What would you do if your child were born with serious special needs? This mother – whose child was born with Apert’s syndrome – was relieved and grateful when a renowned South African team of doctors stepped in to save the day.
Read: Could specialised foetal surgery prevent birth defects?
Meet Professor Frank Graewe, head of the Plastic and Reconstructive Surgery division at the University of Stellenbosch and his incredible team at Tygerberg Academic Hospital, where this surgery was performed:
Health24: Tell us a little about your background and your three biggest achievements (they don’t all have to be professional).
Professor Graewe: I was born and grew up in Germany. After completing my plastic surgery residency at the University of Stellenbosch I spent a few years working at prestigious and internationally renowned plastic surgery units in various parts of the world to gain more experience in my field of specialisation. When I came back to Cape Town in 2006, I became head of the plastic surgery division at Stellenbosch University and started to work in private practice in Cape Town. Highlights and biggest achievements: If I look back are my choice and endeavour to become a plastic surgeon; my family and 3 kids; and professionally, being part of the team that performed the world’s first penile transplant.
Read: World first: penis successfully transplanted in Cape Town
Health24: How many craniofacial surgeries have you performed?
Professor Graewe: I have performed several hundreds of major craniofacial surgery cases during my career. Most major craniofacial procedures are performed by a team of plastic surgeons and neurosurgeons, as well as maxillofacial surgeons in some cases.
Health24: Why did you decide to operate on baby Ashbrite?
Professor Graewe: Ashbrite has been diagnosed with Apert syndrome, a congenital malformation affecting the skull and face, as well as hands and feet. We found that some of the cranial sutures in Ashbrite's skull bone closed to early. This causes abnormal skull growth and shape, and increases the pressure on the growing brain. We did the surgery to relieve that pressure, so that it does not cause permanent damage, and so that Ashbrite can develop more normally. We also tried to improve the skull shape and his appearance with this procedure.
Health24: What exactly was baby Ashbrite’s medical condition and what were/are his specific challenges?
Professor Graewe: Baby Ashbrite has syndromal craniosynostosis. The syndrome is called Apert's syndrome, and he suffers from multiple cranial suture synostosis.
Read: The realities of disability
Health24: What are the typical deformities associated with this syndrome?
Professor Graewe: Typical are skull and facial deformities that can lead to both, functional and aesthetic problems. Pressure can be on the brain, or eyes can be exposed, and some children develop hydrocephalus. The facial deformity can lead to breathing problems and obstructive sleep apnoea. All of these children have symmetric deformities of the hands and feet, where the toes and fingers are fused together.
Health24: What would Ashbrite’s future have been if he did not have the operation?
Professor Graewe: Ashbrite might need more procedures in the future to prevent complications and to improve his appearance. We will address his hands in the near future too. The added pressure on the brain could have exacerbated possible developmental disabilities.
Health24: Craniofacial surgery used to take up to 17 hours to perform. What innovations, including your own, have been made to bring the surgery down to about 7 hours?
Professor Graewe: The first major craniofacial procedures were performed in the late 1970s. Before that these conditions were not addressed surgically, due to the high chance of death and complications. Better anaesthetic techniques, technical equipment to cut and work on bone and a wide experience with this type of work in craniofacial units worldwide have improved technique and experience, and thus shortened the duration of surgery substantially. I developed a technique for mid-face distraction in very young children which has several advantages compared to the traditional procedure. It is less invasive, with a shorter hospital stay and less down time. We perform this procedure when the middle part of the face is underdeveloped and causes problems with breathing. This, on the other hand, can lead to heart failure and other complications, and treatment is essential.
Health24: What could typically go wrong during craniofacial surgery?
Professor Graewe: It is high risk surgery; most of our patients are little babies, and they can lose a lot of blood during surgery. Infections can occur theoretically and lead to meningitis and encephalitis, there can be fistulas where cerebrospinal fluid starts leaking. Fortunately, with improved surgical and anaesthetic techniques, complications are rare.
Health24: When can someone with Aperts or other craniofacial abnormalities not be helped through surgery?
Professor Graewe: Extremely severe cases with craniofacial syndromes can die before surgery can be performed, due to severely increased intracranial pressure.
Health24: You have used 3D print technology in previous operations. Why not in baby Ashbrite’s case?
Professor Graewe: I sometimes use 3D printing technologies to plan difficult cases and to shorten the surgery time, or to reconstruct a three dimensional skull defect. In Asbrite this would have not added sufficient benefit nor shortened the surgery time.
Health24: Can you explain step-by-step what you and the neurosurgeon, respectively, did during the surgery?
Professor Graewe: I first plan and perform the skin incision. After this the important part is the planning of the procedure and what bone pieces need to be removed and redesigned. Then Prof Hartzenberg, the neurosurgeon, took out the bone and we removed the bony part covering and protecting both eyes together. I then reassembled the skull with the remaining bone pieces, and closed the skull.
Health24: What is the future of craniofacial surgery?
Professor Graewe: We are permanently looking to improve the surgery, to find better and less invasive ways to perform these procedures. Bioengineering will play a greater role in the future as will technological improvements, like 3d printing.
It all started with a surgery that we filmed:
How baby Ashbrite looks today:
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