “The surgery that we did last year has brought about the improvement in her heart condition”.
“She had severe sleep apnoea and upper airway obstruction and this was improved much by the surgery to her facial and skull bones, which improved her breathing and function as well as her appearance.”
Akikere’s mother said the surgery not only corrected her daughter’s heart, but had improved her appearance and sleeping patterns dramatically.
“She is looking so much better… she no longer has bulging eyes.
“And, for the first time since she was born, she sleeps throughout the night and is full of energy like kids her age.
“The surgery not only improved Akikere’s life, but it has also improved my life as I no longer have to watch over her 24/7.
Sipokazi Fokazi, Cape Argus