Elena May Reading
Crouzon's Syndrome
Ellie Reading
Elena May Reading, known as Ellie, was born in 1994 with Crouzon's Syndrome which has restricted the growth of her skull and the bones in the middle of her face. She can't close her eyes properly because her eye sockets are too small causing her eyeballs to protrude. She can't sleep very well, has trouble with her breathing and with her teeth.
When Ellie was a baby, the bony cranial plates in her skull fused together too early. Normally, they stay separate for long enough to leave room for the growing brain. Ellie's restricted skull caused pressure to build up and at eighteen months old she had her first major surgery at Great Ormond Street Hospital.
Now, ten years on, the pressure is building up again and her doctors are recommending that she has more surgery. Most children with Crouzon's put off this risky operation until their late teens, but Ellie can't afford to wait any longer.
Ellie's mother, Louise, explains that they hoped to wait until after Ellie finished school, but that one morning, recently, Ellie had awoken to find that her right eye had popped out onto her cheek. They rushed her to Great Ormond Street where the corners of her eyelids were stitched to help hold the eyeballs in place.
Ellie's operation will be extremely complex. Surgeons will cut across the top of her forehead, right down to her jaw bone, releasing the front of her face and creating a gap. A frame will then be fitted to hold the bone in place while new bone grows in the space in between. The frame will be extended every day stretching the new bone, gradually easing her face forward.
Ellie's father, Robert, shares her condition; it runs in the family. Ellie will be the first in her family to have such radical surgery.
David Dunaway
Great Ormond Street's craniofacial unit see up to eight new cases of Crouzon's Syndrome a year. The operation Ellie is having is still relatively new, fifty have been carried out at the hospital in the last five years. The high risk operation could take up to eight hours and Great Ormond Street will be Ellie's home for three weeks.
The man responsible for transforming Ellie's face is craniofacial consultant David Dunaway. He's worked at Great Ormond Street for the past five years and is one of two surgeons responsible for carrying out this complex and risky procedure. Mr Dunaway will be working with neurosurgeon, Miss Silvia Gatscher, because he'll be working so close to Ellie's brain.
Mr Dunaway will begin the surgery by cutting across Ellie's scalp to expose the bone in her forehead. To avoid any scars on the face, the incision is made on the hairline. Once he's made the incision he'll peel back the upper part of Ellie's face. Cutting in to bone can cause a huge amount of blood loss, a special machine is used to save as much of it as possible. The neurosurgeon now smoothes and files Ellie's misshapen forehead.
The most dangerous part of the operation is about to begin. In order to access the middle part of her face, Mr Dunaway has to remove the front part of her skull that protects her brain. The creasing that can be seen on the on the removed skull section shows how much pressure has been put on the skull by Ellie's brain.
Ellie has now been in theatre for three hours and the process of mobilising her facial skeleton has just begun. This crucial part of th operation involves cutting around the bone inside Ellie's eye sockets and the back part of her face down to the upper jaw. This will allow the middle part of her face to be separated from her skull. The bones in Ellie's face are now free to be pulled forward. They put the craniotomy bone flap back in place which was removed to allow access.
The reconstructive surgery is over. Now, the first piece of a frame, made from ultra-light titanium, must be attached to Ellie's head. Ellie will wear the frame for the next few months, every day it will, gently, ease her facial bones forward. The placing of the frame is critical as it will determine the final shape of her face. The surgery has taken six hours.
Ellie with Frame Fitted
Ellie's surgery is complete, but her ordeal isn't over. In a week's time, doctors will begin the slow process of moving her face forward. The screws at the back of Ellie's frame will be turned twice a day, each turn will move her facial skeleton forward by half a millimetre. In two week's, her face will have moved forward by fourteen millimetres.
Ellie's been wearing her frame for two months and is back at school. Later today, she'll return to Great Ormond Street to have the frame removed which is a quick and simple procedure.
CREDITS: All of this information came from the UK Channel 5 "Child in a Million" documentary series
