Children & families · Conditions
Craniosynostosis
Early fusion of the joints of a baby's skull. Depending on the type and your baby's age, treatment may be minimally invasive (including FEASR) or open surgery.
What is it?
Craniosynostosis is early fusion of one or more of the joints (sutures) between the bones of a baby's skull. Normally these joints stay open so the skull can grow with the brain. When one fuses too early, the skull grows in a different direction, changing the shape of the head, and in some children limiting room for the growing brain. It is different from positional (deformational) plagiocephaly, which is flattening from lying in one position and does not need surgery. Most craniosynostosis affects a single suture and occurs on its own; less often several sutures are involved as part of a genetic syndrome.
What symptoms can it cause?
The main sign is an unusual head shape, usually noticed at birth or in the first months of life. The shape depends on which suture has fused: sagittal synostosis gives a long, narrow head; metopic synostosis a triangular forehead; coronal synostosis a flattened forehead and brow on one or both sides; and lambdoid synostosis flattening at the back of the head. Raised pressure inside the head is uncommon in single-suture craniosynostosis but possible, especially when several sutures are involved.
How is it assessed?
Your baby will be examined and the head shape assessed and measured. In many babies the diagnosis is clear on examination; a low-dose CT scan may be used to confirm which sutures are fused and to plan surgery. Babies with more than one fused suture, or features of a syndrome, are assessed with the wider craniofacial team, which may include genetics. Because the minimally invasive options work best in young babies, early referral is helpful.
What are the treatment options?
There are two broad approaches, and which is suitable depends on which suture is fused, your baby's age and their overall health.
Minimally invasive (endoscopic) surgery is usually done in the first months of life, while the skull is thin and growing quickly. Through one or two small incisions, the fused suture is released with the help of an endoscope (a camera), and the baby's rapid brain growth then gradually reshapes the skull. Examples include endoscopic strip craniectomy, usually performed at around two to four months of age (generally before six months) and followed by a custom moulding helmet worn for most of the day for several months; and spring-assisted cranioplasty, in which small metal springs are placed to gently widen the skull over a few months and then removed at a second, shorter operation. For selected babies with sagittal synostosis, Dr Labuschagne uses Flexible Endoscope-Assisted Suture Release (FEASR), a minimally invasive technique he developed and has published on (see below). It uses a flexible endoscope and barrel-stave osteotomies through small incisions. Potential advantages of minimally invasive surgery include smaller scars, shorter operations, less blood loss and fewer transfusions, and a shorter hospital stay; the trade-offs are that it depends on early diagnosis, often relies on helmet therapy, and the final shape correction happens gradually.
Open cranial vault remodelling is usually done later, often between about six and twelve months of age or older. Through a larger incision across the top of the head, the affected bones are reshaped and fixed in position, usually with dissolvable plates, giving an immediate change in shape. It is suitable for older babies and for many types of craniosynostosis, but it is a longer operation with more blood loss, and a blood transfusion is often needed. Open surgery is planned with the craniofacial team.
The options, benefits and risks for your baby, and the timing, are discussed with you in detail.
- Seen from above, a baby's skull is made of plates joined by flexible seams (sutures), with soft spots (fontanelles). Here the sagittal suture has fused too early.
- Because the skull cannot widen at the fused suture, the head grows long and narrow from front to back. This shape is called scaphocephaly.
- In endoscope-assisted surgery, small incisions are made near the soft spots at the front and back of the head.
- Using an endoscope through these openings, the fused strip of suture is released so the skull can widen.
- As the brain grows over the following months, the head shape gradually becomes more rounded. Follow-up is planned individually.
When might surgery be considered?
Surgery is usually recommended when a suture has fused early, to allow the skull to grow normally, give room for the brain and improve head shape. The best timing depends on the approach: the minimally invasive options work best in young babies, while open remodelling is usually done later. Very mild forms, or isolated fusion of the metopic ridge without a triangular forehead, may only need observation.
What to expect from recovery and follow-up
After minimally invasive surgery most babies stay in hospital for a day or two. If a helmet is used, an orthotist fits it soon after surgery and it is adjusted as your baby grows, usually for several months. After open surgery, babies typically stay in hospital for several days and swelling around the eyes is common for a few days. Follow-up continues through childhood to check head shape, growth, development and, where relevant, signs of raised pressure such as vision changes.
When to seek urgent help
If your child is very drowsy, difficult to wake, has a seizure, repeated vomiting or a severe headache, go to the nearest emergency unit or call 10177. If your child is under our care, also call the hospital switchboard and ask for Dr Labuschagne (Netcare Unitas Hospital 012 677 8000; Nelson Mandela Children's Hospital 010 133 0600). After surgery, also seek prompt review for fever, increasing swelling or redness of the wound, or fluid leaking from it.
Dr Labuschagne's publications on this topic
- Labuschagne J, Mutyaba D, Ouma J, Dewan M. Flexible endoscope-assisted suture release and barrel stave osteotomy for the correction of sagittal synostosis. J Neurosurg Pediatr. 2023;31(1):71-77.
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This page is general information for parents and caregivers. Your child's doctor will advise on what applies to your child.